Paediatrics Flashcards

Question

Hepatomegaly causes

Answer 1

* Infection – congenital, infectious mononucleosis, hepatitis, malaria, parasitic infection * Hematological – sickle cell anemia, thalassemia * Liver disease – chronic active hepatitis, portal hypertension, polycystic disease * Malignancy – leukaemia, lymphoma, neuroblastoma, Wilms tumour, hepatoblastoma * Metabolic – glycogen and lipid storage disorders * CV – heart failure * Apparent – chest hyperexpansion from bronchiolitis or astma.

Answer 2

**Appendicitis** = inflammation of the appendix secondary to obstruction (usually by faecolith) or lymphoid hyperplasia. It is the most common surgical emergency and can occur at any age, although it is rare in infancy when lumen of appendix is wider and well drained. **Clinical features:** * Symptoms = Classically abdo pain which starts poorly localized, periumbilical pain and then becomes sharper and localized to RIF. Pain is usually severe and worse on movement to child may lie still. Commonly associated with anorexia, nausea, vomiting, diarrhea or constipation. Anorexia, vomiting. * Signs = Fever, pain aggravated by movement, tenderness and guarding in RIF (McBurneys point- 1/3 RASIS to umbilicus). If unwell, signs peritonism like high temp, abnormal observations. Red flag – Perforated gangrenous appendix can cause peritonitis. Suspect this if high fever \>40, profuse vomiting, severe abdominal tenderness/guarding or absent bowel sounds. **Investigation**s – FBC (increased wcc), CRP, ultrasound scan **Management** – monitor obs + analgesia with fluid resuscitation and IV antibiotics if concerns perforation and unwell. Appendicectomy (laparoscopic or open) with IV antibiotic cover. Complications are perforation, sepsis, abscess, appendiceal mass.

Answer 3

**Pyloric stenosis** – due to hypertrophy of smooth muscle of pylorus causing gastric outlet obstruction and more common in boys, commonly presenting in first 2-8weeks of life. **Clinical** – persistent, projectile nonbilious vomiting after feeds. The infant remains hungry and eager to feed after vomiting. Weight loss, constipation, dehydration and mild jaundice develop after a few days. Hypochloraemic metabolic alkalosis with low plasma sodium and potassium occurs from the vomiting. **Dx** * Test feed: Gastric peristalsis wave moving form L-\>R. Pyloric mass Oliver shaped mass in RUQ. * Capillary blood gas – Hypochloraemic metabolic alkalosis, low sodium and potassium * Ultrasound **Mx** – Rehydration correct electrolyte imbalances, pyloromyotomy.

Answer 4

* Excessive head lag beyond 8w * Persistence of primitive reflexes beyond 6m * Hand preference before 18m * Not interacting with other children at 3y. **Red Flags: Teaching** * 10 week old not smiling -could be visual problem, bell’s palsy, neglect, maternal mental health problems * 9month old left-hand preference (hand preference should be normally after 1), could be birth injury (brachial plexus injury etc), muscle problems, spasticity (cerebral palsy). Could do physiotherapy * 19month old not walking – worried about missing Duchenne muscular dystrophy (proximal muscle weakness, Trendelenburg gait), might just be children find bottom shuffling easier, could be DDH (development hip dysplasia). Blood test to check CK for DMD (would be really high), occupational therapy… * 2 ½ year old not putting words together – could be autism spectrum disorder, cleft palate, tongue tied, refer to speech and language therapy.

Answer 5

**Baby check** – head to toe (head circumference, fontanelle, eyes, red light reflex, pallet, chest wall, heart (murmurs), hip (DDH), back (dimples, spina bifida), is there an anus and intact. Hearing check, blood spot (heel prick) test. * 1-2w old – health visitor check sleeping, vaccination, feeding, adjustment * 6-8weeks – through physical exam by GP * 2yrs and 3months – ASQ ages and stages questionnaire. * Starting nursery check by health visitor

Answer 6

NEWBORN At 6 weeks – developed a couple of skills like hold head up (head control), start to see further (fix + follow), smile at 6weeks. Sleep on back due to risk of sudden infant death syndrome. But to develop gross motor skills need time on tummy when playing. Helps them move on to moving and different positions.

Answer 7

2 years SOme children start toliet trainign 2 1/2

Answer 8

3 years 15horus free per week nursery from 3 for all

Answer 9

4 years line circle square triange = progression of drawing Triangle at 4

Answer 10

* History – birth, family hx, developmental skills, currently, social context, parenteral ICE, regression (in social skills could be autism and in motor etc could be progressive muscular dystrophy etc) * Exam – general (dysmorphic, head size, growth, signs of neglect) and neuro (tone, power, asymmetry, reflexes) * Investigations * Management – MDT approach Play with them, it is very opportunistic. Ask parents, formal assessment tools. Observe in more than one setting at more than one time. Start at where you expect and move back.

Answer 11

* Genetic – downs, fragile X, chromosomal abnormalities * Injury - pre, peri, post-natal * CNS malformation neural tube defect hydrocephalus, structural abnormality brain * Endocrine/metabolic – hypothyroidism * Neurocutaneous- NF, tuberous sclerosis * Idiopathic

Answer 12

* **Autism spectrum disorder** = pervasive developmental disorder which presents in early childhood and is lifelong. Qualitative differences in reciprocal social interaction and communication combined with restricted interest and repetitive behaviours. Usually presents by 3years old. * **More common with:** 1st degree rel, parent with schizophrenia/affective disorder, maternal sodium valproate in pregnancy, gestational age \<35weeks. Also in children with learning disability, congenital CNS malformation/CP/neonatal encephalopathy, downs syndrome and other genetic ones, tuberous sclerosis or neurofibromatosis, muscular dystrophy. * **Clinical:** * Impaired reciprocal social interaction – difficult forming relationships, avoiding eye contact + reduced response to others emotion * Impaired communication – delayed speech, language development, repetitive use of language and difficulty initiating * Restricted interests + repetitive behaviors * Comorbidities – general learning and attention problems, seizures, affective disorders, mental health disorders. * It is a spectrum so may not have significant impairment in all categories. Those with Asperger syndrome or ‘high-functioning’ autism may have normal intelligence quotient and speech development, having specific difficulties with social and communication skills or obsessional interests. 70^ coincidence of psychiatric conditions including ADHD, conduct disorders, depression and OCD.

Answer 13

**Attention deficit hyperactivity disorder.** **3 hallmarks:** * Inattention – manifests as easily distracted child who changes activity frequently and doesn’t persist with tasks * Hyperactivity – excess of movement with persistent fidgeting and restlessness * Impulsiveness – Acting without reflection or prior thought **Physical exam** – developmental delay, clumsiness deficits in hearing or vision and pecific learning difficulties, dysmorphic features. **Diagnosis**: Current NICE guidelines suggests ADHD can be diagnosed where there is hyperactivity/impulsivitiy and/or inattention meeting either ICD-10 or DSM-5 criteria which: * Are present for at least 6m * Persist in 2 or more settings (ie, home and school) * Impair function Behaviour therapy, drug therapy (methylphenidate (Ritalin) or atomoxetine where tics and dexamfetamine where both failed.

Answer 14

Gas exchange changes from placenta to lungs * **Cord is clamped** – cessation of blood flow from placenta and rise in systemic vascular resistance inside baby circulation. Increases left side pressure and Ductus venosus starts to close (3-10days). * **Baby takes their first breath –** * Causes huge decrease in pulmonary vascular resistance (increases lung expansion and decreases hypoxic pulmonary constriction). * Increase in pulmonary venous return to left atrium and red in right atrial pressure. * Pressure in left atrium -\> pressure in right atrial. * Flap closure of foramen ovale in minutes (anatomical closures takes days) * Systemic vascular resistance -\> pulmonary vascular resistance reverse flow through ductus arteriosus * Ductus arteriosus begins to close due to high partial pressure of 02 passing through it (2days) * Over subsequent weeks, right ventricular wall muscle reduces and left ventricular wall thickens. * If babies are born and exhibit significant hypothermia/hypoxia/acidosis can have persistent foetal circulation and won’t achieve this transition.

Answer 15

**Congenital heart disease** or congenital heart defects refers toa variety of malformations of the heart and/or its major blood vessels that are present at birth. **Some General Symptoms:** * Lagging physical development * Cyanosis, pallor, SOB, anxiety in breastfeeding, rejection of the breast * Cyanosis, pallor or of the skin and mucous membranes * Heart murmurs from first days of lfie * Increase in heart rate * ECG signs of overload and hypertrophy of the heart (p-pulmonale, p-mitrale, left, right type of EOS) * Echocardiography signs of hypertrophy of different parts of the heart * Radiography – increase in heart size, depletion or enhancement of pulmonary pattern. * 25% of cases have chromosomal abnormalities * Trisomy 21, VACTERL, Digeorge, charge association * Other risks = maternal infection and maternal teratogen exposure * Commonest lesion = VSD * Majority of cases picked up antenatally * Can be subdivided into: Acyanotic and cyanotic.

Answer 16

* Alcohol in utero – VSD, ASD, PDA, TOF * Congenital Rubella – PDA, Pulmonary artery stenosis, septal defects * Trisomy 21 (Downs) – ASD, VSD, AVSD (endocardial cushion defect) * Mother has DM – TGA * Marfan syndrome – MVP, Throacic aortic aneurysm and dissection, aortic regurgitation * Prenatal lithium – ebstein anomaly * Turner syndrome – Bicuspid aortic valve, coarctation of aorta * Williams syndrome – supravalvular aortic stenosis * 2Q11 (DiGeorge syndrome) – Truncus arteriosus, TOF

Answer 17

Shunt left to right (no mixing of deoxygenated and oxygen bloods) These can be divided into: * Restrictive (allowing little flow left to right): Small ASDs/VSDs, PDAs. May close spontaneously * Non restrictive (large defects, allow signficiant left to right flow): Moderate-Large asds, VSDs, AVSDs. Need surgical closure and carry risks of PHTN & Eisenmengers if untreated * Obstructive (severity of lesion decides age of presentation): Aortic stenosis, co-arctation, pulmonary stenosis.

Answer 18

Deoxygenated blood enters systemic circulation or mix of oxygenated and deozygenated blood entering systemic circulation. **5Ts of Cyanotic Disease:** TOF, TGA, Truncus arteriosus, TAPVD, Tricuspid atresia (also HLHS) * Tetralogy of Fallot * Transposition of greater Arteries – aorta comes off right ventricle. PA comes of left (may be associated with ASD or VSD) * Truncus arteriosus – pulmonary artery and aorta haven’t divided – single common overlying blood vessel * Total anomalous pulmonary venous drainage – pulmonary veins do not connect to left atrium. 3 types described bu where the PVs connect (all have ASD otherwise not compatible with life). * Supracardiac – PVs drain to right atrium * Cardiac – PVs drain to right atrium through coronary sinus * Infracardiac – PVs drain to right atrium through hepatic veins and IVC (has highest risk obstruction) * Tricuspid atresia – tricuspid valve does not form (hypoplastic RV common). Similar defect affects left heart (Hypoplastic left heart syndrome HLHS. * Ebstein anomaly – displaced tricuspid leaflets, artificially atrialising the RV.

Answer 19

Duct Dependent Lesions: If undiagnosed, classically presents days after birth. * Systemic – Hypoxia/ respiratory distress, HF, Absent/reduced femoral pulses, severe metabolic acidosis. * Pulmonary (not enough flow to lungs) – respiratory distress, cyanosis, normal pulses, no response to hyperoxia. Treatment for suspected duct-dependent lesions is to keep patient with prostaglandin E2 (can cause apnoea, and may need intubation and ventilation.

Answer 20

**Eisenmenger:** * When left to right becomes right to left * Eissenmengers syndrome can result dorm untreated left to right shunts * ASDs, VSDs, PDAs * Over time, excess pulmonary blood flow from left to right hsunt causes pulmonary hypertension and right ventricular hypertension * When pulmonary vascular resistance -\> systemic vascular resistance, there is a functional reversal of flow from right to left * Cyanosis now occurs

Answer 21

Common in children and most not associated with pathology. **Interpretation:** * Timing – Is it systolic or diastolic (most are systolic, diastolic is rare and pathological) * Character – pansystolic or ejection systolic * Loudness – Graded 1-6 * Radiation – A murmur that radiations from its site of maximal loudness is more likely significant.

Answer 22

* Murmurs produced by normal flow. Changing the flow should therefore change intensity of murmur. * Maneuvers that decrease the flow of blood returning to the heart through the venous system will decrease the intensity of flow murmurs, suggesting that the murmur is flow related or innocent. * Changing childs position form supine to sitting, then to standing and finally to squatting during exam will change flow and is useful in helping to define innocent murmur. * Child may be asked to push out the abdomen or bear down to perform a Valsalva maneuver, which reduced venous blood flow to the heart and the intensity of innocent murmurs. * Types – stills murmur, venous hum, turbulent flow in pulmonary artery bifurcation. * 25% of full term neonates have a murmur. * Features of an innocent murmur 10S: soft, systolic, short, S1&S2 normal, symptomless, special test (x-ray and ECG) normal, standing/sitting vary with position, sternal depression * **Stills murmur** = soft vibratory murmur heard in LLSB most frequently in childhood when there is normal blood flow an dno cardiac lesion. * **Venous hum** = continuous murmurs heard loudest over clavicles due to venous return form head and neck and this varies with position. * **Turbulent flow in pulmonary artery bifurcation** = A soft ejection systolic murmur caused by turbulent flow in pulmonary artery (PA) bifurcation as the PA bifurcation and branches are small.

Answer 23

* Symptoms = syncope, episodic cyanosis * CVS sings = abnormal pulses, heart sounds, BP or cardiac impulse * Murmur = diastolic, pansystolic, radiating to back or associated with a thrill These can be difficult to distinguish from an innocent murmur. Refer for echocardiography if in doubt.

Answer 24

**Kawasaki disease** = uncommon systemic vasculitis affecting small and medium vessels which can include the coronary arteries leading to aneurysm formation. Typically affects 6m-4y (peak at 1yr) and more common in children of Asian origin. **Clinical criteria for diagnosis**: Fever for 5+ days or more with 4 of the following… * Bilateral non-purulent conjunctivitis * Polymorphous maculopapular rash * Oral changes – red cracked lips or strawberry tongue * Changes to extremities: reddening or oedema of hands and feet, or peeling of skin (classically a late sign) * Cervical lymph nodes \>1.5cm May emerge sequentially and not be present all at once. Characteristically, the child is extremely miserable. Coronary aneurysms will occur in 230% f untreated cases an typically develop in first 4-6weeks of illness. **Investigations** – FBC, CRP, ESR (raised), U&Es, LFTs (bilirubin + AST may be raised), throat swab and antistreptolysin 0 titre, blood culture + viral titres, Echocardiography, electrocardiogram. **DDx** – measles, scarlet fever, rubella, roseola, fifth disease. **Management** – single dose IV immunoglobulin to reduce incidence + severity of coronary artery aneurysm formation and aspirin.

