0
Q
pathogenesis of atopic dermatitis (eczema)
A
- epidermal dysfunction due to improper synthesis of stratum corneum components. Allergens can enter the disrupted skin barrier and generate an inflammatory response
- excessive bathing, dry environments, stress, overheating, and irritating detergents can trigger flares
1
Q
Atopic dermatitis (eczema)
A
- in INFANCY presents w/ pruritis, and scaly erythematous lesions on the face, chest, and extensor surfaces of the extremities
- Flexural involvement is common in children and adults
- topical emollients are the first line treatment (+/- steroid ointment)
- risk factors: low humidity, relatives w/ eczema, allergies, or asthma
- complications: eczema herpeticum, cellulitis/abscess, discomfort interfering w/ daily activities and sleep
2
Q
Contact dermatitis
A
- itchy red rash w/ indistinct margins and occur only in areas of direct allergen contact
- common in older children and adults after sensitization to an allergen (eg poison ivy resin, nickel, neomycin/bacitracin)
3
Q
Eczema herpeticum
A
- potential complication of severe atopic dermatitis
- superinfection w/ herpes simplex virus can cause a vesicular eruption on preexisting inflamed skin
- these pts often have fever and pain
4
Q
Erythroderma (exfoliative dermatitis)
A
- erythema and scaling in > 90% of the body
- bright red patches coalesce and gradually peel
5
Q
Psoriasis
A
- characterized by well-demarcated erythematous plaques w/ silvery scales on the scalp, extensor surfaces, and back
- common in adults but not in children
6
Q
Scabies
A
- caused by Sarcoptes scabiei infection
- small pruritic papules in a linear arrangement (burrows) favor the web spaces, wrists and ankles, genitals, nipples, and waistline
7
Q
Seborrheic dermatitis (“cradle cap”)
A
-adherent greasy scales w/ a mildly erythematous base seen on the scalp
8
Q
Clubfoot (talipes equinovarus)
A
- initially managed w/ stretching and manipulation of the foot, followed by serial plaster casts, malleable splints, or taping
- surgical treatment is indicated if conservative management gives unsatisfactory results, and is preferably performed between 3 and 6 months of age
- remember that the tx of clubfoot should be started IMMEDIATELY
- Equinus and varus of the calcaneum and talus, varus of the midfoot, and adduction of the forefoot
9
Q
Meconium ileus
A
- virtually diagnostic for cystic fibrosis
- inspissated meconium is responsible for life-threatening obstruction at the level of the ileum
10
Q
what two disorders must be considered in any infant who has delayed passage of meconium?
A
- Meconium ileus and Hirschsprung disease (congenital aganglionic megacolon)
- these conditions can be differentiated by the level of the intestinal obstruction and consistency of the meconium
- Hirschsprung: rectosigmoid obstruction, normal consistency
- Meconium ileus: Ileum obstruction, inspissated consistency
11
Q
what is meconium ileus virtually diagnostic for?
A
- Cystic fibrosis
- although only 20% of pts w/ CF develop meconium ileus, almost all newborns w/ meconium ileus have CF
- should prompt SWEAT CHLORIDE TESTING to confirm diagnosis
12
Q
long QT syndrome
A
- pts are at risk of syncope, ventricular arrhythmias, and sudden cardiac death
- those w/ congenital QT prolongation should avoid electrolyte derangements and medications that block potassium channels
- beta blockers w/ pacemaker placement can prevent cardiac arrest
13
Q
developmental milestones for 3 year olds
A
- throw a ball overhead
- build a 6-8 block tower
- copy a circle
- ride a tricycle
14
Q
developmental milestones for 4 year olds
A
- hop on 1 foot
- copy a cross
- brush teeth
15
Q
developmental milestones for 5 year olds
A
- tie a knot, copy squares
- mature pencil grasp, print some letters and numbers
- skip, draw a person w/ 6 or more body parts
16
Q
Thiamine (vitamin B1) deficiency
A
- causes Beriberi or Wernicke-Korsakoff syndrome
- neurologic and psychiatric symptoms, and are often seen in alcoholics or pts who have had weight loss surgery
17
Q
Riboflavin (vitamin B2) deficiency
A
-cheilosis, glossitis, seborrheic dermatitis (often affecting the genital areas), pharyngitis, and edema and/or erythema of the mouth
18
Q
Pyridoxine (vitamin B6) deficiency
A
- irritability, depression, dermatitis, and stomatitis
- can cause elevated serum homocysteine concentration, which is a known risk factor for venous thromboembolic disease and atherosclerosis
19
Q
Cyanocobalamin (vitamin B12) deficiency
A
-macrocytic anemia and peripheral neuropathy
20
Q
Niacin (vitamin B3) deficiency
A
-Pellagra (diarrhea, dermatitis, dementia, death)
21
Q
pyloric stenosis
A
- most commonly seen in boys age 1-2 months w/ NON-bilious projectile vomiting
- visible peristaltic waves and a palpable abdominal mass
- abdominal US is the preferred imaging modality
22
Q
Hirschsprung disease and meconium ileus usually present w/ failure to pass meconium within the first 2 days of life and dilated loops of bowel on x-ray. These should be evaluated by CONTRAST ENEMA.
A
-
23
Q
Malrotation w/ midgut volvulus
A
- usually presents in neonates w/ BILIOUS vomiting and abdominal distension
- untreated volvulus can progress to frank bowel ischemia, bloody stools, and perforation
- an upper GI contrast study is the gold standard for diagnosing malrotation
24
The finding of the ligament of Treitz on the right side of the abdomen reflects MALROTATION, while contrast in a "corkscrew" pattern indicates VOLVULUS on upper GI series.
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25
what is generally the normal duration of crying in an infant?
1-2 hours per day
26
Colic
- excessive crying for 3 or more hrs a day, 3 or more days a week, over a period of 3 or more weeks in an otherwise healthy infant
- usually presents in the first few weeks of life and resolves spontaneously by age 4 months
- crying generally occurs at same time of day, usually early evening
- cause is unknown, but may be overstimulation and parental unfamiliarity w/ alternate soothing methods
- calming techniques include an infant swing, swaddling, minimizing environmental stimuli, and holding and rocking the baby
- feeding patterns should also be reviewed to assess if there is overfeeding, underfeeding, or an inadequate burping technique
27
Intussusception
- typically occurs at age 3-36 months and presents w/ recurrent episodes of severe abdominal pain
- poor appetite and vomiting are common
28
GERD in infants
- usually presents w/ excessive spitting up and crying after feedings
- may arch back during or after feeding
- meds are generally reserved for infants who are not gaining weight appropriately
29
Infantile hypertrophic pyloric stenosis
- most common in first born boys age 3-5 weeks
- protracted projectile, NONbilious vomiting causes a hypochloremic metabolic alkalosis
- abdominal US confirms the diagnosis
- tx is pyloromyotomy
30
Milk protein allergy
- can present w/ vomiting, poor weight gain, and bloody stools
- affected infants should be switched to a hydrolyzed formula and avoid cow's milk-based and soy-based formulas
31
Bilious vomiting in the first 2 days of life and a "double bubble" sign on abdominal x-ray
- Duodenal obstruction
| - duodenal atresia is strongly associated w/ Down syndrome
32
infants w/ biliary atresia
- most are asymptomatic at birth and initially seem healthy
| - jaundice and acholic (pale) stools typically develop by age 2 months
33
meconium ileus
- pathognomonic for cystic fibrosis and typically presents as delayed passage of meconium (> 48 hrs), marked abdominal distension, and sometimes bilious emesis
- obstruction is typically at the level of the terminal ileum, and abdominal x-ray shows multiple dilated loops of small bowel
34
pyloric stenosis
-postprandial NONbilious emesis at ages 3-6 weeks
35
Sigmoid volvulus
- abdominal pain, distention, and constipation due to torsion of the sigmoid colon
- x-ray shows an inverted U-shaped appearance of the distended sigmoid loop ("coffee bean sign")
- typically occurs in elderly pts and is exceptionally rare in children
36
Gastroesophageal reflux (mild, NOT GERD) is common in infants due to their shorter esophagus, incomplete closure of the lower esophageal sphincter, and greater time spent in the supine position. Parents should be reassured about adequate weight gain and advised to hold the infant upright after feeds.
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37
In contrast to physiologic reflux, pathologic features of GERD include failure to thrive or Sandifer syndrome (intermittent opisthotonic posturing). Thickened feeds and antacid therapy are the first steps in management of GERD.
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38
Regurgitation caused by milk protein allergy is associated w/ eczema, poor weight gain, and bloody stools. Infants w/ milk protein allergy should be switched to a hydrolyzed formula. Hydrolyzed formulas are very expensive and unnecessary when no other symptoms are present.
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39
Goat milk is deficient in folate and could result in macrocytic anemia.
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40
Gastroschisis
- usually an ISOLATED defect lateral (right) to the umbilical cord w/ uncovered bowel herniating through the abdominal wall
- maternal serum a-fetoprotein is typically elevated during pregnancy
- after delivery, the exposed bowel should be covered w/ sterile saline dressings and plastic wrap. Immediate surgery is required.
41
Omphalocele
- commonly has other major malformations associated w/ it such as cardiac disease, neural tube defects, or trisomy syndromes
- have a covering sac of peritoneum over healthy bowel
- need immediate surgery after birth by staged closure w/ a SILASTIC SILO
42
Increased gastric residual volume, vomiting, and abdominal distention in a premature neonate
- Necrotizing enterocolitis
- hallmark findings on x-ray include PNEUMATOSIS INTESTINALIS (intramural air) and portal venous air
- leukocytosis and metabolic acidosis are common
43
Infantile hypertrophic pyloric stenosis presents w/ projectile, NONbilious emesis and an olive-shaped mass in RUQ. Prolonged vomiting causes a hypochloremic, hypokalemic metabolic alkalosis. Tx consists of IV rehydration and normalization of electrolytes prior to pyloromyotomy to decrease the risk of post-op apnea.
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44
US is the first-line modality for diagnosing intussusception. A positive "target sign" should prompt reduction w/ air or water-soluble contrast enema.
.
45
Intussusception
- telescoping of one bowel segment into another
- most common cause of intestinal obstruction in children 6-36 months
- ileocolic junction is most frequently involved
- most children have no identifiable lead point (75%)
- possible lead points: Peyer patches (inflammation from viral infection), Meckel's diverticulum, polyps, hematomas (from Henoch-Schonlein purpura)
- intermittent pain
- currant jelly stools are preceded by mucosal ischemia, occult bleeding
- palpable tubular "sausage shaped" mass in RUQ
- US is test of choice ("target sign")
- tx is w/ AIR ENEMA
46
Atopic dermatitis
- presents in INFANCY w/ pruritis, and scaly erythematous lesions on the face, chest, and extensor surfaces of the extremities
- FLEXURAL involvement is common in children and adults
- topical emollients are the first-line treatment
47
Meconium ileus is virtually diagnostic of what?
- Cystic fibrosis
| - Inspissated meconium is responsible for life-threatening obstruction at the level of the ileum
48
family hx of sudden death, congenital sensorineural deafness, and QT interval prolongation
- Jervell and Lange-Nielsen syndrome
| - autosomal recessive long QT syndrome caused by defects in potassium channels
49
Pts w/ prolonged QT intervals are at risk for syncope, ventricular arrhythmias, and sudden cardiac death. Those w/ congenital QT prolongation should avoid electrolyte derangements and medications that block potassium channels. Beta blockers w/ pacemaker placement can prevent cardiac arrest.
.
