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SHELF EXAMS > Pediatric Shelf Exam > Flashcards

Pediatric Shelf Exam Flashcards

1
Q

Dx + DGx + Tx: Newborn with cyanosis, tachypnea, absent breath sounds on left, heart sounds shifted further to the right, scaphoid abdomen, mediastinal shift to right, bowel loops within thoracic cavity

A

Dx: congenital diaphragmatic hernia
DGx:
Tx: intubation + mechanical ventilation, Ng tube placement

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2
Q

Dx + DGx + Tx: tachypnea, subcostal retractions, cyanosis, grunting, decreased breath sounds in pre-term

A

Dx: Neonatal respiratory distress syndrome (NRDS)
DGx: XRay- low lung volumes + diffuse reticulogranular (ground-glass) densities
Tx: CPAP (continuous positive airway pressure ventilation) + get ABG, consider intubation/surfactant

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3
Q

Dx + Dgx + Tx: low grade fever, mild cough, 1- week, patient cries when neck extended, swelling of posterior pharyngeal wall

A

Dx: Retropharyngeal Abscess
DGx: contrast enhanced CT scan
Tx: empiric antibiotic (ampicillin-sulbactam, clindamycin), intubation, abscess drainage

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4
Q

Dx + Tx: URI + coughing spells + high pitched inspiratory sound, incomplete vaccinations

A

Dx: pertussis (whooping cough)
Tx: Macrolide (azithromycin for all ages, but any >1 month

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5
Q

Dx + Tx: 3-day history of fever, headache, earache, sore throat, up-to-date, 102F, 38.9C, enlarged right tonsil + whitish membrane

A

Dx: acute bacterial tonsilitis (strep pyogenes)
Tx: 1st line- penicillin V, macrolides (if clarithromycin, azithromycin, allergic),

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6
Q

Dx: preemee, respiratory distress—> mechanical ventilation for month after delivery, diffuse granular densities, basal atelectasis

A

Dx: bronchopulmonary dysplasia Cx of prolonged mechanical ventilation in premature infants

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7
Q

Dx: toddler, dry barking cough worsens at night, mild rhinorrhea and fever, inspiratory stridor on agitation, febrile,

A

Dx: mild croup (viral etiology—> parainfluenza or RSV
Tx: symptomatic relief—> cold mist- decrease nasal passage irritation, dexamethason- reduce airway swelling

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8
Q

Prevention and treatment of RSV

A

Tx: Palivizumab -passive immunity- infants at risk for severe bronchiolitis

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9
Q

Dx DGx Tx: young boy + coughing, intermittent wheezing, hx of eczema, 3 URIs, otitis media with abx, scattered wheezing on pulm exam

A

Dx: asthma
DGx: spirometry with obstructive lung pattern reversible
Tx: albuterol

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10
Q

Dx: thick purulent ocular discharge, eyelid swelling, newborn within 2-7 days

A

Neonatal conjunctivits with N.gonorrhea

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11
Q

Dx: mucoid watery nasal discharge (rhinorrhea) + tachypnea, wheezing, nasal flaring, low grade fever, intercostal retractions, cyanosis, late neonatal period

A

RSV

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12
Q

Dx: child 6 months to 3 years old—->croup = nasal congestion, barking seal cough, tachypnea, hypoxemia + low grade fever, inspiratory stridor, hoarseness

A

Parainfluenza virus type 1

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13
Q

Dx: within 6 days of birth + pneumonia what bug?

A

Strep Agalactiae

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14
Q

Dx: 5-14 days after birth, mucopurulent eye, discharge, swelling

A

Neonatal conjunctivitis due to Chlamydia

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15
Q

Dx: 4-12 weeks post birth + mucopurulent eye discharge + eyelid swelling + short repetitive coughs with deep inspirations between (staccato cough)

A

Dx: chalmydial pneumonia

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16
Q

Dx: child under 3 y/o + acute onset coughing/shortness of breath, elevated respirations, abnormal hyperinflation of right lung with air trapping.