Answer 25

**Febrile convulsion** = Seizure associated with a fever in a child between 6m-6y in absence of an intracranial infection or identifiable neurological disorder. Most common cause of seizures in childhood (5% children), might be familial predisposition and usually occur when body temp rises rapidly. **Clinical Features:** Typically brief (1-2min), generalised, tonic-clonic seizures. Underlying infection causing fever may be vial/bacterial so need to identify fever cause and ensure no signs of meningitis. * Simple febrile convulsion – \<15mins, generalized, occurring once in 24hours. * Complex - \>15mins, focal or recurring within 24hours. **Management:** * Identify + treat underlying infections – may consider CXR, blood culture, microscopy + culture + LP * Despite no evidence of reduction in seizures, regular antipyretics are recommended * Prolonged convulsions are treated as per status epilepticus * Parenteral education **Prognosis** – v good in simple ones, but 1/3 children will have further ones in lifetime but usually grow out of this by 6yrs. Slightly higher risk of developing epilepsy in lifetime which is increased further by positive H or developmental/neurological abnormalities so EEG may be warranted in complex febrile convulsion or presence RFs.

Answer 26

**Epilepsy** = Chronic disorder characterised by recurrent, unprovoked epileptic seizures. Diagnosis is made in a patient whom epileptic seizures recur spontaneously but an ‘epileptic seizure can be provoked in individuals who do not have epilepsy eg with fever. **DDx** = Syncope, breath holding spell, aspiration, GERD, panic attack, daydreaming, conversion or pseudoseizures, benign sleep myoclonus benign paroxysmal vertigo, complicated migraine, motor tics, complex behaviours, decorticate posturing. **Causes Epilepsy** – idiopathic, cerebral malformations, cortical dysgenesis, tumours, trauma, CNS infection, hypoxic ischaemic encephalopathy, chromosomal abnormalities, neurodegenerative conditions, neurocutaneous syndromes. **Diagnosis** – careful history, distinguish epileptic seizures from the many causes of faints, fits and funny tums. **Investigations** – EEG, neuroimaging, brain imaging (structural-CT/MRI and functional-PET/SPECT), metabolic investigations **Mx –** anti-epileptic drug therapy. Other options like ketogenic diet, vagal nerve stimulation, epilepsy surgery. Acute management – benzodiazepines if still convulsing except in neonates whoud shudl receive phenobarbitone.

Answer 27

**Classification of seizures**: initial division based on where electrical activity started in the brain. * Generalized onset seizures – originate from network in both hemispheres. Can be motor (tonic-clonic and myoclonic\_ or non motor (absence seizure) * Tonic clonic: tonic phase (rigidity with loss of posture) and clonic phase (movements of all four limbs), loss of consciousness, postictal drowsiness. Can be in epilepsy but also in febrile seizures and meningoencephalitis. * Absence seizures: brief unawareness lasting few seconds, no loss of posture, immediate recovery, might be very frequent, associated with automatisms (eg, blinking and lip smacking) * Focal onset seizures – Thought to originate from one hemisphere, either localized point or more widespread. Can be subcategorized by level of self-awareness experienced: * Involvement of only particular part of the body * May be associated with aura * Can have emotional, sensory or cognitive component * Mya spread to involve entire body – focal onset to bilateral tonic-clonic. * Frontal seizure = motor/pre-motor corte which may lead to clonic movements to a tonic seizure. * Temporal lobe seizure – aura, with smell and taste, lip smacking etc. * Occipital seizure– stereotypes visual hallucinations. * Parietal lobe seizures – contralateral dysesthesias (altered sensation) or distorted body image. * Unknown onset seizures * ‘grand mal’, ‘petit mal’, ‘simple’, ‘partial’ are outdated and avoided now.

Answer 28

**Cerebral palsy** = group of conditions affecting motor function and posture due to nonprogressive lesions of developing brain. Even though it isn’t nonprogressive lesion, the clinical manifestations evolve as the child gets older. Cause is uncommon in many but identified RFs can be categorised into antenatal, perinatal and postnatal insults. Perinatal asphyxia is an uncommon cause of cerebral palsy * Antenatal.= cerebrovascular hemorrhage or ischaemia, cortical migration disorder, structural maldevelopment of brain in gestation osme linked ot gene deletions * Birth – hypoxic ischaemic injury before or after deliver * Preterm infants – vulnerable to damage from periventricular leukomalacia secondary to ischemia and or IV hemorrhage and venous infarction. * Postnatal – meningitis/ encephalitis/ encephalopathy, head trauma, symptomatic hypoglycaemia, hydrocephalus and hyperbilirubinemia. **Types:** * Spastic = damage to UMN, spasticity with brisk deep tendon reflexes and extensory plantar responses. Unilateral, bilateral (quadriplegia), bilateral (diplegia). * Dyskinetic = involuntary, uncontrolled movements. chorea, athetosis, dystonia. * Ataxic = Hypotonic. Most genetically dertermined. * Other. Mx – MDT,novel - botulinum toxin injections to muscles, selective dorsal rhizotomy, intrathecal baclofen **Long term complications**: Premature ageing, malnutrition, depression, anxiety, heart and lung disease, OA, chronic pain, scoliosis.

Answer 29

**Cerebral palsy** = group of conditions affecting motor function and posture due to nonprogressive lesions of developing brain. **Clinical features:** * Hypotonia in infancy * Feeding dififuclties due to lack of oromotor coordination * Delayed motor milestones * Speech and language delay * Motor disorders (eg, hypertonia, hemiplegia) Effects can range from having little to no physical disability and thriving in mainstream school to children who are non verbal and have four limb spastic quadriplegia. **Diagnosis**: Usually 1+ * Abnormal tone – hypertonia or hypotonia * Abnormal power (eg delay in motor milestones) * Abnormal reflexes * Abnormal movements – athetosis or chorea * Abnormal posture or gait

Answer 30

**Puberty** = acquire secondary sex characteristics and reproductive capacity attained. Usually 8-13 females and 9-14 males. * **Girls: Average 10years, 1st signs breasts with peak height velocity breast stage 4- (12years). Menarche 11-13.** first sign usually breasts begin to develop and pubic hair starts to grow with more hair on legs and arms. After 1 year..." breasts grown ore, first period (2years after), pubic hair coarser + curlier, underarm hair grows, sweat more, acne, more whit vaginal discharge, grwoth spurt, gain weight. After 4years breasts become adult like, pubic hair spread to inner thih, genitals fully developed, girls stop growing taller * **Boys: Av 12years, 1st sign 4mls testicular vol (from 2mls), peal heigh velocity 10-12mls testicular vol (14years)**. First sign testicle sbigger, scrotum thinns + reddens and pubic hair starts to appear at base of penis. After 1 year or so + continuing: penis _ testicles grow + scrotum darker, pubic hair thicket, udneramr hair, boys sweat more, breast can swell, boys have wet dreams, voice breaks and deeper, acne, growth spurt + muscular. After 4years, genitals look like adults and pubic hair spreads, facial hair, boys taller at slower rate then muscular. Mood changes - unexplained mood swing, low self esteem, aggression, depression. Precocious puberty - before 8years for girls or 9yrs boy. Assoicated growth spurt. most commonly ealry onset of nromal puberty in girls and in boy smostly paholoigcal. Delayed - Absence of secondary sex characteristics at 13years of age in girls or 14years in boys. Can be hypogonadotropic hypogonadism (central delayed puberty) or hypergonadotropi hypogonadism(gonadal failure).

Answer 31

**Diabetes Mellitus** = group of disorders of carbohydrate, fat and protein metabolism, caused by deficiency of or reduced efficacy of endogenous insulin. **T1DM:** * Autoimmune, lack of insulin. T-cell mediated autoimmune destruction of the B cells in pancreatic islets of Langerhans, perhaps triggered by environmental factors (eg, viruses) in people with genetic predisposition. Osmotic diuresis when blood glucose conc exceeds renal threshold. Ketoacidosis develops when insulin efficiency is severe. Associated with HLA-DR3/DR4 * Presentations: Hyperglycaemia, polydipsia/polyuria/polyphagia, weight loss, N/V/abdo pain (DKA) * Mx: Lifelong insulin (high risk, measured in units) replacement, regular blood sugar checks, screen for complications and compliance. Normal BM is 4-7. Noncompliance is big issue for adolescent patients. * Teach – how to treat hypos, sick day rules. * Hyperglycemia – dry mouth, increased thirst, weakness, headache, blurred, frequent urination * Hypoglycemia symptoms – sweating, pallor, irritability, hunger, lack of coordination, sleepiness.

Answer 32

* **Invetsigations** - WHO diagnosis: Fasting plasma glucose ?7mml/L or random plasma glucose of \>11.1mmol/L in absence of other acute illness. If asymptomatic then needs to be confirmed on second occasion. In those with T1DM may be raised levels autoantibodies and in T2DM, raised C peptide. * **Management long term**: Education + psychological support (diet), insulin replacement (basal bolus- long acting before bed, rapid before meals) or subcutaneous pump, diet, exercise, monitoring (finge rprick, fasting 4-7, post meals 5-9), management of acute compications and prveention long term * **Complications**: * Hypoglycaemia - due to mismatch in insulin doe, time, carbs intake,e xercise etc. Initially faint, dizzy, wobbly, sweating, hunger and then more severe are lethargy, bizarre behavior, seizures etc. Need sugary drink, glucose tablet or glucose polymer gel followed by more complex carb and if severe or uncooperating then IV 10% dextrose or IM glucagon. * Diabetic ketoacidosis – abdo pain, vomiting, features severe dehydration and ketoacidosis and can have severe complications including cerebral oedema. * Late complications – CV disease, retinopathy, nephropathy, neuropathy.

Answer 33

* Present at birth * Developmental defects - thyroid agenesis or failure of migration * Dyshormonogenesis - autosomal recessive inborn error of thyroid hormone synthesis * Congenital pituitary lesion * Transient congenital hypothyroidism in cases of materanl thyroid disease with passage of tyrou receptor antibodies or antithyroid medications. * Maternal iodide deficiency * **Clinical features** - May be clinically normal at birth but if untreated then: Prolonged neonatal jaundice, sleepiness, feeding problems, constipation, umbilical hernia, bradycardia and poor peripheral perfusion, coarse facies/ wide posterior fontanelle, large tongue, hypotonia and learning difficulties and goitre. * Testing is part of neonatal screening in the uK.. Most UK labs test for raised levels TSH

Answer 34

* Causes include autoimmune disease (Hashimoto thyroiditis), iodide deficiency and iatrogenic (caused by thyroid surgery or meds). * Clinical Presentation: Feeling cold, weakness + fatigue, low mood, constipation, menorrhagia, dry skin and coarse hair, bradycardia + heart failure, ataxia. * Measures thyroid function tests and thyroid autoantibodies

Answer 35

**Neonatal -** * Causes for neonatal – infants of mothers with Graves Disease or Hashimotos disease from transplacental transfer of thyroid stimulating immunoglobulins. * Clinical presentation – may cause IUGR, tachycardia or fetal hydrops. Get goitre, increased appetite but faltering growth, diarrhea, CNS signs like irritability, CV signs like tachycardia, arrhythmias, hypertension and heart failure. * Usually self-limiting within 1-3month but if severe may require treatment with propranolol or carbimazole. **Acquired –** * Most common by Graves disease(autoimmune) – TSH receptor antibodies * Clinical – feeling hot, diarrhea, increased appetite but weight loss, tremor, tachycardia, palpitations, cardiomyopathy, eye signs like proptosis, cremosis, exposure keratitis and ophthalmoplegia. * T4,T3 elevated, TSH depressed, thyroid autoantibodies. Carbimazole first line with B blockers for symptoms relief.

Answer 36

* Height below 0.4th centile for age * Predicted height less than mid-parenteral arget height * An abnormal growth velocity as indicated by heigh changing by more than the width of one centile band over 1-2years. **Causes**: * Familial short stature * Constitutional delay of growth and puberty, ay be positive FH * IUGR * Endocrine disorder - GH deficiency,hypothyroidism, cushins * Genetic: * Achondroplasia - mutation in GFGR3 gene so other skeletal abnormalities * Mucopolysaccharidosis - short stature, coarse faial features, assoicated learning difficulties * Syndromes – Turner syndrome (45X0 kartotype), down syndrome, prader willi, russel silver syndrome. * Malabsorption (CF/coeliacs) * Any chronic disease (congenital heart disease, chronic kidney disease) * Psychosocial deprivation/ neglect

Answer 37

* Birth weight, length and head circumference * Timeline of how they have grown and whether there has been any pubertal development * Early childhood illness and systemic disorders * Parenteral height – estimate target centile from mid parenteral height. (Father height + mother height)/2 then add 7 for boys and minus 7 for girls. Boys can then devite 10cm either way and girls 8.5cm either way. Can use chart where join mother and father heights and where cross centile line in middle. 9/10 childrens height centiles are within 2 centile spaces of mid parenteral centile. Can also then use adult height predictor. * FH – inherited skeletal dysplasia’s * Dietary and social history **Exam:** * Height – calculate growth velocity in cm/year (min2measurements 6m apart) * Weight * Head circumference * Stage of puberty * Dysmorphic features – might identify syndrome * Visual fields and fundi – might indicate pituitary tumour.

Answer 38

Fetus absorbs iron from mother across placenta. Term infants have adequate reserves for first4m life but preterm have limited stored and higher demands so utilise reserves by 8weeks. Reast milk and unmodified cow milk a=re low in iron but 50% of iron in breast milk is absorbed whereas only 10% from cows milk. Most formula milks are fortified with iron and have 10times concentration in breast milk but 4% absorbed. Dietary sources – red meat, fortified breakfast cereals, dark green veg, bread. About 10-5% dietary iron absorbed by is enhanced by ascorbic acid (Vit C) and reduced by tannin in tea 95% iron supplies in adults is by recycling of broken RBCs but in infants they require 30% from dietary and this demand increases with age, especially with girls who loose iron through menstruation.

Answer 39

**Main causes** – : Inadequate intake (diet, growth spurt), malabsorption (coeliacs), blood loss (menstruation, meckels diverticulum) **Clincial features**: Fatigue, slow feeding, Pica, Pallor (Conjuctiva, palmar creases) **O/E**: only signs might be pallor of mucous membranes and if severe may be flow murmur due to hyperdynamic circulation. In infancy and childhood you get developmental delay and poor growth which is reversible by long term oral iron treatment. Severe anaemia can cause cardiac failure. **Invetsiagtions**: * FBS – Hb, MCV decreased (microcytic, hypochromic anaemia) * Decreased serum ferritin **Management:** : to prevent avoidance unmodified cow milk until 12m, iron supplementation in those vulnerable. Mild to mod then dietary counselling + oral iron supplements (dietician possible) and replacement for 3m after correction to replenish stores. For severe with cardiac compromise then may need transfusion.

