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Peds Flashcards

1
Q

NF 1 vs NF2

A 
Type 1 (von Recklinghausen): chromosome 17 (17 letters in von Recklinghausen). 
•	Café au lait spots, cutaneous neurofibromas (made up of Schwann cells- neural crest origin), pheochromocytomas, Lisch nodule (pigmented iris hamartomas)
•	Autosomal dominant with variable expressivity

Type 2: NF2 gene on chromosome 22.
• Bilateral acoustic schwannomas, juvenile cataracts, meningiomas, ependymomas

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2
Q

Labs in iron deficiency anemia

A

Dec ferritin
Inc transferrin
Inc RDW

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3
Q

What type of sepsis is galactosemia associated with?

A

E. coli

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4
Q

McCune Albright Syndrome

A

Characterized by precocious puberty, café au lait spots that are large and have irregular borders, and multiple bone defects (polyostotic fibrous dysplasia)

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5
Q

What is the cause of Fanconi anemia?

A

Defective DNA repair that results in inc chromosomal breakage

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6
Q

What causes non bullous impetigo and what causes bullous impetigo?

A

Non bullous: GAS and staph

Bullous: staph

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7
Q

Role of vWF

A

Anchors platelets to endothelial wall- essential for platelet aggregation

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8
Q

What urine test can be done to diagnose celiac disease

A

Dec D-xylose in urine (due to dec absorption into the blood from the GI tract)

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9
Q

Triad of Alport syndrome

A

Classic triad: Can’t see, can’t pee, can’t hear a buzzing bee – eye problems, glomerulonephritis, and deafness

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10
Q

hereditary hyperbilirubinemia
Indirect
Direct

A

Indirect: Gilbert, Crigler Najjar

Direct: Dubin-Johnson, Rotor syndrome

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11
Q

3 pathognomonic facies of Fetal Alcohol Syndrome

A
  • Small palpebral fissures
  • Smooth philtrum
  • Thin vermilion border
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12
Q

What causes sickle cell crises and how do you treat them?

A

Intermittent episodes of pain caused by acute vaso-occlusion in the bones

Treat symptomatic pain: NSAIDs and opioids

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13
Q

Female patient with leg pain has increased pain when you press just below the patella

A

Patellofemoral pain syndrome

  • Seen in young athletes, F»M
  • Anterior knee pain associated with activity – exacerbated by going up or down stairs
  • Patellofemoral compression test: reproduction of pain when the patella is compressed into the trochlear groove
    -Tx
    • Activity modification
    • NSAIDs
    • Stretching/strengthening exercises (emphasis on quadriceps)
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14
Q

Patient has tenderness over the tibial tuberosity

A

• Osgood-Schlatter disease
o Most common in adolescent male athletes
o Tenderness and swelling over the tibial tuberosity
o Traction apophysitis of the tibial tubercle
o XR will show anterior soft tissue swelling, lifting of the tubercle from the shaft, and irregularity or fragmentation of the tubercle

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15
Q

Limp in a child that resolves after 1 month who had negative hip xrays

A

Transient tenosynovitis: typically follows a viral infection

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16
Q

Patient with fissures at the corner of his mouth, hyperemic tongue, anemia, and seborrheic dermatitis. what vitamin is deficient?

A

B2 (riboflavin)

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17
Q

Patient has AOM with concurrent conjunctivitis

A

this is likely caused by H influenza, which is a B lactamase producer and needs to be treated with Augmentin

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18
Q

What should the approach be to treatment of close contacts of confirmed pertussis?

A

Treatment with macrolide antibiotics regardless of age, immunization status, or symptoms

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19
Q

Neonate presents with drooling, choking, and vomiting with the first feed

A

Tracheoesophageal abnormality

Clinical test is failure to pass NG tube into stomach

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20
Q

Pinealoma

A

causes obstructive non-communicating hydrocephalus from aqueductal stenosis (papilledema, headache, and vomiting) and dorsal midbrain syndrome aka Parinaud syndrome (paralysis of vertical gaze, ptosis, and pupillary abnormalities) due to direct compression of the pretectal region of the midbrain (superior colliculi)

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21
Q

Jaundice in a baby of a diabetic mother, what do you suspect?