50
Tay-Sachs disease
- progressive autosomal recessive condition
- B-hexosaminidase A deficiency
- Like Niemann-Pick disease, Tay-Sachs also presents w/ loss of motor milestones, hypotonia, and a "cherry-red" macula. However, pts w/ Tay-Sachs have HYPERREFLEXIA and NO hepatosplenomegaly
51
Galactocerebrosidase deficiency
- Krabbe disease
- autosomal recessive LYSOSOMAL storage disorder that presents in early infancy w/ developmental regression, hypotonia, and areflexia
52
Gaucher disease
- Glucocerebrosidase deficiency
| - anemia, thrombocytopenia, and hepatosplenomegaly
53
Hurler syndrome
- one of the mucopolysaccharidoses
- LYSOSOMAL storage disease due to lysosomal HYDROLASE deficiency
- presents at age 6 months- 2 years w/ coarse facial features, inguinal or umbilical hernias, corneal clouding, and hepatosplenomegaly
54
Niemann-Pick disease type A
- due to SPHINGOMYELINASE deficiency
- presents at age 2-6 months w/ areflexia, hepatosplenomegaly, a "cherry-red" macula, and developmental milestone regression
- Although Tay-Sachs disease presents in a similar manner, hepatosplenomegaly and areflexia are NOT seen
55
Down syndrome (trisomy 21)
- hypotonia, flat face, upward and slanted palpebral fissures, epicanthal folds, Brushfield spots, dysplasia of the pelvis, cardiac malformations, simian crease, short and broad hands, hypoplasia of the middle phalanx of the 5th finger, intestinal atresia, and high arched palate
- most common trisomy
56
Patau's syndrome (trisomy 13)
-cleft lip, flexed fingers w/ polydactyly, ocular hypotelorism, bulbous nose, low-set malformed ears, small abnormal skull, cerebral malformation, microphthalmia, cardiac malformations, scalp defects, hypoplastic or absent ribs, visceral and genital anomalies
57
Wolf-Hirschhorn's syndrome
- very low yield for USMLE
- chromosome 4p deletion
- "greek helmet" facies w/ ocular hypertelorism, prominent glabella and frontal bossing, ptosis, and strabismus
58
Cri-du-chat syndrome
- microcephaly w/ protruding metopic suture
- cat-like cry
- hypotonia, short stature, moonlike face, hypertelorism, bilateral epicanthic folds, high arched palate, wide and flat nasal bridge
- 5p deletion
59
Edwards' syndrome (trisomy 18)
-low birth weight, clenched fists w/ index finger overlapping the 3rd digit and the 5th digit overlapping the 4th, microcephaly, prominent occiput, micrognathia, rocker-bottom feet
60
The diagnosis of ADHD is based on clinical evaluation of symptoms and associated impairment in 2 different settings. Teacher evaluations are an important tool for assessing behavior in the school environment.
.
61
Complete AV canal defect
- strongly associated w/ Down syndrome
- aka endocardial cushion defect
- chest x-ray findings include increased pulmonary markings and cardiomegaly from excessive pulmonary blood flow and biventricular volume overload
62
Ebstein's anomaly
- associated w/ maternal lithium use during pregnancy
- displacement of a malformed tricuspid valve into the right ventricle
- the droopy tricuspid valve results in severe tricuspid regurgitation and right atrial enlargement, resulting in tall P waves and right axis deviation on ECG
- extreme cardiomegaly from heart failure can be seen on chest x-ray
63
Tetralogy of Fallot
- can have paucity of vascular marking on CXR due to pulmonary outflow tract obstruction
- "boot-shaped heart"
- right axis deviation on ECG
64
Total anomalous pulmonary venous return
- defect in which all 4 pulmonary veins fail to make their normal connection to the left atrium
- the right atrium receives blood from both the pulmonary and systemic venous systems, resulting in right atrial and ventricular enlargement
- ECG can show right ventricular hypertrophy and right axis deviation
- CXR can show increased pulmonary markings due to pulmonary overcirculation
65
Truncus arteriosus
- strongly associated w/ DiGeorge syndrome
- ECG can be normal in neonate
- CXR typically shows cardiomegaly and increased pulmonary vascular markings from heart failure and pulmonary overcirculation
66
Left axis deviation on neonatal ECG is NEVER NORMAL.
.
67
Tricuspid valve atresia
-cyanotic congenital heart defect characterized by left axis deviation on ECG and decreased pulmonary markings on CXR due to hypoplasia of the right ventricle and pulmonary outflow tract
68
IgA deficiency
- consider in pts w/ mild immunodeficiency consisting of recurrent sinopulmonary and GI infections, and anaphylactic transfusion reaction
- Diagnosis may be confirmed by measurement of a low serum IgA concentration w/ normal serum IgM and IgG levels
- Pts may form antibodies to IgA, predisposing them to anaphylactic transfusion reactions
69
DiGeorge syndrome
- thymic aplasia and consequent T cell deficiency
- susceptible to fungal infections
- hypoparathyroidism w/ hypocalcemia, cleft palate, and congenital heart disease (truncus arteriosis) are also associated
70
X-linked agammaglobulinemia (Bruton's)
- affects males
- pts lack B cells and all classes of Ig
- sinopulmonary infections and Pseudomonas are common
71
Wiskott-Aldrich syndrome
- X-linked combined B and T cell disorder
| - pts present w/ recurrent otitis media, eczema, and thrombocytopenia
72
Cystic fibrosis
-recurrent respiratory infections, sinus disease, pancreatic insufficiency, meconium ileus, and infertility
73
Hyper-IgM syndrome
- pts have elevated IgM and depressed IgG and IgA
- they are susceptible to both Giardia and recurrent sinopulmonary infection
- Lymphoid hyperplasia is common
74
Osteogenesis imperfecta
-associated w/ blue sclerae, hearing loss, recurrent fractures, and OPALESCENT teeth (dentinogenesis imperfecta; due to discolored dentin shining through the translucent and weak enamel; both primary and permanent teeth are affected)
75
Osteogenesis imperfecta
- associated w/ blue sclera, hearing loss, recurrent fractures, and opalescent teeth
- Pts w/ OI have normal intelligence
76
Full term infants are born w/ adequate iron stores to prevent anemia for the first 4-6 months of life regardless of dietary intake. Preterm infants are at significantly increased risk for iron deficiency anemia. Iron supplementation should be started at birth in exclusively breastfed preterm infants and continued until age 1 year. All exclusively breastfed infants should be started on Vitamin D 400 IU daily.
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77
what factors increase risk of iron deficiency anemia in infants
- maternal iron deficiency
- prematurity
- early introduction of cow's milk before age 12 months
78
what is the first step in management of a newborn w/ respiratory compromise and suspected congenital diaphragmatic hernia (CDH)?
- Endotracheal intubation!
- note that bag-and-mask ventilation can exacerbate respiratory decline
- a gastric tube should be placed immediately to decompress the stomach and bowel.
79
Bilateral choanal atresia
-presents w/ cyanosis and respiratory distress during feeding that improve when the infant cries
80
Congenital diaphragmatic hernia
- cyanosis w/ respiratory distress immediately after birth
- polyhydramnios can occur as a result of esophageal compression
- deviation of abdominal viscera into the thorax results in a scaphoid-appearing abdomen
- x-ray shows a displaced cardiac silhouette, bowel in the thorax, and a gasless abdomen
81
Duodenal atresia
- can cause polyhydramnios and vomiting w/ initial feeds but not respiratory distress
- the abdomen would NOT be distended and intestinal gas would be absent on x-ray as air cannot pass the duodenum
- gastric tubes can be inserted without resistance and x-ray would show air in the stomach and proximal duodenum ("double-bubble sign")
82
Esophageal atresia without fistulous connection to the trachea is rare and would not cause sudden respiratory distress w/ feeding.
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83
Anomalous vascular branches of the aortic arch (vascular rings)
-can cause stridor and dysphagia due to compression of the trachea and esophagus
84
Esophageal atresia w/ TE fistula
- should be suspected when a newborn chokes and coughs during the first feeding
- attempts at naso- or orogastric tube insertion will fail and x-ray will show the tube in the proximal esophageal pouch
- most common is a PROXIMAL atresia with a fistula between the trachea and DISTAL esophagus
- Polyhydramnios is typically seen
- aspiration pneumonia and distended abdomen is common
- half of pts have other anomalies; VACTERL (vertebral, anal atresia, cardiac, TE fistula, renal, limb)
85
genetic B-lymphocyte deficiency
- begin to develop recurrent infections AFTER passing 6 months of age due to the decline of passive immunity from mom's Ig
- The deficient humoral immune response in these pts impairs the body's ability to destroy ENCAPSULATED organisms (recurrent sinopulmonary infections w/ H. influenza and S. pneumo are common)
- lack of IgA also predisposes to Giardia infection
86
Chronic granulomatous disease (CGD)
- impaired oxidative metabolism
- a defect in the NADPH-oxidase system of phagocytic cells results in defective intracellular killing
- pts are prone to ABSCESSES secondary to catalase-producing organisms like ASPERGILLUS and STAPHYLOCOCCUS
87
Complement deficiency results in common infections with what?
-Gonococcal and meningococcal infections (due to Neisseria species)
88
Thymic hypoplasia associated w/ DiGeorge syndrome
-T-cell deficiency puts pts at high risk for viral and fungal infections
89
Adenosine deaminase deficiency
- commonly the genetic defect underlying severe combined immunodeficiency (SCID)
- pts w/ SCID have a deficiency of both B and T cells
- T cell deficiency predisposis to viral and fungal infections and the B cell deficiency predisposes to infection by bacteria
90
Meningococcal meningitis
- most common cause of bacterial meningitis in children and young adults
- typically presents w/ fever, headache, neck stiffness, altered mental status, and a PETECHIAL OR PURPURITIC RASH
- prompt diagnosis and treatment are critical given that it has a high morbidity and mortality rate even w/ appropriate treatment
91
Borrelia burgdorferia
- causes Lyme disease
- usually acquired in the late summer months after an Ixodes sp. tick bite
- presents w/ rash (ie erythema chronicum migrans), headaches, fevers, chills, and malaise
- skin rash is very characteristic and is large, annular, and erythematous, occassionally w/ central clearing
92
Cytomegalovirus
-can cause infectious mononucleosis like illness, which presents w/ high fevers, fatigue, malaise, splenomegaly, and pharyngitis
93
Group B strep
- most common cause of meningitis in infants
| - usually acquired from the mother during childbirth
94
Haemophilus influenza
- may cause meningitis but typically does NOT present w/ a rash
- may also cause rhinorrea, fever, epiglottitis, and ear infections
- the rate of infection has decreased dramatically since the vaccine was introduced
95
Herpes simplex virus
-generally causes temporal lobe encephalitis in neonates and infants and typically presents w/ seizures
96
Listeria monocytogenes
- transmitted vaginally
- 1 of the 3 most common causes of meningitis in newborns but does NOT present w/ a rash
- another mode of transmission would be ingestion of unpasteurized milk or cheese from infected cows
97
Hemolytic Uremic Syndrome (HUS)
- suspect HUS in a child who recently recovered from a diarrheal illness and presents w/ acute renal failure, microangiopathic hemolytic anemia, fever, thrombocytopenia and characteristic peripheral smear finding of schistocytes
- E. coli 0157:H7 is often the organism (can also be w/ Shigella, Salmonella, Yersinia, and Campylobacter)
98
Thalassemia presentation
- hepatosplenomegaly
- peripheral blood smear may reveal target cells, microcytosis, hypochromia, and anisopoikilocytosis, but NOT schistocytes
- BUN and creatinine levels are normal
99
B12/Folate deficiency
-macrocytosis and hypersegmented PMNs on blood smear
100
Lead poisoning
- causes a MICROCYTIC anemia, w/ BASOPHILIC STIPPLING of RBCs on peripheral smear
- pts typically also present with GI symptoms
101
PT and aPTT are prolonged in pts w/ DIC
.
