Pediatrics

Question 1

hydrops foetalis is a

Answer 1

• condition in the fetus characterized by an accumulation of fluid, or edema, in at least two fetal compartments.
• including ascites, pleural effusion, pericardial effusion, and skin edema.
• In some patients, it may also be associated with polyhydramnios and placental edema.

Question 2

Fetal hydrops occurs in:

Answer 2

Hematological disorders
Lysosomal diseases
Disorders of steroid metabolism

Question 3

name 2

Hematological disorders

Answer 3

G6PD deficiency
Pyruvate kinase deficiency

Question 4

defiene

G6PD deficiency

Answer 4

• Glucose-6-phosphate dehydrogenase deficiency (G6PDD), which is the most common enzyme deficiency worldwide
• is an inborn error of metabolism that predisposes to red blood cell breakdown (hemolysis).
• Most of the time, those who are affected have no symptoms.
• Following a specific trigger, symptoms such as yellowish skin, dark urine, shortness of breath, and feeling tired may develop.
• Complications can include anemia and newborn jaundice.
• Glucose-6-phosphate dehydrogenase is an enzyme which protects red blood cells, which carry oxygen from the lungs to tissues throughout the body.
• A defect of the enzyme results in the premature breakdown of red blood cells.

Question 5

Coombs test

Answer 5

• is an immunological test used to detect antibodies that bind antigens found on red blood cells.
• A positive Coombs test in a patient with hemolysis is a sign of immunological hemolytic anemia.

Question 6

atypical hemolytic uremic syndrome →

Answer 6

→ in this syndrome, there is uncontrolled activation of the complement system, which causes damage to the endothelium, platelet aggregation and thrombosis in the microcirculation
- this process leads to the destruction of erythrocytes and normocytic anemia , the destruction of blood cells is not caused by antibodies.

Question 7

define

Carney complex

Answer 7

• rare genetic disorder characterized by multiple benign tumors (multiple neoplasia) most often affecting the heart, skin and endocrine system and abnormalities in skin coloring (pigment) resulting in a spotty appearance to the skin of affected areas.

Question 8

Carney syndrome →
symptoms

Answer 8

→ it is a genetic syndrome with many components such as:

• obesity above the waist (but with thin arms)
• round face, skin changes
• weak bones and muscles
• slow growth rate in children
• micronodular hyperplasia of the adrenal glands
• myxomas of the skin, heart and mammary glands
• occurrence of light brown spots on the skin
• testicular tumors
• other endocrine disorders, e.g. acromegaly it is a genetic syndrome with many components such as:

Question 9

What causes Carney complex?

Answer 9

• caused by an inactivating mutation of the PRKAR1A gene (chromosome 17q22-24) encoding the regulatory subunit 1 of kinase A
• This gene provides instructions for making one part (subunit) of an enzyme called protein kinase A, which promotes cell growth and division (proliferation).

Question 10

DiGeorge syndrome

Answer 10

→ it is a congenital defect causing disturbances in the development of the thymus gland, which in embryonic development develops from the same gill pouch as the parathyroid glands.
- DiGeorge syndrome results in hypoparathyroidism.

Question 11

type 1 autoimmune polyendocrinopathy syndrome

Answer 11

→ it is an autosomal recessively inherited polyglandular hypofunction syndrome caused by a mutation of the AIRE gene responsible for the regulation of immune tolerance; the team consists of:

• treatment-resistant mucosal candidiasis
• hypoparathyroidism
• adrenal insufficiency

Question 12

maternal hyperparathyroidism during pregnancy

Answer 12

→ fetal complications associated with maternal hyperparathyroidism include intrauterine fetal growth restriction, low birth weight, premature delivery, intrauterine death, miscarriages, and neonatal tetany.
- Hypocalcemia may occur in newborns of mothers with hyperparathyroidism due to suppression of PTH secretion by the newborn’s parathyroid glands (hypoparathyroidism).

Question 13

CaSR inactivating mutation

Answer 13

→ Ca-SR inactivating mutations cause blockage of signal transfer to parathyroid cells, which leads to excessive PTH synthesis, which is the cause of symptoms of hyperparathyroidism .

Mutations occur in two disease entities:
- Familial benign hypocalciuric hypercalcemia (FBHH, FHH)
- Severe neonatal hyperparathyroidism (NSHPT, FNHP)

Question 14

in 80% of children , acute pharyngitis and tonsillitis is caused by

Answer 14

viruses
- Most often, these are rhinoviruses, adenoviruses, and coronaviruses.
- If bacterial etiology is suspected, most often it is B-hemolytic streptococcus from group A
Streptoccocus pyogenes , and in these few cases antibiotic therapy is indicated.

Question 15

the incubation period of hepatitis A in children is

Answer 15

14-42 days

Question 16

According to Pediatrics Kawalec, factors that may disturb fetal growth include:

Answer 16

• young mother’s age (under 16 years of age)
• mother’s body weight deficiency before pregnancy and too little weight gain during pregnancy
• obesity or too much weight gain during pregnancy
• deficiencies in maternal nutrition , especially deficiency of protein, calcium, iron, B vitamins, including folic acid (vitamin B9 )
• mother’s diseases - hypertension, hyperthyroidism, pre-pregnancy and gestational diabetes , chronic diseases of the respiratory and circulatory system (hypoxia of the mother’s body and the fetus)
• some medications and psychoactive substances taken during pregnancy
• fetal development defects

Question 17

Pharmacotherapy is considered in patients with recurrent syncope despite education and lifestyle modifications. The drugs used include:

Answer 17

fludrocortisone
alpha-adrenergic receptor agonists
β-receptor blockers (propranolol, metoprolol)

Question 18

Cystic fibrosis is a congenital dysfunction of the

Answer 18

• chloride channel

Question 19

Cystic fibrosis is a congenital dysfunction of the chloride channel
-There is a decrease in ?

Answer 19

• There is a decrease in chlorine secretion and an increase in sodium absorption into cells, which leads to ionic disturbances and dehydration of the secretion of exocrine glands.

Question 20

name 5 conditions

children with cystic fibrosis more often than healthy children develop ?

Answer 20

1) bronchiectasis may occur;

2) cholestasis and gallstones may occur;

3) sinusitis and nasal polyps may occur;

Question 21

Cystic fibrosis is a genetic disease, inherited in an

Answer 21

• autosomal recessive manner.
• The cause is mutations in the gene encoding the membrane protein CFTR , which is a chloride channel.
• The absence or incorrect structure of this protein causes blocking or impairment of the transport of chlorine from the cell and increases the absorption of sodium into the cell , which reduces the water content in the secretion of the exocrine glands.
• Too thick secretion is produced, which causes many ailments, mainly in the respiratory and digestive systems

Question 22

Cystic fibrosis

Changes in the respiratory system:

Answer 22

• Increased secretion of thick mucus prevents proper cleansing of the respiratory tract, and the high concentration of NaCl and change in the pH of the fluid reduce the antibacterial properties, which is the cause of chronic infections .
• Atelectasis, dilation and cysts appear .
• Chronic inflammation of the nasal mucosa and paranasal sinuses develops with the presence of polyps

Question 23

Cystic fibrosis

Changes in the digestive system:

Answer 23

• They mainly affect the pancreas, where pancreatic fluid stagnates and its pH drops, which in turn causes activation of proteolytic enzymes and inflammation, followed by fibrosis and exocrine insufficiency, and over time also endocrine failure.
• Due to impaired bile secretion in the liver , cholestasis, biliary cirrhosis and cholelithiasis occur.
• Meconium ileus may occur in infants - thick meconium blocks the intestinal lumen, meconium does not pass in the first days of life, and symptoms of obstruction appear.

Question 24

Cystic fibrosis

Tuberculosis is an infectious disease caused by

Answer 24

• bacillus Mycobacterium tuerculosis.

Question 25

Tuberculosis The most common route of infection is the

Answer 25

respiratory tract (95%), less often the alimentary tract.

Question 26

# Tuberculosis Children most often become infected from

Answer 26

- adults in the household

Question 27

# Tuberculosis The incubation period from the day of infection to the appearance of a positive tuberculin test is

Answer 27

2-12 weeks

Question 28

# Tuberulosis Factors predisposing to infection are:

Answer 28

- young age of the child, especially infancy ( we can subtract subpart B here , I didn't find the specific age) - pubertal age - immunodeficiency (especially that caused by HIV syndrome) - drug-induced immunosuppression - diseases that impair immunity - poor nutritional status - recovery period

Question 29

# Tuberculosis The incubation period from the day of infection to the appearance of a positive tuberculin test is 2-12 weeks. Most often, after infection, the disease enters a

Answer 29

- a latent (inactive) state and after some time, usually 6 months to 2 years, the disease becomes active.

