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Master the Boards Step 3 > MTB - Pediatrics > Flashcards

MTB - Pediatrics Flashcards

1
Q

Routine newborn screening tests

A

PKU
Galactosemia
Hypothyroidism

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2
Q

erythema toxicum

A

firm, white-yellow pustules with erythematous base, peak on 2nd DOL; self-limited

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3
Q

Sturge Weber syndrome

A

AV malformation causing port wine stain along with seizures, MR and glaucoma

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4
Q

Tx. port wine stain

A

pulsed laser therapy

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5
Q

Tx. hemangioma

A

steroids or pulsed laser therapy if large or interferes with organ function; normally involutes by age 9

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6
Q

what are pre-auricular tags/pits associated with?

A

Hearing loss

Genitourinary abnormalities

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7
Q

what tests should be ordered if baby has preauricular tags/pits?

A

Hearing Test

Kidney USG

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8
Q

Coloboma of the Iris

A

defect in the iris; assoc. with CHARGE

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9
Q

CHARGE

A 
Coloboma
Heart Defects
Atresia of nasal coanae
Retarded growth
Genitourinary abnormalities
Ear abnormalities
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10
Q

Aniridia - what is it and what is it assoc. with

A

Absence of Iris

Assoc. with Wilms tumor

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11
Q

Screening for Wilms tumor

A

abdominal USG Q3 months until age 8

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12
Q

Dx. neck mass, lateral to the midline

A

Branchial Cleft Cyst

- remnant of embryonic development

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13
Q

Mngmt. Branchial Cleft Cyst

A

Infected? abx

Large? surgery

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14
Q

Dx. neck mass in the midline with moves with swallowing or tongue protrusion

A

thyroglossal duct cyst

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15
Q

Tx. thyroglossal duct cyst

A
  1. Surgery

2. Get thyroid scans and TFTs pre-op

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16
Q

what is an umbilical hernia in a newborn assoc. with

A

congenital hypothyroidism

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17
Q

what screening test should be done if baby has umbilical hernia

A

TSH

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18
Q

Management: Cryptorchidism

A

No tx until age 1

  1. hormones - bhcg or testosterone
  2. orchiopexy
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19
Q

what is hypospadias assoc. with

A

undescended testes

inguinal hernias

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20
Q

Mngmt. Epispadias

A

Surgical eval. for bladder exstrophy

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21
Q

Large, newborn baby is jittery after his bath. On exam he is plethoric and tremulous; there is a pansystolic murmur heard. His delivery was complicated by shoulder dystocia - likely diagnosis?

A

Infant of Diabetic mother

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22
Q

Lab abnormalities in an Infant of a Diabetic Mother

A 
hypoglycemia
hypocalcemia
hypomagnesemia
hyperbilirubinemia
polycythemia
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23
Q

IODM is assoc. with

A
  1. cardiac abnormalities (VSD, ASD, truncus)

2. small left colon syndrome

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24
Q

best initial test for resp. distress in newborn

A

CXR

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25
Q

best initial treatment for resp. distress in newborn

A
  1. oxygen
  2. give nasal CPAP if O2 requirements are high enough
  3. consider empiric abx
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26
Q

in a newborn with resp. distress, if hypoxia does not improve with O2 what should you be considering?

A

congenital heart defects –> cardiac causes

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27
Q

Term newborn that was delivered by C/S presents with tachypnea - dx?

A

transient tachypnea of newborn

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28
Q

Dx. transient tachypnea of newborn

A

CXR

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29
Q

Signs of TTN on CXR

A
  1. air trapping
  2. fluid in fissures
  3. perihilar streaking
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30
Q

Tx, TTN

A

min. O2

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31
Q

Tx. Meconium aspiration

A

PPV
High frequency ventilation
Nitric oxide therapy
ECMO

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32
Q

what dz’s can cause meconium plugs

A
  1. CF
  2. Small left colon (IODM)
  3. Hirschsprung
  4. Maternal drug abuse
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33
Q

When is hyperbilirubinemia pathologic? (5)

A
  1. first DOL
  2. bilirubin rises > 5 mg/dl/day
  3. bilirubin > 12 mg/dl in term infant
  4. lasts greater than 2 weeks old
  5. direct (CB) bili > 2 mg/dl at any time
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34
Q

what should you consider if there is prolonged jaundice (>2 weeks) and NO CB?