102
ITP
- characterized by an ISOLATED thrombocytopenia
| - on peripheral smear, the morphology of red cells is normal
103
Bleeding in renal failure is usually due to dysfunctional platelets from uremia.
.
104
Human milk is the ideal form of nutrition for term infants. The major protein source is whey, which is more easily digested than casein and helps to improve gastric emptying.
.
105
what is the most common type of brain tumor seen in pediatric patients?
- benign astrocytomas
- in the pediatric population, infratentorial tumors are more common than supratentorial tumors, and benign astrocytomas are the most common histologic type in both groups
106
Medulloblastoma
- second most common tumor of the posterior fossa in children
- approximately 90% of cases arise from the vermis
107
Glioblastoma multiforme
- highly malignant astrocytic tumor
| - usually seen in adults
108
Pinealoma
- develops in the dorsal aspect of the midbrain
- symptoms consist of endocrine syndrome, intracranial hypertension, Parinaud's sign (paralysis of vertical gaze), and Collier's sign (retraction of the eyelid)
109
Craniopharyngioma
- arise in the sella turcica, and symptoms usually include visual field defects and an endocrine syndrome
- characterized by a cystic structure w/ calcifications
110
Pediatric OCD
- repetitive mental and physical rituals that interfere w/ social and occupational functioning
- Tx involves high-dose SSRI and psychotherapy
- onset may be associated w/ recent streptococcal infection (PANDAS)
111
Spondylolisthesis
- developmental disorder characterized by a forward slip of vertebrae (usually L5 over S1) that usually manifests in preadolescent children.
- in the typical clincial scenario, back pain, neurologic dysfunction (ie urinary incontinence), and a palpable "step-off" at the lumbosacral area are present if the disease is severe
112
Patients w/ sickle cell anemia
- anemia w/ a high reticulocyte count, previous admissions for pain, hematuria, african american
- become functionally asplenic at an early age due to splenic autoinfarction, thus more susceptible to encapsulated organisms
- pneumococcal vaccination plus penicillin prophylaxis (until 5 years of age; twice daily) can prevent almost all cases of pneumococcal sepsis in pts w/ sickle cell anemia
113
live attenuated virus vaccines
-measles, mumps, rubella, and chicken pox (varicella)
114
bacterial toxoid vaccines
-tetanus and diptheria
115
Sickle cell anemia
- associated w/ functional asplenia due to recurrent splenic infarction, which puts these pts at risk of dangerous infection w/ encapsulated organisms (S. pneumo, H. influenza type B, N. meningitides, and Salmonella species)
- Pneumococcus is the most common cause of sepsis in this population
- vaccination and penicillin prophylaxis are extremely important infection prevention measures
116
what vaccinations should sickle cell pts receive?
- both the 13 valent and 23 valent polysaccharide pneumococcal vaccines
- meningococcal conjugate vaccines
- H influenza type B vaccines
117
common cause of bacteremia in pts w/ burn wound and neutropenia?
Pseudomonas aeruginosa
118
most common causes of osteomyelitis in sickle cell pts in the U.S.
-Salmonella species and Staph aureus
119
Brain abscess
- can present w/ fever, headache, focal neurologic deficits, and seizure
- congenital heart disease (right-to-left shunts bypass the lungs, an important filter) and recurrent sinusitis (direct spread to frontal lobe) are important predisposing factors
120
bacterial meningitis presentation
-headache, lethargy, fever, fast onset, photophobia, nuchal rigidity
121
Carotid artery dissection
-typically occurs after trauma or spontaneously in pts w/ connective tissue disorders
122
Systemic Lupus Erythematosus (SLE)
- often associated w/ false positive RPR test resuts
- 1/5 of all SLE cases occur in children
- confirmatory tests are a positive ANTI-SMITH antibody and/or ANTI-DOUBLE STRANDED DNA antibodies (highly specific)
123
Neonatal abstinence syndrome
- caused by infant withdrawal to opiates and usually presents in the first few days of life
- characterized by irritability, a high-pitched cry, poor sleeping, tremors, seizures, sweating, sneezing, tachypnea, poor feeding, vomiting, and diarrhea
- tx is symptomatic care such as swaddling, providing small frequent feeds, and keeping the infant in a low stimulation environment
- pharmacologic tx should be used when supportive tx does not control the infant's withdrawal symptoms. Morphine can be administered and systematically weaned to help control opiate withdrawal
124
fetal exposure to valproic acid
-risk of neural tube defects, may result in cardiac anomalies, and can produce dysmorphic facies, including a cleft lip, narrow bifrontal diameter, midface hypoplasia, broad and depressed nasal bridge, and long philtrum
125
prenatal exposure to phenytoin
- fetal hydantoin syndrome, which is characterized by nail and digit hypoplasia, dysmorphic facies, and mental retardation
- growth deficiency can also be seen
126
fetal alcohol syndrome
- growth deficiency, presence of a long, smooth philtrum, small palpebral fissures, and a thin upper lip in addition to CNS abnormalities such as mental retardation
- withdrawal symptoms are usually not seen unless the mother was drinking just prior to delivery
127
Prenatal exposure to cocaine
- jitteriness, excessive sucking, and a hyperactive Moro reflex
- withdrawal symptoms are usually not as severe as with opiates
- long term effects on behavior, attention level, and intelligence may be seen
128
Guillain-Barre syndrome
- most commonly presents w/ ASCENDING flaccid paralysis
- involvement of respiratory and bulbar muscles puts pts at high risk for respiratory failure
- serial measurements of FVC are the best means of monitoring respiratory function (use SPIROMETRY)
- severe autonomic dysfunction, flaccid quadriparesis, inability to hold the neck erect, and bulbar palsy are other indications for critical care
129
Botulism has DESCENDING progression of weakness!
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130
Congenital hypothyroidism
- infants initially appear normal at birth, but gradually develop apathy, weakness, hypotonia, large tongue, sluggish movement, abdominal bloating, and an umbilical hernia
- for this reason, screening newborns for hypothyroidism, along w/ PKU and galactosemia, is standardly performed in all states
- tx is levothyroxine
- most common cause is thyroid dysgenesis (ie aplasia, hypoplasia, or ectopic gland)
131
infant botulism
-occurs most commonly in infants whose diet includes honey or canned food
132
Werdnig-Hoffman syndrome
- autosomal recessive disorder that involves degeneration of the anterior horn cells and cranial nerve motor nuclei
- it is a cause of "floppy baby" syndrome (the other cause is infant botulism)
133
Myotonic congenital myopathy
-autosomal dominant disorder characterized by muscle weakness and atrophy (most predominant in the distal muscles of the upper and lower extremities), myotonia, testicular atrophy, and baldness
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Myasthenia gravis
- presents w/ easy fatigability and weakness that improves w/ rest
- the pediatric form occurs in late childhood or adolescence
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Galactosemia
- newborn or young infant w/ failure to thrive, bilateral cataracts, jaundice and hypoglycemia
- caused by galactose-1-phosphate uridyl transferase deficiency
- typically present w/ vomiting, poor weight gain, jaundice, hepatomegaly, convulsions, and cataracts
- other common manifestations include aminoaciduria, hepatic cirrhosis, hypoglycemia, and mental retardation. Pts are at increased risk of E. coli neonatal sepsis
- Early diagnosis and tx by elimination of galactose from the diet are mandatory
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Galactokinase deficiency
-present w/ cataracts only, and are otherwise asymptomatic
137
Uridyl diphosphate galactose-4-epimerase deficiency
- rare disease compared to uridyl-transferase deficiency
| - pts have hypotonia and nerve deafness
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Malrotation with midgut volvulus
- usually presents in neonates w/ bilious vomiting and abdominal distension
- untreated volvulus can progress to frank bowel ischemia, bloody stools, and perforation
- an upper GI contrast study is the gold standard for diagnosing malrotation
- finding is Ligament of Treitz on the right side of the abdomen (malrotation) while contrast in a "corkscrew" pattern indicates volvulus
- Ladd procedure consists of fixing the bowel in a non-rotated position to minimize recurrent volvulus risk
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Pyloric stenosis
- most commonly seen in boys age 1-2 months w/ NON-bilious projectile vomiting
- finding can include a visible peristaltic waves and a palpable abdominal mass
- abdominal US is the preferred imaging modality for pyloric stenosis
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Hirschsprung disease and meconium ileus
- both usually present w/ failure to pass meconium within the first 2 days of life and dilated loops of bowel on x-ray
- these diagnoses should be evaluated by contrast enema
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Serial abdominal x-rays are used in infants to follow the progression of non-surgical conditions, such as ileus or mild cases of necrotizing enterocolitis.
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Cri-du-chat syndrome
- due to 5p deletion and presents as microcephaly, hypotonia, short stature, and a cat-like cry
- other features: protruding metopic suture, moonlike face, hypertelorism, bilateral epicanthal folds, high arched palate, wide and flat nasal bridge, and mental retardation
143
Chronic constipation is a risk factor for what in toddlers?
- Recurrent cystitis
- impacted stool can cause rectal distension, which in turn compresses the bladder, prevents complete voiding, and leads to urinary stasis
144
any male adolescent who presents with epistaxis, a localized mass, and a bony erosion on the back of the nose has what?
-an ANGIOFIBROMA until proven otherwise (juvenile angiofibroma)
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Reactive nasal polyps
- normally do NOT cause bony erosions
| - usually associated w/ chronic infections or allergies, and the main complaint is obstruction rather than bleeding
146
Fragile X syndrome
- characterized by low to normal IQ w/ learning disabilities, generalized language disability, short attention span, autism, large head, prominent jaw, large low set ears and macroorchidism
- results from full mutation in the FMR1 gene caused by an increased number of CGG trinucleotide repeats accompanied by aberrant methylation of the FMR1 gene
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Fetal alcohol syndrome
mild to moderate mental retardation, hypo-plastic maxilla, long philtrum, and microcephaly
148
Immune Thrombocytopenia (ITP)
- should be suspected in children who develop isolated thrombocytopenia and petechia after a viral infection
- children usually spontaneously recover within 6 months and require only observation, regardless of platelet count
- children w/ bleeding should receive IVIG or glucocorticoids
149
Risk factors for renal calculi
-diet high in animal protein and sodium and low in calcium and fluid
150
risk factors for the development of renal cell carcinoma
-cigarette smoking, hypertension, and diabetes mellitus
151
Vesicoureteral reflux (VUR)
- risk factor for recurrent UTIs, which can lead to progressive renal scarring
- all children w/ a first febrile UTI at age 2-24 months should undergo a renal US to evaluate for anatomic abnormalities
- those w/ recurrent UTIs should also undergo a voiding cystourethrogram to evaluate for VUR
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almost all infants w/ congenital toxoplasmosis are asymptomatic until adulthood, when they develop chorioretinitis
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HIV infection in infants
- should be suspected when an infant has failure to thrive, lymphadenopathy, and thrush in the setting of maternal IV drug abuse
- Pregnant pts w/ risky behavior should undergo HIV antibody testing in the 1st and 3rd trimesters as it can take up to 3 months to develop detectable antibody
- diagnosis in infants consists of PCR testing
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Friedreich Ataxia
- autosomal recessive condition and genetic counseling is recommended for prenatal diagnosis for parents with one affected child
- due to an excessive number of trinucleotide repeats, resulting in an abnormality of a tocopherol transfer protein
- the disorder is progressive w/ a poor prognosis; wheelchair bound by 25 and death by 30-35
- unsteady gait, weakness in lower limbs, decreased vibratory and position sense in lower extremities, high plantar arches, atrophy of cervical spinal cord and minimal cerebellar atrophy
155
Differential for T-wave inversion?