Question 30

# Tuberculosis Mycobacterium infection progresses into an active disease much more easily in

Answer 30

- infants and young children than in adults. - Unlike adults, children suffer from the oligomycobacterial form, which is usually not infectious

Question 31

# Tuberculosis- in children diseases that impair immunity

Answer 31

- Hodgkin's disease - lymphocytic leukemia - diabetes - chronic renal failure (end-stage renal failure requires dialysis )

Question 32

Diagnosing tuberculosis in children is difficult, the younger the child, the more non-specific and general symptoms there are. Most often, tuberculosis is diagnosed in children secondary to its diagnosis in adult family members. Tests to detect active tuberculosis:

Answer 32

- bacteriological (staining of sputum samples using the Ziehl-Neelsen method to visualize mycobacteria, cultures of gastric lavage, in certain cases urine, CSF) - PCR tests - radiological - invasive, i.e. bronchoscopy - biopsy of organs suspected of tuberculous lesions.

Question 33

# Tuberculosis Tests that detect infection in the latent stage:

Answer 33

1. tuberculin test - We administer 2 units of tuberculin intradermally and the result is read after 48-72 hours, in Poland a positive result >=10mm in unvaccinated children, <=15 in vaccinated children, while in the UK an infiltration of >=5 is considered positive 2. IGRA test (C) - Interferon gamma release test as a result of the immune response to antigens specific for M. tuberculosis. - They have an advantage over the tuberculin skin test because vaccinations do not influence the results. - IGRA tests are less sensitive in children <5 years of age. - It is recommended to perform the tuberculin skin test and the IGRA test; a positive result of one of them is the basis for confirming infection

Question 34

# Tuberculosis in children Treatment involves the use of

Answer 34

- 3 or 4 drugs ( rifampicin, isoniazid, pyrazinamide and ethambutol ).

Question 35

Tuberculosis - After 2 months, we limit the treatment to

Answer 35

- two drugs: rifampicin and isoniazid .

Question 36

Tuberculosis Treatment of uncomplicated tuberculosis usually lasts about

Answer 36

- 6 months, disseminated tuberculosis, osteoarticular tuberculosis, and death longer.

Question 37

Tuberculosis TX in adolescents:

Answer 37

- pyridoxine - vitamin B 6 , is additionally used (to prevent peripheral neuropathy), and in children with dementia, dexamethasone .

Question 38

# age & reason Tetracyclines (doxycycline) should be avoided in children under

Answer 38

- 12 years of age - because there is a risk of permanent discoloration and damage to teeth - or a delay in skeletal development

Question 39

# lyme disease Antibiotics that are safe for children and effective against Borrelia burgdorferi (erythema migrans ) include

Answer 39

amoxicillin azithromycin clarithromycin cefuroxime axetyl

Question 40

symptoms ( low fever, dry cough ) and changes in the physical examination ( single rales ) and imaging ( interstitial changes and enlargement of the lung lymph nodes ) suggest

Answer 40

- atypical pneumonia (Chlamydia pneumoniae, Mycoplasma pneumoniae).

Question 41

# Child (14 yr) atypical pneumonia (Chlamydia pneumoniae, Mycoplasma pneumoniae). - The drug of first choice in such an infection are

Answer 41

macrolides: clarithromycin or azithromycin

Question 42

12-year-old suffering from nausea, vomiting, pain during micturition radiating to the left groin and pain in the left lumbar region since the morning. Physical examination revealed abnormalities in the abdominal cavity and a positive Goldflam sign on the left side. Laboratory tests showed normal blood count and kidney function parameters, elevated CRP, and erythrocyturia in general urine test. Ultrasound of the abdominal cavity showed left-sided hydronephrosis, the left ureter was not visible. The correct diagnosis is:

Answer 42

Urinary tract stones

Question 43

# Acute pancreatitis AP is a consequence of an infection ascending from the

Answer 43

- lower urinary tract - The clinical picture varies: from cystitis to urosepsis. - pain in the lumbar region - positive Goldflam sign - nausea and vomiting - indicator of renal parenchymal involvement would be a fever above 38.5 degrees - In general urine examination: we observe significant bacteremia and leukocytosis in morphology.

Question 44

markers for monitoring the cytokine storm are | Cytokine release syndrome (CRS) sometimes called cytokine storm

Answer 44

IL-6, ferritin, LDH, D-dimer.

Question 45

# also known as hypercytokinemia

Answer 45

- A cytokine storm - is a physiological reaction in humans and other animals in which the innate immune system causes an uncontrolled and excessive release of pro-inflammatory signaling molecules called cytokines. - Normally, cytokines are part of the body's immune response to infection, but their sudden release in large quantities may cause multisystem organ failure and death.

Question 46

A 3-year-old boy was admitted to the emergency room due to high fever > 39.5°C lasting the second day, abdominal pain, apathy, lack of appetite and unpleasant urine odor. A general urine test showed leukocyturia and the presence of nitrites. Indicate the correct action:

Answer 46

- High fever >38.5°C and abdominal pain suggest acute pyelonephritis - unpleasant urine odor indicates inflammation of the urinary bladder and urethra. - Inflammation in children may result in apathy, loss of appetite and vitality of the child. The most common pathogen of urinary tract infections is E. coli.

Question 47

A 3-year-old boy was admitted to the emergency room due to high fever > 39.5°C lasting the second day, abdominal pain, apathy, lack of appetite and unpleasant urine odor. A general urine test showed leukocyturia and the presence of nitrites. The drugs of choice is?

Answer 47

The drugs of choice in OOZN are : - cephalosporins - amoxicillin with cavulanic acid - piperacillin with tazobactam - aminoglycosides - The route of administration depends on the patient's clinical condition.

Question 48

Wilms tumor

Answer 48

(nephroblastoma) originates from the germ cells of the kidney and is the most common kidney tumor in children

Question 49

Neuroblastoma (NBL) and related tumors arise from the

Answer 49

- primary neural lamina, which forms the adrenal medulla and the sympathetic nervous system - Classically, the starting point of an abdominal tumor is the adrenal medulla

Question 50

non-Hodgkin's lymphoma

Answer 50

- B-cell tumors more often present as NHL [non-Hodgkin's lymphoma], with enlarged lymph nodes in the head and neck or abdominal cavity

Question 51

Medulloblastoma

Answer 51

(medulloblastoma, approx. 20%) are located in the midline of the posterior cranial fossa

Question 52

dysgerminoma

Answer 52

- Germ cell tumors (GOT) can be both benign and malignant. - They develop from primitive reproductive cells that migrate from the yolk sac to form the gonads of the fetus. - Benign tumors most often appear in the sacrococcygeal area , while malignant tumors are usually located in the gonads .

Question 53

# X-ray image and probable etiology of infection: Streptococcus pneumoniae

Answer 53

- lobar pneumonia

Question 54

# X-ray image and probable etiology of infection: Staphylococcus aureus

Answer 54

- multifocal lesions, abscesses, emphysematous blisters

Question 55

# X-ray image and probable etiology of infection: atypical bacteria

Answer 55

- interstitial, perihilar, band-shaped inflammatory changes, possibly peripheral distension, possibly fluid in the pleura,

Question 56

# X-ray image and probable etiology of infection: viruses

Answer 56

- bronchial, interstitial, bilateral, airy lesions,

Question 57

# X-ray image and probable etiology of infection: whooping cough

Answer 57

- the image of a "tent",

Question 58

# X-ray image and probable etiology of infection: pneumonia complicated by pleural empyema

Answer 58

- most often S. pneumoniae, S. aureus , Streptococcuspyogenes

Question 59

- The presence of abscesses, emphysematous bullae, pneumothorax and empyema in the radiological image of the child's lungs supports the diagnosis of

Answer 59

staphylococcal pneumonia

Question 60

In the course of iron deficiency anemia the following are observed

Answer 60

1) a decrease in the average concentration of hemoglobin in the blood - this is one of the basic changes in the blood count of people suffering from anemia. 2) erythrocyte poikilocytosis

Question 61

erythrocyte poikilocytosis

Answer 61

- poikilocytosis is a phenomenon of the presence of blood cells of different shapes. - In the case of anemia, anisocytosis, or the presence of blood cells of different sizes, may also appear.