Answer 40

I**diopathic thrombocytopenic purpura (ITP)** = most common cause of thrombocytopenia in children. Immune destruction of platelets by autoantibodies. Autoimmune **Clinical Features**: May be history of recent viral illness. ITP mainly affects children between 2-10, often 1-2 weeks after viral infection. Presentation is with purpura and minor bleeding eg, from mucosal surfaces like epistaxis. Spleen is palpable in minority of cases. **Diagnosis**: Of exclusion (rule out more sinister causes): Acute leukaemia, aplastic anaemia. Organomegaly, lymphadenopathy, anaemia or neutropenia. * FBC, low platelets * Blood film * (Bone marrow biopsy) **Treatment** – Observation as usually self-resolves in 6-8 weeks. May need steroids or IV Ig. **DDx** – acute leukaemia, nonaccidental injury, Henoch-Schonlein purpura.

Answer 41

**Henoch-Schönlein Purpura** = commonest vasculitis in children. Multisystem vasculitis involving small blood vessels. Immune mediated IgA vasculitis, usually triggered by viral URTI. Common in boys and most are under 10 (2-10). **Clinical Features**: Affects skin, joints, GI tract and kidneys. Painful, palpable purpura. * Non-blanching rash (100%) – buttocks, extensor surfaces of legs/arms * Painful, swollen joints (60-80%) * Abdominal pain (80%) * Hematuria (20-60%) **Investigations** – Rule out other patho and measure extent of organ involvement. FBC, CRP, blood cultures, U&Es) Estimated 2% with HSP develop renal failure. **Management** – usually self resolves within6weeks, simple analgesia. Steroids sometimes used to treat joint pain.

Answer 42

Eczema = chronic inflammatory skin condition usually starting in childhood. 3 main variants: Atopic eczema, infantile seborrheic eczema and napkin dermatitis. * Atopic * Infantile seborrheic eczema * napkin dermatitis

Answer 43

* Often FH of atopic disorders (eczema, asthma, allergic rhinitis), reflecting genetic predisposition that confers an abnormal immune response to environmental allergy. * **Clinical:** Usually episodic but may be continuous in serious. Dry, red itchy rash usually on extensor surface and face and flexures in older children. Skin can vary from acute, weeping papulovesicular eruption to chronic, dry, scaly, thickened skin that develops in older children. Itching is often most problematic symptom. * **Investigations** – clinical but IgE likely raised. Food and environmental allergens play role so exclusion diets or radioallergosorbent testing and skin pricks may be helpful. * **Management** – regular use emollients even when under control and apply topical steroids. If not enough then topical therapies like tacrolimus and pimercrolimus may be used. Systemic therapy with immunsuppressive agents for severe. Education essential and many grow out of eczema by teens. * **Complictions** – seocndayr infection with viruses or bacteria. Infection with herpes simplex is potentially serious and treat with aciclovir.

Answer 44

Presents mostly in first 2m life with scaly, non itchy rash initially on the scalp (cradle cap) which might spread to involve the face, flexures and nappy areas. Treatment is emollients and mild topical steroids.

Answer 45

Rashes in nappy area are common and can be due: Irritant contact dermatitis (nappy rash) – prolonged contact urine and faeces with ski. Include skin wetness and ammonia from breakdown or urine by faecal enzymes. Skin is red, moist and might ulcerate. Inguinal folds are spared. Can be prevented by frequent nappy changes and barrier creams. Exposure to air can hasten recovery. Candidiasis – bright red skin with satellite lesions and involvement of ksin folds. Can be treated with topical and oral antifungal like nystatin Seborrheic dermatitis.

Answer 46

Used to monitor neonatal patients when collecting arterial sample is not practical. Lateral area of heel preferred but can use fingertip, earlobe, toe etc. * Respiratory acidosis – excess carbonic acid as increased CO2. Eg from CNS depression (asphyxia), obstructed airways. Can be compensated (kidneys cnrease H+ secretion and bicarb reabsorption) when low normal pH with increased CO2/Bicarbonate * Respiratory alkalosis – alveolar hyperventilation so deficiency carbonic acid. Cause eg, iatrogenic, hypoxemia, pain. Compensation is retain chloride and excrete fewer acid salts by kidneys decreased hydrogen secretion and when pH high normal with low CO2/bicarb * Metabolic acidosis – deficiency in bicarbonate in ECF. Caused by any systemic disease that causes increased acid production or retention or anything caucusing excessive base losses (diarrhea, renal failure sepsis).Compensation if lungs blow off additional CO2 through hyperventilation and kidneys increased excreitona cid salts and reabsorption bicarbonate ions. pH below normal with low levels CO2/bicarb * Metabolic alkalosis. Excess bicarbonate in ECF where problems lead to increased loss acid. Severe vomiting, diuretic therapy, iatrogenic etc. Compensate by retaining CO2 through hypoventilation so pH high normal, high co2 and bicarbonate

Answer 47

Needed to test the fluid around the brain and spinal cord. May be done to see if a child has meningitis. Lie on side with knees tucked into chest and head bent forward. Babies will be curled in a ball to get a better position. Young children and babies will be held in place. Needle L4 level or lower as conus medullaris finishes near L3 at birth (L1-2 by adulthood). Preferable to get before antibiotics. **Interpretation:** Normal CSF parameters vary with age. Any neutrophils present is unusual and should raise concern about bacterial meningitis. If CSF abnormal, safest course is to treat as bacterial meningitis. **Factors that can affect result** – Ab prior, seizures, traumatic (blood stained) tap, tie between smapling and analysis

Answer 48

Measurements must be accurate for meaningful monitoring of growth. Groth parameters should be plotted on charts and dont assess a single growth parameter in isolation. The pattern of growth is essential when assessing the health of a child, consider genetics, nutrition, general health and hormones as potential causes of abnormal growth. **Measuring** – remove footwear, without nappy using a length board or mat. Good to do 3 measurements and use average. \<2 years = length board or mat \>2years = rigid upright measure with T piece or stadiometer **Significant abnormalities:** * Measurements below the 0.4th or above the 99.6th centiles or outside the midparental height range * If marked discrepant from weight * Serial measurements, which cross growth centile lines after 1st year of life.

Answer 49

height below 2nd centile (2SDs below mean). Most of these 1 in50 children will be normal as short parents but the further below centile, the more likely pathological. Measuring height velocity is a good indicator of growth failure. Must compare heigh centile of a child with weight centile and expected genetic height. This is done by mean of father and mothers height with cm added for midparenteral height for boy and -7 for girl. The 9th to 91st centile range of this estimate is given by +/-10cm in boy and +/-8,5c, in a girl. * Familial – may be small from parents to check parents and child odnt have inherited growth disorder like skeletal dysplasia * Constitutional delay in growth and puberty – variation of normal growth with short stature in teens as delay in onset of puberty, bone age is delayed etc and onset of sexual secondary characteristics, but final height is normal. * Small for gestational age and extreme prematurity * Chromosomal disorder/syndromes – downs syndrome, turner, russell-silver, noonan. * Nutritional/ Long term illness – coeliac, crohn’s, chronic kidney disease * Psychosocial deprivation * Endocrine – hypothyroidisim, GH deficiency, IGF-1 deficiency and associated with children beig relatively overweight. * Extreme short staure – idiopathic short stature, abdnormlaities in SHOX gene

Answer 50

Obesity fuels early growth but doesn’t increase final height. Secondary endocrine causes are rare. Both congenital adrenal hyperplasia and precocious puberty lead to early epiphyseal fusion so that eventual height is reduced after an early excessive growth rate. Marfan and Klinefelter’s syndrome cause long legged tall stature and in XXY there is infertility and learning difficulties. Excessive height in prepubertal or early pubertal adolescents can be treated with oestrogen therapy and testosterone therapy, but serious S/E and mixed results. Surgical destruction of epiphyses in extreme cases.

Answer 51

Measuring - Without clothes or nappies. Children over 2years can be weighed in a vest and pants but without shoes, footwear, or anything in their hand. Compare to the charts for growth standards which allow for lower weight of exclusively breast-fed infants, so they aren’t labelled as underweight and allow earlier identification of formula fed babies gaining weight too rapidly. Asses with things like height as small and a bit under isn’t as significant as tall and low weight. Weight faltering – covered above and obesity.

Answer 52

**Measuring** – Head circumference should be measured with a narrow plastic or disposable paper tape and measurement should be taken where the head circumference is widest. Take 3 and get average. Hat or bonnet removed. **Plotting**: Record measurement and date in ink but plot in pencil. Abnormal head growth: Most head growth in first 2 years lufe and 80% of adult head size is by 5years. Posterior fontonelle closed by 8weeks and anterior by 12-18m. If rapid increase in head circumference, raised intracranial pressure should be excluded. * Microcephaly – head circumference below 2nd centile. May be familial, autosomal recessive, from congenital infection or acquired after insult to developing brain. * Macrocephaly – head circumference above 98th centile. Familial, obesity, secondary (hyperthyroidism, excess sex steroids, excess adrenal androgen steroids true gigantism), syndromes (long legged tall stature- marfan syndrome, homocystinuria, klinefelters syndrome & sotos syndrome), excessive growth at birth (maternal diabetes, primary hyperinsulinism, beckwith syndrome) * Asymmetric heads – imbalance from growth sutures. * Craniosynostosis

Answer 53

**Capillary refill time**: 2 or less when on finger, and if foot/chest then 4 or less seconds * High HR – Hypovalaemia, sepsis, fever pain, hypoxia, anaemia * Low HR – Hypothermia, hypothyroidism, anorexia nervosa, malnutrition, hypokalaemia, hypoxia. * High RR – can be transient (less than 24hours), bronchiolitis, pneumonia, infection of lower airways. * Low RR – hypothyroidism, opioids, toxins, head injury, * High SBP – often related to other health conditions like heart defects, kidney disease, genetic conditions or hormone disorders and for older children it may be primary hypertension. * Low SBP – anaphylaxis, arrhythmia, medications, dehydration, drinking alcohol, heart conditions, infections.

Answer 54

Clinical Exam – General principles: Newborn clinical examination (NIPE): Screening exam, head to toe exam, medicolegal significance- don’t miss: Eyes – red reflex: clear media (cataract) Heart – murmurs, femoral pulses (congenital heart disease) Hips – any dislocation, unstable (DDH- developmental dysplasia of hips) Testes: are they descended Exam od young child (toddler/preschool): separation anxiety, stranger danger, get to child’s level or lower, make eye contact, smile, play, speak about general things first to help anxiety, use parents o cam them and examine on their lap if needed. Exam older child- involve them in history, get verbal consent, respect personal space, explain all steps, pre warn If going to be uncomfortable and stop if they say so. (Male child with abdo pain, always look at genitalia no matter what they say in case testicular torsion). Exam adolescent – verbal consent, check privacy, comfortable position, respect wishes, chaperone. Don’t do invasive exam like PR/PV (leave for surgeons/gynae)

Answer 55

* Frequency – how often, single event, recurrent * Timing – certain time od the day eg, during sleep, on waking * Triggers – tiredness, travel, exercise or excitement, painful stimulus * Warning beforehand – called aura * Duration – how long, if occurs in clusters, is consciousness regained in between * Colour change – pallor, cyanosis * Alteration in consciousness – or impaired * Recovery and any symptoms afterwards – does the child behave normally * Record accurately – what happened just before, during and after the event.

Answer 56

May be epileptic or non-epileptic. Frequently the word seizure is used interchangeably with epilepsy, but this isn’t correct. * Epileptic seizures are unprovoked and usually recurrent. * Acute symptomatic seizures secondary to acute insults are NOT epilepsy. Eg, hypoglycaemia, metabolic disturbances, decreased Na (\<120), Increased NA-hypernatreamic seizure(\>145), decreased Calcium. * Role of EEG is limited and must be used judiciously, as both false positives and false negatives occur. EEG only answers the type of seizures, and can categorise epileptic syndromes.

Answer 57

* **Tonic** = prolonged period of contraction of one or several muscle groups. * **Clonic** = rhythmic contractions followed by a slower relaxation phase * **Atonic** = sudden loss of muscle tone, called drop attacks or slumps * **Myoclonic** = isolated, brief, fats contractions followed my muscle relaxation. * **Vacant** = * Absences characterized by arrest of activity, are brief and multiple, vacant gaze, with automatism such as lip smacking, eyelid flickering. * Focal seizures (complex) characterized by impaired consciousness.

Answer 58

* Benign neonatal sleep myoclonus * Benign neonatal seizures * Parasomias – sleep paralysis, sleepwalking, night terrors and confusional arousal. * Syncope (vasovagal/reflex) * Cyanotic Breath Holding spells (crying convulsions), blue spells. * Reflex anoxic seizures (eg, pain (eg hit head),asystole, loose consciousness, fit), Pallid syncope * Sandifer syndrome with GORD, apnoea, and dystonic reaction * Hyperekplexia – whole body stiffening in response to sudden noise * Daydreaming – occurs when bored or watching TV/screen, self-gratification (vs absent seizure, where in hyperventilation if its absent seizure they will stop and freeze) * Febrile seizures, tics, infantile shudder, shivering due to excitement, cataplexy

Answer 59

* Usually 4-7m, can be as late as 12m * Sudden jerking of neck, trunk + limbs (salam attacks) * There are runs or clusters of these attacks, may be dozens or hundreads per day * The baby cries at the end of a run of spasms. * Insidious onset, encephalopathic pattern, hypsarrhytmia on EEG * Loss of smole, visual nattention wigh regression (lennox Gestaut syndrome).

Answer 60

* Typical 3-12years, from sleep. * Focal onset (acial or perioral). Sensory and/or motor * Tingling of one side of the mouth * Expressive aphasia or guttural sounds. * Post icta drooling * Can experence secondary generalization with brief tonic clonic movements. * EEG shows centro temporal spoke sactivated by sleep and may or may not need treatment, usually remits in adolescence.

Answer 61

* 4-8years (onset before 3years is rare). * More common in girls, typical clinical features are (child freezes typically \<5secs, perioral or periocular flickering movements, unrousable/unresponsive during episode * Rapid recovery, as if nothing ever happened (No post-ictal phase). Occurs 10s or hundreds of times in day and can induce by hyperventilation. * Eeg shows 3Hz generalized spike and wave pattern.

Answer 62

* Typically 12-18yrs. * Typical features: first generalized tonic clonic seizures then GTC seizures often preceded by several myoclonic jerks. * Awareness retained during myoclonic jerks: history of dropping objects while preparing breakfast is common. * Absences occur in up to 1/3. EEG typically shows polyspike discharges followed by irregular 1-3Hz slow waves. Photosensitivity is common.