- Myocardial infarction
- Myocarditis
- Old pericarditis
- Myocardial contusion
- Digoxin toxicity
156
language development of a 2 year old
- vocabulary of several hundred words and be able to combine words into short sentences
- in addition, half of their speech should be understandable to a stranger
157
language development of a 1 year old
- "mama" and "dada", and one other word
| - should be able to follow simple commands w/ gestures
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language development of 15 month old
- ''mama", "dada", and 3-5 other words
| - can understand simple commands even without a gesture
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language development of 18 month old
-vocabulary of approx. 5-20 words
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language development of 3 year old
- vocab of close to 1000 words, and about 75% of child's speech can be understood by a stranger
- they speak in 3-4 word sentences and use pronouns correctly
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Pyloric stenosis
- presents at age 3-5 weeks w/ projectile, nonbilious vomiting that occurs after each feed
- protracted vomiting produces a hypochloremic, hypokalemic metabolic alkalosis that should be corrected prior to pyloromyotomy to decrease risk of postoperative apnea
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normal acid-base status
- pH of 7.35-7.45
- PaCO2 of 35-45
- Bicarb of 22-26
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Nephrotic syndrome
- edema, fatigue, proteinuria, absence of hematuria, hypoalbuminemia
- MINIMAL CHANGE DISEASE in peds
- FSGS, Membranous nephropathy, Membranoproliferative glomerulonephritis in adults
164
Nephritic syndrome
- hypertension, oliguria, hematuria, proteinuria, casts
- HUS, Poststrep glomerulonephritis in peds
- IgA nephropathy, Crescentic glomerulonephritis, Membranoproliferative glomerulonephritis in adults
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Membranous nephropathy
- common cause of nephrotic syndrome (edema, proteinuria, and hypoalbuminemia) in adolescents and adults
- active hepatitis B infection is an important risk factor, and vaccination reduces the risk
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FSGS
- common cause of nephrotic syndrome in adults and adolescents
- HIV is the most commonly associated infection
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Membranoproliferative glomerulonephritis
-can cause Nephrotic syndrome or nephritic syndrome, most commonly in adults, and has been associated with hepatitis B infection. However, it is significantly less common than membranous nephropathy in hepatitis B infection
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Minimal change disease
-most common cause of nephrotic syndrome in preadolescent children
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Poststreptococcal glomerulonephritis
-most common cause of nephritic syndrome in children
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Whooping cough
- aka 100 days cough
- Bordetella pertussis (gram negative coccobacillus)
- 3 phases: catarrhal, paroxysmal, and convalescent
- the paroxysmal phase is characterized by severe paroxysms of coughing and posttussive emesis
- Macrolide antibiotics are the first-line treatment
- may have lymphocytosis on CBC
- infants
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Adenovirus
-common URI in children and is often accompanied by fever, pharyngitis, rhinitis, conjunctivitis, and diarrhea
172
Chlamydophila pneumoniae and Mycoplasma pneumoniae
- causes of ATYPICAL pneumonias, which present w/ a gradual onset of symptoms, mild cough, and low-grade fevers
173
Mycobacterium TB
-causes TB, a serious illness that presents w/ prolonged fever, night sweats, chronic cough, and weight loss
174
Nontypeable Haemophilus influenza
-common cause of community-acquired pneumonia
175
Parainfluenza
- very common cause of laryngotracheobronchitis (croup) in children
- croup presents w/ fever, a "barky" cough, and inspiratory stridor
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Respiratory syncytial virus
-most common cause of bronchiolitis in children age
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Streptococcus pneumoniae
-most common cause of community-acquired pneumonia in children and presents w/ fever, cough, and tachypnea
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Acute Iron Poisoning
- disrupts basic cell processes from free radical production and lipid peroxidation
- abdominal pain, hematemesis, shock and metabolic acidosis
- commonly occurs in children of pregnant women taking pre-natal vitamins because children often confuse brightly colored iron pills for candy
- tx of iron poisoning involves DEFEROXAMINE, which binds ferric iron, allowing urinary excretion
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antidote to TCA and aspirin overdose
Sodium Bicarbonate
180
antidote to Torsades de Points induced by a prolonged QT interval?
Magnesium sulfate
181
Tx for lithium toxicity?
Hemodialysis (most dialyzable toxin)
182
tx for moderate to severe lead poisoning?
- Calcium EDTA (a lead chelator)
| - children present w/ irritability, poor appetite, headaches, abdominal pain and anemia
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tx for hypercalemia?
Calcium gluconate (cardio-protective)
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Tx for mild to moderate lead poisoning?
-Oral succimer (chelating agent)
185
tx for acetaminophen toxicity?
N-Acetylcysteine
186
Osgood-Schlatter disease
- traction apophysitis of the tibial tubercle
- Radiographic findings include anterior soft tissue swelling, lifting of tubercle from the shaft, and irregularity or fragmentation of the tubercle
- common in adolescent male athletes
- worsened by sports that involve repetitive running, jumping, or kneeling, and it improves with rest
- 1/4 have bilateral disease
- PE: edema and tenderness over the tibial tubercle
- pain can be reproduced by extending the knee against resistance
- tx is activity restriction, stretching exercises, and NSAIDs
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Prepatellar bursitis
- occurs w/ chronic irritation of the anterior knee
| - symptoms include pain w/ direct pressure and superficial swelling over the patella
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Patellar tendonitis
- overuse syndrome resulting from repetitive jumping or kicking
- pts present w/ anterior knee pain after exercise
- pts have point tenderness at the inferior pole of the patella
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Tibial osteomyelitis
- bone infection, usually bacterial in origin
- sx include pain, swelling, tenderness, and erythema
- pts classically present w/ refusal to bear weight on the affected extremity. systemic symptoms may also be present
- the pain from osteomyelitis does not remit with rest
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Patellofemoral stress syndrome
- overuse injury commonly seen in runners
- pts present w/ anterior knee pain that worsens upon descending steps or hills
- pain is localized to the patella and radiographs do not demonstrate separation at the tibial tubercle
191
Intussusception
- risk factors: recent viral illness or rotavirus vaccine
- pathological lead points: Meckel's diverticulum, Henoch-Schonlein purpura, Celiac disease, Intestinal tumor, Polyps
- Presentation: episodic, crampy abdominal pain; currant jelly stools, sausage shaped abdominal mass
- diagnosis: "target sign" on US
- Tx: enema (air or water soluble contrast), surgical removal of lead point if present
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Recurrent intussusception
- a pathological lead point should be suspected in older children w/ recurrent intussusception
- Meckel's diverticulum is the most common anomaly of the GI tract and can be asymptomatic between episodes of telescoping
- Surgical resection is required to treat and prevent complications of obstruction
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Inspissated stool in cystic fibrosis may lead to intussusception.
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Henoch-Schonlein purpura
- increases risk of intussusception due to small-bowel hematoma formation from the autoimmune vasculitis
- palpable purpura and joint point are usually the first manifestations
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Hereditary angioedema
- rapid-onset edema of the face, acral extremities, genitals, trachea, and abdominal organs without urticaria
- due to a deficiency or dysfunction in C1 inhibitor, which results in elevated levels of the edema-producing factors C2b and bradykinin
196
Antibody-mediated (type 2) hypersensitivity
- can be seen in immune hemolytic anemia and Rh hemolytic disease of the newborn
- involves specific rxns of IgG or IgM antibodies w/ cell bound antigens leading to complement activation and cell destruction
- note that type 1 (immediate hypersensitivity) also involve the antibody IgE but result in mast cell and basophil degranulation w/ associated urticaria and pruritis
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Immune complex mediated (type 3) hypersensitivity
- can be seen in serum sickness
- antibodies form complexes w/ allergens that are present in tissues, leading to activation of the complement cascade wherever the immune complexes deposit
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cell-mediated (type 4) hypersensitivity
- common examples are the TB skin test and allergic contact dermatitis
- in allergic contact dermatitis, the allergen causes dermal inflammation following direct contact w/ the skin after a latent period of 1-2 days
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Low C1q levels
-associated w/ acquired angioedema, which usually presents in patients age > 30 (most common cause is ACE-inhibitor use)
200
Circumstances in which minors do not require consent
- Medical: emergency care, STI's, substance abuse, Prenatal care
- Emancipated minor: Homeless, Parent, Married, Military, Financially independent, High school graduate
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In general, parents who are themselves minors can give consent for medical treatment of their child. However, challenges in obtaining consent should not delay potentially life-saving, emergent procedures such as intubation for respiratory failure or emergent surgery.
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Homocystinuria
- autosomal recessive disorder caused by CYSTATHIONINE SYNTHASE DEFICIENCY
- in addition to Marfanoid body habitus, pts w/ homocystinuria also have intellectual disability, downward lens dislocation, and hypercoagulability
- tx involves vitamin B6, folate, and vitamin B12 to lower homocysteine levels, as well as antiplatelet or anticoagulation to prevent thromboembolic events
- fair hair and eyes, developmental delay, and cerebrovascular accident are virtually pathognomic for homocystinuria
203
Ehlers-Danlos syndrome
- collagen disorder
| - scoliosis, joint laxity, and skin hyperelasticity
204
Fabry disease (a-galactosidase deficiency)
- angiokeratomas, peripheral neuropathy, and asymptomatic corneal dystrophy
- pts may also develop renal and heart failure and are at risk for thromboembolic events
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Krabbe disease
- autosomal recessive lysosomal storage disorder caused by galactocerebrosidase deficiency
- intellectual disability, blindness, deafness, paralysis, neuropathy, and seizures
206
PKU
- inborn error of metabolism that results from a deficiency of phenylalanine hydroxylase
- intellectual disability, fair complexion, eczema, and a musty body odor
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Tay-Sachs disease
- autosomal recessive GANGLIOSIDOSIS caused by a deficiency of B-hexosaminidase A
- intellectual disability, weakness, seizures, and presence of a cherry-red macula on exam
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Theophylline
- methylxanthine that may be used to treat asthma
- has numerous side effects, must be given IV, and levels must be closely monitored
- NOT indicated in the tx of acute status asthmaticus
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Tracheostomy
- surgical procedure performed to relieve upper airway obstruction
- almost always an elective procedure and is never done to treat asthma, which is a disease process of the lower airways
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Racemic epinephrine
-should always be tried prior to endotracheal intubation in pts w/ CROUP
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Endotracheal intubation and mechanical ventilation are indicated in pts w/ severe asthma unresponsive to medications who have fatigue, altered mental status, CO2 retention, worsening hypoxemia, and poor air movement on exam
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Hemolytic-uremic syndrome
- clinical syndrome of hemolytic anemia, thrombocytopenia, and acute renal failure
- most often caused by Shiga-toxin mediated endothelial cell damage, after infection w/ E. coli O157:H7 or Shigella
- Tx is supportive; approx. 50% of children w/ HUS require dialysis
213
Henoch-Schonlein purpura
- presents w/ purpura on the legs and buttocks in the setting of a NORMAL platelet count
- often presents as joint and abdominal pain
214
Rocky mountain spotted fever (RMSF)
- can cause a vasculitis, including petechiae, anemia, thrombocytopenia, and hematuria
- children are almost invariably febrile
- tick borne infection
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Coarctation of the aorta
- hypertension in the upper part of the body and relative hypoperfusion in the lower part of the body
- a mild, continuous murmur heard all over the chest is due to the development of collaterals between the hypertensive and hypoperfused vessels
- rib notching, caused by the dilatation of the collateral chest wall vessels, is specific for coarctation
216
supracondylar humerus fracture
- Compartment syndrome is a potential complication; severe pain, pallor, poikilothermia, paresthesias, and the late findings of pulselessness and paralysis
- brachial artery injury is another concern; monitor pulses frequently
- tx of compartment syndrome includes removal of any bandages, measurement of compartment pressures, and emergent orthopedic evaluation for possible fasciotomy
217
blue-grey macule on infant
-congenital dermal melanocytosis ("Mongolian spots"); benign birthmarks prevalent in asian babies
218
All neonates and infants should be screened for developmental dysplasia of the hip. How so?