A

Polycythemia (due to inc O2 demand in utero)

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22
Q

Which metabolic disorder is a complete contraindication for breastfeeding?

A

Classic Galactosemia

Initiated soy formula feeds

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23
Q

Congenital GI issues in Down syndrome

A

Hirschsprung disease
Annular pancreas
Duodenal atresia

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24
Q

An infant with Down Syndrome has not passed meconium 48 hours after birth, what do you suspect?

A

Hirschsprung disease

congenital megacolon characterized by lack of ganglion cells in distal colon → due to failure of neural crest migration

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25
Q

What age do you typically see staph scalded skin syndrome?

A

Infancy, rarely beyond age 5

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26
Q

What is the most common type of ectopic mucosa in Meckel diverticulum?

A

Pancreatic and gastric

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27
Q

What happens if you give an EBV patient amoxicillin (mistakenly thinking they have strep pharyngitis)?

A

• They will develop a diffuse maculopapular rash

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28
Q

Pulmonary hypoplasia
Flat facies
Limb deformities

A 
POTTER sequence
o	Pulmonary hypoplasia (cause of death) 
o	Oligohydramnios (insufficient amniotic fluid = trigger)
o	Twisted face
o	Twisted skin
o	Extremity defects (club feet)
o	Renal failure in utero
o	Caused by: ARPKD, renal agenesis, obstructive uropathy
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29
Q

Gold standard for diagnosis of Duchenne muscular dystrophy

A

Genetic testing: deletion of the dystrophin gene

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30
Q

Hemihyperplesia

A

Beckwith-Wiedemann

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31
Q

Prematurity

A

<37 weeks EGA

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32
Q

S/S of myotonic dystrophy

A 
Myotonia
Muscle wasting
Cataracts
Testicular atrophy
Frontal balding
Arrhythmia
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33
Q

Immunodeficiency with periodontal disease

A

Leukocyte adhesion deficiency

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34
Q

Immunodeficiency that presents with partial albinism, peripheral neuropathy, pancytopenia and inc risk of pyogenic infections

A

Chediak Higashi syndrome:

defective lysosomal trafficking regulator gene – microtubule defect: phagosome cannot travel across the neutrophil to merge with the lysosome

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35
Q

Prader Willi Syndrome is caused by…

A

loss of paternal copy of 15q11-q13

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36
Q

Sandifer syndrome

A

Seen in GERD

a condition in which infants will arch and become tonic to protect their airway from the refluxing gastric contents

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37
Q

Mechanism of severe combined immunodeficiency

A

Failure of T cell development, which then causes B cell dysfunction

High risk for infections with viruses, fungi, and opportunistic infections
(PCP pneumonia)

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38
Q

Why do infants receive a shot of vitamin k?

A

To prevent bleeding – inc production of coagulation factors

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39
Q

Xray findings:

Bacterial
Viral
Atypical pneumonia

A

Bacterial: lobar consolidation
Viral: diffuse interstitial infiltrates
Atypical pneumonia: diffuse interstitial infiltrates

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40
Q

Age distribution of Legg-Calve- perthes vs SCFE

A

Legg-Calve: 4-8 years old

SCFE: mean age 12 (associated with obesity)

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41
Q

Bite cells with heinz bodies

A

G6PD deficiency: increased vulnerability of RBCs to oxidative stress

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42
Q

Infant of a T1 diabetic mother vs a gestational diabetic

A

o Gestational diabetes → macrosomia
Delivery can be difficult – shoulder dystocia, clavicle fx, brachial plexus injury

o T1DM → small for gestational age (IUGR)
Think of the placenta as an end organ – DM causes damage to the placenta and it becomes insufficient

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43
Q

Classic labs/test in hereditary spherocytosis

A

Elevated MCHC

Osmotic fragility test

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44
Q

CSF in guillain barre

A

o CSF is significant for albuminocytologic dissociation (increased protein with normal WBC cell count)

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45
Q

Anemia of prematurity

A

o Normocytic normochromic RBCs with a low retic count
• Decreased EPO causes decreased reticulocyte production in bone marrow
• Shorter RBC life span in prematurity as well as frequent phlebotomy in the NICU

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46
Q

What type of anemia presents in the first 3 months of life? What type of anemia is this?