A

Foreign Body Aspiration
DGx + Tx: bronchoscopy

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17
Q

Dx + Tx: teenager + fever and sore throat worsening, dysphagia, hoarseness, febrile, anterior cervical lymphadenopathy, erythematous tonsils, swelling, white exudate on tonsillar pillar, uvula deviated to left

A

Tonsilitis, peritonsillar abscess

Incision, drainage + IV ampicillin-sulbactam therapy

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18
Q

Most common side effect of inhaled corticosteroids

A

Oropharyngeal candidiasis (thrush)

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19
Q

Tx: child from TB endemic contry with HBsAb + and Interferon 𝛄 release assay- + , chest X-ray is negative

A

Latent TB infection:
Tx: start isoniazid + rifapentine for 3 months or 4 months of rifampin
Give Vit B6 (pyridoxine) to avoid peripheral neuropathy + sideroblastic anemia

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20
Q

Vertical gaze palsy, nystagmus, eyelid retraction when looking upward, pupils that accommodate but don’t react to light

A

Parinaud syndrome- compression of the tectum + medial longitudinal fasciculus

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21
Q

Wt loss, headache, insomnia, + parinaud syndrome

A

Pinealoma

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22
Q

Common sequel of untreated pinealoma

A

Obstructive hydrocephalus (headaches, vision abnormalities, nausea, vomiting, ataxia

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23
Q

Complication of precocious puberty

A

Increased short stature,- trigger early epiphyseal plate closure

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24
Q

Dx: 17 y.o no menarche, normal breast development, scant axillary hair, firm, nontender left inguinal mass, does not show a uterus or ovaries

A

AIS- XY—> formation of testes undifferentiated fetal gonad, testes remain in inguinal canal, female external genitalia, cutaneous andorgen receptors lack sensitivity

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25
Q

6 mo infant - cataracts in early infancy,

A

Galactokinase deficiency

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26
Q

Infant hepatomegaly, hypoglycemia, generalized muscle weakness, cramps, hyperlipidemia, ketosis

A

Debranching enzyme

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27
Q

Infant, jaundice, vomiting, hepatosplenomegaly, infantile cataracts, failure to thrive

A

Galactose-1 Phosphate Uridyl transferase

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28
Q

Infant, vomiting, poor feeding, hypoglycemia, jaundice, hepatomegaly, around time of weaning from breastmilk

A

Aldolase B deficiency- lifelong fructose + sucrose free diet

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29
Q

Fructose in urine,

A

Fructokinase deficiency

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30
Q

Short stature, delayed puberty, delayed bone age, and family history of delayed growth

A

CDGP- constitutional delay of growth and puberty

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31
Q

Development of secondary sex characteristics under 8

A

Precocious puberty, bone age > 1yr difference- measure LH or low LH + GnRH stimulation-> check if high—> GnRH-dependent precocious puberty,

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32
Q

Menorrhagia, fatigue, weight gain, bradycardia, facial myxedema (puffy appearance), thinning of the eyebrows, diminished deep tendon reflexes

A

Hypothyroidism

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33
Q

Cx of Gestational Diabetes Mellitus

A

Fetal macrosomia— big birth weight, shoulder dystocia- upper brachial plexus palsy

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34
Q

Severe acne, excess hair growth, accelerated bone maturation)

A

Hyperandrogenism

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35
Q

Hyperandrogenism before age 9, elevated 17-hydrocyprogesterone, androstenedione, DHEAS, low levels of deoxycorticosterone and cortisol despite administering ACTH

A

Non-classic congenital adrenal hyperplasia (late-onset CAH)

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36
Q

Dx + DGx + Tx: newborn, abdominal distention, multiple episodes of bilious vomiting, increased anal sphincter tone/empty rectal vault, delayed passage of meconium, narrow sigmoid colon, squirt sign

A

Dx: Hirschsprung Disease- defective migration of Neural Crest Cells from proximal to distal bowel- aganglionic rectum/distal sigmoid
DGx: ABXR- megacolon + rectal biopsy
Tx:

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37
Q

Mechanism of appendicitis

A

Lymphatic Tissue Hyperplasia- children
Fecalith obstruction- adults

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38
Q

Dx+ DGx + Tx: 3-month history stool incontinence, frequent nocturnal urination, large hard stools + painful bowel movements- firmness to palpation in left lower quadrant.