- Barlow and Ortolani maneuvers
- a palpable clunk should prompt referral to an orthopedic surgeon
- a soft click, leg-length discrepancy, or asymmetric inguinal skin folds require diagnostic imaging with US (age 4-6 months)
- the tx of choice for age
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Clinical manifestations of neonatal sepsis (including meningitis)
-Present in > 50% of cases: Temperature instability (> 38C or
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Neonatal sepsis
- untreated neonatal sepsis can result in significant morbidity and mortality, and there should be a very low threshold for evaluation and empiric treatment
- clinical manifestations are often nonspecific and include temperature instability (low or high), poor feeding, lethargy, and jaundice
- preterm infants are more likely to present w/ hypothermia than with fever
- antibiotics should be administered immediately after blood cultures in ill-appearing infants without waiting for a lumbar puncture
- workup includes a CBC, BLOOD CULTURE, LUMBAR PUNCTURE, URINALYSIS, AND URINE CULTURE
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Procalcitonin
-acute phase reactant similar to C-reactive protein and is released in response to bacterial toxins; in contrast, levels are reduced in viral infection
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Breast milk jaundice
- caused by a factor in human breast milk, and results from increased intestinal absorption of bilirubin and generally peaks around age 2 weeks
- even infants w/ breast milk jaundice should continue to be breastfed as hyperbilirubinemia from breast milk rarely requires intervention
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Wiskott-Aldrich syndrome
- X-linked disorder characterized by the triad of thrombocytopenia, eczema, and recurrent bacterial infections
- the thrombocytopenia is caused by decreased platelet production, and the few platelets that exist are typically quite small
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Childhood leukemias
- can cause anemia, leukopenia, and thrombocytopenia by infiltrating the bone marrow
- an abnormal leukocyte differential w/ a high percentage of blast cells would be seen on the lab studies in children w/ leukemia
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Idiopathic thrombocytopenic purpura (ITP)
- immune-mediated disorder caused by antibodies that bind to platelets and facilitate their removal from the circulation
- affected children typically present w/ easy bruisability or bleeding
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Thrombocytopenia as a result of increased activation, aggregation, and consumption can occur in pts w/ DIC, TTP, or HUS
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Measles (rubeola)
- presentation: prodrome- fever, malaise, anorexia, CONJUNCTIVITIS, CORYZA, COUGH, KOPLIK SPOTS; exanthem- blanching, reddish-brown maculopapular rash, cephalocaudal and centrifugal spread, usually spares palms/soles
- diagnosis: PCR, acute and convalescent serology for anti-measles IgM and IgG
- Prevention: live attenuated measles vaccine
- Tx: supportive care, VITAMIN A for hospitalized children
- Complications: otitis media, pneumonia, encephalitis (within days), acute disseminated encephalomyelitis (within weeks), subacute sclerosing panencephalitis (within years), gastroenteritis
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Measles
- prodrome of Cough, Coryza, Conjunctivitis, and Koplik spots followed by a maculopapular rash that spreads in a cranial-caudal pattern
- the disease affects only humans and is transmitted by respiratory droplets that can remain airborne for hours
- prevention is best achieved by vaccination with the live attenuated measles vaccine
- people are most contagious when they have coryza and cough, but can spread disease from 5 days before the appearance of the rash to 4 days after the rash resolves
- manifests 1-3 weeks after inhalation of respiratory droplets
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Medulloblastoma
- second most common infratentorial tumor in children (after cerebellar astrocytoma) and arises from the cerebellar vermis
- recognize posterior vermis syndrome (truncal dystaxia)
- it is highly radiosensitive and can metastasize through the CSF tract
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Cerebellar hemispheric syndrome
- consists of arm, leg, and gait dystaxia, with ipsilateral cerebellar signs
- cerebellar hemispheres are the site of astrocytoma and cerebellar abscess rather than medulloblastoma
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peripheral smear of sickle cell anemia
- sickled red cells, polychromasia (due to reticulocytosis), and Howell-Jolly bodies (due to splenic infarction)
- acute pain crises due to vasoocclusion and ischemia are the most common manifestations of sickle cell anemia
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sickle cell anemia
- pathophys: autosomal recessive inheritance of B globin mutation in blacks and hispanics
- features: hemolytic anemia, dactylitis, acute vasoocclusive pain crises
- labs: decrease hematocrit, increased reticulocytes, serum LDL, and unconjugated bilirubin; peripheral smear shows sickled red cells, Howell-Jolly bodies
- management: maintenance- vaccination, penicillin until age 5, folic acid, hydroxyurea; acute pain crises- hydration, analgesia, +/- transfusion
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radial head subluxation (nursemaid's elbow)
- common in preschool children
- the classic mechanism is swinging or pulling a child by the arm
- full recovery after closed reduction by forearm hyperpronation confirms the diagnosis
- arm is classically held EXTENDED AND PRONATED and there is no swelling, deformity, or focal tenderness
- tx: supination of forearm and flexion of elbow, OR hyperpronation of forearm (preferred); no post-reduction films are needed when the pt resumes full use of extremity
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in patients with apparent subcutaneous emphysema secondary to severe coughing paroxysms, chest x-rays must be obtained first to rule out pneumothorax!
-sever coughing paroxysms may result in subcutaneous emphysema, wherein air leaks from the chest wall due to high intraalveolar pressure provoked by the cough; by a similar process, pneumothorax can occur
235
Remember to always evaluate precocious puberty (girls
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Gonadotropin-dependent (central) precocious puberty
- characterized by premature adrenarche, premature thelarche, and advanced bone age
- LH is elevated at baseline or after stimulation w/ GnRH agonist
- tx consists of GnRH agonist therapy to maximize adult height
- GnRH agonist therapy should NOT be initiated until after MRI has been performed, as idiopathic GDPP is a diagnosis of exclusion. Surgical resection would be the appropriate tx for certain tumors.
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Danazol
- testosterone derivative w/ progestin-like effects
| - used for the treatment of endometriosis
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Pts w/ gonadotropin-independent (peripheral) precocious puberty
- have low basal LH levels with no response to GnRH
- US of the adrenal glands, ovaries, and testes should be performed to look for a peripheral source of excessive sex hormone secretion
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Slipped capital femoral epiphysis
- common hip disorder seen in OVERWEIGHT adolescents age 10-16
- urgent surgical fixation is required to prevent avascular necrosis of the hip
- additional risk factors: endocrinopathies (hypothyroidism, GH deficiency), renal failure, and radiation hx
- always check for BILATERAL disease
- insidious onset of dull hip or referred knee pain and altered gait w/ no preceding trauma
- pts tend to hold the affected hip in passive external rotation, and exhibit decreased internal rotation, abduction, and flexion
- gold standard for tx is IMMEDIATE SURGICAL SCREW FIXATION
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Developmental hip dysplasia
- caused by abnormal development of the hip in utero
| - leg length discrepancy is present and radiographs show a poorly formed femoral head
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Legg-Calve-Perthes disease
-idiopathic AVN of the hip that most commonly affects boys age 5-7 years
242
Anemia in sickle cell disease
- suffer from chronic extravascular and intravascular hemolysis
- lab findings include mild to moderate anemia w/ reticulocytosis and unconjugated hyperbilirubinemia, increased LDH, decreased haptoglobin
- an acute worsening of anemia can occur from splenic sequestration, which manifests as splenomegaly and increasing fatigue
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Respiratory distress syndrome
- should be suspected when a premature infant presents w/ grunting, flaring, and retractions immediately after birth
- CXR includes characteristic fine reticular granularity of the lungs
- tx includes early CONTINUOUS POSITIVE AIR PRESSURE VENTILATION
- due to surfactant deficiency in most cases of preterm infants
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Congenital diaphragmatic hernia
- loops of bowel in the hemithorax, a displaced cardiac silhouette, and a gasless abdomen
- pts worsen w/ noninvasive positive-pressure ventilation as air pumped into the GI tract can compress the lungs and should be intubated instead
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Meconium aspiration syndrome
- occurs in term or post-term infants born through meconium stained fluid
- meconium obstructs the airways and causes respiratory distress
- x-ray would show patchy infiltrates, coarse streaking of both lung fields, and flattening of the diaphragm
246
Persistent pulmonary hypertension of the newborn
- should be suspected in all term and post-term neonates w/ cyanosis
- high pulmonary vascular resistance results in right-to-left shunting of deoxygenated blood through the foramen ovale and ductus arteriosus, resulting in hypoxia
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Transient tachypnea
-most common cause of respiratory distress in full-term infants and has no long-term sequelae
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neonatal displaced clavicular fracture
- babies who are large for gestational age are at increased risk for birth injuries such as clavicular fractures
- most clavicular fractures heal rapidly without complications
- risk factors: fetal macrosomia (maternal diabetes, post-term pregnancy), instrumental delivery (vacuum or forceps), shoulder dystocia
- features: crying/pain w/ passive motion of affected extremity, crepitus over clavicle, asymmetric Moro reflex
- diagnosis: x-ray
- tx: reassurance, gentle handling, analgesics (NOT aspirin), place affected arm in a long-sleeved garment and pin sleeve to chest w/ elbow flexed at 90 degrees
- heals in 7-10 days
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Newborns w/ DiGeorge syndrome
- must be assessed immediately for potentially life-threatening hypocalcemia
- the diagnosis should be suspected in the setting of craniofacial anomalies, thymic hypoplasia, and congenital heart disease
- CATCH-22 (conotruncal cardiac defects, abnormal facies, thymic hypoplasia, cleft palate, hypocalcemia, chromosome 22)
250
Todd's paralysis
- represents a postictal condition that usually rapidly improves w/ restoration of motor function within 24 hours
- may follow generalized as well as focal seizures
- usually indicates that a structural abnormality underlying the seizure is present
251
Cholesteatomas
- in children can either be congenital or acquired secondary to chronic middle ear disease
- new-onset hearing loss or chronic ear drainage despite antibiotic therapy are typical presenting symptoms of cholesteatomas, and granulation tissue and skin debris may be seen within retraction pockets of the tympanic membrane on otoscopy
- complications: hearing loss, cranial nerve palsies, vertigo, brain abscesses or meningitis
- refer to ENT for dedicated otologic exam
252
Meniere's disease
-accumulation of fluid in the inner ear that leads to hearing loss, vertigo, and tinnitus
253
Craniopharyngioma
- tumor that can occur in children
| - derived from Rathke's pouch, which is in the suprasellar space
254
Otosclerosis
-condition in which there is bony overgrowth of the stapes footplate that results in conductive hearing loss
255
osteoma
-benign, solitary area of bony overgrowth that can form in the outer ear and lead to heard loss
256
Refeeding syndrome
- potentially fatal complication of nutritional rehabilitation in anorexia nervosa
- carb intake stimultes INSULIN activity, which in turn promotes cellular uptake of phosphorus, potassium, and magnesium
- clinical manifestations include arrhythmias and cardiopulmonary failure
- phosphorus is the primary deficient electrolyte as it is required for energy (ATP)
- potassium and magnesium deficiency can cause arrhythmias
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muscle degeneration in the muscular dystrophies releases muscle enzymes into the blood, resulting in markedly elevated serum creatine phosphokinase and aldolase
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Myotonic dystrophy
- autosomal dominant disease that generally presents in the teenage yrs w/ muscle weakness, myotonia, cataracts, and cardiac conduction abnormalities
- the muscular groups that are most affected include the facial muscles, intrinsic hand muscles, and ankle dorsiflexors
- myotonia, or delayed muscle relaxatioin, is a prominent feature of the disease and manifests as a myoTONIC pattern on EMG; in contrast, a myoPATHIC pattern would be seen in DMD and BMD.