A

Diamond-Blackfan anemia: pure RBC aplasia

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47
Q

Centor criteria, strep pharyngitis

Strep treatment?

A
  • Fever
  • No cough
  • Tender lymphadenopathy
  • Sore throat with tonsillar exudate/petechiae

Treat with PCN (really Amoxicillin)

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48
Q

Which is worse, Gilbert syndrome or Crigler-Najjar syndrome?

A

Crigler-Najjar (absent UDP-glucuronosyltransferase) and die within a few years

Gilbert has decreased enzyme– patients have symptoms during times of stress, fasting, physical exertion

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49
Q

What would you see on histo of a erythema toxicum lesion?

A

Eosinophilic infiltrate

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50
Q

What diagnosis should you consider when a child presents with hepatosplenomegaly and cervical lymphadenopathy?

A

Acute lymphoblastic leukemia

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51
Q

Xray finding in NEC

A

XR shows air in the bowel wall (double line or train track appearance) leading to the hallmark finding of pneumatosis intestinalis

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52
Q

Tinea capitis treatment

A

Oral terbinafine or griseofulvin

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53
Q

Desquamating rash differential

A

Scarlet fever
Kawasaki disease
Scalded skin syndrome
Toxic shock syndrome

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54
Q

Transferrin and ferritin in iron deficiency anemia

A

Low ferritin

High transferrin

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55
Q

Myotonic muscular dystrophy inheritance

A

AD inheritance
Trinucleotide repeat: CTG

Anticipation: onset of disease is earlier with each generation

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56
Q

Patient presents for WCC. He is noted to have weight, height, and OFC in the 99th percentile, his right upper and lower extremities are smaller than his left extremities, he has a history of macroglossia and umbilical hernia. what do you suspect and what is a common complication of this? How will you monitor him?

A

Beckwith-Wiedemann syndrome

An overgrowth disorder characterized by a predisposition to neoplasms (Wilms tumor, hepatoblastoma)

Abdominal US and AFP levels every 3 months from birht to age 4

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57
Q

What do labs look like in Fanconi anemia? What do the patients look like?

A

Labs: pancytopenia

PE: short stature, inc incidence of tumors/leukemia, café au lait spots and thumb/radial defects, renal abnormalities

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58
Q

What is most commonly associated with erythema multiforme?

A

HSV

less commonly, M pneuminia, sulfa, PCN, SLE, malignancy

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59
Q

Anemia with low retic count

A

Anemia of prematurity

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60
Q

PAS positive:
TdT positive:
Peroxidase positive:

A

PAS positive: ALL
TdT positive: ALL
Peroxidase positive: AML (Auer rods)

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61
Q

What diseases are associated with intussusception?

A

Rotavirus
HSP
Meckel diverticulum

Do not give rotavirus vaccine to a child with hx of intussusception

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62
Q

Mom brings in 3 year old child after finding an abdominal mass. The child is otherwise asymptomatic. What do you suspect?

A

Wilms Tumor

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63
Q

Leukocytosis with neutrophilia is seen in which immunodeficiency? Why?

A

Leukocyte adhesion deficiency

Neutrophils are unable to exit the blood due to defect in integrins

64
Q

Risk factors for developmental dysplasia of the hips

A 
Breech presentation
Female sex
Caucasian
Family history
**Most patients have no risk factors
65
Q

Most common pathogen causing infection in kids with CF?

A

Staph aureus

Pseudomonas is the most common cause of pneumonia in adults, and contributes to life threatening decline of pulmonary function

66
Q

What can hyperbilirubinemia lead to? What are the common initial signs?

A

Elevated unconjugated bilirubinemia can lead to neurotoxicity (kernicterus) – a neurologic syndrome resulting from unconjugated bilirubin deposition in brain cells, especially in the basal ganglia, hippocampus and cerebellum

S/S: poor feeding, lethargy, loss of Moro reflex

67
Q

How do you treat staph scalded skin syndrome?