Answer 63

* Very common 6m-6yr * Occur in a febrile illness in absence of intracranial pathology. * In 3% children under 6. * Genetic predisposition – 10% risk in first degree relative of index case. * Usually occur in early part of illness when temp is rising quickly (not the height of the temperature it’s how quickly it goes up) . * 30-40% risk of recurrence **Classification of Febrile seizures:** * Simple febrile convulsion – short (usually couple of minutes or less but anything \<15mins), generalized tonic-clonic, full recovery. Occur only one per episode of an illness/ No increased risk of future development of epilepsy (1-2% pop risk) * Complex febrile seizure – Prolonged \>15mins (may end up in febrile status), focal, multiple seizures in the same episode of n illness. Persistent cognitive impairment (remains drowsy). Increased risk fo future development of epilepsy (4-12%). * Generalized epilepsy with febrile convulsion plus (GEFC+) – due to sodium channel mutation (hence why also called channelopathy).

Answer 64

**Management at home:** * If it happens again, the plan… * Place the child on a safe flat surface, no risk of falling (floor) * Make sure nothing is close to child that can harm them while jerking * Don’t panic * Time and if beyond 5mins then 999 * Recovery position * Most children are post-ictal so allow to recover (10-15mins) * Then see GP – need to see what infection is causing it. **Acute Management:** * ABCD: airway, breathing, circulation, disability, exposure, glucose. * Given oxygen (as most become cyanotic and hypoxic and body seizes) * Note the time * Check BM (blood glucose) * Get IV access if possible then a benzodiazepine (if not then rectal diazepam). Can have 2 doses benzodiazepines ( eg, 1 paramedic, 1 hospital) * APLS algorithm for convulsion child (tells you when to give next medication)

Answer 65

* **Chickenpox**: 1-3 weeks after infection with most infectious 1-2days before rash. Spots develop which become red and fluid filled blisters within day or two and then become scabs which drop off. Keep hydrates, pain killers (not ibuprofen), baths, loose clothes, calamine lotion, distract form scratching, let school know and kepe away from pregnant women. * **Measles** – Symptoms 7-12days after infection. Infectious 4days before rash, 4 days after. Begins as bad cold with cough and sore, watery eyes. Gradually more unwell, with fever. Rash appears after 3/4th day. Spots are red and slightly raised, may be blotchy but not itchy and begins behind ears and spreads to face and neck. Illness usually lasts a week. Complications are pneumonia and death. Plenty rest, drink, warm drinks, paracetamol/ibuprofen, if eyelids are crusty gently wash with water and if trouble breathing, seizure, coughing lots, drowsy etc then medical advice urgently. * **Mumps** – Symptoms 14-25 after infection. Infectious 6days before swelling in face and 5 days after. Feel unwell, high temperature, pain and swelling on side of face and under the chin, discomfort when chewing. Rare to affect boys testes. Give paracetamol/ ibuprofen, lots to drank (not fruit juices), only see GP if other symptoms like severe headache etc. * **Slapped Cheek Disease (f**ifth disease or parvovius B19) : symptoms 1-20days after infection and infectious few days before rash starts and then not contagious when rahs appears. Symptoms are fever, nasal discharge, bridgh red rash on cheeks which spread to trunk and limbs. Those with blood disorders may become anaemic. Make sure fluids, painkillers. * **German Measles** (Rubella): Symptoms 15-20days after infection, and infectious 1 week before symptoms to 4days after rash disappeared. Starts like mild cold, rash appears in a day or 2 onf ace then body (flat and ale pink), glands in neck may be swollen and child usually wont fele unwell. Plenty to drink, painkillers. * **Whooping cough**: Symptoms 6-21days after infection and infectious from first sin illness till 3weeks after coughing starts(if antibiotic given then stops 5days after treatment) . Similar to cold. Cough but cough gets worse, coughing fits after 2 weeks and may choke and omit. Can go on for several weeks. If gets worse or mor coughing see GP.

Answer 66

**Sepsis** – fever, poor feeding, lethargy, focal infection history, predisposing conditions, tachycardic, purpuric rash, multiorgan failure. Bacteria proliferate in the blood stream and the host response releases inflammatory cytokines and activation of endothelial cells which can lead to sepsis. Most common are coagulase negative staphylococcus, staphylococcus aureus, non-pyogenic streptococci and streptococcus pneumonia (Neisseria meningitiidis and E.coli still prevalent). In neonates mostly GBS and E.Coli. Need antibiotics, fluids, circulatory support and can get disseminated iV coagulation.

Answer 67

– inflammation of meninges mostly by viral and self resolving but bacterial can be very severe. Fungal/parasitic in immunocompromised. Can lead to hydrocephalus. * Bacterial - Fever, purpuric rash, neck stiffness, bulging fontanelle, Brudzinski/kernig sign, signs shock, altered conscious level. Headache, laetheragy, seizures... lumbar puncture to diagnosi. Need antibiotics and supportive therapy. Mostly 3rdgen cephalosporine(ceftriaxone) * Viral – form enterovirus, EBV, adenovirus, mumples. Culture or PCR o CSF, stool, urine, nasopharyngeal apsiriate and throat swabs. * If febrile and purpuric rash give IM benzylpenicillin straight away and hospital.

Answer 68

Encephalitis – inflammation of brain substance which may be from direct invasion of brain b neurotoxic virus (HSV)delayed brain swelling or slow virus infection. Mostly fever, altered consciousness and often seizures. High dose IV acyclovir until rule out HSV by CSF.

Answer 69

– Toxin producing S.aureus and G A streptococcus. Fever over 39, hypotension, diffuse erythematous macular rash, GI dysfunction, renal impairment, liver impairment, clotting abnormalities, CCNS altered consciousness. Intensive care and antibiotics (3rd gen cephalosporin, ceftriaxone and clindamycin. May give iV immunoglobulin

Answer 70

– rare severe subcutaneous infection often involving tissue panes from kin down to fascia and muscle. Severe pain and systemic illness. May be S.aureus OR group A streptococcus. IV antibiotic therapy and surgical intervention to stop the spread and maybe IVIG.

Answer 71

* Look at weight loss * Loon at fontonelle * Look at capillary refill time on chest * Check for sunken eyes out * Skin turgor on abdomen **Management:** Rehydration with correction of fluid and electrolyte imbalance. * No signs dehydration then encourages normal fluid intake and oral rehydration salts as supplemental fluids if worsens. * Evidence clinical dehydration – give 50mL/kg ORS over 4hours in addition to maintenance fluids as ORS. * Evidence shock (decreased conscious levels, poor perfusion, hypotension) then give 20mL/kg 0.5% saline rapidly and repeat if needed then continue with IV rehydration with 0.9% saline adding 100mL/kg to maintenance requirements. **Calculating maintenance fluids:** * 100mL/kg/24h for 0-10kg bodyweight * 50mL/kg/24h for 10-20kg bodyweight * 20mL/kg/24h for \>20kg bodyweight * If diarrhoea continues, give additional 5mL/kg ORS for each large watery stool.

Answer 72

* Effort of breathing – recession, respiratory rate, inspiratory or expiratory noises, grunting, accessory muscle use, flare flaring of the alae nasi. * Efficacy of breathing – breath sounds, chest expansion, abdominal excursion * Effects of inadequate respiration – HR, skin colour, mental status

Answer 73

* Narrowed airways: Secretions, foreign body (luminal), inflammation, smooth muscle contraction, thickening (wall), fibrosis, tumour, congenital abnormality (extrinsic), Intra of extra-thoracic * Failure of gas exchange – above pus alveolar pathologies: fibrosis, surfactant deficiency, pulmonary embolus * Reduced lung compliance – CF + chronic lung disease of prematurity (chronic fibrosis), prematurity (surfactant deficiency), heart failure secondary to CHD (pulmonary venous congestion). * Acidosis – metabolic disorder, especially neonate or infant with acute decompensation, ketoacidosis with diabetes or ingestion of poison eg, aspirin. * Afferent receptor stimulation – tumour (caused by hiccupping) * Psychogenic – panic attack * Restrictive abnormality – kyphoscoliosis * Chest wall defect – flail segment in trauma

Answer 74

* Physical assessment – Functional effects ( posture, activity, speech, feeds, sleep, conscious level0, Work of breathing (RR, recession, accessory muscle usage, nasal flaring, grunting) * 02 sats – O2 sats, cyanosis, pallor, arterial and capillary gases * PaCO2 – increased (exhaustion, chronic + acute lung disease, asphyxia, uncompensated metabolic alkalosis), decreased (mild + moderate asthma, psychogenic) * Trajectory – improving or worsening? Diagnosis specific eg, epiglottis (get ENT surgeon and anaethetist)

Answer 75

* Infection * Viruses – RSV, parainfluenza, rhinovirus, corona virus, adenovirus * Bacteria – strep penumoniae, haemophilus, staphylococcus aureus, mycoplasma, chlamydia, mycobacteria, listeria, strep B * Fungi- aspergillus * Site -Coryza (nasal), pharyngitis, tonsillitis, epiglottitis, laryngo-tracheobronchitis (croup), tracheitis, bronchitis, broncholitis, bronchopneumonia, lobar pneumonia. * Complications – effusion, empyema, pneumothorax * Transmission – nursery, siblings, grandparents, hospital. Droplet vs direct * Allergy – asthma (coryzal illness, cold, damp, exercise, pollen, house dust mite), anaphylaxis (peanut, tree nuts, bee stings (venom) * Prematurity – surfactant deficiency * Congenital – genetic (CF, atopy, metabolic disorder), organogenesis (bronchogenic cyst, congenital heart disease) * Psychological – Anxiety (predicament, panic attack) * Accident – trauma (pneumothorac, flail chest), inhaled foreign body, ingested toxin (eg aspirin) * Neurological – aspiration (on swallowing or with reflux), scoliosis * Diabetes – ketoacidosis ‘air hunger’ * Tumour – compression or invasion, effusion

Answer 76

* Bedside – SpO2, PEFR (8+, pre/post reliver), vitalograph (8+, obstructive/restrictive), capillary blood gas (esp/ when reduced conscious level. Exhaustion, chronic lung disease has increased co2 and compensation by raised gas excess. Metabolic disorder i low base excess. * Common – CXR (avoided in typical bronchiolitis, for penumona, foreign body, pneumothroac etc), NPA (nasopharyngeal aspirate for RSV antigen), pernasal swab (pertussis), blood culture (pneumonia), infection bloods (WCC + differential count, CRP, ESR all non specific). Therapeutic trial (salbutamold). * Uncommon – USS (effusion), CT (effusion from pneumatocele of staph aureus pneumonia), spial ct (tracheal/bronchial morphology), contrast swallow (external compression), bronchoscopy (biopsy, microbiology…), co-oximetry (CO poisoning) * Specific – DeltaF508 (CF), sweat test (CF), histamine challenge (asthma) * Heart – ECHO (morph, direction/velocity blood flow), ECG (arrhythmia, ventricular hypertrophy), cardiac catheter (blood gas sample, pressure measurements an XR screening).

Answer 77

* Symptomatic support – Explanation, 2, airway suction, CPAP, ventilation, nasogastric tube feed * Inflamed airways – Abs, anti-virals, steroid, immune modulators (leukotriene antagonists), adrenaline * Narrowed airways – beta agonists (salbutamol), anticholinergics (Atrovent), Xanthines (aminophylline) * Mucus/secretions – anticholinergics, DNAase, physiotherapy, bronchoscopy * Foreign body – bronchoscopy * Acidosis – specific to cause eg, insulin for DKA, dialysis for toxin, substarte for metabolic disorder * Heart failure – diuretics, calciuk antagonists, ACE antagonists, digoxin * Surfactant deficieny – give surfactant, ventilation, prevention by steroids to mother * Spacer +/- mask inhalation – asthma treatments * Nebulization – 02 driven * Chest drain - for effusion or exudate (empyema), rarely for penumothroac esp, neonates on ventilator * NG tube – where feeding impaired

Answer 78

Acute illness – RR, sats, PEFR, rexamine, transcutaneous CO2, capillary gas, arterial gas Chronic illness – pEFR (diurnal variation), symptom + treatment diaries (beta agonist usage), theraptuic compliance, inhaler technique, school attendance, participation (eg school trips).

Answer 79

**Patient counselling/ education:** * Explain asthma * Explain use of metered dose inhaler and spaces * Explain roles of reliever and preventer * Explain how to do PEFR, monitor it and what it means * Explain preventative measures to ameliorate asthma * Explain time course of bronchiolitis and role of health interventions * Break news of CF * Explain recessive inheritance **Protecting individual/ pop:** * Effect of smoking on admission for breathing disorders * Immunization: pertussis, TB, Hib, diphtheria, pneumococcus, measles * Monoclonal human antibody for RS (Paluvizimab) for at risk groups * Attendance at nursery, school during acute illness * Management in nursery or school with chronic illness. *

Answer 80

Speed of temperature increase, response to antipyretics?, HR, RR, BP, CRT, Hydration, behavior of child, interaction with carer, hydration, skin colour, conscious level (AVPU)

Answer 81

* Under 4w then electronic thermometer under axilla. In 4w-5yrs, electronic or chemical dot thermometer in axilla or infrared tympanic thermometer. * Body’s response to inflammation/ infection (but doesn’t always mean infection). Induced by the cytokines TNF-a, IL-1, IL-6. * Inflammatory disorders like JCA, CT disorders, malignancy (leukemia, sold organ tumours like neuroblastoma) also causes. * Pyrexia of unknow origin: \>38 on several occasions, accompanied by more than 3 weeks of illness, failure to reach diagnosis after one week of inpatient investigation.

Answer 82

* Blanching or non-blanching (tumbler test) * Most viral and some bacterial fade under pressure * Non blanching: * Petechial (1-2mm) red purple dots causes by capillary bleeding. Can be IPP, check for non platelet causes or non accidental injury, severe bout coughing * Purpuric \>2mm larger spots form capillary bleeding – HSP (vasculitis), meningococcal (infections) * Bruises and echymoses will be larger than 1cm (bleeding disorder).