Question 62

# Crede's procedure: Prophylaxis of gonococcal conjunctivitis - prophylaxis involves the use of

Answer 62

- 1% silver nitrate solution (Crede's procedure) or - 0.5% erythromycin in ointment or - solution or 1% tetracycline solution or ointment. Note: Every child after birth – including newborns born by caesarean section – is given gonococcal conjunctivitis prophylaxis. Currently, 0.5% erythromycin ointment

Question 63

# Inhaled glucocorticosteroids Therapeutic procedures for subglottic laryngitis: Mild croup

Answer 63

- dexamethasone: 0.15 mg/kg bw (max: 8 mg) intramuscularly or orally or - budesonide: 2 mg nebulized

Question 64

# Inhaled glucocorticosteroids Therapeutic procedures for subglottic laryngitis: Croup moderate

Answer 64

- dexamethasone: 0.3 mg/kg bw (max: 8 mg) intramuscularly or orally and/or - budesonide: 2 mg in nebulization and/or - 0.1% epinephrine in nebulization: 0.5 ml/kg bw (max: 5 ml)

Question 65

# Inhaled glucocorticosteroids Therapeutic procedures for subglottic laryngitis: Heavy groats

Answer 65

- 0.1% epinephrine in nebulization: 0.5 ml/kg bw (max: 5 ml) (first) and - dexamethasone: 0.6 mg/kg bw (max: 8 mg) intramuscularly and/or - budesonide: 2 mg in nebulization

Question 66

diagnosis of IgA-related vasculitis: (formerly Schönlein–Henoch disease ):

Answer 66

- the development of the disease is often preceded by an upper respiratory tract infection ; - a typical and obligatory symptom of the disease is a rash - it appears symmetrically on both buttocks, extensor surfaces of the arms and lower legs and around the ankle joints; skin lesions are maculopapular, then hemorrhagic , palpable under the fingers when palpating the skin; - joint pain, especially in the knee and ankle joints, occurs in 2/3 of patients; - more than 80% of patients have micro- or macroscopic hematuria with moderate proteinuria . Additionally, patients with Schönlein–Henoch disease may experience colicky abdominal pain .

Question 67

# Acute bronchiolitis Treatment Acute bronchiolitis is self-limiting and the most important thing is to ensure proper?

Answer 67

- hydration and nutrition of the child, and in case of SpO2 < 90% – inclusion of oxygen therapy . The use of : 1) antibiotic therapy, 2) ribavirin or anti-RSV antibodies, 3) glucocorticosteroids, 4) bronchodilators (beta-mimetics, adrenaline), 5) 3% NaCl in nebulization – used only in hospitalized patients, 6) physical therapy.

Question 68

The clinical picture presented in the question corresponds to Alport syndrome :

Answer 68

- a genetically determined syndrome - inherited in a recessive manner linked to the X chromosome; - leads to progressive end-stage renal disease in young male adults; - coexists with neurosensory deafness and visual impairment

Question 69

Alport Syndrome Definition

Answer 69

- HEREDITARY NEPHROPATHY caused by disturbances in the synthesis of α chains of type IV collagen, which leads to damage to the structure of the BASEMENT MEMBRANE of the renal glomeruli and disturbances in the organ of HEARING and VISION . - In 85% of cases the sex-linked form (XLAS) – the full-blown form occurs in men , women are healthy carriers or have a mild disease. - It is the most common congenital progressive kidney disease. Its incidence is 1/10,000 live births.

Question 70

Alport Syndrome Symptoms

Answer 70

1) End-stage renal disease in all men and 15% of women. 2) Sensorineural hearing loss , which in some patients leads to deafness before the age of 40. 3) Half of patients develop ocular symptoms during adolescence (keratoconus, macular abnormalities). 4) Urine test: hematuria , proteinuria.

Question 71

# Urin test Alport Syndrome

Answer 71

- *Hematuria *Hematuria is a dominant symptom of Alport syndrome, which is present from birth or appears in the 1st year of life. - *Proteinuria* appears after several years with the progression of kidney disease and in 30% of patients leads to the development of nephrotic syndrome .

Question 72

Alport Syndrome Treatment

Answer 72

- Treatment is symptomatic only (ACEI may be used to reduce proteinuria) . Kidney transplant.

Question 73

Landau reflex

Answer 73

- is elicited by holding the infant horizontally in the air with the face turned to the ground. - The infant should lift the head up, then arch the trunk and straighten the lower limbs at the hip joints. - The Landau reflex is present from 5 months of age and may persist until 3 years of age

Question 74

Lust's sign

Answer 74

- is a contraction of the peroneal muscles and abduction of the foot in response to a percussion hammer on the common peroneal nerve (below the head of the fibula). - It indicates excessive neuromuscular excitability and is one of the symptoms characteristic of tetany .

Question 75

Levine's sign

Answer 75

- is a symptom found in patients after myocardial infarction or with unstable angina , which consists of pointing to the sternum with a clenched fist when describing the location of pain.

Question 76

Lasègue's sign

Answer 76

- is a symptom of inability to raise the lower limb in a lying position. It indicates compression of the sciatic nerve. - In addition, it may be accompanied by difficulty in raising the hips and the whole body from a lying position with the lower limbs straight, caused by the pain that occurs at that time. - The patient can perform this activity only after previously bending the legs at the knee joints. Lasègue's sign is characteristic of sciatica . It has two varieties: - the first consists of the occurrence of pain along the straight lower limb during its passive lifting; - the second is elicited by flexing the limb at the hip and knee joints and then straightening it; similarly, a positive symptom is indicated by the occurrence of sudden pain along the limb

Question 77

Leigh syndrome (subacute necrotizing encephalomyelopathy):

Answer 77

- is a rare, etiologically heterogeneous mitochondrial disease associated with a disturbance in ATP production in mitochondria ; - it may result from mutations in both mitochondrial and nuclear genetic material, since both contain sequences encoding enzyme proteins involved in mitochondrial metabolism. - Oxidative decarboxylation of pyruvate, electron transport in the respiratory chain, or ATP production by ATP synthase may be impaired; - It appears at the age of 3-24 months and progresses rapidly ;

Question 78

# Leigh syndrome (subacute necrotizing encephalomyelopathy) the clinical picture includes symptoms on the part of :

Answer 78

- nervous system - encephalomyelopathy , - skeletal muscles, - hearts - hypertrophic cardiomyopathy is common , - liver, - kidneys; - Plasma lactate and pyruvate levels are elevated , leading to metabolic acidosis .

Question 79

Glomerulopathies Idiopathic Nephrotic Syndrome - Diagnostics - Laboratory tests

Answer 79

1) General urine test – proteinuria , rarely hematuria. 2) Assessment of the extent of proteinuria – 24-hour urine collection for protein or protein/creatinine ratio (or albumin/creatinine) in a morning urine sample. 3) Reduced total protein level and hypoalbuminemia, dysproteinemia. 4) Hypercholesterolemia and hypertriglyceridemia.

Question 80

# Glomerulopathies Idiopathic Nephrotic Syndrome - Laboratory tests ratio (or albumin/creatinine) in a morning urine sample.

Answer 80

- Taking a urine sample in the morning, immediately after a night's rest, allows for the exclusion of orthostatic proteinuria (increased protein excretion after standing), which is a benign condition that does not require treatment and, according to some researchers [ 1 ], is even considered a variant of the norm.

Question 81

# The proteinogram shows Glomerulopathies Idiopathic Nephrotic Syndrome - Diagnostics - Laboratory tests Reduced total protein level and hypoalbuminemia, dysproteinemia.

Answer 81

- The proteinogram shows increased levels of α2- and β-globulins and decreased levels of γ-globulins . - This is due to the loss of albumin and γ-globulin in the urine, which increases the percentage of remaining protein fractions.

Question 82

# café au lait stains Phakomatoses Neurofibromatosis type 1 - Symptoms

Answer 82

- café au lait stains (from the first months of life) - freckled spots - 2-3 mm in diameter in the armpits and groin area (from puberty) - Lisch nodules - dark yellow or brown hamartoma-type nodules of the iris (in 90% of patients over 6 years of age)

Question 83

# subcutaneous nodules Phakomatoses Neurofibromatosis type 1 - Symptoms

Answer 83

- subcutaneous nodules – neuromas or neurofibromas : (from about 10 years of age) - are located along peripheral nerves, blood vessels, and within visceral organs, - located in the spinal canal, they may cause pain, - may develop into neurofibrosarcoma or malignant schwannoma .

Question 85

# plexiform neurofibromas Phakomatoses Neurofibromatosis type 1 - Symptoms

Answer 85

- plexiform neurofibromas (from the first months of life) : - often located in the orbital area, on the neck, in the groin, - grow into the spinal canal, internal organs, - may cause hypertension , bone deformations , - the skin overlying the plexiform neurofibroma is sometimes severely discolored.