A

Anti-staph meds

Severe: treat as though they have a second degree burn with fluid management and IV oxacillin

68
Q

What is thought to cause necrotizing enterocolitis? What can be done in premature infants to help prevent NEC?

A

Combo of gut immaturity and exposure to bacteria from enteral feeds leading to a cascade of inflammation and damage to the bowel wall

Premature infants should receive breast milk

69
Q

What is metatarsus adductus and what is the treatment?

A

Medial deviation of the forefoot with a normal neutral position of the hindfoot

Commonly seen in first born infants due to crowded positioning in the small, primigravid uterus

Usually will spontaneously self-correct

70
Q

Hyperinsulinemia in infant of a diabetic mother

A

After delivery and leaving the high sugar in utero environment, the infant’s hyperinsulinemia can lead to severe hypoglycemia → seizures, obtundation and respiratory distress
• If baby is asymptomatic, have mom feed him and then recheck glucose after 30 mins
• If baby is symptomatic, give IV glucose

71
Q

3 year old presents with arm pain. Holding elbow in flexed position and forearm in pronated position. What do you suspect?

A

Radial head subluxation (nursemaid’s elbow)

72
Q

Difference between Fanconi and Diamond-Blackfan

A

Fanconi: pancytopenia
DM: pure red blood cell aplasia

73
Q

What is the treatment of intussusception?

A

Air enema

74
Q

Diagnosis of von willebrand disease and treatment

A

Dx with ristocetin aggregation test: Ristocetin activates GP1b receptors on platelets and makes them available for vWF bindings → When the vWF level is decreased, there is poor platelet aggregation in the presence of ristocetin

Tx with desmopressin: inc release of vWF and F8 from endothelial wall

75
Q

Recurrent sinopulmonary and GI infections (immunodefiency)

A

X linked agammaglobulinemia

Low B cells and immunoglobulin levels

76
Q

Maintenance fluids for pediatrics

A

D5 1/4NS + 20KCL

77
Q

How do people present with acute leukemia (AML and ALL)?

A

Increased blast production crowds out normal hematopoiesis in the bone marrow – resulting in an acute presentation with anemia (fatigue), thrombocytopenia (bleeding), or neutropenia (infection)

78
Q

Diagnosis of leukemia

A

> 25% lymphoblasts

79
Q

What labs can be done to help diagnose neuroblastoma?

A

Typically have increased levels of catecholamines: homovanillic acid and vanillylmandelic acid

80
Q

Treatment of hemophilia

A

Desmopressin – inc release of F8 from endothelial cells

81
Q

Treatment for a cat bite

A

Augmentin

82
Q

Treatment of CAP

A

Outpatient: Amoxicillin or Augmenting
Inpatient: Ampicillin
Atypical: azithromycin

83
Q

What past medical history is a contraindication to getting the rotavirus vaccine?

A

Past history of intusussception or uncorrected meckel diverticulum

84
Q

Heart defect in Edwards syndrome

A

VSD

85
Q

3 year old patient presents with petechiae and bruising.Only history is a URI 2 weeks ago. CBC shows thrombocytopenia but is otherwise normal. Diagnosis and treatment?

A

Immune thrombocytopenic purpura (ITP_• Autoimmune destruction of platelets by circulating antibodies (usually anti GP2b/3a)

Usually self limited but cases with severe bleeding may require IVIG or steroids, in severe cases can perform spelenectomy

86
Q

Todd Paralysis

A

A self-limited, focal weakness that occurs after a focal or generalized seizure

o Presents in the postictal period with a partial or complete hemiplegia involving an ipsilateral upper and lower extremity, although one may be more affected than the other

87
Q

Patient with primary amenorrhea has a normal US, and a high FSH. What is the next step?

A

Karyotype – likely a peripheral cause of primary amenorrhea, look for possible Turner’s syndrome

88
Q

Histology of Reye Syndrome

A

Microvesicular steatosis

89
Q

How to diagnose intestinal malrotation?