Answer 83

1. Measles (Rubeola) 2. Scarlatina (scarlett fever) 3. German measles (Rubella) 4. Staphylococcal scalded skin syndrome 5. Erythema infectiousim (5th disease) 6. Roseola infantum

Answer 84

* Blanching * Uncommon, few outbreaks, coryzal illness, cough + conjunctivitis, intense photophobia, rash starts at hairlines, spreads cephalo-caudally. * Miserbale, high temperature. * Diagnosis is clinical, Koplik spots in mouth (last only 1day or two, like salt sprinkled on cheek), morbilliform rash (maculo papula). * Labs, measles IgM Ab. Complication are pneumonia and SPPE * Begins face and behind ears spreading to trunk and extremities within 36hrs. Fades after 3-4days. Palmar sparing. Complications are otitis media (deafness), pneumonia and contact In pregnancy

Answer 85

* Blanching * due to streptococcus spp, causing sore throat. * Toxin mediated diseas. * Red strawberry tongue, characteristic rash (maculo papula but sandpaper feel). * High temp and miserable. * Need 10days penicillin * 2-4 day incubation, rash 12-48hrs after

Answer 86

* Blanching * Self limiting, milder disease, similar to measles with upper respiratory features. * Tender posterior cervical and occipital lymphadenopathy. * Starts behind ears and spreads lower down. * Infectious 7days before and 7days after. Pink/red macules lasting up to 5days. Severe if contact in pregnancy

Answer 87

* blanching * Usually in neonates, skin appears as scalded, around umbilical, flexural areas, toxin mediated. * Mild fever, very painful rash when baby handled. Flucloxicillin *

Answer 88

* Blanching * (5th disease) Parvovirus B19, common in winter and spring and often asymptomatic. * 3 stages – slapped cheek, lacy rash on extremetiies, rash on trunk. * Spares palms and soles, risk of miscarriage in pregnant contacts

Answer 89

* blanching * exanthem subitem, caused by human herpes virus 6 and 7. High grade fever in relatively well playful child. After 3-4days, fever suddenly subsides and pink maculopapular rash breaks out * High fever, runny nse, irritability 3days then rash. Complications ar every rare (liver involvement/encephalopathy)

Answer 90

* blanching * also caused by: measles, rubella, enteroviruses (coxsackie, echovirus), respiratory viruses (adenovirus, SRV, rhinovirus), Epstein Barr Virus, human herpes viruses, parvoviruses, drugs eg amoxicillin in EBV

Answer 91

* blanching rash * Unknown aetiology, set diagnostic criteria: fever 5days plus 4 or more of following (bilateral non exudative conjunctivitis, cervical lymphadenopathy\>1.5cm, changes in lips and tongue-becomes red strawberry tongue, polymorphous rash, red palms and sole with indurative oedema (sausage fingers)) Peeling of fingertips about day10. * Bloods will show high platelet counts, cultures will be negative. Associated with coronary aneurysms and myocarditis. * Very miserable and temp doesn’t come down unless high dose aspirin or immunoglobulin.

Answer 92

Mild self-limiting rash usually associated with infections mycoplasma, herpes. Target lesions, symmetrical fixed urticarial. Abrupt onset. Last for good few weeks (2weeks) . Central zone dusky purple may develop into blister. Severe forms with mucosal (eyes, oral cavity, genitalia) involvement is Stephen Johnson syndrome. Causes – hypersensitivity to drugs or infection (NSAIDs, baributrates, penicillins, HSV, Varicella, mycoplasma)

Answer 93

May be sinister or less dangerous than it looks. Mainly meningococcal, pneumococcal septicaemia. Others are invasive Group A STREP. Could be Henock cholein prupura. Tests – bloods, cultures, coagulation.

Answer 94

Very common, febrile illness with rash that occurs in crops. Rash develops through various stages and eventually scabs off. Maculo-paula, vesiculopustular, haemorrhagic, scabs (depends on ages etc) . Intensely itchy (can use calamine illness, anti-histamines) , highly infectious. Complication’s – superadded bacterial infection, pneumonia, meningo-encephalitis, late reactivation causing herpes zoster (shingles-dermaotme, doesn’t cross midline). Dangerous 5 days before to 5 days after delivery if pregnant. Treatment is nothing for mild, just symptomatic. Lasts 1-3weeks and may scar.

Answer 95

* Vomiting center stimulation: * Toxin/Drug – emetic, chemo, alcohol, uraemia, ketones, hyperammonemia, toxin producing staphylococcus and bacillus cereus, irradiation * Sensory input – vestibular system (motion sickness, labyrinthitis), pharyngeal stimulation (self induced), pain from viscus eg, torsion testis * Pressure – space occupying lesion, raised intracranial pressure * Autonomic input – excess fluid accumulation in duodenum and stomach * Unknown – congenital drenal hyperplasia and Addisonian crises, cyclical vomtiign syndrome like migraine? * Intestinal fluid accumulation – malabsorption (cholera, coeliac, small bowel bacterial overgrowth…), over production of fluid (infective stimulation of secretion). Dysmotility and stasis (ifnectin, opiates, inflammation mucosa). * Obstruction – luminal, mural and extra mural * GORD – lower esophageal sphincter dysfunction (constituitional, postural, hiatus hernia, drugs, hormones, intraabdominal pressure) * Obstruction – Luminal (bezoar and other foreign body, worms, constipation, meconium ileus), mural (pyloric stenosis, hirschprungs, leoima, stricture), extra mural (incarcerated hernia, malrotation and volvulus).

Answer 96

**Quantity** – volume, frequency, duration **Quality –** effort, blood (effect of vomiting?), bile (obstruction or problem distal to pylorus) **Effects** * Dehydration- exam of hydration, change in weight, history birth, oral intake, urine output. Inevstigate with urea, creatinine, serum, urine osmolarity * Electrolyte depletion – sodium + potassium in blood + urine. Capillary/arteria gas for pH. Blood for chloride * Long term weight gain/loss – outcome marker for significant infant gord, skinfold thickness and other measures of fatness

Answer 97

**Common:** * Viral illness – multisystem illness often mild or fluctuating in severity ie, pyrexia, vomiting, misery, few loose stools, rash, runny nose, muscle aches, mild headache etc. Eg common cold viruses and influenzas also hepatitis causing illnesses (HepA, glandular fever, CMV) * Gastroenteritis with virus or bacteria – with diarrhoea especially enteroviruses, rotaviruses and Norwalk agent. Less frequently zoonoes and food poisoning like E.cOLI 507, salmonella, shigella (each can cause bloody diarrhoea) and campylobacter (abdominal pain). Toxin producing but 4 day illness too long. * GORD with exacerbation – long history vomiting with posture and feeds. Exacerbations mostl likely from intercurrent illness * Ingestion of toxin – especially alcohol in teens * Vestibular – motion sickness or labyrinthitis * Iatrogenic -chemo, radiotherapy, ‘antabuse reaction’ **Serious/important:** * Meningitis (through raised ICP especially), DKA (check glucose, U&Es acid base), appendicitis, pyloric stenosis (3-7wks usually), intussusception (6m-3yrs), incarcerated hernia, septicaemia and other significant bacterial infection. Concealed pregnancy. Bulimia (back of teeth erosions) **Rare/particular:** * Newborn apparent intestinal obstruction (oesophageal atresia, duodenal atresia, malrotation, volvulus, necrotizing enterocolitis, meconium ileus, Hirschsprungs, anal atresia and stenosis. * Renal failure – can present insidiously * IBD – Crohns and UC * Brain tumour – headache and neuro signs * Entriculo-peritoneal shunt failure * Metabolic disorders – particularly with altered conscious level * Torsion of testis

Answer 98

* Quality * Common associated symptoms – general infection (pyrexia, rash, coryza, urinary symptoms), Gastroenteritis (diarrhoea?), surgical disorders * Effects – hydration (weight change, urine output, thirst, fluid intake etc), demeanor * Hypothesis as to cause: * Gastroenteritis – recent contacts with symptoms, recent parties, travel etc * GORD – long history, effortless, postural, association with feeding * Ingestion or iatrogenic – tablet available * Food allergy or malabsorption – particular food * Diabetes – recent weight loss, thirst, polyuria, polydipsia * Constipation – soiling, infrequent bowel actions, abdominal distention * IBD – weight loss, blood, mucus in stool * Hernia – lump? * Exotic infective illness -travel

Answer 99

* General: State nutrition, hydration, foetor (significant bowel patho and diabetes) * Abdo: Inspect (distention, girth, cough impulse), palpate (tender, guarding, pylorus, hernia), percuss (tympanic bowel), auscultate (bowel sounds of obstructuob/ileus), rectal (constipation, Hirshprung, tender with appendicitis, blood with enterocolitis + IBD) * CNS: GCS (play + interest in environment), cranial nerves (raised ICP/SOL), peripheral nerves (focal impairment attribute to SOL- space occupying lesions) *

Answer 100

**Biochemistry:** * U&Es + creatinine – electrolyte derangement and hydration status * Blood glucse- exclude diabetes or indicate rare metabolic disorder with hypoglycaemia * Capillary gas – with excessive vomiting, possible diabetes or depressed consciousness especially * Urine electrolytes + creatinine – useful with long term and ongoing losses that need supplementation * Blood and urine osmolarity – hydration when syndrome or inappropriate ADH production * Ammonia – if associated depressed conscious level without obvious cause * Alcohol, drug screen * LFTs – persistent malaise suggestive of hepatitis, follow up with bilirubin and clotting If abnormal. **Haematology** – FBC + film – blood in vomit or diarrhoe,a low platetes, schistocytes in haemolytic uraemic syndrome **Microbiology:** * Stool – culture + sensitivity for salmonella, campylobacter. Viruses for gastroenteritis. Clostridia difficule toxin for pseudomembranous colitis. * Urine – M,C,S for infection * LP- for meningitis if no signs raised ICP **Radiology:** * AXXR – bowl obstruction * Abdo USS – appendicitis, malrotation, intussuseption * Contrast swallow – neuro control of swallow, GORD, malrotation * Abdo CT + contrast – small bowel patho * Barium enema – for large bowel patho (before GI contrast or have to wait) * Cranial MRI – if intracranial lesion suspected **GI:** * pH study – proportion of time acid reflux at 1/3 up, 2/3 down esophagus (\>4 abnormal). * Upper GI endoscopy – visualization of oesophageal erosions, biopsy * Colonoscopy – visual and biopsy IBD

Answer 101

**Monitoring:** * Acute illness – hydration, electrolyte balance, urine output * Chronic illness- weight **Patient counselling + education:** * Use of oral rehydration fluids * Hygiene to prevent cross infection * Patient held acute illness instructions for patient with metabolic disorder * Avoidance of alcohol with metronidazole * Feeding through D&V symptoms **Protecting individual and population:** * Hygiene measures on wards and if gastroenteritis * How long to stay off school with D/V (24horus after lats diarrhoea) * Which forms Gastroenteritis notifiable * Going back to work in food industry after salmonella * Mnaagenging outbreak at scgool

Answer 102

**Metabolic Acidosis** = Decreased Ph, same PCO2, decreased bicarbonate HCO2\<22, DM ketoacidosis, urate acidosis (renal failure), lactic acidosis (decreased perfusion, severe hypoxemia/sepsis), drugs (eg, salicylates), anion gap. **Respiratory acidosis** = decreased Ph, Increased PCO2, Noemal bicarbonate . PACO2\>6, hypoventilation, 12 RD-impaired gas exchange COPD, heroin OD, Chest wall defect, respiratory muscle weakness eg, G.Barre **Metabolic alkalosis** = Increased ph, normal PCO2, increased bicarbonate Hco3 \>26, XS loss (eg, vomiting), ingestion of alkali. **Respiratory Alkalosis** = increased ph, decreased OCO2 and normal bicarbonate. Alkalosis (PaCO2\<4.7), hyperventilation

Answer 103

On air: Pao2 should be \>10KpA On oxygen: \<10Kpa less than % inspired concentration. Eg, 15L/min delivers pprox 50-60% O2 so should have PAO2 of about 40.

Answer 104

* Delivers 24-60% oxygen * Different colors deliver different rates * Flow rate: varies with colour. The correct flow rate to use with each colour is shown on mask along wth percentage of oxygen delivered. * Blue = 2-4L/min = 24% O2 * White = 4-6L/min = 28% O2 * Yellow = 8-10L/min = 35% O2 * Red = 10-12L/min = 40% O2 * Green = 12-15L/min = 60% O2 * Often used in COPD where it is important to not over-oxygenate the patient.

Answer 105

Type 1: One problem: PA02 \<10 Type 2: Two problems: PAO2 \<10 and PACO2\>6

Answer 106

* Only to be calculate in metabolic acidosis * Calculates level of unmeasured anions * Anion gap = measured positive ions- measured negative ions = Na+ - (Cl- +HCO3-) * Normal = 6-12 * High anion gap \>12 is lactic/keto/urate- acidosis * Normal anion gap is diarrhoea, renal tubal acidosis.

Answer 107

**Compensation:** * Through homeostasis this is a response to the initial problem as method of correction * Is not able to compensate “over”. * Respiratory compensation = quick * Metabolic compensation = slow * When there is an attempt made by the body to compensate but the PH remains abnormal then this is referred to as “partial compensation” or “uncompensation” * ABG Interpretation: * How is the patient * Assess oxygenation * Acidosis vs alkalosis * Respiratory component * Metabolic component

Answer 108

Acute epiglottis - inflammation of epiglottis, can cause airway obstruction. Typically present with Dysphagia, drooling and respiratory distress. Was most commonly caused by hameophilius influenzae type B but now mostly vaccinated. Paediatric airway trained clinical to secure airway management, antibiotics, etc. pe B but now mostly vaccinated. Paediatric airway trained clinical to secure airway management, antibiotics, etc. don’t examine thriat as can become full obstructed

Answer 109

Progressive destruction of the extrahepatic biliary system in neonatal period, idiopathic cause. Ohi classification based on anatomy (1, CBD, 2, hepatic ducts, 3, porta hepatitis atresia). Presents with jaundice (Birth to 8weeks anytime). Intraoperative cholangiogram showing biliary obstruction. Conjugated hyperbilirubinemia. If biliary atresia suspected then abdo USS to exclude, hepatobiliary scintigraphy, liver biopsy, cholangiogram. Hepatoportoenterostomy (Kasai procedure) – roux-en-Y loop small intestine joined at hilum of liver after removing biliary system. Should be within 45-60days of life and most go on to need liver transplant. Cholanguogram is gold standard

Answer 110

* Most common cause of LRTI in children under 2. Most commonly by Respiratory syncytial virus (RSV- incubation period 2-5days, shedding 6-21says after symptoms). * RFs - \<3yrs, premature, LWB, male, low socioeconomic group, parenteral smoking, chronic lug disease, CHD, NM disorders, immunodeficiency. * Clinical – start subtle. Coryzal illness (wheeze etc) start then other symptoms pea at 3-5days. Typically apnoea, low grade fever, coryza + often significant nasal congestion, difficulty feeding, signs dehydration, tachypnoea, accessory muscles, fine end inspiratory crepitations + wheeze, hypoxia/cyanosis. * Rapid viral antigen or nucleic acid amplification test of nasopharyngeal secretions can be used to confirm RSV. * Clinical diagnosis * Investigations – vital signs, pulse oximetry, viral throat swabs. * Management – supportive care (antipyretics, airways, fluids, o2 etc) and CR monitoring. * Will continue to cough for weeks after episode bronchiolitis, usually 2 weeks or so but can be up to6 weeks. This is normal and fine as long as not working hard and feeding okay.