Question 86

# bone abnormalities, optic nerve glioma Phakomatoses Neurofibromatosis type 1 - Symptoms

Answer 86

- *bone abnormalities* : - spinal curvature, macrocephaly, sphenoid dysplasia, fibula and tibia deformities, pathological fractures with a tendency to develop pseudoarthroses. - *optic nerve gliomas* (mainly in the first decade of life) : - occur in approximately 15% of patients, - the growing tumor compresses the optic chiasm and the hypothalamus.

Question 87

# neurological observations Phakomatoses Neurofibromatosis type 1 - Symptoms

Answer 87

- epilepsy in 10-15% of patients , - delayed mental development in 20% of patients , - hyperactivity, dyslexia, difficulties at school.

Question 88

# define Phakomatoses,

Answer 88

- also known as neurocutaneous syndromes, are a group of multisystemic diseases that most prominently affect structures primarily derived from the ectoderm such as the central nervous system, skin and eyes. - The majority of phakomatoses are single-gene disorders that may be inherited in an autosomal dominant, autosomal recessive or X-linked pattern.

Question 89

Fainting - Causes

Answer 89

- Reflex syncope - vasovagal syncope - situational faints - orthostatic hypotension

Question 90

# Diabetes Diagnosis - OGTT - Results A venous blood serum glucose value of 156 mg/dl at the 120th minute of the OGTT test in a child is interpreted as:

Answer 90

impaired glucose tolerance.

Question 91

# JIA Clinical picture The clinical picture of JIA varies depending on the form of the disease. - Common symptoms include:

Answer 91

1) Arthritis : - symptoms – swelling , joint effusion, pain , excessive joint heat, - as a consequence, mobility is limited , muscle atrophy and joint contractures occur, - the KNEE, wrist and ankle joints are most commonly affected , but any joint may be affected. 2) Inflammation of tendons and tendon sheaths: - most often pain in the heel area caused by inflammation of the Achilles tendon attachment . 3) Morning stiffness - a feeling of stiffness in the joints that occurs after waking up and disappears during movement. 4) Uveitis - the most common extra-articular manifestation of JIA: - chronic, it typically occurs in the oligoarticular form in little girls, less frequently in the polyarticular form, - acute (photophobia, redness, eye pain) occurs in the form of enthesitis and psoriatic arthritis.

Question 92

# Exclusion criteria - Characters - Oligoarticular Character Assignment to a specific subgroup is made after 6 months from the onset of the disease, but it is necessary to consider the following exclusion criteria :

Answer 92

A: Physician-confirmed psoriasis in the patient or a first-degree relative. B: Arthritis in a boy > 6 years of age with HLA-B27+. C: HLA-B27-related condition in a first-degree relative. D: Demonstration of RF on two occasions > 3 months apart. E: Demonstration of systemic arthritis. In the individual forms of JIA, all or only some of the above criteria are taken into account ( e.g. in the case of psoriatic arthritis, criterion A is not taken into account ) – detailed information on this subject can be found on the slides with the characteristics of the individual forms of JIA.

Question 93

Uveitis in JIA :

Answer 93

- most often it affects little girls or older boys with JIA with the onset involving only a few joints ; - It usually occurs secondary to arthritis , at a variable time interval, but can sometimes precede the symptoms of arthritis by several years; -it develops more frequently in patients with the presence of antinuclear antibodies in the blood serum (especially in girls < 7 years of age); may occur in any clinical form of JIA: - in patients with psoriatic arthritis and arthritis with concomitant enthesitis, especially with the presence of the HLA-B27 antigen , it is most often acute , with photophobia, redness and pain of the eyeball, - in other forms, JIA may have a completely silent – ​​“cold” – clinical course.

Question 94

Cystic fibrosis Symptoms – Subjective

Answer 94

1) Cough – usually the first symptom, initially sporadic, then occurring daily with expectoration of thick, purulent secretion (often after waking up). 2) Hemoptysis . 3) Shortness of breath . 4) Limited patency and chronic purulent rhinitis . 5) Passage of large, foul-smelling, fatty stools . 6) Abdominal distension and pain with episodes of intestinal obstruction. 7) Weight loss . ## Footnote Cystic fibrosis – meconium ileus Thick meconium is produced in the intestines, causing meconium ileus in about 10–20 % of newborns with CF. […] The initial treatment involves performing gastrografin enemas, but most patients need surgical treatment.

Question 95

Cystic fibrosis Course – Natural

Answer 95

- The disease usually manifests itself in early childhood ( rarely later, when the symptoms are less severe or atypical). - Chronic, slow destruction of the bronchi occurs with involvement of the lung parenchyma , which leads to respiratory failure and death (in Poland on average around 25 years of age ).

Question 96

Cystic fibrosis Diagnostic criteria

Answer 96

Confirmation of diagnosis – demonstration of ≥1 of the following: – sweat chloride concentration ≥60 mmol/l in 2 measurements on different days, – demonstration of known pathogenic mutations of both alleles of the CFTR gene (of fundamental importance in the case of a non-diagnostic sweat test result), ​– abnormal result of the measurement of the transepithelial potential difference in the nasal mucosa (test not performed in Poland).

Question 97

# Glomerulopathies Acute glomerulonephritis - Diagnostics 1) General urine examination :

Answer 97

- hematuria – dysmorphic and leached erythrocytes, - granular and erythrocytic casts (so-called "active urine sediment"), - non-nephrotic proteinuria (< 50 mg/kg/day), - sterile pyuria – leukocyturia with negative urine culture.

Question 98

# Glomerulopathies Acute glomerulonephritis - Diagnostics 2) Blood tests :

Answer 98

- increased creatinine and urea levels – temporary deterioration of kidney function; acute renal failure occurs in 5% of patients, - decreased concentration of the C3 complement component.

Question 99

# Acute glomerulonephritis - Diagnostics 3) Confirmation of streptococcal infection –

Answer 99

elevated antistreptolysin test (ASO): - a diagnostic serological test that examines the titer of specific antibodies directed against streptolysin O, a protein produced by group A streptococcus, - increased ASO indicates a streptococcal infection. 4) Renal biopsy – performed rarely, in case of suspected other causes of symptoms, chronic course or occurrence of acute renal failure.

Question 100

The main features of the WAGR syndrome are:

Answer 100

W - Wilms tumor - nephroblastoma = renal tumor in children A - Aniridia - congenital absence of the iris G - Genitourinary malformation - congenital defects of the urinary tract R - Mental Retardation

Question 101

Congenital nephrotic syndrome Finnish type is caused

Answer 101

- s a congenital nephrotic syndrome inherited in an autosomal recessive manner (NPHS1 mutation) and occurs mainly in newborns in Scandinavian countries, - severe proteinuria often occurs already in the intrauterine period, - many children are born prematurely, at 35–37 weeks of gestation, with a birth weight that is too low for gestational age, - a large placenta (placentomegaly) is characteristic, - edema is present from birth or occurs in the first weeks of life, - the newborn has a characteristic appearance: big belly, small nose, low-set ears, often also limb deformities secondary to a large placenta, wide cranial sutures due to ossification disorders, - In many cases, muscle weakness and neurological disorders are observed.

Question 102

Nephrotic AND Nephritic Syndrome

Answer 102

Question 103

# Urinary tract infections Procedure The basis of UTI treatment is antibiotic therapy , which should be initiated after making the diagnosis based on clinical symptoms and the results of a general urinalysis, before obtaining the result of a urine culture (but AFTER collecting urine for microbiological testing). The route of antibiotic administration depends on the child's age and the severity of the infection:

Answer 103

1) intravenous - children < 3 months of age, serious condition of the child, severe vomiting, feeding difficulties, complications, urosepsis, 2) oral - children treated on an outpatient basis, 3) sequential therapy - iv in the initial phase of treatment, after the patient's condition has stabilized.

Question 104

# Urinary tract infections Treatment - Upper urinary tract infection

Answer 104

1) In children up to the end of the 2nd year of life, all UTIs are treated as upper urinary tract infections due to the impossibility of determining their location. 2) Treatment duration: 7–14 days . 3) Newborns and infants < 3 months of age – hospitalization and intravenous treatment. 4) Other children – outpatient treatment possible, hospitalization if indicated. Route of antibiotic administration depending on the patient's condition.