A
  1. UTI /infection
  2. bilirubin conjugation defects
  3. hemolysis
  4. intrinsic RBC mb or enzyme defects
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35
Q

what should you consider if there is prolonged jaundice and elevated CB?

A

cholestasis

- get LFTS and ultrasound

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36
Q

when do you use phototherapy for treatment of hyperbilirubinemia?

A

if bili is > 10-12 mg/dl

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37
Q

signs of kernicterus

A 
hypotonia
seizures
opisthotonos
delayed motor skills
choreoathetosis
sensorineural hearing loss
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38
Q

what entails a sepsis work-up?

A
  1. CBC w/ diff
  2. blood culture
  3. urinalysis/culture
  4. CXR prior to antibiotics
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39
Q

MCC of early onset sepsis (first 24 hours)

A

pneumonia

  • Group B strep
  • E.coli
  • H.flu
  • Listeria
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40
Q

MCC of late onset sepsis (> 7 days)

A

meningitis and bacteremia

  • Staph aureus
  • E.coli
  • Klebsiella
  • Pseudomonas
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41
Q

Tx. neonatal sepsis

A

ampicillin + gentamicin until 48-72 hr cultures negative

if meningitis, add cefotaxime

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42
Q

MC extraneural complication of myelomeningocele

A

involvement of genitourinary system (bladder dysfunction)

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43
Q

extraneural complications of myelomeningocele

A
  1. bladder dysfunction
  2. GI tract dysfunction - fecal incontinence due to external anal sphincter dysfunction
  3. fractures of lower extremities
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44
Q

genera diagnostic test for all TORCHES infections

A

elevated total cord blood IgM

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45
Q

in NICU, infant noted to be jittery and has repetitive sucking movements, tongue thrusting and brief apneic spells; the jitteriness fails to subside with stimulus - dx?

A

seizures

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46
Q

Diagnostic Workup in Neonatal Seizures

A
  1. EEG
  2. CBC, electrolytes, calcium, Mg, glucose
  3. Amino acid assay and urine organic acids
  4. Infectious causes:
    - TORCH cord blood IGM, bcx, urine cx, LP
  5. if preterm, USG of head to look for IVH
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47
Q

Tx. acute neonatal seizures

A

lorazepam or diazepam rectally

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48
Q

which drugs cause respiratory and CNS depression in the newborn?

A

Anesthetics
Barbiturates
Mg sulfate (resp)

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49
Q

Effect of phenobarbital on neonate

A

vitamin K deficiency

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50
Q

Effect of sulfonamides on neonate

A

displace bilirubin from albumin = hyperbili

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51
Q

Effect of NSAIDs on neonate

A

premature closure of PDA

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52
Q

Effect of ACEI on neonate

A

craniofacial abnormalities

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53
Q

Effect of isotretnoin on neonate

A

facial /ear anomalies

CHD

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54
Q

Effect of Phenytoin on neonate

A

Hypoplastic nails
Typical Facies
IUGR

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55
Q

Effect of warfarin on neonate

A

facial dysmorphism

chondrodysplasia

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56
Q

Diagnostic W/U in baby born with trisomy 21

A
  1. Hearing exam
  2. ECHO
  3. GI - TEF, duodenal atresia
  4. TSH - hypothyroidism
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57
Q

Dx. W/U in baby born with trisomy 18

A
  1. ECHO

2. Renal USG - polcystic kidneys, ectopic/double ureter

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58
Q

Dx. W/U in baby born with trisomy 13 (Patau)