A

Dx: retentive encopresis (involuntary elimination of feces)
DGx: Clinical
Tx: fecal disimpaction + enemas for 6-24 months

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39
Q

What is Meckel Diverticulum

A

incomplete obliteration/ of vitelline duct
can cause lower GI bleeding

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40
Q

Dx + DGx + Tx: young child, multiple episodes of n/v + ab pain, missed several days of school intermittently, recent psych stressor

A

Dx: cyclical vomiting syndrome
DGx: diagnosis of exclusion no anatomical anomalies
Tx:

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41
Q

Failure of duodenal recanalization

A

duodenal atresia- complete intestinal obstruction- double bubble sign, gasless distal small bowel, maternal polyhydramnios

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42
Q

nonrotation of the intestine

A

complete non-rotation–> cecum in left lower quadrant, small bowel obstruction, small bowel on right, colon on left

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43
Q

incomplete intestinal rotation

A

malpositioned duodenojejunal junction (located left of midline, cecum) + duodenal obstruction–> bilous vomit

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44
Q

Dx + DGx + Tx: infant + acute watery diarrhea + dehydration + not fully vaccinated + no abdominal pain

A

Dx: rotavirus
DGx: enzyme immunoassay of stool
Tx: Oral Hydration Solution, IV fluids

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45
Q

Dx: newborn with poor feeding, lethargy, prolonged newborn jaundice, pale facies, increased head circumference, abdominal distention, macroglossia

A

congenital hypothyroidism

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46
Q

dx: congenital hypothyroidism + abdominal protrusion + enlarges with crying, easily reducible

A

Dx: congenital umbilical hernia

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47
Q

Dx + DGx + Tx: child with recurrent skin infections + catalase positive bacteria/fungi (stapha A/ Serratia marcescens) + diffuse granulomas

A

Dx: CGD - Chronic granulomatous disease (X-linked recessive mutation of NADPH oxidase)- decreased superoxide
DGx: Dihydrorhodamine flow cytometry
Tx: prophylaxis: TMP- SMX + Itraconazole + INF-y, Hematopoietic cell transplant

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48
Q

Dx + DGx + Tx: recurrent swelling episodes w/o urticaria/pruritis no trigger, colicky ab pain, vomit, diarrhea, laryngospasm

A

Dx: Hereditary Angioedema
DGx: low C4/ C1 inhibitor protein/function- C2 inhibitor deficiency/dysfunction
Tx: C1 inhibitor concentrate + bradykinin antagonists (icatibant) + kallikrein inhibitor ecallantide)

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49
Q

Low risk vs high risk food allergy management

A

Low Risk: early introduction- trial at home
High Risk: trial in office referral to allergy testing

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50
Q

Wiskott- Aldrich Syndrome

A

Eczema, recurrent infections, microthrombocytopenia (low platelets

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51
Q

X-linked agammaglobulinemia (Bruton)

A

B-cell deficiency- disorder of B-cell maturation, low on all levels of IgGs and no response to vaccines

52
Q

Dx + DGx + Tx: recurrent non purulent infections (cellulitis/periodontitis), impaired wound healing, delayed umbilical cord separation > 3weeks), leukocytosis neutrophilia

A

Dx: LAD leukocyte adhesion deficiency
DGx: reduced CD1 antigen
Tx:

53
Q

Dx + DGx + Tx: recurrent severe sinopulmonary infections, poor growth, low IgA, low IgG, elevated IgM, encapsulated bacteria, increased risk of opportunistic infections

A

Dx: hyper IgM syndrome X-linked defect in CD40 ligand
Dgx: clinical + Lab
Tx:

54
Q

Selective IgA deficiency

A

Asymptomatic or recurrent respiratory, GI, or UTI
IgA low
IgM, IgG- normal

55
Q

Hyper IgE syndrome

A

Everything normal except IgE elevated
Defective JAK-Stat Th17
Severe chronic eczema, non inflammatory abscesses (cold)- staph or candida
IgE + Eosinophils high,WBC always normal
Dysmorphic facies (broad nose, prominent forehead, retained primary teeth

56
Q

Hyper IgM syndrome

A

Severe recurrent sinopulmonary infections
- B cell normal
- IgG + IgA + IgE low
- IgM high

57
Q

Common Variable Immunodeficiency

A

Normal B cell count but all Ig’s are Low
Presents in adolescence- recurrent sinopulmonary and GI infections, failure to thrive, chronic pulmonary disease
Tx: IV IG

58
Q

Bruton’s agammaglobuliemia (x-linked)

A

B cells and Igs all low
Recurrent sinupulmonary infections

59
Q

Immunizations

A

All kids pre-term or not receive it on chronological age

60
Q

ICU infant, respiratory distress, multiple episodes of pneumonia, chronic thrush, otitis media,

A

No T cells, and dysfunctional B cells- Severe Combined Immunodeficiency
Tx: urgent stem cell transplantation

61
Q

Recurrent sinopulmonary infections + anaphylaxis with blood transfusion

A

Selective IgA deficiency

62
Q

Associated Complications of DiGeorge 22qDS syndrome?

A

CATCH-22
C- cardiac outflow anomalies- ToF + persistent truncus arteriosus
A- anomalous face- nasal bridge, low set ears,
T- thymic hypoplasia- decreased T-cell immunity
C- cleft palate
H- hypoparathyroidism- hypocalcemia

63
Q

cyanosis, fatigue with feeding,
Harsh crescendo-decrescendo systolic murmur at left upper sternal border

A

Tetralogy of Fallot
-> R Ventricular outflow tract obstruction
- R ventricular hypertrophy
- Overriding Aorta
-VSD
Diversion- r A—> aorta—> hypoxemia
Tx: Knee chest positioning, O2, surgical intervention

64
Q

Dx + Tx: syncope + normal BP, no trauma to head, returns to alert state, reports events which occurred during episode

A

Dx: psychogenic pseudosyncope- conversion disorder

65
Q

Dx + DGx + Tx: infant + noisy breathing with birth, no improvement with steroid, afebrile, nml vitals, biphasic stridor, improves with neck extension

A

Dx: vascular rings (great vessels encircle/compress trachea)
DGx: CT scan
Tx: surgical division of structures creating ring

66
Q

Which murmurs must be evaluated?

A

Holosystolic/ Diastolic Murmurs
Harsh
Grade > 3 intensity persists with standing and Valsalva maneuver
DGx: get echo

67
Q

Dx + Tx: febrile illness with development of pain in swelling in multiple joints, 2/6 holosystolic murmur at apex, mild mitral regurg

A

Dx: Rheumatic Heart Disease- chronic valvular damage- group A strep
Tx: IM benzathine Penicillin G every 4 weeks
Cx:

68
Q

Common heart defect in trisomy 18 (edwards syndrome)

A

VSD- holosystolic murmur
Trisomy 18- micrognathia, microcephaly, rocker bottom feet, overlapping fingers, absent palmar creases

69
Q

Tx of Persistent Pulmonary Hypertension of the Newborn ( PPHN)

A

Inhaled Nitric Oxide, O2,

70
Q

Dx: post surgery infant, —> decreased appetite/ vomiting, fever + tachycardic, tachypnea, distal heart sounds, enlarged cardiac silhouette

A

Dx: pericardial effusion
DGx: ECHO
Tx: drainage

71
Q

Dx + DGx + Tx: abrupt onset of fever, pharyngitis, severe myalgias, and pale mottled skin, strep and flu are negative

A

Dx: Meningococcal meningitis
DGx: Lumbar Puncture
Tx: Ceftriaxone

72
Q

Difference between orbital vs. preseptal cellulitis

A

Preseptal- Eyelid Erythema/Swelling + Chemosis—> Tx with Oral Abx

Orbital cellulitis- Pain with EOM, proptosis/opthalmoplegia with diplopia—> IV abx