259
Becker's vs Duchenne muscular dystrophy
- BMD is a similar but milder version of DMD, in which the dystrophin gene deletion preserves the reading frame for dystrophin, resulting in decreased (but not absent) dystrophin and muscle weakness later in childhood
- DMD should be suspected in a boy age
260
Neuroblastoma
- 3rd most common cancer in the pediatric population (after leukemia and CNS tumors)
- the tumor arises from neural crest cells, which are also the precursor cells of the sympathetic chains and adrenal medulla
- the most common site is the abdomen
- calcifications and hemorrhages are seen on plain x-ray and CT scan
- the levels of serum and urine catecholamines and their metabolites (HVA and VMA) are usually elevated
261
embryologic precursor of the renal parenchyma
-Metanephros
262
embryologic structure that gives rise to the seminal vesicles, epididymis, ejaculatory ducts, and ductus deferens?
-MeSonephros (S = sperm)
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Paramesonephron gives rise to what?
-fallopian tubes, uterus, and part of the vagina
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Strabismus
- strabismus after age 4 months is usually abnormal and requires treatment to prevent amblyopia
- Asymmetric corneal light reflections and deviation on cover test are concerning findings
- the standard tx is occlusion (patching) or penalization (blurring) of the normal eye
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New onset of strabismus
- can be a sign of retinoblastoma if accompanied by a white eye reflex
- acute onset can also result from intracranial hemorrhage, brain abscess, or encephalitis
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Hand-foot syndrome or dactylitis
- the earliest manifestation of vaso-occlusion in sickle cell anemia, thereby warranting a complete workup for previously asymptomatic sickle cell patients
- usually present between 6 months - 2 yrs of age w/ an acute onset of pain and symmetric swelling of the hands and feet
- pathophys involves vascular necrosis of metacarpals and metatarsals
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mutation in sickle cell disease
-point mutation which results in the substitution of VALINE for GLUTAMIC ACID on the surface of the HbS molecule in the sixth codon of the beta globin chain
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Impetigo
- initially presents as papules which rapidly become pustules that rupture into honey-colored crusted exudates
- Staphylococcus aureus and Streptococcus pyogenes are the most common causes of this contagious infection
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Chickenpox
- starts as a pruritic vesicular rash that eventually ruptures to leave scabs
- usually, several stages of lesions are present simultaneously throughout the body
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Eczema herpeticum
- a superinfection of HSV in areas of severe eczema
| - the rash can progress rapidly and is accompanied by fever
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Erysipelas
- begins as a small erythematous patch that progresses to a red, indurated, tense, and shiny plaque
- the presence of a raised, sharply demarcated margin is a classic feature
- overlying skin streaking and regional lymphadenopathy indicate lymphatic involvement
- these pts usually have fever and chills
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Hand-foot-mouth disease
- characterized by vesicles on the posterior oropharynx, hands, and/or feet
- the rash is not typically present on the face and does not crust
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Fibromuscular dysplasia
- can present as new onset hypertension in children
- bruit or venous hum may be heard at the costovertebral angle
- angiogram reveals the "string of beads" sign
- it is the most common cause of secondary hypertension in children
- the right renal artery is more affected than the left
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Conn's syndrome
- due to an aldosterone secreting adenoma or bilateral adrenal hyperplasia
- usually presents in older pts as hypokalemia and hypertension
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Primary amenorrhea evaluation
- defined as absence of menarche by age 15 yrs
- first do a pelvic exam or US to determine if uterus is present or absent
- if uterus is PRESENT, get a serum FSH; if FSH increased, get a karyotype; if FSH decreased, get a cranial MRI
- if uterus is ABSENT, get a karyotype and serum testosterone
- Turner syndrome is one of the most common causes of primary amenorrhea
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Primary amenorhea, delayed puberty, short stature, and bicuspid aortic valve
- strongly suggestive of Turner syndrome
- one of the most common causes of primary amenorrhea
- Pelvic US should be performed to evaluate internal female anatomy
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Erb-Duchenne palsy
- excessive traction on the neck during a difficult delivery can result in this and the characteristic "waiter's tip" posture
- fortunately, most infants recover arm function spontaneously within a few months
- african american boys are at increased risk for fetal macrosomia, which in turn is a risk factor for shoulder dystocia and brachial plexus injury
- involves C5, C6, and sometime C7
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Cephalohematoma
- subperiosteal scalp swelling that can occur in any delivery but is more common in the setting of instrumental delivery
- usually resolves spontaneously w/o any sequelae
- on the other hand, intracranial hemorrhage is a potential complication of vacuum use that can cause upper motor neuron injury leading to decreased arm movement, hyperreflexia, and hypertonia
279
Vitamin K deficiency in newborns
- the body derives vitamin K from the diet and from gut flora synthesis
- deficiency in newborns is the result of poor placental transfer, absent gut flora, immature liver function, and inadequate levels in breast milk
- all newborns should receive a vitamin K injection to prevent vitamin K deficient bleeding (ie intracranial hemorrhage)
- severe vitamin K deficiency typically causes prolonged PT and PTT, but mild deficiency may present w/ a prolonged PT and a normal PTT
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pts w/ impaired platelet function present w/ mucocutaneous bleeding and petechiae in the setting of normal PT, aPTT, and platelet count. Some causes include medications (aspirin, clopidogrel), metabolic problems (uremia, liver disease), and congenital disorders (Glanzmann thrombasthenia).
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affected individuals w/ von Willebrand disease will have a normal PT interval but may have a prolonged aPTT due to decreased factor VIII activity
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Osteosarcoma
- most common primary bone tumor in children and young adults and typically involves the metaphyses of long bones (distal femur, proximal tibia, proximal humerus)
- physical exam typically shows a large and tender mass
- classic x-ray findings include "sunburst" periosteal reaction and Codman triangle
- constitutional symptoms are usually absent
283
Ewing sarcoma
- second most common primary bone malignancy
- incidence is highest in adolescent males and up to 20% may have systemic symptoms
- x-ray shows an OSTEOLYTIC lesion w/ a periosteal reaction that produces layers of reactive bone, known as "ONION SKIN" appearance
284
Osteoid osteoma
- appears as a sclerotic, cortical lesion on imaging w/ a central nidus of lucency
- typically causes pain that is worse at night and unrelated to activity
- pain is quickly relieved by NSAID medications
285
Absence (petit mal) seizures
- suspect absence seizures in a 4 to 8 year old child w/ frequent daydreaming episodes, brief staring spells, decline in school performance, and no post-ictal phase
- the preferred tx is ETHOSUXIMIDE or VALPROATE
- classic EEG pattern is generalized, symmetrical 3-Hz spike and wave activity on a normal background (this finding is provoked by hyperventilation of the patient during EEG)
286
APGAR score
- helpful in assessing the status of a neonate and response to neonatal resuscitation
- routinely measured at 1 and 5 minutes of life
- scores of
287
Rabies
- transmission of neurotropic virus typically involves exposure to infected saliva; rare cases may be from contact w/ mucous membranes, infected aerosol particles, and corneal and organ transplantations
- raccoons are the most prevalent rabid animal in the US, especially on east coast
- post-exposure prophylaxis can be life-saving and should be initiated after a bite wound from a wild carnivore; IVIG and rabies vaccine
- hydrophobia and aerophobia are pathognomonic for encephalitic rabies, and affected pts typically die within weeks of illness onset
- ascending flaccid paralysis
- almost all pts die within weeks
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Epiglottitis
- life-threatening cause of airway obstruction that is most commonly caused by bacterial infection
- the first priority is to secure the patient's airway with endotracheal intubation in a controlled environment (eg operating room)!
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epiglottitis
- organisms: H. flu type B, nontypable H. flu, Strep, Staph
- presentation: Distress ("tripod" positioning, inspiratory stridor), Dysphagia, Drooling
- x-ray findings: "Thumbprint" sign (enlarged epiglottis), loss of vallecular space
- management: keep child calm and comfortable, emergency ENDOTRACHEAL INTUBATION in the operating room
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Cricothyroidotomy in children
- should be avoided in pre-pubescent children
| - the upper portion of the trachea is incompletely developed and children age
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Croup
- viral laryngotracheobronchitis
- nebulized racemic epinephrine can provide relief
- barky cough and inspiratory stridor
- "steeple" sign
292
Laryngomalacia
-inspiratory stridor that WORSENS IN THE SUPINE POSITION and w/ crying and feeding 2
and peaks at age 4-8 MONTHS
-diagnosis is confirmed w/ flexible laryngoscopy, which shows collapse of the supraglottic structures during inspiration and OMEGA-shaped epiglottis
-self-resolves by age 18 months
-increased laxity of supraglottic structures
-reassurance for most cases; supraglottoplasty for severe symptoms (poor weight gain, apnea, cyanosis, rapidly worsening stridor)
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common causes of pediatric stridor
-croup, foreign body aspiration, anatomic abnormalities such as laryngomalacia or vascular rings
294
Vascular rings
- BIPHASIC STRIDOR and feeding difficulties if an anomalous branch of the aortic arch encircles the trachea and esophagus
- workup consists of screening w/ barium swallow followed by confirmation w/ MRI w/ angiography
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Spirometry is used to diagnose VOCAL CORD PARALYSIS and ASTHMA in children age > 6 yrs.
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Prevertebral soft-tissue thickening
-retropharyngeal abscess
297
subglottic edema
-croup
298
All sexually active women age
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Neonatal tetanus
- generally seen in infants born to unimmunized mothers, frequently following umbilical stump infection
- affected infants initially present in the first two weeks of life w/ poor suckling and fatigue, followed by rigidity, spasms and opisthotonus
300
Fanconi anemia
-autosomal recessive disorder that causes congenital marrow failure, poor growth, morphologic abnormalities, and usually macrocytic anemia
301
recurrent intussusception
- a pathological lead point should be suspected in older children w/ recurrent intussusception
- Meckel's diverticulum is the most common congenital anomaly of the GI tract and can be asymptomatic between episodes of telescoping
- Surgical resection is required to treat and prevent complications of obstruction
- most cases of intussusception (~75%) occur before age 2 following a viral illness and have no identifiable lead point
302
most common cause of nephrotic syndrome in pre-adolescent children?
- Minimal Change Disease (80%)
| - renal biopsy shows normal kidney architecture but is not routinely obtained in pts age
303
Focal segmental glomerulonephritis
- crescent formation is typical finding
- most common cause of nephrotic syndrome in adults
- may be rapidly progressive
304
Thickened basement membrane and subEPITHELIAL "spikes"
- Membranous glomerulonephritis
| - second most common cause of nephrotic syndrome in adults (behind FSGS)
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Masangial hypercellularity
- suggestive of membranoproliferative glomerulonephritis
- commonly presents w/ NEPHRITIC syndrome rather than nephrotic syndrome
- uncommon in young children
306
nephrotic syndrome
-edema, fatigue, abdominal pain (from rapid fluid accumulation), and proteinuria
307
Indications for renal and bladder US in children
-infants and children age
308
indications for voiding cystourethrogram
- can be considered if hydronephrosis or scarring is seen in renal US
- newborns age
309
Children age
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common cause of childhood stroke?