A
  1. ECHO

2. Renal USG - polycystic kidneys

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59
Q

WAGR syndrome

A

Wilms tumor
Aniridia
GU anomalies
Retardation

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60
Q

Dx. W/U in baby born with Klinefelters

A

Testosterone levels - hypogonadism and hypogenitalism

- may require testosterone replacement at age 11-12

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61
Q

Dx. W/U in baby born with Turner’s syndrome

A
  1. renal US: horseshoe kidney, double renal pelvis
  2. Cardiac US: bicuspid aortic valve, coarctation of aorta
  3. TFTs: hypothyroidism
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62
Q

what psych disorder are patients with Fragile X prone to?

A

ADHD

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63
Q

MCC of mental retardation in boys

A

Fragile X syndrome

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64
Q

CF: Beckwith Wiedemann syndrome

A 
macrosomia
macroglossia
pancreatic B cell hyperplasia (hypoglycemia)
large kidneys
neonatal polycythemia
omphalocele
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65
Q

Dx. W/U in baby born with Beckwith Weideman syndrome

A

increased risk of abdominal tumors

–> US and serum AFP every 6 months through 6 yo to look for Wilms tumor and hepatoblastoma

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66
Q

CF. Angelmann syndrome

A

MR
inappropriate laughter
absent speech or < 6 words
ataxia and jerky arm movements (puppet gait)
recurrent seizures (80% chance of epilepsy)

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67
Q

Pierre Robin sequence - associations (1), CF (2) and diagnostic W/U (3)

A
  1. fetal alcohol syndrome, Edwards syndrome
  2. mandibular hypoplasia, cleft palate
  3. monitor airway for obstruction over first 4 weeks
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68
Q

best indicator for acute malnutrition

A

height and weight < 5th percentile

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69
Q

DDX for normal weight gain in a child but decrease length/height

A

GH or thyroid hormone deficiency
excessive cortisol secretion
skeletal dysplasias

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70
Q

C/I to breast feeding

A
  1. Infections: HIV, CMV, HSV if lesions on breast, HBV until infant is immunized, TB
  2. Breast cancer
  3. Substance abuse
  4. Drugs: antineoplastics, iodide/mercurials, lithium, chloramphenicol, nicotine, alcohol
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71
Q

baby age: cruising, says 1 or more words, plays ball

A

12 months

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72
Q

baby age: builds 4 cube tower, walks down stairs, say 10 words and can feed self

A

18 months

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73
Q

Baby age: can walk downstairs alternating feet, rides tricycle, knows age and sex and understands taking turns

A

36 months (3 yo)

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74
Q

baby age: has pincer grasp, creeps and crawls, knows own name

A

9 months

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75
Q

baby age: builds 3 cube tower, walks alone, makes lines and scribbles

A

15 months

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76
Q

baby age: builds 7 cube toward, runs well, goes up and down stairs, jumps with two feet, threads shoelaces, handles spoon, says 2-3 sentences

A

24 months

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77
Q

up to what age is bedwetting “normal”

A

age 5

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78
Q

Enuresis

A

involuntary voiding of urine, occuring atleast 2x/week for atleast 3 months in children over age 5

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79
Q

diurnal enuresis

A

MC among girls; higher rate of UTIs

MCC - UTI, DI, seizure, constipation, abuse

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80
Q

best initial test for enuresis

A

urinalysis

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81
Q

best initial therapy for enuresis

A

behavioral therapy

- if this fails: imipramie, desmopressin

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82
Q

Encopresis

A

unintentional or involuntary passage of feces in inappropriate settings, in children > 4 yo

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83
Q

best initial test: encopresis

A

AXR

- distinguishes between retentive and nonretentive

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84
Q

best initial therapy encopresis

A

retentive: disimpaction, stool softeners, behavioral modification
non retentive: behavioral modification