73
Q

Dgx and Tx of Retropharyngeal Abscess

A

Dx: fever, difficulty/ pain with swallowing, neck pain, drooling, muffled hot potato voice
DGx; widening of pre-vertebral space lateral neck X-ray + CT
Tx: IV abx (ampicillin-sulbactam, clindamycin) + surgical drainage

74
Q

high fever, drooling stridor, non-vaccinated, thumb sign

A

epiglottitis

75
Q

fever, muffled voice, throat pain, uvular deviation to contralateral tonsillar pillar

A

peritonsillar abscess

76
Q

URI–> croup-like symptoms, fever, stridor, tracheal narrowing, steeple sign

A

Tracheitis

77
Q

when to use topical vs. oral abx in impetigo?

A

non-bullous impetigo-> topical
bullous impetigo–> oral (amox, cephalexin, dicloxacillin

78
Q

Dx + Dgx + Tx + Cx: newborn + sensorineural hearing loss, cataracts, PDA, red reflex absent, loud harsh murmur, blueberry muffin rash

A

Dx: Rubella
DGx: IgM serology
Tx: Nothing
Prevention: Vaccination
Cx:

79
Q

Dx, Dgx, Tx: chid, uncontrollable writhing of arms/grimacing of face, previous sore throat/fever resolved without treatment, pericardial friction rub, prolonged PR interval/diffuse ST elevations

A

Dx: Acute Rheumatic Fever - Strep Pyogenes- (Joints (migratory arthritis), Carditis, Nodules (subq), Erythema marginatum, Sydenham chorea

DGx: clinical + major/minor
Tx: prophylatic oral penicillin, Tx: IM benzathine penicillin G

80
Q

Dx + DGx + Tx: neonate + irritability, poor feeding, lethargy, leukopenic,

A

Dx: neonatal sepsis- Group B strep, E Coli, Listeria Monocytogenes
Tx: IV ampicillin + gentamicin

81
Q

Dx + DGx + Tx: neonate, macrocephaly, jaundice, mother travel abroad + fever/swollen lymph nodes, spontaneous resolution hepatomegaly, hydrocephalus, diffuse intracranial calcifications

A

Dx: Congenital Toxoplasmosis
DGx: toxoplasma serology/ PCR
Tx: pyrimethamine, sulfadiazine, folinic acid

82
Q

Dx + DGx + Tx: neonate + microcephaly + ventriculomegaly, periventricular califications growth restriction, hepatosplenomegaly, thrombocytopenia

A

Dx: Congenital Cytomegalovirus- transmission by urine/saliva
DGx: PCR testing, viral culture
Tx: antiviral therapy (valganciclovir)

83
Q

Bugs to watch out for in SCD sepsis?

A

Strep Pneumo, H Flu, N Meningitidis

84
Q

Bugs to watch out for in CF, recurrent pneumonia?

A

Staph A- kids/young children Pseudomonas Aeruginosa- adults

85
Q

Good vs. Bad Lymphnodes

A

Good: soft, mobile, <2cm, localized, no symptoms
Bad: firm, hard, immobile, > 2cm, systemic symptoms

86
Q

Transmission + Dx + DGx + Tx + Cx: Congenital Toxoplasmosis

A

Tr: Feces/ Uncooked Food Meat/ Unpasteurized Goat Milk
Dx: Chorioretinitis, Hydrocephalus, Diffuse, Intracranial Calcifications
DGx: PCR + IgM Toxo
Tx: Mom- spiramycin
Baby: pyrimethamine, sulfadiazine, folinic acid
Cx: epilepsy/intellectual disability, visual/hearing impairments

87
Q

Tr + Dx + DGx + Tx + Cx: Congenital Syphillis

A

Tr: Sexual
Dx: Early: < 2 yearsRhinorrhea, Skeletal Anomalies, Desquamating Rash (Palms/Soles), Lymphadenopathy
Late: > 2 years- saddle nose, frontal bossing, notched, widely spaced teeth, vision/hearing impairment
DGx: RPR + VDRL, dark field microscopy, PCR
Tx: IV penicillin
Cx: miscarriage, stillbirth,