Answer 111

* Highly contagious viral illness causes classic vesicular rash caused by varicella-zoster virus. (human herpes virus 3). Can be primary VZV or reactivation (shingles), restricted rash to dermatomal distribution. * After recovery the virus lies dormant within dorsal root ganglia. * Incubation 14-16days, infective 48horus before rash onset until lesions fully crusted over. * Clinical – classic generalized vesicular rash (macules -\> papules -\> Intensely pruritic vesicles then pustules which burst and crust over). Mya also get fever, malaise, feeding problems, vomiting, headache, diarrhea. * Clinical diagnosis but if formal then PCR from swab or VZV antibodies checked * Management – self limiting and supportive. Avoid NSAIDs calamine lotion can help and antihistamines \>1years. Hydration etc and avoid high risk people. Aciclovir only 14+ for presenting with chickenpox within 24hours of rash onset. * Dangerous in pregnancy as can get varicella pneumonia. If within 28weeks can get fetal varicella syndrome (intrautrien infection with skin scarring, hypoplasia alimbs, neuro disorders, eye disorder). * Can complicate with bacterial infection

Answer 112

* Common upper respiratory illness of childhood characterized by barking cough and inspiratory stridor. Most cases are self-limiting with full recovery. * Most common in 6m-3y, boys more than girls. * Parainfluenza type 1 virus is most common. May get secondary bacterial infection. * Viruses infect nasopharyngeal mucosa and extend to larynx and subglottic airways. * Clinical – corzyal symptoms, barking cough, stridor. Clinical diagnosis. * Westley score for severity to mil d/ moderate/ severe to determine need for dexamethasone (bets treatment) , nebulized adrenaline, inpatient care and admission to PICU. * On plain film radiograph is steeple sign (Pic) * Management – based on severity and centres on administration of dexamethasone. * NEVER EXAMINE THROAT IS SUPECT AS ditubrign child can cause full obstruction

Answer 113

* Autosomal recessive multisystem disease predominately characterized by respiratory failure. Caused by mutations to CF transmembrane conductance regulator gene on chromosome 7. Encodes chloride channel. * Most commonly identified mutation is DF508 but there are many and differently affect CFTR production/regulation etc. * Patho – dehydration of airway surface fluid resulting in mucociliary dysfunction. As kids grow older the disease is characterized by chronic and recurrent pulmonary infection. Similar issue in other organs with impaired biliary and pancreatic drainage due to viscous secretions so impaired digestion and malabsorption. * Screened as part of newborn heel prick test * Clinical manifestations – respiratory disease (productive cough and recurrent chest infections that can damage bronchial walls), pancreatic disease (fatty stools and malabsorption), GI disease (constipation), increased risk GI malignancies. * Diagnosis – after a positive heel prick test and confirmatory testing. Immuno-reactive trypsin test/swet test/genetic test * Management – encouraging clearance of secretions and treating/preventing infections. Airway clearance techniques, mucoactive agents to aid secretions (rhDNase, hypertonic sodium chloride, mannitol dry poweder for inhalation). * Pulmonary infection – staphylococcus aureus (oral flucloxacillin), pseudomonas aeruginosa – eradication therpay with oral/IV and inhaled Abs, * Lung transplants for progressive respiratory failure. * Nutrition + pancreatic insufficiency, liver disease (screened) , CR related (glucose monitoring tc) * Novel therapies – CFTR modulators * Median predicted survival age – 49.1 years old newborns

Answer 114

* Genetic condition caused by trisomy 21. * Aetiology – most common is nondisjunction of chromosome 21 during meiosis on the maternal side resulting in trisomy 21. Also is Robertsonian translocation (translocation on long arm C21), or trisomy 21 mosaicism (after fertilization). * Rfs – maternal age. * Clinical features – characteristic facial features (flat nasal bridge, protruding tongue) and can affects multiple organ systems (joint hyperflexible, sort, broad hands), intellectual disability, dementia, cardiac disease, fertility and reproductive problems, haematological problems… * Prenatal screening – combined (ds, patau’s and Edwards at 12weeks, blood tests then or from 10weeks alongside) or quadruple test (DS when NT cant be measured and head circumference between 101 and 172mm. AFP, hCG, inhibitin A, unconjugated oestriol)to be offered to all pregnant people. There is also non invasive prenatal testing rollout. * Prenatal diagnosis – those undergone combined test or quadruple test with higher chance result. Chorionic villus sampling (11-14wks, US) or amniocentesis (15-20) US but both offer risk of miscarriage. * Management – individual support offered and newborns screen for karyoptying, FBC, CHD screening, npnatal hearing screen and eyes. * Mean life expectancy UK is 58years

Answer 115

* Acute viral illness, usually mild and self-limiting. Characterized by papulovesicular lesions on the distal limbs and vesicular eruptions in the mouth. Mostly late summer and early autumn and mostly in children under 10 (\<4) * Most commonly caused by Coxsackie A16 virus but can also be caused by other group A and B Coxsackie viruses or Enterovirus71. Transmitted between humans only. * Not all get lesions on hand feet or mouth and has incubation of 3-7days. There is prodromal period 12-36hrs of fever, malaise, decreased appetite, cough, abdominal pain and myalgia. Mouth lesions which go to vesicles and erode. Distal limb lesions often elliptical with long axis parallel with skin lines. Atypical is nail shedding and widespread. * Investigations + diagnosis- clinical * Management – supportive and treatment of complications.

Answer 116

* IgA vasculitis commonly presenting in childhood. Commonly in 3-15years old. Precipitated by viral illness leading to immune mediated reaction In blood vessels. * Immune mediated due to deposition of IgA immunoglobulin in blood vessels. * Patho – inflammation of blood vessels. * Clinical – Palpable purpura in symmetrical distribution over lower limb. Arthritis/arthralgia, GI upset, severe GI features, hematuria, nephritic syndrome, other… * Diagnostic criteria - Based on EULAR and PRES diagnostic criteria. Must have palpable purpura in absence of thrombocytopenia or coagulopathy. Must have at least one of others like diffuse abdo pain, acute arthritis, renal involvement etc. * DDx – sepsis, ITP, medication induced thrombocytopania, bone marrow failure, CT diseases * Investigations – investigations help rule out things so do urine, protein/creatinine ratio, BP and bloods, imaging not routine * Management – most make a fully recovery and monitored as outpatient. Most over 6weeks supportive therapy. Steroids can shorten duration symptoms but don’t affect clinical course * Estimated up to 50% of children have preceding upper respiratory tract infections.

Answer 117

* Telescoping of intestine into neighbouring segment mostly in infants and young children. * Common abdo emergency 3m-3y presenting with distress and severe abdo pain accompanied by vomiting and bloody stools. * Mesentery of telescoped segment of intestine is impaired leading to reduced venous and lymphatic outflow. Intestinal oedema, obstruction + ischemia develop. * Patho – most are idiopathic. Most commonly ileocolic (terminal ileum invaginates into colon). The mesentery of the affected segment becomes involved, and pressure prevents normal venous and lymphatic drainage. Lead points refers to abnormalities that predisposes – Meckel’s diverticulum, vascular malformation, lymphoma, parasites, thick stool, polyps. * Clinical Features – worsening abdominal pain and vomiting that becomes bilious in a distressed child. * Investigations – abdominal US, bloods, (Xray but poor sens.) * Management – often managed with non-operative radiologically guided reduction. Either using hydostatic approach with saline or contrast or pneumatic so air. Can operate for those unstable.

Answer 118

* Jaundice in preterm or term babies within 1st month life. * Most commonly due to physiological jaundice occurring in first weeks of life. * Physiological jaundice – harmless, with no underlying cause, breastfeeding closely linked. * Pathological jaundice – haemolysis, inborn errors metabolism (gilbert and crigler-najir syndrome) , obstruction (biliary), systemic disease. * Affects up to 80% of neonates born before 37weejs gestation * RFs – preterm babies more likely, ethnicity, maternal factors (DM, \>25, exclusive breastfeeding) * Patho – bilirubin is breakdown product of hemoglobin. You get increased level causing yellowing of skin and mucous membranes. Can get kernicterus which is yellow staining of cerebral tissue due to bilirubin deposition (as unconjugated can cross BBB) and so acute/chronic bilirubin encephalopathy * Clinical features – yellow discoloration of skin + mucous membranes., * Visual/ clinica, * Acute bilirubin encephalopathy – lethargy, irritability, abdnormal muscle tone/posture, apnoea episodes, convulsions * Chronic bilirubin encephalopathy – cerebral palsy, hearing loss, visual, dental dysplasia. * Diagnosis + investigations – clinically then confirmed with serum bilirubin measurements. * Management – depends on treatment threshold graph/table and includes photodynamic therapy or exchange transfusion. Need to prevent irreversible neurological damage for bilirubin deposition. * Phototherapy – blue green light 460-490nm which converts unconjugated bilirubin into water soluble molecules that can be excreted. * Red cell exchange – removal of newborns blood and simultaneously replacing it with compatible donor. This leads to dramatic reduction in bilirubin levels.

Answer 119

* Affects hip joint in children and characterized by idiopathic avascular necrosis (death of bone tissue secondary to impaired blood supply) of femoral epiphysis (proximal end) an resulting in structural changes. * Mostly males, Caucasians, 3-1years old * RFs – males North European descent. * Patho – idiopathic avascular necrosis of the hip in children * Clinical – Atraumatic hip pain and limp. * DDx – septic arthritis, slipped capital femoral epiphysis, transient synovitis… * Imaging – pelvic x rays and MRI hips to diagnosis and monitor Perthes. * Symptomatic relief and physiotherapy * Risk OA later in life.

Answer 120

* Also known as infantile hypertrophic pyloric stenosis * Narrowing of pylorus leading to intestinal obstruction in infants. Hypertrophy + hyperplasia of pylorus * Aetiology – multifactorial involving environmental and genetic factors. * Presentation – non-bilious vomiting (mostly projectile). Often 2-8weeks, normally 30-60mins after being fed. * Investigations – Abdominal USS. Bloods can evaluate effects of vomiting. * Management – Fluid resus and electrolyte abnormalities corrected. Surgically with pyloromyotomy ehich is cutting thickened muscle to release stenosis. There are Ramstedt’s and laparascopic.

Answer 121

* Infrequent passage of dry, hardened stool. Associated with straining, pain or bleeding * Clinical – infrequent bowel movements, straining, abdominal pain, loss of appetite, soiling, overflow diarrhea. * Examination – well child, abdomen soft, palpable faecal mass in lower abdomen. * Laxatives – disimpaction regimen, stimulant, osmotic, stool softener * Management – encourage oral fluids, encourage health, fibre rich diet and toileting routine **Red flags:** * Failure to pass meconium in 24h of life (Hirshsprungs) * Faltering growth (Hypothyroidism, Coeliac disease) * Abnormal lower limb neuro/deformity (Lumbosacral pathology) * Sacral dimple over natal cleft (Spina bifida occulta) * Perianal bruising (sexual abuse)

Answer 122

Caused by H influenzae B. Intense swelling of epiglottitis and sepsis Emergency – higher risk of airway obstruction Clinical – high grade fever, unwell looking child, drooling, no cough, soft stridor.

Answer 123

* Anaphylaxis = severe life-threatening systemic hypersensitivity reaction. * Presentation is sudden onset and rapidly progressing. * Clinical – Angioedema, stridor, wheeze, tachypnoea, tachycardia, shock, vomiting, urticaria, collapse. * Acute management – ABCDE, adrenaline 1:1000IM, oxygen, nebulizers, fluid bolus, hydrocortisone chlorphenamine.

Answer 124

* High pitched whistle sound, usually hear don expiration, indicated narrowed airway * Causes – asthma, bronchiolitis, viruses, foreign body aspiration, structural abnormalities, congenital heart defects, GOERD * Treatment – 02 as needed, SABA (if doesn’t respond to SABA ask why) * Approx 50% will have one episode before age 6 but only 20% go on to have asthma. * Absence wheezing in patient who presents with acute asthma may suggest impending respiratory failure so make sure you hear movement through the lungs.

Answer 125

* Common causes – RSV, Rhinovirus, Coronaviruses, Parainfluenza, Influenza * Remember to ask: Prior episodes, admissions, ITU trips, ever needed IV therapy, steroids given previously, FH atopy, use inhalers at home and hwo often, explore triggers.

Answer 126

* **T1DM** - Autoimmune, lack of insulin. T-cell mediated autoimmune destruction of the B cells in pancreatic islets of Langerhans, perhaps triggered by environmental factors (eg, viruses) in people with genetic predisposition. Osmotic diuresis when blood glucose conc exceeds renal threshold. Ketoacidosis develops when insulin efficiency is severe. Associated with HLA-DR3/DR4 * T2DM = reduced sensitivity to insulin (more common in adults). RFs are obesity, positive FH, S.Asian or African/Caribbean heritage, female sex. T2DM may have acanthosis nigricans (velvety-brown-black patches of skin) or present with infections (skin infections or UTIs) * Maturity onset diabetes of the young: autosomal dominant form with similar presentation to T2DM * Diabetes secondary to pancreatic destruction/failure: CF associated diabetes, pancreatitis, pancreatic trauma or surgery. * Associated with genetic syndromes (eg Downs, Wolfran, DIDMOAD (diabetes insipidus, diabetes mellitus, optic atrophy and deafness.))

Answer 127

* Presentation – dehydration, shock, N/V, abdominal pain, drowsiness, acidotic respirations * ABCDEFG – don’t ever forget glucose * Ketonemia, hyper-glycaemia, acidemia * Treatment – Complex and always requires senior support, often needs 1:1 Nursing care. Combo of fluids, insulin and potassium. * Complications – Hypokalaemia, cerebral oedema, hypoglycaemia, VTE. * pH\<7.3 (\>7.1 mild-mod/ \<7.1 severe) Glucose \>11 and dehydration, drowsy, vomiting, bicarb \<15

Answer 128

* Transient synovitis - associated with viral infection, sudden onset, resolvs \<1wk * Septoc arthritis - red/hot/painful joint, fever, mostly under 2yrs, abs, surgery * Perthes diease - avascula rnecrosis of capitla femoral epiphysis, 5-10, rest * Slipped capital femorla epiphysis - displaced epiphysis of fmoral head postero-inferiroly, mostly 10-15, surgicalmanagement promptly.

Answer 129

* –“irritable hip syndrome”: * Most common cause of acute hip pain in children * Associated with viral infection * Sudden onset hip pain or limp * No pain at rest * Reduced ROM – internal rotation * Usually resolves within 1 week. * Age 2-12

Answer 130

* Red, hot, painful joint, reduced ROM * Fever and unwell child * Limp/unable to weightbear * Most common under 2 * Investigations – FBC, CRP, ESR, Blood cultures, joint aspirate, X-Ray * Treat with antibiotics +/- surgical washout

Answer 131

* : Avascular necrosis of capital femoral epiphysis * Occurs due to interruption of blood supply, followed by revascularization and reossification over 18-36m * Mainly affects boys and 5-10years * Xray hip including frogs leg views * Management – Rest, physio, casts and someties surgery

Answer 132

* Displacement of epiphysis of femoral head postero-inferiorly * Most commo at 10-15 * Commonly occurs during adolescent growth spurt * Increased risk associated with obesity * Restricted abduction and internal rotation * Xray hip including frog lateral views * Surgical management and should be prompty to prevent avascular necrosis.