Sickle cell anemia
311
Electromyography (EMG)
- procedure that tests peripheral nerve function
| - can be helpful in distinguishing nerve disease from muscle disease
312
Neuroblastoma
- 3rd most common pediatric cancer after leukemia and brain tumors, but the most common cancer in the FIRST YEAR OF LIFE
- can arise anywhere in the sympathetic nervous system but typically involves the adrenal glands and presents as an abdominal mass that CROSSES THE MIDLINE w/ systemic symptoms
313
Posterior urethral valves
- can cause bilateral obstructive uropathy and are usually diagnosed prenatally
- boys who present later in life typically have frequent UTI and signs of renal failure
- associated hydronephrosis
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Renal cell carcinoma
- most common PRIMARY renal neoplasm
| - occurs predominantly in men age 50-80 years and is rare in children
315
Wilms tumor (nephroblastoma)
- most common pediatric renal malignancy
- should be suspected in a toddler w/ a firm, smooth, unilateral abdominal mass and hematuria
- fourth most common childhood cancer
- peak age 2-5 years
- does NOT CROSS THE MIDLINE
- very high 5 year survival rate
316
Biliary atresia
- newborns w/ conjugated (direct) hyperbilirubinemia and hepatomegaly should be evaluated for biliary atresia
- the first step in evaluation is abdominal US, which may show an absent or abnormal gallbladder
- early tx w/ a Kasai procedure (hepatoportoenterostomy) dramatically improves outcomes
317
Breast milk jaundice
- generally appears in the second week of life
- INDIRECT hyperbilirubinemia that may rise as high as s10-30 mg/dL
- generally is benign, but in rare cases phototherapy may be indicated
318
Crigler-Najjar and Gilbert's syndrome
- inherited deficienies of UDP-glucuronyl transferase that result in UNCONJUGATED hyperbilirubinemia
- in Gilbert's, the deficiency is mild and pts are asymptomatic or have mild jaundice particularly during times of stress
- in Crigler-Najjar, the enzyme is ABSENT and so pts present early in life and require liver transplant
319
Erythroblastosis fetalis
- unconjugated hyperbilirubinemia and Coombs-positive hemolytic anemia
- caused by mismatch between infant and maternal blood types (Rh disease, ABO incompatibility, minor blood group antigens)
320
Physiologic jaundice
- UNCONJUGATED hyperbilirubinemia that appears after the first 24 hours of life and resolves within the first week
- remember that CONJUGATED hyperbilirubinemia is always pathologic
321
Primary humoral immune deficiency syndromes present w/ recurrent or severe sinopulmonary infections
- X-linked (bruton's) agammaglobulinemia
- Common variable immunodeficiency
- IgA deficiency
- Hyper IgM syndrome
- IgG subclass deficiency
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Hyper-IgM syndrome
- caused by a defect in the CD40 ligand (allows for class switching from IgM to others and formation of plasma cells)
- high IgM levels, low or absent IgG and IgA, and normal lymphocyte populations
323
Bruton agammaglobulinemia (X-linked)
- low IgG, IgM, and IgA
| - low or absent B-lymphocytes
324
Common variable immunodeficiency
- low levels of IgG, IgM, and IgA
| - NORMAL B-lymphocyte levels
325
Selective IgA deficiency
- relatively common
- most pts are asymptomatic but some have recurrent respiratory, GI, and UG infections
- IgA is low while IgM is normal
326
Selective IgG subclass deficiency
-recurrent infections, low total IgG, normal IgM
327
Transient hypogammaglobulinemia of infancy
- low serum IgG levels after age 6 months due to the waning maternal derived antibody from birth
- usually resolves by 12 months of age
- B and T cells and IgA and IgM levels are all normal
328
Cystic fibrosis
- autosomal recessive
- recurrent sinopulmonary infections, nasal polyps, digital clubbing
- bilateral nasal polyps exacerbate nasal obstruction and chronic rhinosinusitis
- sweat testing and QUANTITATIVE PILOCARPINE IONTOPHORESIS are gold standard for diagnosis
329
ALL
- most common cause of childhood cancer
- can present w/ numerous infections and weight loss
- hepatosplenomegaly, lymphadenopathy, and petechiae are often seen
330
Primary ciliary dyskinesia (immotile-cilia syndrome)
- autosomal recessive disorder of mucociliary clearance
| - recurrent sinopulmonary infections, nasal polyposis, and digital clubbing
331
Sturge-Weber syndrome
- neurocutaneous syndrome that is characterized by a congenital unilateral cavernous hemangioma along the trigeminal nerve distribution and radiographic evidence of intra-cranial calcifications that resemble a tramline
- focal or generalized seizures, mental retardation, port wine stain or nevus flammeus, hemianopia, hemiparesis, hemisensory disturbance, ipsilateral glaucoma
- tx is aimed at controlling the seizures and reducing IOP
- argon laser therapy is successful in removing skin lesions
332
Tuberous sclerosis
- initial presentation w/ seizure
| - cutaneous manifestation is called adenoma sebaceum, which appears between 5-10 years of age
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capillary hemangioma
- usually presents at birth or appears by 6 months of age
- small capillaries and presents as in irregular, raised lesion that blanches on pressure
- approximately 75% regress by age 7
334
Meningiomas
- non-invasive tumors derived from arachnoid cap cells, and consist of concentric whorls and calcified psammoma bodies
- these may indent the brain and cause hyperostosis
335
empty sella syndrome
- sella turcica is often enlarged and contains no discernible pituitary gland
- syndrome may be primarily due to an incompetent sellar diaphragm w/ compression of the pituitary gland by the herniating arachnoid, or secondary to surgery or radiotherapy
336
ependymomas are gliomas and are benign.
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a young boy w/ symptoms of increased intracranial pressure, bitemporal hemianopsia, and a calcified lesion above the sella?
- Craniopharyngioma
- presence of a cystic calcified parasellar lesion on MRI is almost diagnostic
- tx of choice is surgical removal
338
Serum creatinine phosphokinase and aldolase levels are elevated in screening for muscular dystrophies. Fibrosis and fatty infiltration on calf muscle biopsy support the diagnosis. Genetic studies are the gold standard for confirmation.
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contraindications to rotavirus vaccine
- anaphylaxis to vaccine ingredients
- hx of intussusception
- hx of uncorrected congenital malformation of the GI tract (eg, Meckel's diverticulum)
- SCID
340
The administration of multiple vaccinations in a single office visit is safe and increases vaccine compliance and optimal protection at a young age. The exception is live-virus vaccines, which should be administered 4 weeks apart due to a possible interference of immune response.
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Live virus vaccinations can be safely administered to household contacts of pregnant women because the virus is weak and not contagious. Vaccination of household contacts reduces the potential spread of infection to pts who cannot receive vaccinations.
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Physiologic jaundice of the newborn
-total bilirubin
343
Breastfeeding jaundice
- "lack of breast milk jaundice"
- happens early in first week of life when the milk supply is low, resulting in limited enteral intake
- low intake causes decreased GI motility and in turn retention of meconium
- the B-glucuronidase in meconium deconjugates bilirubin and the unconjugated bilirubin is reabsorbed via the enterohepatic circulation, causing elevated serum levels
- improves once milk let-down occurs
- often difficult to distinguish from that of physiologic jaundice
344
Breast-milk jaundice
- begins in first 4 to 7 days of life, but may not peak until about 10-14 days
- NOT the result of low breast milk volume
- possibly due to B-glucuronidase in breast milk deconjugating bilirubin in GI tract, allowing for increased reabsorption via enterohepatic circulation
- can persist for up to 12 weeks, but total bilirubin concentrations rarely, if ever, get to concerning levels
345
Biliary atresia
- healthy-appearing infant who develops jaundice, dark urine, and acholic (pale) stools between 3 and 6 weeks of age
- any infant who develops jaundice after 2 weeks of age must be evaluated w/ fractionated bilirubin
- tx is w/ Kasai procedure
346
Kernig's sign
-resistance to extension of the knee
347
Brudzinski's sign
-flexion of hip and knee in response to flexion of the neck by the examiner
348
Difference between "fever without source" and "fever of unknown origin"?
- FWS: time of presentation (2-3 days) after onset of fever, which there is not a clearly identified source
- FUO: 7-10 days after onset and workup has been negative and viral infections should have run their course. more likely to have unusual infections, malignancies, or collagen vascular disorders
349
Simple vs Complex seizures
-simple: more common, 15 minutes, more than once in a 24 hr period, focal
350
Causes of altered mental status
- A,E,I,O,U and TIPS
- Alcohol, ingested toxins
- Epilepsy, encephalitis, endocrine, electrolytes
- Infection, insulin
- Overdose, opiates, oxygen deprivation
- Uremia (renal failure)
- Trauma, temperature
- Insulin, infection
- Psychosis
- Stroke, shock, space occupying lesion
351
Acute iron poisoning
- abdominal pain, hematemesis, and metabolic acidosis
- iron is radiopaque, and ingested tablets can be seen on x-ray
- chelation therapy w/ deferoxamine should be provided for moderately to severely ill pts
352
acute aspirin poisoning
-tinnitus, fever, hyperpnea (respiratory alkalosis), and metabolic acidosis
353
acute overdose of vitamin A
- nausea, vomiting, and blurry vision
| - chronic toxicity result in increased intracranial pressure (pseudotumor cerebri)
354
meds used for tx of absence seizures
Ethosuximide and valproic acid
355
Lamotrigine
first line agent for mixed seizures
356
phenytoin and carbamazepine
-first line drugs for the tx of primary generalized tonic-clonic seizures or partial seizures w/ or w/o secondary generalization
357
Diamond-Blackfan syndrome
-macrocytic pure red aplasia associated w/ several congenital anomalies such as short stature, webbed neck, cleft lip, shielded chest and triphalangeal thumbs
358
Wiskott-Aldrich syndrome
-X linked disorder w/ eczema, thrombocytopenia, and hypogammaglobulinemia
359
Fanconi's anemia
- autosomal recessive
- progressive pancytopenia and macrocytosis
- 8 yrs average age at diagnosis
- cafe-au-lait spots, microcephaly, microphthalmia, short stature, horseshoe kidneys and absent thumbs
360
Celiac disease
frequent cause of malabsorption and can lead to iron deficiency anemia
-common associations w/ celiac disease include type 1 DM and dermatitis herpetiformis
361
Enterobius vermicularis
- infection is highly contagious and manifests as nocturnal perianal pruritis
- ALBENDAZOLE and PYRANTEL PAMOATE are first-line tx options (the latter is best for pregnant women)
- "pinworms"
362
Chagas disease (Trypanosoma cruzi)
- primarily affects the heart (cardiomyopathy, right BBB) and GI tract (megacolon, megaesophagus)
- BENZNIDAZOLE is the first-line treatment
363
Strongyloidiasis
- IVERMECTIN is the first-line tx
- presents w/ urticaria, abdominal pain, and respiratory symptoms (dry cough, dyspnea, wheezing)
- Onchocerciasis "river blindness" causes ocular lesions and dermatitis and is also tx w/ ivermectin
- these are the worms that live in soil and penetrate the bottoms of your feet
364
Reye syndrome
- seen exclusively in children less than 15 years old who were tx w/ salicylates for a viral infection
- vomiting, agitation, irrational behavior, lethargy, stupor, restlessness
- hyperammonemia, prolonged PT, severe elevations in AST, ALT, and LDH
- microvesicular steatosis
365
Sunscreens are rated by the strength of their SPF. The amount of UVB radiation filtered by SPF 15, 30, and 50 sunscreens is 93%, 97%, and 98%, respectively. Sunscreens w/ SPF > 50 provide a neglibible increase in UV protection (eg, SPF 100 sunscreen filters 99% of UVB rays).
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366
Sun avoidance is the best form of photo-protection. Sunscreen w/ SPF 15-30 or higher should be applied 15-30 minutes prior to sun exposure to allow time for development of a protective film.