88
Q

Tr + Dx + DGx + Tx + Cx: Congential Rubella

A

Tr: Airborne Droplets, transplacental
Dx: miscarriage, preterm, fetal growth restriction
PDA, Cataracts, Cochlear Defects, Hepatosplenomegaly, Jaundice, hemolytic anemia, thrombocytopenia, blue berry muffin rash,
DGx: PCR, IgM antibody serology,
Tx: Immunization, <16 weeks, termination, >16weeks self-resolving
Cx: microcephaly, intellectual disability, skeletal abnormalities.

89
Q

Tr + Dx + DGx + Tx + Cx: Congenital CMV

A

Tr: Any Fluids, Transplacental
Dx: Sensorineural Hearing Loss, Chorioretinities, Microcephaly, Periventricular Calcifications,
DGx: CMV IgM antibodies or PCR
Tx: Baby: Ganciclovir, foscarnet, blood/platelet transfusions
Mother: Valacyclovir
Cx: Audio/Visual Complications

90
Q

Tr + Dx + DGx + Tx + Cx: Congenital HSV

A

Tr: HSV-2, oral, transplacental, perinatal
Dx: vescicular/ ulcerative rash, keratocunjunctivitis, meningoencephalitis
DGx: viral culture + PCR
Tx: IV acyclovir/valacyclovir
Prevention

91
Q

When to Proxphlaxis with Varicella Vaccine vs. Varicella IG

A

No immunizations + immunocompetent–> vaccine only

No immunization + immunocompromised–> VZIG

92
Q

Dx + DGx: 2 y/o- recurrent cough/ SOB, multiple lobar pneumonias, mild retractions, inspiratory crackles, hyperresonance, clubbing of nail beds, generalized hyperinflatio, bronchial thickening, patchy atelectasis

A

CF
Sweat Chloride Test

93
Q

Dx + DGx + Tx: tachypnea, hypoxia, accessory muscle use, cyanosis,

A

Dx: NRDS or hyaline membrane disease- not enough pulmonary surfact

94
Q

treatement of Von Willebrand Disease

A

platelet count normal, PT elevated
supportive care + desmopressin

95
Q

hypotonia, downturned palpebral fissures, prominent epicanthal folds, flat facies, single palmar creases, intellectual disability,

A

Downs Syndrome

96
Q

intellectual disability, elongated face with prominent jaw, high arched palate, large ears, hyperextensible joints, postpubertal macroorchidism

A

Fragile X syndorme (X linked Dominant)

97
Q

dysgenesis of seminiferous tubules, testicular atrophy, decreased spermatogenesis, eunuchoid body shape, long extremities, gynecomastia

A

Klinefelter Syndrome XXY

98
Q

intellectual disability, cleft lip/palate, holoprosencephaly, microphthalmia, cutis aplasia,

A

Trisomy 13 (Patau Syndrom)

99
Q

widening of the wrist in infant

A

Rickettes + Vitamin D deficiency –> increased alkaline phosphatase activity

100
Q

female, low estrogen, underdeveloped sexual characteristics, increased LH,FSH

A

Turner Syndrome (Gonadal Dysgenesis)

Tx: growth hormone replacement, estrogen replacement

101
Q

easy bruising, active, scattered petechiae + ecchymoses at various stages of healing, very low platelet count

A

ITP (Immune Thrombocytopenic Purpura)- self limiting

102
Q

virilization, hirsutism, elevation of dehydroepiandrosterone sulfate, 17-ketosteroid excretion, normal testosterone, normal pelvic US

A

Adrenal Adenoma in zona reticularis

103
Q

neonate, hydronephrosis, urine flow is poor with dribbling, bilateral hydronephrosis/hydroureter, thick walled bladder

A

posterior urethral valve- abnormal remnant of the mesonephric (wolfian duct)
DGx: US- hydronephrosis, oligohydramnios, fetus, voiding cystourethrogram
Tx: endoscopic valve ablation