Answer 133

* Leukemia = cancer of blood cells * Most common cancer in children * Presentation insidious over weeks – lethargy. Pallor, generally unwell, non blanching rash, frequent infectins, lymphadenopathy, hepatosplenomegaly. * Incidence peaks age 2-3 * 80% of leukemias in children are ALL * Investigations – FBC, Blood film, bone marrow aspirate/biopsy, lumbar puncture * Survival rate of ALL after treatment with chemo is over 90%

Answer 134

* A ¼ of cases of IBD present in childhood * Crohn’s disease – affects any part of GI tract from mouth to anus. Non caseating epithelioid cell granulomata * Ulcerative colitis – confined to colon. Mucosal inflammation and crypt cell damage * Presentation – Abdo pai, diarrhoea, failure to thrive, weight loss, delayed puberty, extra-intestinal manifestations (oral lesions, uveitis, arthralgia, erythema nodosum) * Investigations – FBC, CRP/ESR, Fecal elastase, Biopsy. * Crohn’s Mx – nutritional therapy, systemic steroids, immunosuppressants, Anti-TNF (Infliximab) * UC – Aminosalicylates (Mesalazine), topical or systemic steroids, immunosuppressants

Answer 135

Red Flags: * First seizure with accompanying fever, confusion, fluctuating consciousness, coma, or abnormal exam findings * Rapidly recurring convulsive seizures without other accompanying symptoms/ signs * A child who has sustained significant injury either before or as a result of the event * Syncope with abnormal vital signs, cardiac exam and/or ECG

Answer 136

* Syncope during exercise or when supine * Syncope preceded by palpitations * Syncope in a child with known congenital heart disease * Heart murmur or other abnormalities on CV exam * Any abnormal findings on 12 lead ECG * FH of sudden death, prolonged QT syndrome or hypertrophic cardiomyopathy *

Answer 137

* Insulin = Stimulates glucose uptake into liver + muscle, stimulates liver + muscle to store glucose as glycogen. Stops production of glucose in liver * Lack of insulin = stops uptake of glucose from blood into liver + muscle, stimulates breakdown of glycogen into FFA and ketones. Stimulates hepatic gluconeogenesis. * Breakdown of glycogen/fat stores -\> ketone body production -\> acidosis * Hyperglycemia -\> glycosuria -\> loss of water + electrolytes **Injectable Insulins:** * Short acting – Novorapid/Humalog (onset 15mins, peak action 2hrs, last at most 3/4hrs) and Actrapid (IV for DKA * Long acting – Degludec (Tresiba- onset 2-4hr,lasts 24-48hrs), Levimir (Detemir-onset 2-4hrs,lasts 24hrs), Glargine (Lantus- onset 2-4hrs, lasts 24hrs) * Basal Bolus/MDI: long acting at night, short acting with meals. More injections, more flexibility. * Pumps: Variable basal rates, boluses with food and for correction of hights. Can programme temporary rates for eg, illness, but needs more monitoring. Short onset of DKA and always attached

Answer 138

* Total daily dose insulin = 0.65 units/kg/day (0.6 \<8) * Basal insulin (Degludec) = 0.35 U/kg/day above T.D.D at bed time (0.3\<8) * Carb ratio: * \<8 = 300/T.D.D * 8+ = 500/T.D.D * I.S.I (insulin sensitivity index)= 100/T.D.D. Correct blood sugar to 6mmol/l * Bolus doses (Hovorapid) = dose for carbs + correction bolus * Carb counting **Correcting Hypo (\<4 DM):** * Short acting – 1glucose tablet 3g, 3-4 tables. * 10-15g carbs also (3 jelly beans, 2/3jelly babies, 3 wine gums, 120ml orange juice etc) * Long acting – 10-15g (1 packet crisps, 1 apple, 1 pear, 300mls milk…)

Answer 139

True/central (Gonadotrophin dependent) = Idiopathic, CNS tumour, Neurofibromatosis, septo-optic dysplasia False (Gonadotrophin independent) = peripheral causes

Answer 140

Premature Thelarche (breast development): * Temporary activation of FSH- oestridiol axis, self limiting and innocent, girls age 6/12-3 years * Never more than stage 3, no other signs puberty, normal growth and boen age * Pre pubertal gonadotrophin levels Premature Adrenarche: * Normal increase in adrenal androgen release in mid-childhood (6-8) with maturation zona reticularis * Can produce early pubic hair and small growth spurt * Care if pubic hair and or growth continue or if \<6yrs Gynaecomastia: * Normal puberty, obesity, Testicular/germ cell tumours, kleinfelters.

Answer 141

* Androgen excess – congenital adrenal hyperplasia, cushings disease, adrenal neoplasm * Breast development only – ovarian hyperstimulation, testicular/germ cell tumours * Testosterone Excess – tumours, testotoxicosis

Answer 142

* Constitutional delay in growth and puberty * Hypothalamic/Pituitary disorders (Hypogonadotrophic hypogonadism): idiopathic, isolated gonadotrophin deficiency (Kallmans), pituitary hormone deficiency (post radiation, chemo, surgery, tumour) and chronic ill health/anorexia * Syndromes – Noonans, Prada-Willi * Hyergonadotrophic hypogonadism: Gonadal dysgenesis (Turners, Kleinfelters, XY gonadal dysgenesis), Gonadal damage (radiation, chemo vascular accident)

Answer 143

* Inadequate breast development – gonadal dysgenesis, Poland anomaly * Inadequate pubic hair- androgen insensitivity, adrenal failure * No menarche – Polycystic ovary syndrome, absent ovaries/uterus, Imperforate uterus, pregnancy * No growth spurt – Hypothalamic-pituitary disorders, skeletal dysplasia

Answer 144

* Due to mutations in CYP21B gene on Ch 6 * Variable phenotype * Salt wasting – 2 severe mutations – complete or near complete loss (\<1%)of 21 hydroxylase function * Simple virilizing – 2 mutations but 1 relatively mild – 1-5% of 21 hydroxylase function, rarely get salt loosing crisis but can get high renin. * Non classical/late onset – 2 mutation but one is mild – Present in adolescents with androgenicity +/- PCOS * Presentation: * Neonatal presentation – virilization at birth 9easier to spot with girls) or salt loosing crisis (2nd week, low Na/High K/ Low or normal glucose) * Androgen excess in childhood – enlarged penis/clitoris, pubic hair, greasy skin, excess growth, advanced bone age * Precocious puberty – early priming of the hypothalamic-pituitary axis to sex steroids * Hyperandrogenism in adolescents

Answer 145

Primary * Congenital – adrenal hypoplasia, congenital adrenal hyperplasia, familial glucocorticoid deficiency, stAR deficiency * Acquired – autoimmune (isolated-Addison’s, multiple autoimmune problems-A.P.S), Adrenoleucodystrophy, T.B, Idiopathic (bilateral adrenalectomy), Drugs – Corticosteroids. Secondary (Tertiary) * Hypopituitarism, septo-optic dysplasia, isolated ACTH deficiency, craniopharyngioma. Cranial irradiation, surgery. Treatment: * Initial crisis and severely sick days – fluid resuscitation glucose/dextrose (0.9% saline/5%dextrose), IV hydrocortisone 6hourly * Long term – hydrocortisone TDS, fludrocortisone * Monitor growth

Answer 146

* Symptoms: Usually asymptomatic, sleepiness,s poor feeding, jaundice, constipation, Goitre, Dry skin, wide fontenelle, 1:4000 * Causes: iodine def (commonest), thyroid agenesis 70-80% (agenesis vs ectopia), thyroid hormone synthesis problem, TSH/TRH deficiency, G-Protein problem * Transient: maternal- iodine deficiency, antibodies, prematurity, sickness. *

Answer 147

Infection of the bone caused by bacteria. Common in \<2m. Commonly affects the femur and tibia. Spread often from haematogenous/infected wound. Management by IV antibiotivs (extended course) and may require surgical debridement.

Answer 148

* Viral 2/3, EBV * Bacterial 1/3 – Group A strep * CENTOR criteria for group A strep (LEAF): Tendor anterior cervical lymph nodes, tonsillar exudate, absence of cough, history of fever. \<1 unlikely GAS, 2-sondier test with throat swab, \>3 likely GAS so antibiotics * If viral – self limiting * If bacterial then penicillin V for 10days (not amoxicillin-glandular fever)) * Tonsillectomy if recurrent infections.

Answer 149

* Infancy: period rapid growth, weight loss initially (up to 10%) but regain by 3weeks, double birth weight by 6m, triple birth weight by 12m. 150ml/kg/day, breast feeding/formula until 12m and weaning around 6m * Toddlers + preschool: rapid -\> slow, limbs, portion sizes, picky eaters, sugary drinks * School Age + Adolescent: Rapid growth in puberty then slows until adult height (female 15, male 17). High calroic needs. At risk of too much fat/salt/sugar and too little vitamins/minerals/exercise *

Answer 150

* Infants = breast milk/formula alone for first 4-6m, solids 6m, finger feeding 7-9. Discourage bottles\>1year, supplemental vitamins, don’t add salt. 110kcal/day * Young children = limit 500ml milk/day, diet with all food groups, 3meals + 2snacks, minimize sugar/sugary drinks, avoid choking hazard foods an food battles. 1000+ 100xage kcal/day

Answer 151

* Breast – how long/often, regular soaked nappies, stool, feed through night, difficulty breathing, signs neur abnormalities or cardiac, rashes * Bottle – how much/often, what type formula. Feeding vary on time started weaning, textural problems, regular vom refuse food. * Older children – meals, snacks, takeaway, last meal, time of mealtime, vegetables, sweets, traditions, cultural influences.

Answer 152

Measuring children: * Weight – birth, 2, 8, 12, 16weeks, 1 year, annually * Length – until 2 * Height – until 2 * Centiel charts * BMI = weight kg/ height^2 (M^2) for 2years+

Answer 153

* Bets indication – weight trends * Classification – mild (falls across 2 centile lines), severe (falls across 3 centile lines) * Thresholds for concern: * Fall across \>/1 centile if birth weight \<9th centile * Fall across \>/2 centiles if birth weight 9-91st centile * Fall across \>3/3 centiles if birth weight ?91st centile * Current weight below 2nd centile, whatever birth weight.

Answer 154

**Exam:** * ABCDE – consciousness, hydration? * Weight/height/length and head circumference * Nutritional status – pallor, wasting of muscle, thin hair? * Evidence chronic illness * Neuro/developmental assessment * Signs of organic/non organic causes **Investigations:**Mostly none but usually guided by history and exam. FBC + ferritin, other bloods, urinanalysis/stools, M,C&S, environmental assessment, hospital admission. **Management:** * ABCDE * Determine severity: Red flags are cardiac, developmental delay, dysmorphic features, failure to gain wait despite ehigh calories, organomegaly, lymphadenopathy, recurrent respiratory infections, recurrent UTI, recurrent vomiting/diarrhoea/dehydration, signs neglect. * Depends on underlying cause * Hospital mx – IV fluids, NG feeds, Gastrostomy for chronic issues * Outpatient – food behaviour diary, paediatric dietician, health visitor meal time obs, social work, close follow up * Age appropriate counselling * Make family aware of resources, admission last resort. * For chornic: Gastrostomy – CNS disorders (intake), GI disorders (absorption), management (2-3day admission, pain management, slowly starting feeds, monitoring fluid + electrolyte status closely.)

Answer 155

Fraser Competent is a term used to describe a child under 16 who is considered to be of sufficient age and understanding to be competent to receive contraceptive advice without parental knowledge or consent.

Answer 156

* Genetic condition, trisomy 21 (non disjunction or robertsonian translocaiton or trisomy 21 mosaicism) * **RFs-** maternal age * **Clinical** - Facial features (flat nasal bridge, protruding tongue), organ systems (joitn hyperflexible broad hands, short), intellectual disability, dementia, cardiac disease, fertility, repro problems, haem problems * **Prenatal screening** - Combined (ds, patau, edwards12wks), quadruple (AFP, Hcg, inhibitin A, unconjugated oestriol). * Diagnosis - chorionic villus sample 11-14 or amniocentesis 15-20 by US but risk miscarriage * Mx - support, hearing etc * mean L/E is 58

Answer 157

* Acute viral illnes,s usually mild and self limiting. Mostly under 10 * **Clinical** - Prodromal period 12-36hrs, fever, malaise, decreased app, cough, pain, myalgia). Papulovesicular lesions on distal limbs and vesicular eruptions in mouth. * **Cause** - coxsackie A16 (can be others0CA/B or enterovirus 71) * **Incubation** 3-7days. * M x- supportive and treat complications

Answer 158

* Telescoping of intestine into neighbouring segment * 3m-3y * **CLinical** - distress and severe abdo pain with vomiting and bloody stools * **Patho** - most idiopathic, mostly ileocoli * **Ix** - abdo US, bloods * **Mx** - non operative radiologically guided reduction. Hydrostatic with saline or contrast or pneumatic so air.

Answer 159

* Jaundice in pretemr or term babies within first 1m life * Physiological - harmless, no cause, breastfeeding linked * Pathological - hemolysis, inborn error metabolusmm systemic disease * RFs - preterm, ethnicity, maternal factors * Patho - (Mostly unconjugated), mroe breakdown Hb. Can get kernicterus which is yellow tsaining cerebral dissuse. * Clinical -yellowing, encpehalopathy (acute vs chronic) * Dx - serum bilirubin * Mx - photodynamic therapy or exchange transfusion.

Answer 160

(Hyaline membrane disease) = Deficiency of surfactant (mix of phsopholipids + proteins excreted by type II pneumocytes) which lowers surface tension. * Patho = Deficiency of surfactant leads to widespread alveolar collapse + inadequate gas exchange. * Epidemiology – More common in the more preterm, usually before 28weeks gestations and more boys. Rare at term but may be in diabetic mothers or genetic mutations. * Prevention = Glucocorticoids antenatally if preterm anticipated which significantly reduces RDS, bronchopulmnary dysplasia, IV hemorrhage in those \<34weeks. * Clinical signs at delivery or \<4hrs = * Tachypnea over 60bpm * Labored breathing with chest wall recession and nasal flaring * Expiratory grunting in order to try creating positive airway pressure during expiration and maintain functional residual capacity * Cyanosis if severe * Treatment – Raised ambient oxygen and surfactant therapy may be by instilling surfactant directly into lungs via tracheal tube or catheter. May. Need additional respiratory support with continuous positive airway pressure (CPAP) or high flow nasal cannula therapy or IV with mechanical ventilation via tracheal tube. Non-invasive preferred. * Complications * Pneumothorax – pulmonary interstitial emphysema from air leaking from alveoli. It can also leak into pleural cavity causing pneumothorax. Usually O2 requirement increases and breath sounds and chest movement reduced onside.