Question 105

# Urinary tract infections Treatment - Upper urinary tract infection 5) Selection of antibiotic:

Answer 105

- 2nd generation cephalosporins ( cefuroxime ), - 3rd generation cephalosporins ( ceftriaxone , cefotaxime, cefixime, ceftibuten) - amoxicillin with clavulanic acid , - ampicillin + aminoglycoside – 1st choice in newborns, - fluoroquinolones (ciprofloxacin) - from 12 years of age, possibly from 1 year of age as a "rescue" antibiotic.

Question 106

The basic conditions necessary for the diagnosis of bronchopulmonary dysplasia are the need

Answer 106

- to use oxygen therapy in a child > 36 weeks of postconceptional age or oxygen therapy conducted for at least 28 days.

Question 107

# bronchopulmonary dysplasia Symptoms include:

Answer 107

- tachypnea , - dyspnoea , - retraction of the intercostal spaces , - zygomatic fossa , - growth and weight gain disorders, - bronchial hyperreactivity, - tendency to respiratory tract infections, e.g. RSV infection. - The radiological image shows diffuse shadowing in the lung fields with visible cystic changes, as well as areas of distension or atelectasis mentioned in some sources.

Question 108

Bronchopulmonary dysplasia - General information

Answer 108

- Bronchopulmonary dysplasia (BPD) is characterized by abnormal lung structure. - It results from impaired lung development and damage to the airways and alveoli by damaging factors, including oxygen therapy, positive airway pressure, and inflammation. - There is also a disruption in the development of pulmonary vessels, which may cause pulmonary hypertension. - The criterion for diagnosing BPD is the need for oxygen therapy in the neonatal period for at least 28 days.

Question 109

# BPD classification Depending on the result of the assessment at week 36, counting from the date of the last menstrual period (postmenstrual age, PMA), we can distinguish:

Answer 109

* mild BPD - the baby does not require oxygen therapy in the 36th week of PMA, * moderate BPD - the child requires oxygen therapy with an oxygen supply of < 30% (FiO2 < 30%), * severe BPD - the child requires oxygen therapy with FiO2 > 30% and/or the need to use positive airway pressure (mechanical ventilation or CPAP). The use of oxygen therapy for reasons other than BPD is not a criterion for qualification to the BPD group.

Question 110

Pathophysiology of BPD The pathophysiology of BPD, or bronchopulmonary dysplasia,

Answer 110

- The basic problem is the impaired development of the lungs – a reduction in the number of interalveolar septa and underdevelopment of the alveoli. There are fewer alveoli and they are large, which disrupts gas exchange. - Additionally, factors such as intrauterine infection, pneumonia, mechanical damage due to respirator therapy, and oxygen therapy may exacerbate changes in the pulmonary alveoli. - Damage to the alveoli can cause fibrosis of the interalveolar septa, which limits further alveolar division and creates a vicious circle. - Disturbances of the development of pulmonary alveoli are also accompanied by disorders of the development of pulmonary vessels – disorders of their distribution and hypertrophy of the muscular layer of the arteries in the pulmonary circulation, which may cause pulmonary hypertension.

Question 111

# Prematurity Bronchopulmonary dysplasia - Symptoms

Answer 111

1) the child tires easily, 2) feeding difficulties – apnea during feeding, cyanosis, 3) shortness of breath, tachypnea, use of additional respiratory muscles, 4) auscultation – rales, constant and localized crackles, and expiratory wheezing, 5) limited growth rate. 6) delayed psychomotor development.

Question 112

Bronchopulmonary dysplasia - Diagnostics - to confirm the disease

Answer 112

- The diagnosis of bronchopulmonary dysplasia is based on typical history and clinical symptoms. No additional tests are needed to confirm the disease , but in children with BPD the following are performed: 1) arterial blood gasometry (assessment of the severity of respiratory disorders) , 2) blood count, CRP (suspected infection) , 3) Lung ultrasound, chest X-ray, less frequently chest CT, 4) echocardiographic examination (features of pulmonary hypertension), BNP/NT-proBNP determination . In the differential diagnosis, infections and respiratory tract defects should be taken into account .

Question 113

# BPD image in imaging studies Features of bronchopulmonary dysplasia on chest X-ray:

Answer 113

- uneven aeration of the lungs, - uneven parenchymal densities, - blurring of the heart silhouette,

Question 114

# BPD image in imaging studies Features of bronchopulmonary dysplasia on chest CT:

Answer 114

- decreased lung aeration, - thickening of interlobular septa, - fibrosis, - "honeycomb" image – emphysematous areas surrounded by zones of fibrosis, visible areas of poor vascularization.

Question 115

Rare Malignant Bone Tumors in Children

Answer 115

Non-Hodgkin's lymphomas, fibrosarcomas, chondrosarcomas.

Question 116

# Bone tumors Osteosarcoma - General Information

Answer 116

- Osteosarcoma is the most common malignant bone tumor in children, whose characteristic feature is the production of osteoid (approx. 70% of all malignant bone tumors in children) . - The peak incidence in children occurs during the growth spurt, more often in boys ( ♀ 10–14 years of age, ♂ 15–19 years of age). - The most common location is the growth cartilage area of ​​the long bones of the knee (distal femoral metaphysis and proximal tibial metaphysis).

Question 117

Osteosarcoma – location

Answer 117

- Knee area – 50% of tumors, - proximal metaphysis of the humerus – 35% of tumors, - other locations – fibula, scapula, pelvic bone.

Question 118

Acute bronchitis Epidemiology and etiology

Answer 118

- Acute bronchitis ( ABP ) is an inflammation of the lower respiratory tract in which the dominant symptom is a cough lasting no longer than 3 weeks – dry or productive – which may be accompanied by auscultatory symptoms ( ruffling, wheezing, rales ). - Acute bronchitis mainly affects children up to 2 years of age and school children . - The etiology is usually viral (RSV, influenza and parainfluenza viruses, adenoviruses, metapneumoviruses, rhinoviruses). - 1% of cases are caused by atypical bacteria – Chlamydophila pneumoniae and Mycoplasma pneumoniae. - Whooping cough may also be the cause of bronchitis with persistent cough .

Question 119

# Clinical picture Symptoms of erythema infectiosum:

Answer 119

1) prodromal symptoms – fever, malaise, joint pain, headache, 2) characteristic skin lesions: – intense redness on the face, mainly on the cheeks ( image of a "slapped" child ), – pink, fading from the center, garland-like rash on the trunk, spreading circumferentially. It may go away and then reappear over the next few weeks. 3) less frequently – joint pain or inflammation, 4) gloves and socks syndrome – sharply circumscribed erythema followed by a papular or hemorrhagic rash on the hands and feet with accompanying pain and itching (more often in adults) , 5) bone marrow aplasia – in patients with hemolytic diseases and immunodeficiencies .

Question 120

The Tanner scale

Answer 120

is a scale that allows determining the stage of sexual maturity in children, adolescents and adults based on morphological features - the structure of the genitals and breasts.

Question 121

The Glasgow Coma Scale:

Answer 121

assessment of disorders of consciousness , assessment of the severity of AP .

Question 122

The NYHA scale

Answer 122

is used to assess the severity of heart failure symptoms

Question 123

The Ballard scale is used to assess the maturity of a newborn .

Answer 123

- The Ballard Maturity Test includes a score assessment of 7 morphological and 6 neurological features of the newborn. - The sum of the points allows for an approximate determination of the duration of pregnancy with an accuracy of ± 2 weeks:

Question 124

Apgar scale

Answer 124

- is a scale used in medicine to determine the condition of a newborn immediately after birth: at 1, 3, 5 and 10 minutes of life.

Question 125

Classification criteria for Schönlein–Henoch disease:

Answer 125

- palpable skin purpura or petechiae , predominantly involving the lower extremities, not associated with thrombocytopenia, and at least one of the following four criteria: - diffuse colic abdominal pain (possible intussusception, gastrointestinal bleeding); - typical histopathological changes in the skin (leukocytoclastic vasculitis with predominance of IgA deposits) and kidneys (proliferative glomerulonephritis with predominance of IgA deposits); - joint changes - inflammation (swelling or pain with limited mobility), joint pain; - nephropathy - proteinuria > 0.3 g per day or presence of albumin in the morning urine, hematuria or erythrocyturia.