A

Upper GI series – illustrates abnormal position of the ligament of Treitz and the cecum

Lactic acidosis = bowel ischemia

90
Q

Patient with delayed puberty and complaints of anosmia… What is the condition and what labs will you see?

A

Kallmann syndrome

Defective migration of GnRH cells and impaired formation olfactory bulb leads to dec GnRH synthesis (leading to dec LH, FSH, and testosterone)

91
Q

Gastroschisis vs Omphalocele

A

Gastroschisis: extrustion of the abdominal contents through abdominal folds

  • typically right of umbilical folds
  • not covered by peritoneum

Omphalocele: persistence of herniation of the abdominal contents into the umbilical cord

  • midline
  • covered by peritoneum
92
Q

Jones criteria for rheumatic fever

A
  • J- migratory polyarthritis
  • O-carditis **primary cause of morbidity
  • N-subcutaneous nodules
  • E- erythema marginatum
  • S- Sydenham chorea (non rhythmic movements of the hands, feet, and face – due to antistreptococcal antibodies that cross react with basal ganglia).
93
Q

What are bugs are patients with Chronic Granulomatous Disease susceptible to? How do you test for this?

A

Recurrent pulmonary and cutaneous infections with catalase positive organisms

Dihydrorhodamine flow cytometry test will show absence of green fluorescence that is characteristic of normal neutrophils

94
Q

3 most common causes of bacterial meningitis in neonates:

A

GBS, E coli, listeria

95
Q

Common pathogens of AOM

A

Strep pneumo
H. flu (nontypeable)
Moraxella

96
Q

WAGR syndrome

A

Wilms Tumor, Aniridia, Genitourinary anomalies, mental Retardation

97
Q

Type of immunodeficiency in DiGeorge

A

Lack of normal thymic development = T cell deficiency

98
Q

Treatment of acute stroke in sickle cell disease

A

Transfusion therapy

99
Q

Triad of Kartagener syndrome

A

Situs inversus
Chronic sinusitis
Bronchiectasis

100
Q

Mongolian spot

What can this be confused with?

A

Dermal melanocytosis
Looks like a bruise, can be confused with abuse –> important to document presence of this in chart to prevent abuse accusation

101
Q

What are risks in babies who are SGA?

A

Hypoglycemia
Hypothermia
Polycythemia

102
Q

Erb palsy

A

C5/C6 nerve root damage

103
Q

When should the umbilical cord fall off? What does delayed cord separation suggest?

A

Within 3-4 weeks

Delayed cord separation suggests neutrophil abnormalities: leukocyte adhesion deficiency

104
Q

Where can you find a neuroblastoma?

A

Abdomen, thoracic cavity, head or neck (anywhere along the postganglionic sympathetic nervous system)

105
Q

Xray findings in Ewing

A

Ewing: a lytic bone lesion with calcified periosteal elevation (onion skin)

106
Q

CATCH 22 of DiGeorge syndrome

A

Maldevelopment of the 3rd and 4th pharyngeal pouches
CATCH 22
o Cardiac (tetralogy of fallot, truncus arteriosus)
o Abnormal facies
o Thymic abnormal development – T cell deficiency
o Cleft palate
o Hypocalcemia (secondary to parathyroid hypoplasia)
o Chromosome 22q11 microdeletion

107
Q

Clenched hands with overlapping fingers

A

Edwards Syndrome (Trisomy 18)

108
Q

Patient with primary amenorrhea has a normal US, and a low FSH. What is the next step?

A

MRI brain– likely central cause of primary amenorrhea, look for pituitary lesion

109
Q

Patient with chronic back pain has a palpable vertebral step off is noted on physical exam, what do you suspect?

A

Spondylolithesis: a developmental disorder characterized y a forward slip of the vertebrae

110
Q

What causes osteogenesis imperfecta?

A

Impaired synthesis of type 1 collagen – impaired bone matrix formation

111
Q

What is an infant of a diabetic mother at risk for after delivery?