Answer 161

* Serious illness, more incidence in more premature. Bowel of preterm infant vulnerable to ischaemic injury and necrotizing enterocolitis. * RFs – more premature, fed cows milk formula (supplementing milk feeds with prebiotics and probiotics can be beneficial). * Early signs – feed intolerance vomiting (may be bile stained), abdomen distended and stool can contain fresh blood. May rapidly become shocked and need mechanical ventilation from pain and distention. * Dx – Xray features – distended loops bowel + thickening of bowel wall with intramural gas, and may be gas in portal venous tract. Disease may progress to bowel perforation. * Treatment – stop oral feeding and given broad spectrum antibiotics (aerobic + anaerobic organisms)/ Parenteral nutrition needed, possible mechanical ventilation and circulatory support and then surgery for bowel perforation

Answer 162

* Now called bronchopulmonary dysplasia): * Infants who have oxygen requirement still at postmenstrual age of 36weeks. Lung damage mainly from delay in lung maturation but may also be from pressure + volume trauma from artificial ventilation, oxygen toxicity and infection. * CXR – widespread opacification, with possible cystic changes * Tx – some need prolonged aritifical ventilation but most weaned onto CPAP or high flow nasal cannula with additional ambient oxygen. Corticosteroids may facilitate earlier weaning but concerns with these with abnormal neurodevelopment. * Complication – Few with severe disease may die of intercurrent infection or pulmonary hypertension. Subsequent pertussis and respiratory viral infection may cause respiratory failure needed ICU.

Answer 163

* Apnoea + bradycardia + desaturation – Common until 32weeks gestational age, need to exclude underlying cause and need respiratory stimulant caffeine and possible CPAP/ mechanical ventilation. * Temp control – hypothermia caused increased energy consumption ad may result in hypoxia and hypoglycaemia, failure to gain weight etc. * Patent ductus arteriosus – no symptoms or apnoea and bradycardia, increased 02 requirement. * Fluid balance, Nutrition (high requirements due to rapid growth) * Increased risk infection * Preterm brain injury * Retinopathy of prematurity. *Long term outcomes/ Ex-Preterm going home:* 5-10% of very low birthweight infants develop Cerebral palsy but most common impairments are learning difficulties (fine motor skills, concentration, behavioral problems, abstract reasoning, processes several tasks simultaneously. 02 therapy neonatal – recommended 21-20% oxygen to start and avoid over 95%. Avoid \<91% (increased risk necrotising enterocolitis and death) and avoid \>95% (increased risk of retinopathy of prematurity) .

Answer 164

02 therapy neonatal – recommended 21-20% oxygen to start and avoid over 95%. Avoid \<91% (increased risk necrotising enterocolitis and death) and avoid \>95% (increased risk of retinopathy of prematurity) .

Answer 165

* **Causes** – marked physiological release of Hb from break down red cells, red cell lifespan of newborns infants is shorter than adults and hepatic bilirubin metabolism is less efficient in first few days of life. * \<24h = hemolytic disorders, congenital infections * 24h -2 weeks = physiological, breast milk jaundice, infection, bruising …. * \>2 weeks = unconjugated (physiological. breast milk, hypothryoidisim, haemolytic anaemia…) Conjugated (bile duct obstruction, neonatal hepatitis). persistent/prolonged neonatal jaundice. May be caused by biliary atresia. In prolonged unconjugated hyperbilirubinaemia breats milk jauncide is most common and check UTI, congenital hypothyroidism. * Physiological – no underlying cause, infant is adapting * Importance – may be sign of another disorder and unconjugated bilirubin can be deposited in the brain causing kernicterus (encephalopathy due to unconjugated bilirubin in basal ganglia and brainstem with neurotoxic effects). * Clinical – jaundiced when bilirubin reaches about 80umol/l. * Severity – blanching of skin with one finger. Jaundice tends to start head/face and spread down. check bilirubin. * Management: * Phototherapy – light (wavelength 450nm) from blue-green band of visible spectrum converts unconjugated bilirubin into harmless water-soluble pigment excreted mostly in urine. Disruptive to normal care of infant. Can result in temp instability. * Exchange transfusion – if bilirubin rises to levels potentially dangerous. Blood removed and replaced with donor blood. Phototherapy reduces need for this.

Answer 166

Breastfeeding or formula recommended until 12months. After 6m age breast milk isn’t enough so solid foods recommended ot be introduced between 17weeks – 26weeks Milk aspiration – more frequent in preterm + respiratory distress or neuro damage. Those with bronchopulmonary dysplasa often have GORD which predisposes and cleft palate.

Answer 167

* Infestation with mites which burrow down epidermis along stratum corneum. Severe itching 2-6weeks after infestation and worse in warm conditions at night. * Complciations – skin excoriated due to scratching anf secondary eczematous or urticarial reaction. Secondayr bacterial infections. * Tx – whole family treted too. Permethrin cream below neck and washed off after 8-12hurs. Also malathion lotion is good.

Answer 168

**Viral infections**: * Viral warts – human papillomavirus. Tx is daily application proprietary salicyclic acid and lactic acid paint or glutaraldehydte lotion. Also crytohterpay with liquid nitrogen. * Molluscum contagiousum – poxvirus, small skin coloured perly papules with central umbilication. Topical antibacterial and crytotherapy. **Fungal Infections**: * Ringworm, - dermatophyte fungi invade dead keratinous structures like nails, skin, hair. Topical antifungals. **Parasitic:** * Scabies. * Pediculosis – head lice. Dimeticode 4% lotion or aqueous solution malathion rubbed, left, shampooed off.

Answer 169

* **Febrile neutropenia** = neutrophil count of less than 1.0x10^9/L and temperature of 38C+ or above on one occasion. (Low temps may also indicate sepsis and same guidelines to follow) * Empiric therapy with broad spectrum Abs. * Fluid bolus * Ix – FBC, UEC, blood culture, blood lactate, G&S, urine

Answer 170

* **Primary (uncommon)** – genetically determined defect in the immune system. Should be considered in those with Severe, Prolonged, Unusual or Recurrent infections. * Mx – Antimicrobial proph, antibiotic treatment, screening for end organ disease, immunoglobulin replacement therapy, bone marrow transplant, gene therapy * **Secondary (more common)** – caused by another disease or treatment like malignancy, chemo malnutrition, HIV infection, immunosuppressive therapy, splenectomy, nephrotic syndrome.

Answer 171

**Child abuse** = Term which describes all the ways in which a child’s development and health are damaged by the actions, or inactions of others, either by commission or omission. * Physical abuse – shrinking, fear home/parents, unexplained absences, missed appointments * Emotional – developmental delay, misbehaviour, fear new situations, self harm… * Neglect – missed school, failure to thrive, avoid health appointments * Sexual abuse – inappropriate sexual awareness,s aggressive, withdrawn etc (\<13 cant consent to sexual,16 is legal age consent) * Radicalization – isolating, scripted speech, disrespectful attitude, increased anger, secretiveness. **Sfaeguarding** = Protecting children from abuse and maltreatment, preventing harm to their health or development, ensure they grow up safe and enabling them with the best outcomes. Look for RFs. younger, MH, domestic abuse, social/environment **Red flags** = delayed presentation, unexplained injuries, improbable explanations, frequent admissions, missed appointments. Look bruising, roal, fractures, abusive head trauma etc. Raise concern, prioritise child welfase, communicaiton, docs

Answer 172

**Space occupying lesion Red flags:** * Worse on lying down or with coughing and straining * Wakes up child * Associated confusion and/or morning or persistent nausea or vomiting * Recent change in personality, behaviour ot educational performance. * Growth failure * Visual field defects (craniopharyngioma) * Squint * Cranial nerve abnormality * Torticollis * Abnormal coordination (for cerebella lesions) * Gait (UMN or cerebellar signs) * Fundi (papilloedema) * Bradycardia * Cranial bruits (Arteriovenous malformation

Answer 173

* **Patho** – Autosomal recessive disease, HbS inherited. HbS polymeries within RBC, forming spirl bdies which deform RBCs into sickle shape and these hsve reduced lifespan and may be trapped leading to blood vessel occlusion and ischemia of organ o bone. * **Clinical** – Anaemia, infection, painful crises, acute anemia, priapism, splenomegaly * **Long term problems** – stroke, cognitive, cardiac enlargement, HF, renal dysfunction, leg ulcers, psychosocial problems. * **Mx** – prophylacis for infection. Once daily folic acid. Avoid exposure to cold, dehydration, ecesive exercise, undue stress or hypoxia to minimize vaso-occlusie crisis. * **Acute crises** – painful crises should be treated with oral/IV analgesia, antibiotics, o2. * **Chronic** – hydroxycarbamide to increase Hbf production if frequently in hospital. If stroke so dont respond then bone marrow transplant.

Answer 174

**Thalassaemia** = Name from group of inherited conditions that affect a substance in the blood called Hb. * Produce too litter or no Hb so can be very anaemia. * Mainly in Mediterranean, S.Asian, SE Asian and middle eastern origin. * Main health problems associated – anaemia (tiredness, weak, SOB, pounding, palpitations, pale skin), too much iron in body (from reg blood transfusions). Also delayed growth, weak, fragile bones (osteoporosis) and reduced fertility. * Screening – preg or soon after birth * Tx – blood transfusion, chelation therpay (remove excess iron), healthy diet, exercise etc. * **B Thalassaemia: media and intermedia types.** * Clinical features – severe anemia from 3m-6m of age and jaundice, faltering growth/growth failure, extramedullary haemopoiesis prevented by reg blood transfusions. * Complications- Pallor, jaundice, bossingof skull, maxillary overgrowth, splenomegaly + hepatomegaly. * Mx – regular blood transfusions to reduce growth failure and prevent bone deformation. * B-thalassaemia trait – heteroxygotes usually asymptomatic. Hypochromic and microcytic red cells. Anaemia mild/absent and reduction MCH, red blood cell count increased. Raised HbA2. * **A-thalassaemia:** * How severe depends on number a-globin genes (should be 4) * Red cells hypochromic and microcytic * Clinical

Answer 175

* Downs Syndrome: Trisomy 21 * **Patho** – nondisjunction, (mostly due to maternal age), translocation, mosaicism * **Presentation** – antenatal screening, prenatal diagnosis, or clinical presentation confirmed on chromosome analysis. * **Clinical** – hypotonic, flat occiput, single palmar creases, incurved 5th finger, wide ‘scandal’ gap between big and scond toes. rtPCR test or FISH test. * Craniofacial appearance. Round face, flat nasal bridge, upslanted palpebral fissures, epicranthic folds, small mouth and protruding tongue, small ears * Other – short neck, single palmar creases, hypotonia, congenital heart defects… * Later medical problems – delayed motor milestones, learning difficulties, short statures, increased susceptibility to infections, hearing impairment, epilepsy… * Immediate medical complications – increased risk of duodenal atresia, congenital herat disease *

Answer 176

* **Haemophilia** = severe inherited coagulation disorder. X-linked recessive. Hemophilia A – FVIII deficiency / Hemophilia B – FIX deficiency. * Clinical- recurrent spontaneous bleeding into joints and muscles which can lead to crippling arthritis I not properly treated. Most present end of 1st year life. In neonatal period then intracranial hemorrhage, bleeding post circumcision or prolonged oozing from heel prick or venipuncture sites. * Severity: * \<1% FVIII – severe – spontaneous joint/muscle bleeds * 1-5% FVIII – moderate – bleed after minor trauma * \>5-40% FVIII – mild – bleed after surgery * Management – Recombinant FVIII (HA), Recombinant FIX (HB) prompt infusion if bleeding. Prophylactic too. * Complications – transfusion transmitted infections, vascular access.

Answer 177

* Spectrum disorders ranging from dysplasia to subluxation through to frank dislocation of the hip. * **Neonatal screening** – Barlow manouvre or ortolani manouvre. Then repeated at routine surveillance 8w) * **Presentation** – limp or abnormal gait. Asymmetyr o skinfolds around hip, limited abduction of hip or shortening of affected leg. * Need US * May have splint or harness to keep hip flexed or abducted. Porgress with repeat USs or Xray.

Answer 178

**Acute Arthritis:** Pain, swelling, heat, redness, restricted movement in a joint. In acute monoarthritic also systemically unwell with fever and if septic arthritis/osteomyelitis is the cause then need urgent diagnosis and treatment. * **Reactive arthritis** – transient joint swelling (\<6weeks) often on ankles or needs and often evidence extra articular infection * **Septic arthritis** – serious infection joint space, usually in \<2yrs. Warm, acutely tender joitn and reduced ROM in acutely unwell, febrile child. * **Juvenile idiopathic arthritis** – persistent joint swelling \>6weeks before 16years age with absence of cause.

Answer 179

* **Patho** – pathological immune response is mounted against a specific food protein. Usually IgE mediated. Non-immunological hypersensitivity reaction to a specific food is called a food intolerance. * **Primary common allergie**s – Infants (milk, egg, peanut), older children (peanut, tree nut, fish, shellfish) * **Secondary allergies** – due to cross reactivity between proteins in fresh fruit/veg/nuts and those in pollens * **Clinical**: * IgE – urticaria to anaphylaxis usually 10-15mins after ingestion * Non IgE.- diarrhoea, vomiting, abo pain, faltering growth and possible colic and eczema and may have blood in stools in first few weeks of life. * **Diagnosis**: for IgE then mostly skin prick tests and measure IgE Abs in blood. Greater response, more likely allergic. For non IgE then history, exam, pos endoscopy/intestinal biopsy. sometimes need uspervised food challenge. * **Mx** – avoid food. must be bale to manage allergic attack. for mild then non-sedating antihistamines and severe is adrenaline.

Answer 180

* Blood spot test * Blood sample to find out if baby has one: Sickle cell disease, CF, congenital hypothyroidism, inherited metabolic diseases (PKI, MCADD, MSUD, IVA, GA1, HCU), SCID, * 5days old, collect 4 drops bloods.

Answer 181

1. Intro, history 2. Weight 3. General inspection 4. Tone 5. Head – circumference, fontonella 6. Skin 7. Face 8. Eyes – appearance + movement, check cataracts, fundal reflex 9. Ears 10. Mouth and palate 11. Neck and clavicles 12. Upper limbs 13. Chest – lungs, heart, oximetry 14. Abdo – and palpate 15. Genitalia – testicles descended, position urethral meats, absent testicle, fused labia 16. Lower limbs – tone, movement, pulses, barlows/Ortolanis test, DDH 17. Back and spine 18. Anus 19. Reflexes

Paediatrics Flashcards

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