Question 126

symptoms of neurofibromatosis type 1:

Answer 126

- café au lait type stains , - painful lumps on the abdomen - neurofibromas , - freckled speckling under the arms, - dyslexia , which is a common problem in patients with NF1. - Neurofibromatosis type 1 is a neurocutaneous disease that may progress to neoplastic changes

Question 127

# symptoms typical of systemic lupus erythematosus: Skin symptoms:

Answer 127

- acute - butterfly-shaped redness on the face, bullous/papular/vascular lesions, - hypersensitivity to UV light; chronic - limited/generalized skin lesions with a discoid erythema, cellulitis and others (alopecia without scarring, reticularis reticularis, maculopapular lesions

Question 128

# symptoms typical of systemic lupus erythematosus: Hematological disorders:

Answer 128

- leukopenia with lymphopenia, - thrombocytopenia, - hemolytic anemia

Question 129

# symptoms typical of systemic lupus erythematosus: Neurological symptoms:

Answer 129

- seizures, mental disorders, headaches , - encephalopathy, strokes, disseminated mononeuropathy, - chorea, myelitis, - demyelinating changes in the CNS

Question 130

scintigraphy with 123I-labelled meta-iodobenzylguanidine (MIBG) also enables the diagnosis of

Answer 130

- pheochromocytoma (and consequently, pheochromocytoma with extra-adrenal location, i.e. paraganglioma )

Question 131

# diag/clinical/radiological ganglion cell tumor (neuroblastoma).

Answer 131

- diagnosis [of neuroblastoma] is based on the characteristic clinical and radiological findings and elevated urinary catecholamine (VMA/HVA) levels. - A biopsy confirming the diagnosis is usually performed, as well as tests for possible dissemination of the disease, i.e. bone marrow biopsy, bone scintigraphy and meta-iodobenzyl guanidine scintigraphy - MIBG .

Question 132

# X-ray image and probable etiology of infection: Streptococcus pneumoniae

Answer 132

- lobar pneumonia,

Question 133

# X-ray image and probable etiology of infection: Staphylococcus aureus

Answer 133

- multifocal lesions, abscesses, emphysematous bullae, pneumothorax and empyema | Pneumonia

Question 134

# X-ray image and probable etiology of infection: atypical bacteria

Answer 134

- interstitial, perihilar, band-like inflammatory changes, possibly peripheral distension, possibly pleural fluid,

Question 135

# X-ray image and probable etiology of infection: viruses

Answer 135

- bronchial, interstitial, bilateral, airborne lesions,

Question 136

# X-ray image and probable etiology of infection: whooping cough

Answer 136

- "tent" image,

Question 137

# Pneumonia It may be caused by the measles virus or secondary bacterial superinfection. In the latter case, the most common etiological factors are:

Answer 137

S. Pneumoniae , H. Influenzae , S. Aureus . Pneumonia is more common in patients with immunodeficiency , cancer and heart defects . It is the leading cause of death in measles.

Question 138

Provide a brief summary of the post-measles complication, subacute sclerosing panencephalitis:

Answer 138

- (Latin: leucoencephalitis subacuta scleroticans, subacute sclerosing panencephalitis, SSPE) - 7–10 years after measles - related to the persistence of infection (result of virus mutation) - the risk is much higher if measles is contracted in the first 2 years of life - boys more often - progressive course - symptoms: behavioral disorders, convulsions, dementia coma - Death occurs within 6–9 months - high concentration of γ-globulins in MR fluid - characteristic EEG recording - Imaging tests --> brain atrophy

Question 139

# Pneumonia Atypical pneumonia – Course of the disease

Answer 139

1) The patient's condition is usually good and the onset is less abrupt. 2) The predominant symptoms are a dry, long-lasting cough , low-grade fever , rhinitis , sore throat , muscle and joint pain , vomiting , and diarrhea . 3) Auscultatory changes are often slight.

Question 140

# Pneumonia Atypical Pneumonia – Treatment

Answer 140

Mycoplasma and Chlamydophila : 1) Macrolide (azithromycin, clarithromycin or erythromycin). 2) Tetracyclines , e.g. doxycycline. 3) Fluoroquinolone . Legionella : 1) Fluoroquinolone . 2) Macrolide or doxycycline (opposite of above)

Question 141

# M. pneumoniae Recognition If M. pneumoniae infection is clinically suspected,

Answer 141

- empirical therapy is initiated and the diagnosis is made retrospectively. - The EIA test is commonly used for this purpose. It involves determining antibodies against M. pneumoniae: -- in the acute phase of the disease, -- after 2–3 weeks, during the recovery period. - Both tests are necessary! M. pneumoniae infection is indicated by at least a four-fold increase in the IgG antibody titer .

Question 142

Pneumonia Etiology

Answer 142

Question 143

Clinical picture

Answer 143

- In case of infection caused by typical bacteria (mainly Streptococcus pneumoniae ) fever, dyspnea, tachypnea, cough (dry, then productive) , chest pain and abdominal pain are observed . - In pneumonia caused by atypical bacteria ( Chlamydophila pneumoniae, Mycoplasma pneumoniae ) dry cough , headache and slightly increased body temperature dominate . - Wheezing heard in a preschool child strongly indicates viral etiology .

Question 144

# Pneumonia Treatment - Antibiotic therapy

Answer 144

- Children from 5 to 15 years of age - In this age group, CAP is caused equally often by atypical bacteria and S. pneumoniae . Therefore, there is no consensus on whether the first-line drug should be a macrolide or amoxicillin . - Of the macrolides, clarithromycin or azithromycin are used in first-line treatment . - In more severe forms of CAP , a beta-lactam antibiotic (amoxicillin, ampicillin, ceftriaxone, cefotaxime ) combined with a macrolide is used . - In milder cases, treatment lasts 5 days , and in more severe cases – 7–10 days .

Question 145

# Community acquired Pneumonia treatment of CAP in children

Answer 145

Macrolides in the treatment of CAP in children Clarithromycin 15 mg/kg/day, divided every 12 hours. Azithromycin 10 mg/kg on the first day, then 5 mg/kg every 24 hours for 4 days OR 10 mg/kg/day every 24 hours for 3 days

Question 146

# Community acquired pneumonia- name 3 Antibiotic dosage in the treatment of CAP in older children

Answer 146

- AMOXICILLIN: Children > 40 kg – orally at a dose of 75–90 mg/kg/day in three divided doses (i.e. every 8 hours), or in hospital conditions intravenously 1–2 g in a dose every 6 hours, children < 40 kg – orally at a dose of 75–90 mg/kg/day in three divided doses (i.e. every 8 hours) or intravenously at a dose of 100–200 mg/kg/day, in four divided doses (i.e. every 6 hours, not more than 4 g/day). - CEFTRIAXONE: Children > 40 kg – 1000–2000 mg in one daily dose (maximum dose 4000 mg/day), children < 40 kg – 50–100 mg/kg in one daily dose. - CEFOTAXIMUM: Children > 40 kg – 500–1000 mg every 8 hours, children < 40 kg– 50–180 mg/kg every 6–8 hours

Question 147

# Clinical picture Pneumonia caused by typical bacteria -name bacteria -clinical picture

Answer 147

- mainly Streptococcus pneumoniae - fever, dyspnea, tachypnea, cough (dry, then productive) , chest pain and abdominal pain are observed . Wheezing heard in a preschool child strongly indicates viral etiology .

Question 148

# Clinical picture In pneumonia caused by atypical bacteria -name bacteria -clinical picture

Answer 148

- ( Chlamydophila pneumoniae, Mycoplasma pneumoniae ) - dry cough , headache and slightly increased body temperature dominate . Wheezing heard in a preschool child strongly indicates viral etiology .

Question 149

# Systemic tumors Hodgkin's Lymphoma - Clinical Picture - Lymph Nodes

Answer 149

- The most common symptom is unilateral (less often bilateral) enlargement of the cervical (60–80%) or supraclavicular (less often axillary and inguinal). - Concomitant involvement of mediastinal lymph nodes is found in 60% of cases. - Increased susceptibility to infections additionally affects the size of lymph nodes, and antibiotic therapy may contribute to their temporary reduction, delaying diagnosis.

Question 150

# Systemic tumors Hodgkin's Lymphoma - Clinical Picture - Lymph Nodes Lymphoma lymph nodes: describe

Answer 150

1) hard , painless, 2) grow together into packets , 3) do not soften, ulcerate or break through to the skin surface. Note: peripheral lymphadenopathy may coexist with mediastinal lymphadenopathy, generating symptoms

Question 151

# Systemic tumors Hodgkin's Lymphoma - Clinical Picture - General Symptoms -

Answer 151

1) General symptoms – occur in about 25%-30% of children – the so-called “ B ” symptoms , 2) pruritus – very rare in children (unlike in adult patients) , 3) immunological disorders – hemolytic anemia, thrombocytopenia, nephrotic syndrome, 4) non-specific symptoms – cough, recurrent respiratory tract infections, fatigue, abdominal pain, skin rashes, 5) extrapulmonary changes – enlarged liver and/or spleen.