104
Q

acute onset of weakness, headache, nausea/vomiting, AMS, hx of recent viral gastroenteritis, large amounts of water, no meals, edema on hands/feet

A

dilutional hyponatremia
Tx: hypertonic saline

105
Q

infant, poor linear growth, no vomit/diarrhea, hypokalemia, hyperchloremia, non-anion gap metabolic acidosis, respiratory alkalosis

A

Renal Tubular Acidosis

106
Q

neonate, systolic ejection murmur radiation to back/ lung fields and loudest at right axilla, ejection click/ split S2, left parasternal heave

A

Pulmonary Stenosis

107
Q

crescendo-decrescendo systolic murmur upper right sternal border, radiating to carotid arteries

A

aortic stenosis (bicuspid aortic valve)

108
Q

fixed widely split S2, pulmonary hypertension,

A

Atrial Septal Defect

109
Q

extracardiac left-right shunt, continuous machine-like murmur

A

PDA

110
Q

holosystolic murmur at lower left sternal border + palpable thrill

A

VSD

111
Q

infant, recurrent episodes of severe abdominal pain, nausea, vomiting, bloody/mucoid stools,

A

Dx: Intussusception
DGx: history, exam, imaging
Tx: contrast enema- diagnostic/therapeutic, surgery

112
Q

female, pain in lower abdomen/pelvis, nausea, diarrhea, headaches, flushing,

A

Dx: primary dysmenorrhea
DGx: clinical, exclusion
Tx: NSAIDS, topical heat, oral contraceptives + progestin IUD

113
Q

Definitive Treatment of SCFE

A

Physis stabilization with screw fixation

114
Q

microthrombocytopenia, eczema, recurrent infections

A

Wiskott- Aldrich Syndrome- X-linked recessive defect in WAS protein- impaired cytoskeleton
Tx: stem cell transplant

115
Q

What increases the risk of shoulder dystocia (brachial nerve palsy/clavicle fractures) during birth

A

Gestational Diabetes, hyperglycemia + Fetal hyperglycemia

116
Q

post- birth what can happen due to withdrawal of maternal hormones

A

neonatal withdrawal bleeding in females via vagina

117
Q

Tx: child, pruritic vulva, labial adhesions

A

estrogen cream

118
Q

Tx of neonatal clavicular fractures

A

Reassurance, Gentle Handling, Analgesics, Long-sleeve garment, elbow flexed at 90

119
Q

Dx + DGx + Tx: macrocytic anemia, webbed neck, cleft palate, mild hypertelorism, flat nasal bridge, pale mucous membranes, triphalangeal thumbs

A

Dx: Diamond-Blackfan Anemia
DGx: Clinical + Lab
Tx: corticosteroids + RBC transfusions

120
Q

Dx: pigmented peri oral macules, GI hamartomatous polyps, abdominal pain, anemia of chronic bledding, rectal prolapse

A

Peutz-Jeghers Syndrome
Genetic Testing
Annual Anemia Screening
Upper/Lower Endoscopy

121
Q

Mother with HBsAg positive, Anti-HBsAg negative, IgM Anti-HBcAg negative, and Anti- HBcAg positive, how to treat neonate

A

Administer Hep B IG and Hep B Vaccine

122
Q

child, lethargy + AMS, post- URI, —> nausea, vomit, hepatomegaly, diffuse cerebral edema

A

Reye’s Syndrome- Aspirin for viral illness–> cerebral edema
Tx: supportive, but no aspirin for kids

123
Q

NF1 vs NF2

A

NF1 - neurofibromin, Chromosome 17- Cafe au Lait Spots, multiple neurofibromas, optic pathway glioma, lisch nodules

NF2- merlin- Chromosone 22, Bilateral Acoustic neuromas

124
Q

nasal sinusitis, unilateral, foul-smelling purulent, bloody

A

nasal foreign body–> mechanical extraction/positive pressure expulsion

125
Q

treatment of Left Ventricular Outflow Obstruction

A

Propranolol

SHELF EXAMS (3 decks)

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