A
  • After delivery and the high sugar in utero environment, the infant’s hyperinsulinemia can lead to severe hypoglycemia → seizures, obtundation and respiratory distress
  • Birth defects like congenital heart disease and neural tube defects
  • Increased fetal O2 requirement → polycythemia
  • Hyperinsulinemia has an antagonistic effect on surfactant production – respiratory distress syndrome
112
Q

How to close PDA? How to keep it open?

A

Close: indomethacin

Keep open: prostaglandin E1

113
Q

Clinical features in vitamin D deficiency in peds

A

craniotabes (softening of the skull, ping pong ball skull), delayed fontanel closure, enlarged skull with frontal bossing, enlarged costochondral joints, and enlarged long bone joints.
Also have leg bowing (Genu varum).

114
Q

First step in the evaluation of primary amenorrhea?

A

Pelvic ultrasound

115
Q

Why are infants of a diabetic mother at risk for respiratory distress syndrome?

A

Hyperinsulinemia has an antagonistic effect on surfactant production – respiratory distress syndrome

116
Q

Duchenne vs Becker muscular dystrophy

A

Duchenne: deletion in dystrophin, appears in young children

Becker: mutation in dystrophin, appears in adolescence or early adulthood

117
Q

Child with signs of meningitis develops purpura and hypotension… what do you suspect?

A

N meningitidis causing waterhouse friedrichsen syndrome: acute primary adrenal insufficiency

118
Q

Treatment for molluscum contagiosum

A

Will typically resolve over 1-2 years but families typically request treatment with cryotherapy

119
Q

Treatment of lyme disease by age

A

<8 yo: oral amoxicillin or cefuroxine
>8 yo: doxycycline

Those with evidence of cardiac or neurological disease should get high dose penicillin G or ceftriaxone

120
Q

Two most important consequences of sickling in sickle cell anemia

A

Hemolysis

Vaso-occlusion

121
Q

Wiskott Aldrich syndrome

A

Combined B and T cell deficiency due to disruption of the cytoskeleton

WATER
Wiskott
Aldrich
Thrombocytopenia
Eczema
Recurrent infections
122
Q

Severe combine immunodeficiency

A

Failure of T cell development, which then causes B cell dysfunction

High risk for infections with viruses, fungi, and opportunistic infections

Treat with a stem cell transplant

123
Q

What type of bilirubin can cause neurotoxicity?

A

Unconjugated bilirubin (indirect): When serum levels of unconjugated bilirubin exceed the binding capacity of albumin, excess free bilirubin can cross the BBB

124
Q

Posterior cervical lymphadenopathy

A

EBV

125
Q

What does rheumatic fever follow?

A

Strep pharyngitis but not impetigo!

126
Q

What electrolyte abnormality is associated with pyloric stenosis?

A

Hypokalemic, hypochloremic metabolic alkalosis

127
Q

Aplastic crisis in sickle cell anemia: characteristics and most common cause

A

An acute drop in hemoglobin accompanied by a low retic count without splenomegaly

Often caused by parvo infection

128
Q

Howell jolly bodies

A

Sickle cell anemia

129
Q

Painless rectal bleeding in a pediatric patient

A

Meckel’s diverticulum

130
Q

Conjunctivitis in the newborn

A

Gonorrhea: profuse discharge
Chlamydia: milder, watery

  • Early onset = gonorrhea
    (tx with IV or IM ceftriaxone)
  • After several days = chlamydia
    (tx with oral erythromycin)
131
Q

Clinical tetrad of Henoch Schonlein Purpura

A
  1. Palpable purpura on legs and buttocks (most common presenting symptom)
  2. Arthritis/arthralgia (2nd most common)
  3. Abdominal pain: Colicky pain and GI bleeding
    4: Renal disease
    - Ranges from microscopic hematuria and proteinuria to ESRD
    - IgA nephropathy
132
Q

Results of congenital diaphragmatic hernia

A

Results in pulmonary hypoplasia and pulmonary HTN

Polyhydramnios can occur as a result of esophageal compression

133
Q

Salmon patch aka stork bite aka angel kisses

A

Nevus simplex

134
Q

Treatment of tylenol overdose

A

N-acetylcysteine

135
Q

Location of bone lesion in Ewing vs Osteosarcoma

A

Ewing is in the diaphysis (mid shaft)

Osteosarcoma is in the metaphysis (near the growth plate)

136
Q

What does a normal PaCO2 during an acute asthma exacerbation suggest?