Question 152

# Systemic tumors-Symptoms B Hodgkin's Lymphoma - Clinical Picture: - general symptoms that make up the so-called B symptoms:

Answer 152

- Fever >38°C without any obvious cause (e.g. no signs of infection), - weight loss >10% in the last 6 months, - drenching (heavy) night sweats. - The cause of B symptoms is immunological disorders (secretion of cytokines) accompanying neoplastic growth.

Question 153

# Hodgkin's Lymphoma - Clinical Picture General symptoms – occur in about 25%-30% of children – the so-called “ B ” symptoms: Mnemonics

Answer 153

- In the illustration you can see a young person with an enlarged cervical lymph node. Our patient is slim, consumed by cancer. We see symbols of general symptoms that make up the so-called B symptoms: = drenching night sweats – the night surrounds him, and we see a pool of sweat on the scale and in his surroundings; the scale indicates a loss of body weight, and the kettlebell weighing on our patient reminds us of the criterion of over 10% in the last 6 months; finally he looks at the thermometer with fear. Again... again this fever..

Question 154

# tick-borne encephalitis virus: name 4 Meningitis is also caused by:

Answer 154

- flaviviruses other than tick-borne encephalitis virus (West Nile virus, Japanese encephalitis virus, St. Louis encephalitis virus), - adenoviruses (in immunocompromised individuals), - HIV, - LCMV (lymphocytic encephalitis virus).

Question 155

# Neuroinfections in children and adults ZOMR - Etiology - Viral and fungal ZOMR

Answer 155

1) Viral ( aseptic ) Meningitis is caused by: - most often enteroviruses (ECHO and Coxsackie A), - mumps virus, - herpesviruses (HSV, VZV, rarely EBV, CMV, HHV-6) , - tick-borne encephalitis virus. 2) Fungal: - Candida (most often C. Albicans ) , - Cryptococcus neoformans , - Aspergillus .

Question 156

# MMR Rules: Primary dosing times Measles = Piggy = Little Rose =

Answer 156

Measles = Measles Piggy = Mumps Little Rose = Rubella - 13th–15th month of life & - 6th year of life

Question 157

MMR Vaccination - 2024 Immunization Program

Answer 157

- The primary vaccination dose should be administered at 13–15 months of age . - A booster dose should be given at 6 years of age. - Exceptions: Children who have not been vaccinated by the age of 6 should receive the missed vaccination as soon as possible, but no later than by the age of 19. - previous vaccination against measles is not a contraindication to be vaccinated with a combined vaccine at the age of 6, - having had measles, mumps or rubella is also not a contraindication , you just need to wait until the patient recovers. - you should not be vaccinated 4 weeks before a planned pregnancy .

Question 158

What inflammatory parameters in CSF are typical for viral infection?

Answer 158

- protein, - glucose - lactic acid

Question 159

pleocytosis with a predominance of lymphocytes is characteristic of

Answer 159

- viral meningitis - However, it is worth noting that in viral meningitis, especially those caused by enteroviruses, neutrophils may also dominate in pleocytosis in the first 24-48 hours and constitute about 60% of all cells. In these cases, the remaining inflammatory parameters in CSF (protein, glucose and lactic acid) are typical for viral infection

Question 160

# bacterial meningitis bacterial meningitis - in the cytogram, we pay attention to the presence of

Answer 160

- pleocytosis with a predominance of multinucleated cells (neutrophils) - their increased number is observed in acute bacterial infections, but rarely in chronic infections such as syphilis, tuberculosis, viral, fungal. - Increased lactate concentration in the cerebrospinal fluid with accompanying leukocytosis is also a deviation indicating bacterial meningitis. - Reduced glucose concentration is also characteristic of bacterial infections ( lowered cerebrospinal fluid/serum index )

Question 161

# bacterial meningitis bacterial meningitis: Treatment

Answer 161

- requires immediate antibiotic therapy - meningitis is a life-threatening condition, therefore in the case of bacterial etiology, antibiotic therapy must be implemented as soon as possible, - but preferably after performing a lumbar puncture (if there are no contraindications to it), taking blood for culture and other material if possible (e.g. material from skin lesions, synovial fluid).

Question 162

# Neuroinfections in children and adults Meningitis - Diagnostics - Cerebrospinal fluid examination

Answer 162

1) General examination of PMR . 2) Direct preparation – centrifuged sediment stained using the Gram method (enables preliminary identification of bacteria). 3) Culture – final determination of the bacterial etiology of meningitis and drug sensitivity of the isolated bacteria.

Question 163

# Neuroinfections in children and adults Meningitis - Treatment

Answer 163

- After collecting material for microbiological testing,* empirical antibiotic therapy* should be started immediately. - If purulent meningitis is suspected, treatment should be initiated no later than : - 3 hours – from the first contact with a medical professional, - 1 h – from admission to the hospital ward, - 30 min – if meningococcal etiology is suspected .

Question 164

# In type 1 diabetes in children, Criteria for diagnosing type I diabetes:

Answer 164

- randomly determined glucose concentration > 200 mg/dl (≥11.1 mmol/l), - fasting glucose level > 126 mg/dl (≥7.0 mmol/l), - 2-hour oGTT glucose level > 200 mg/dl (≥11.1 mmol/l), - HbA1c ≥ 6.5% (1)

Question 165

# In type 1 diabetes in children, Criteria for diagnosing type I diabetes: - Other selected deviations in laboratory tests:

Answer 165

- glucosuria (3) and ketonuria, - metabolic acidosis in blood gasometry , - ↑ Ht in blood count, - insulin (low blood concentration)

Question 166

# In all children with suspected diabetes What should be determined to confirm type 1 diabetes

Answer 166

- In all children with suspected diabetes, *antibodies against pancreatic antigens* should be determined to confirm type 1 diabetes - anti-GAD - against glutamic acid decarboxylase - anti-IA2 - against tyrosine phosphatase, or - less frequently determined anti-IAA - against insulin - Znt8 - against the zinc transporter, - ICA - anti-islet antibodies)

Question 167

# Respiratory diseases - streptococcal pharyngitis - The patient received 3 points on the Centor scale:

Answer 167

- fever > 38 degrees C - 1 point ; - absence of cough - 1 point ; - enlarged anterior cervical lymph nodes - 1 point ; - no fibrinous coating and tonsil edema - 0 points ; - age 15-44 - 0 points .

Question 168

# Respiratory diseases Acute pharyngitis and tonsillitis – Treatment – ​​Antibiotic therapy for streptococcal infection

Answer 168

1) Phenoxymethylpenicillin – first choice → 1 million IU every 12 hours for 10 days. - If there is any doubt as to whether the patient will take the antibiotic, - benzathine penicillin 1.2 million units im once may be used.

Question 169

# Respiratory diseases Acute pharyngitis and tonsillitis – Test - TX - Due to the moderate risk of streptococcal pharyngitis, before deciding to implement antibiotic therapy, it is necessary to perform a

Answer 169

- bacteriological test/quick test for S. pyogenes infection. - If the bacterial etiology of the infection is confirmed , the antibiotic of first choice is *phenoxymethyl penicillin*, used for 10 days.

Question 170

In newborns, meningitis is most often caused by:

Answer 170

- Escherichia coli , - Streptococcus agalactiae , - Listeria monocytogenes , - Klebsiella sp. - and other Gram-negative intestinal bacilli.

Question 171

# Neuroinfections in children and adults Meningitis - Etiology - Bacterial Meningitis

Answer 171

*Purulent : * Most often caused by encapsulated bacteria, responsible for 90% of bacterial meningitis : - Neisseria meningitidis , - Streptococcus pneumoniae , - Haemophilus influenzae type b (Hib). The predominant etiology varies depending on the age of the patients. *Non-purulent : *

Question 172

Purulent inflammations are caused by

Answer 172

- bacteria that multiply extracellularly . - The inflammatory reaction in these cases is granulocytic .

Question 173

The etiological factors of subglottic laryngitis are viruses

Answer 173

most often: - parainfluenza, - influenza, - adenoviruses , - ECHO viruses - RSV.

Question 174

# Graves-Basedow's disease or graves disease The most common symptoms of hyperthyroidism are:

Answer 174

- behavioral disorders (nervousness, concentration problems, learning problems ), - increased sweating and fatigue, constant feeling of warmth, - trouble falling asleep, - weight loss resulting from increased metabolism despite good appetite, - diarrhea, - feeling of heart palpitations, - positive family history of thyroid disease.