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367
Intraventricular hemorrhage
- common complication in premature and underweight neonates
- accumulation of blood in subarachnoid space can impair the ability of the arachnoid villi to absorb CSF, resulting in communicating hydrocephalus
- affected neonates are at risk for permanent neurodevelopmental problems and death
368
Hemophilic arthropathy
- delayed consequence of recurrent hemarthrosis (often seen in hemophilia A or B)
- associated w/ hemosiderin deposition leading to synovitis and fibrosis within the joint
- risk can be significantly reduced by prophylactic tx w/ factor concentrates
369
idopathic avascular necrosis of the femoral head
- Legg-Calve-Perthes disease
- limp or hip pain and is most common in children age 4-12
- note that sickle cell can also cause avascular necrosis of femoral head
370
Allergic contact dermatitis
- erythema, edema, pruritis, tiny vesicles and weepy or crusted lesions 24-48 hours after contact w/ the allergen
- due to CELL-MEDIATED (DELAYED, TYPE IV) HYPERSENSITIVITY
- remember that type IV requires the host be sensitized before a reaction will develop, so a pt must have had prior exposure to the allergen before developing a reaction
371
type I hypersensitivity
- IgE-mediated
- occurs when an allergen binds and cross-links two IgE molecules attached to the mast cell
- Atopy, urticaria, and anaphylaxis are examples
- immediate
372
type II hypersensitivity
- antibody-mediated hypersensitivity
- CYTOTOXIC reactions involve specific reactions of IgG or IgM to cell-bound antigens. The antibodies then activate complement resulting in cell damage
- examples are immune hemolytic anemia and Rh hemolytic disease in the newborn
373
type III hypersensitivity
- immune complex-mediated hypersensitivity
- antibodies of IgG or IgM form complexes w/ antigens and nonspecifically active the complement cascade and other inflammatory processes
- examples are serum sickness and the Arthus reaction
374
6 month developmental milestones
gross motor: sits momentarily propped on hands (unsupported by 7 months)
fine motor: transfers objects hand to hand, raking grasp
language: responds to name, babbles
social/cognitive: stranger anxiety
375
12 month developmental milestones
gross motor: stands well, walks first steps independently, throws ball
fine motor: 2-finger pincer grasp
language: says first words
social/cognitive: separation anxiety, comes when called
376
By age 4 months, primitive reflexes (Moro, grasp) have either already disappeared, or are starting to disappear as the infant initiates purposeful movement of the extremities. The Babinski reflex is the only primitive reflex that may persist in healthy children up to age 2 years, but can disappear as early as age 12 months.
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377
what age does stranger anxiety develop?
6 months
378
what is the most common cause of bacterial pneumonia in young children with cystic fibrosis?
- Staph aureus (especially w/ coexisting influenza infection)
- pts w/ CF suffer from life-threatening, recurrent sinopulmonary infections due to accumulation of viscous airway secretions
- IV VANCOMYCIN is the drug of choice for severe pneumonia
- (switches from S. aureus to P. aeruginosa at about age 20)
379
antibiotics effective for Pseudomonas?
- Amikacin, ceftazidime, and ciprofloxacin
| - most common cause of pneumonia in ADULTS w/ CF, but is less common in young children
380
drug of choice for outpatient tx of community-acquired pneumonia for coverage of Strep pneumo?
High dose oral amoxicillin
381
what vitamin has been shown to reduce the morbidity and mortality rates of pts w/ measles?
Vitamin A
| -immune enhancement and helps GI and respiratory epithelium to regenerate
382
what lab values might you see in measles?
-Leukopenia (T-cell cytopenia) and thrombocytopenia
383
Lesch-Nyhan syndrome
- secondary to a deficiency in hypoxanthine-guanine phosphoribosyl transferase (HGPRT)
- self-mutilation, neurologic features (mental retardation, dystonia, choreoathetosis, spasticity), gouty arthritis, and tophus formation
- gout is usually seen in pts above 50 yrs of age; therefore, suspect Lesch-Nyhan if you see a BOY with gout
- X-linked recessive
- accumulation of uric acid due to enzyme deficieny involving purine metabolism; give ALLOPURINOL
384
A large thymic silhouette is a normal finding on frontal chest x-ray in children age
"Sail sign"
385
Cradle cap
- seborrheic dermatitis
- papular, scaly rash tends to affect the eyebrows, nasolabial folds, and scalp
- tx consists of moisturizers, antifungals, anti-dandruff shampoos, and topical steroids
386
Atopic dermatitis
- recurrent, pruritic, eczematous eruption that tends to affect flexural and extensor surfaces
- scaling red papules and plaques
387
Trisomy 18 (Edward's syndrome)
- micrognathia, microcephaly, rocker bottom feet, overlapping fingers, and absent palmar creases
- congenital heart disease occurs in > 50%; VENTRICULAR SEPTAL DEFECT IS THE MOST COMMON
- 90% die in first year of life, the rest are mentally retarded
388
heart disease in Trisomy 21
- Endocardial cushion defects
| - Atrial septal defects
389
heart disease in Williams' Syndrome
supravalvular aortic stenosis
390
heart disease in DiGeorge syndrome
-Conotruncal abnormalities (Truncus arteriosus, Tetralogy of Fallot, interrupted aortic arch) (CATCH-22 syndrome)
391
heart disease in neonatal lupus
Congenital heart block
392
Neonatal polycythemia
- defined as hematocrit >65% in term neonates
- risk factors: delayed cord clamping, maternal HTN, and maternal DM
- symptomatic neonates generally have a combination of respiratory distress, hypoglycemia, and neurologic manifestations
393
Tetralogy of Fallot
- varying degrees of cyanosis depending on the degree of RIGHT VENTRICULAR OUTFLOW TRACT OBSTRUCTION
- squatting decreases right-to-left ventricular shunting, which improves cyanosis and increases the intensity of systolic murmur due to increased blood flow across the right ventricular outflow tract
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D-transposition of great arteries is a CYANOTIC congenital heart disease
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395
Hypertrophic cardiomyopathy
- fatigue, dyspnea, chest pain, palpitations, or syncope; NO CYANOSIS
- systolic murmur that DECREASES in intensity with INCREASING AFTERLOAD (squatting, handgrip)
396
heart sound in atrial septal defect?
wide and fixed splitting of the second heart sound
397
What are the most common causes of viral meningitis?
the non-polio enteroviruses, such as echoviruses and coxsackieviruses
398
A type 1 collagen assay is used to diagnose osteogenesis imperfecta, or "brittle bone disease". Common findings include numerous fractures in multiple stages of healing, presence of blue sclerae, and short stature.
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399
Hemarthrosis after minor trauma in a young child is suspicious for what?
bleeding disorder
- screening should include coagulation studies and a CBC
- nonaccidental trauma should be suspected in children w/ injuries inconsistent w/ the hx or developmental stage
400
Bacterial meningitis in infants and children > 1 month
- lethargy, fever, poor oral intake, vomiting
- its CRUCIAL to administer empiric antibiotics (CEFTRIAXONE and VANCOMYCIN) FIRST, and then perform a lumbar puncture as soon as possible
- Imaging should be performed before the lumbar puncture in infants and children in coma, w/ focal neurologic findings, or w/ a hx of a neurosurgical procedure
- note that in neonates
401
Vaginal discharge in the newborn is due to the effects of maternal estrogens. In such cases, reassurance of the mother is all that is required. Female infants less than 3 months of age.
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402
Chlamydial infection in the newborn
-may cause pneumonia or conjunctivitis
403
Contraindications for further administration of pertussis-containing vaccines
- immediate anaphylaxis, unstable neurologic disorders, and encephalopathy within a week of administration of the DTaP vaccine
- immunization against pertussis should be provided in the absence of these conditions
404
Acetaminophen and ibuprofen can be administered after vaccination for fever or discomfort; they have NOT been shown to prevent fever or febrile seizures. In addition, prophylactic antipyretics may reduce antibody response to vaccines.
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405
Complex partial seizure
- brief episodes of impaired consciousness, failure to respond to various stimuli during the episode, staring spells, automatisms, and post-ictal confusion
- the EEG pattern is usually normal or may show brief discharges; unlike in absence seizures, hyperventilation during the EEG cannot simulate a complex partial seizure
406
typical absence seizure
- brief (lasting for a few seconds) periods of imparied consciousness
- pts may also have automatisms, but do NOT have a post-ictal state
- hyperventilation during the EEG reveals a gerneralized 3 Hz spike and wave pattern on a normal background
407
Lennox-Gastaut syndrome
-usually presents in childhood as seizures of multiple types, impaired cognitive function, and slow spike and wave activity on EEG
408
Henoch-Schonlein purpura
- IgA mediated vasculitis of the small vessels that is most common in children
- classic manifestations include palpable purpura, arthralgias, abdominal pain, and renal disease
- Immunofluorescence microscopy shows IgA deposition in the kidney
409
Minimal change disease
- most common form of idiopathic nephrotic syndrome in children and often presents w/ edema and hematuria
- EM findings include fusion or flattening of the podocytes ("foot processes")
410
Retropharyngeal abscess
-should be suspected in children who present w/ fever, dysphagia, inability to extend the neck, muffled voice, and lateral x-ray showing a widened prevertebral space
411
what does Diphtheria pharyngitis look like on exam?
Grey-colored pseudomembrane formation
412
Healthy infants normally lose up to 7% of birth weight in the first 5 days of life. No treatment is required, and exclusive breastfeeding should be continued. Birth weight should be regained by age 10-14 days. Pink discoloration in neonatal diaper is common and usually represents URIC ACID CRYSTALS, which is a NORMAL finding.
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413
Infants have immature kidneys that cannot reabsorb sodium effectively. Plain water should never be given to infants age
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414
Lyme arthritis
- most common late manifestation of untreated Lyme disease
| - the diagnosis should be suspected in pts w/ hx of travel to the northeastern or upper midwestern US
415
Reactive arthritis
- typically an acute and asymmetric oligoarthritis that occurs 1-4 weeks after urethritis or diarrheal infection
- malaise and fever can be present
416
Simple febrile seizures
- do NOT require any workup
| - pts can be discharged home from the ED w/ reassurance about a good prognosis
417
Brain imaging should be considered in a child w/ COMPLEX febrile seizures, with a persistently abnormal neuro exam, macrocephaly, or signs/symptoms of elevated intracranial pressure.
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418
Congenital hypothyroidism
- associated w/ neurodevelopmental injury if not recognized and treated early
- decreased activity, hoarse cry, and jaundice are commonly associated w/ congenital hypothyroidism, but the majority of infants are asymptomatic
- THYROID DYSGENESIS is the most common cause of congenital hypothyroidism worldwide
419
Routine neonatal ophthalmic prophylaxis w/ topical erythromycin is NOT effective against chlamydial conjunctivitis. Maternal prenatal testing and treatment is the only effective strategy for the prevention of this condition. Typically presents 5-14 days after birth w/ chemosis, mild eyelid swelling, and watery or mucopurulent discharge. Tx is w/ ORAL ERYTHROMYCIN for 14 days despite the risk of hypertrophic pyloric stenosis w/ oral erythromycin use.
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420
TOPICAL erythromycin ointment is only effective against gonococcal conjunctivitis (ophthalmia neonatorum), which presents 2-5 days after birth.
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421
All immunoCOMPETENT pts who are asymptomatic and NONimmune should receive the varicella vaccine for post-exposure prophylaxis within 3-5 days of exposure. ImmunoCOMPROMISED hosts who are asymptomatic and NONimmune should receive varicella zoster immune globulin.
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USMLE Step 2
flashcards
Decks in class (7)
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