A

A normal PaCO2 in the face of tachypnea and fatigue is an ominous sign because the PaCO2 should be well below 40 mmHg in the pateint with rapid respiratory rate

137
Q

Most common cause of infection in kids with sickle cell disease

A

Osteomyelitis: salmonella
Other: staph aureus

138
Q

Newborn with cyanosis and hypoxia that is aggravated by feeding and relieved by crying. What should you do to confirm the diagnosis?

A

Choanal atresia

Congenital nasal malformation caused by a failure of the posterior nasal passage to canalize completely, leaving either a bony or membranous obstruction
o Check by attempting to pass a catheter through the nose to the pharynx

139
Q

Temperature in a neonate with sepsis

A

Hyper or hypothermia

140
Q

AOM treatment by age

A

<2 years old = treat with antibiotics

>2 years old = observation, or antibiotics

141
Q

Infant of a diabetic mother: early gestation complications vs late gestation complications

A

Early: neural tube defects and congenital heart disease (transposition of the great arteries)

Late: macrosomia –> shoulder dystocia, clavicle fx
Polycythemia due to inc fetal O2 requirement

142
Q

Treatment of rocky mountain spotted fever

A

Since RMSF is so rapidly progressive, it is essential to start treatment based on clinical suspicion rather than waiting for confirmatory testing to come back

143
Q

Asymptomatic abdominal mass that does not cross midline found incidentally in a 3 year old child

A

Wilms tumor

144
Q

What does opsoclonus-myoclonus raise suspicion for?

A

Neuroblastoma

145
Q

Neonate with painless bloody stools +/- eczema

A

Milk or soy protein induced colitis

o Non-IgE mediated immune response to proteins in formula or breast milk that causes rectal and colonic inflammation

146
Q

What lab is characteristically elevated in muscular dystophy?

A

CK

147
Q

Fixed split S2

A

ASD

148
Q

Prader Willi presentation early vs late

Complications

A
  • Early: Hypotonia and failure to thrive
  • Late: Compulsive binge eating and obesity
  • Short stature
  • Hypogonadism
  • Intellectual disability
  • Dysmorphic facies
  • Narrow forehead
  • Almond shaped eyes
  • Downturned mouth

Complications (related to obesity): Sleep apnea, T2DM, gastric distention

149
Q

Patient with episodes of gross hematuria after URIs

A

Alport syndrome: Mutation in type IV collagen → thinning and splitting of GBM

X linked dominant

150
Q

Transient erythroblastopenia of childhood (TEC)

A
  • An acquired red cell aplasia which occurs in healthy children between 6 months and 4 years old
  • Gradual onset of symptoms: pallor, dec activity
  • The physical exam is typically unremarkable: mild pallor
  • Normocytic normochromic anemia
  • Complete spontaneous recovery within 1-2 months
151
Q

Patient with primary amenorrhea has a normal US, what is the next step in management?

A

FSH level

High FSH indicates a peripheral cause
Low FSH indicates a central cause

152
Q

What type of deficiency causes bleeding in patients with CF?

A

Vitamin K deficiency due to fat malabsorption

Dec factors 2, 7, 9, 10

153
Q

Bilateral hemianopia

A

Craniopharyngioma

154
Q

Treatment of croup vs treatment of bronchiolitis

A

Croup: Racemic epi and steroids

Bronchiolitis: supportive care

155
Q

Hemophilia A

A

Factor 8 (A-ight)

Treat with desmopressin (DDAVP) to inc release of F8 from endothelial cells

156
Q

Klumpke paralysis

A

C7/C8/T1 nerve root damage

157
Q

What bone lesion is seen with retinoblastomas?

A

Osteosarcoma (RB mutations)

Shelf Exams (8 decks)

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