Question 175

# most common cause of hyperthyroidism in children is Graves' disease The most characteristic symptoms visible on physical examination are:

Answer 175

- tachycardia , - exophthalmos and other eye symptoms, - warm and moist skin , - systolic hypertension, - weakening of muscle strength, - tremors

Question 176

# Neuroblastoma - Diagnostics - Laboratory tests in the diagnosis of neuroblastoma, what are determined in a 24-hour urine collection

Answer 176

- VMA and HVA - Catecholamine metabolites ( vanillylmandelic acid - VMA and homovanillin - HVA) are excreted in the urine

Question 177

# Neuroblastoma - Diagnostics - Laboratory tests It is particularly important to mark: serum/urine/others

Answer 177

1) from serum – neurospecific enolase (NSE), LDH, ferritin. Their increased concentration or activity constitutes a high-risk group. 2) from urine – catecholamine metabolites – vanillylmandelic acid (VMA) and homovanillic acid (HVA) and dopamine . Additionally: blood count, creatinine, assessment of kidney and liver function

Question 178

Congenital factor IX deficiency causes

Answer 178

hemophilia B

Question 179

# Plasma hemorrhagic diathesis Hemophilia - Definition

Answer 179

1) They are caused by deficiency or reduced activity of a coagulation factor: – haemophilia A → coagulation factor VIII , – hemophilia B → coagulation factor IX . 2) The genes for both factor VIII and IX are located on the X chromosome . Although women can be symptomatic carriers of the disease, hemophilia primarily affects men . 3) In 30% of patients the mutation occurs spontaneously and the family history is negative.

Question 180

Factor VIII (antihemophilic factor A )

Answer 180

- Factor VIII is involved in the activation of factor Xa in the intrinsic pathway, being part of the intrinsic tenase complex . - Factor VIII circulates in plasma bound to von Willebrand factor (vWF). - Once activated, vWF regulates its concentration in plasma by protecting it from degradation by activated protein C. - Factor VIII deficiency leads to hemophilia A ( antihemophilic factor A ).

Question 181

Factor IX (antihemophilic factor B , Christmas factor)

Answer 181

- Factor IX is activated by the VIIa-TF complex and also by factor XIa. - Factor IXa together with factor VIIIa activates factor X to factor Xa in the so-called intrinsic tenase complex , which initiates the phase of thrombin propagation, i.e. the rapid increase in its amount ("thrombin burst"). - Activation of factor X in the IXa-VIIIa complex is approximately 50-fold more efficient than activation of factor X occurring via the VIIa-TF complex. - Congenital factor IX deficiency causes hemophilia B.

Question 182

Congenital factor IX deficiency causes

Answer 182

hemophilia B

Question 183

Factor VIII deficiency

Answer 183

leads to hemophilia A ( antihemophilic factor A ).

Question 184

Haemophilia B is __ to__ times ____than haemophilia A.

Answer 184

6–7 times rarer than haemophilia A.

Question 185

# Erythema infectiosum - General information Erythema infectiosum is caused by

Answer 185

- parvovirus B19 from the Parvoviridae family . - The virus is transmitted mainly by droplets, and humans are the only source of infection. - The incubation period is from several days to 2–3 weeks. - The patient is infectious for about a week until skin lesions appear. - The course is usually mild, but it can be more severe for specific groups of patients - for people with hemolytic diseases, immunodeficiencies and for pregnant women. (they are associated with the risk of profound anemia and non-immune fetal hydrop

Question 186

Glycogenosis type VI also known as :

Answer 186

( Hers' disease ) is : - inherited in an autosomal recessive manner , the enzymatic defect concerns glycogen phosphorylase , glycogen is stored in the liver, manifests itself with hepatomegaly, hypoglycemia.

Question 187

Glycogenosis type VII known as :

Answer 187

( Tarui's disease ) is : - inherited in an autosomal recessive manner , - the enzymatic defect concerns phosphofructokinase , - glycogen is stored in the muscles , appears in childhood, - manifests itself with muscle flaccidity, muscle cramps after load, easy fatigue, and myoglobinuria.

Question 188

Glycogenosis type III known as:

Answer 188

( Cori disease ) is : - inherited in an autosomal recessive manner , - the enzymatic defect concerns amylo-1,6-glucosidase , - glycogen is stored in the liver , muscles , heart and red blood cells , - manifests itself, among others, with hypoglycemia, hepatomegaly, and myopathy.

Question 189

Glycogenosis type V known as :

Answer 189

( McArdle's disease ) is : - inherited in an autosomal recessive manner , - the enzymatic defect concerns glycogen phosphorylase glycogen is stored in the muscles , - appears in adolescence or adulthood, - manifests itself with muscle flaccidity, muscle cramps after load, easy fatigue, and myoglobinuria.

Question 190

Glycogenoses are diseases in which

Answer 190

- glycogen is stored in various organs , including the liver and muscles (the exception is glycogenosis type 0, in which glycogen synthesis is reduced).

Question 191

Glycogenosis type II also known as:

Answer 191

( Pompe disease ) is : - inherited in an autosomal recessive manner , - the enzymatic defect concerns lysosomal α-glucosidase - glycogen storage is generalized, - the following characters are distinguished: - childhood – manifested by cardiomegaly, hepatomegaly, progressive muscle hypotonia, - juvenile – presenting as myopathy.

Question 192

# Infectious diseases Lyme disease - Early disseminated stage - Inflammation of the nervous system ( neuroborreliosis ) Clinical picture:

Answer 192

- simultaneous or gradual involvement of the central and peripheral nervous system at various levels, - lymphocytic meningitis (usually mild; headache may be the only symptom), - inflammation of the cranial nerves (most often facial nerve paralysis, may be bilateral).

Question 194

In the treatment of acute pharyngitis and tonsillitis caused by ________, what should be administered and what rout?

Answer 194

- Streptococcus pyogenes, - phenoxymethylpenicillin - should be administered orally : - adults and children weighing more than 40 kg at a dose of 2-3 million IU/day administered in 2 divided doses every 12 hours for 10 days, - - children weighing less than 40 kg at a dose of 100,000-200,000 IU/kg/day, administered in 2 divided doses every 12 hours for 10 days.

Question 195

most common cause of primary hypothyroidism in children is

Answer 195

chronic lymphocytic thyroiditis (Hashimoto's disease)

Question 196

The most common cause of hyperthyroidism in children

Answer 196

Graves' disease

Question 197

Symptoms of hypothyroidism depend on the child's age, in children and adolescents they are:

Answer 197

- slowing down the pace of growth, - disorders of sexual maturation (pseudo-premature or delayed), - delayed eruption of primary or permanent teeth, - bradycardia , -- drowsiness, fatigue, - deterioration of academic performance, - cold intolerance, - dry skin , - hair loss, - menstrual cycle disturbances (prolonged or heavy bleeding).v

Question 198

The most common symptoms of hyperthyroidism are:

Answer 198

-- behavioral disorders (nervousness, concentration problems, learning problems ), - increased sweating and fatigue, constant feeling of warmth, - trouble falling asleep, - weight loss resulting from increased metabolism despite good appetite, - diarrhea, - feeling of heart palpitations, - positive family history of thyroid disease.

Question 199

# common symptoms of hyperthyroidism The most characteristic symptoms visible on physical examination are:

Answer 199

- tachycardia , - exophthalmos and other eye symptoms, - warm and moist skin , - systolic hypertension, - weakening of muscle strength, tremors .

Question 200

# Erythema infectiosum - General information Erythema infectiosum is caused by parvovirus B19 from the Parvoviridae family .

Answer 200

- The virus is transmitted mainly by droplets, and humans are the only source of infection. - The incubation period is from several days to 2–3 weeks. - The patient is infectious for about a week until skin lesions appear. - The course is usually mild, but it can be more severe for specific groups of patients - for people with hemolytic diseases, immunodeficiencies and for pregnant women. (they are associated with the risk of profound anemia and non-immune fetal hydrops) .

Question 201

Aplastic crisis; complication of what? occurs in whom?

Answer 201

- the most severe complication of parvovirus infection - occurs in children with chronic hemolytic anemia, in whom the erythrocyte turnover time is shortened (e.g. sickle cell anemia and thalassemia), - and in children with impaired immunity (e.g. due to cancer), who are unable to produce immune antibodies that can stop the infection

Question 202

The *Apgar* score includes assessment of the following characteristics

Answer 202

- A appearance - skin color - P ulse - heart rate - Grimace - reaction to irritation - Activity - muscle tension - R espiration - breathing