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85
Q

C/I to MMR vaccine

A 
anaphylaxis to neomycin or gelatin
pregnancy
immunodeficient state
thrombocytopenia
recent IVIG treatment
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86
Q

egg allergy contraindicates to what vaccine

A

yellow fever only

  • MMR no
  • influenza: give inactivated vaccine
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87
Q

Measles exposure prophylaxis in:

1) 0-6 month old
2) 6-12 month old
3) > 12 months old

A
  1. IG only
  2. IG + vaccine with booster at 12-15 months
  3. vaccine only w/in 72 hours of exposure
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88
Q

hep b prophylaxis in infant born to hep b positive mom

A

HBIG + vaccine at birth, 1 month and 6 months

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89
Q

details about DTap Vaccine

A

total 5 doses prior to school (last dose 4-6 years)
TdaP booster once in adolescence
Td at age 11-12 and every 10 years

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90
Q

meningococcal conjugate vaccine

A

given at age 11-12 or at age 15

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91
Q

preferred method of identifying small joint effusions

A

USG

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92
Q

transient synovitis of the hip

A

young boy presents with pain in the hip and refusal to walk; he is febrile. P/E: one hip is kept externally s

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93
Q

what antibiotic is linked to pyloric stenosis

A

erythromycin

- also usage of macrolides in breast feeding women

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94
Q

fatigued child with difficulty breathing and apneic spells

A

bronchiolitis

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95
Q

precocious pseudo-puberty

A

early onset puberty caused by a gonadotrophin-independent process, typically an excess of sex steroids.
- may be caused by late onset congenital adrenal hyperplasia

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96
Q

sequence of events in precocious puberty due to hypothalamic dysfunction

A

testicular enlargement –> penis enlargement –> pubic hair growth –> growth spurt

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97
Q

manifestation of 47 XYY karyotype

A

severe acne

98
Q

you suspect infant botulism –> what do you look for on exam?

A

gag reflex –> frequently impaired

99
Q

abx active against pseudomonas

A 
cefepime, ceftazidime
amikacin
carbapenems
Zosyn
aztreonam
colistin
- should start two drugs when treating empirically for pseudomonas
100
Q

neonatal polycythemia

A

peripheral venous Htc > 65%

101
Q

tx. neonatal polycythemia

A

adequate hydration

partial exchange transfusion

102
Q

pathogenesis of infant botulism

A

ingestion of C. botulinum spores from environmental dust (california, PA and Utah - esp. in construction and farming sites) and from ingestion of raw honey

103
Q

Tx. infant botulism

A

human derived botulism immune globulin

104
Q

when do you use equine-derived botulinum antitoxic

A

foodborne or wound botulism; usually avoided in infants

105
Q

management of bronchiolitis case

A

respiratory isolation
trial of inhaled bronchodilators (albuterol/epinephrine)
steroids are NOT indicated

106
Q

standard of diagnosis for bronchiolitis

A

primarily clinical but can use;

  1. best initial test = CXR
  2. RSV antigen detection in nasal or pulm secretions (IFA or ELISA) [most accurate test]
    alt: NAAT testing
107
Q

complications of RSV bronchiolitis

A

apnea (esp. < 2 mos old)

respiratory failure

108
Q

RSV bronchiolitis associated with development of what disease(s)

A

AOM in 20% of children

reactive airway disease in 30% of children

109
Q

idiopathic thrombocytopenic purpura in children - management

A

symptomatic pts with mod-severe thrombocytopenia (<30 000) should be tx. with corticosteroids and/or IVIG

110
Q

Tx. lead poisoning with levels between 45-69

A

DMSA (Succimer) - PO

if they cannot tolerate PO –> IV EDTA

111
Q

Tx. lead poisoning with levels > 70

A

IM Dimercaprol PLUS IV EDTA

112
Q

Earliest sign of puberty in males

A

nocturnal increase in the LH surge followed by a daytime increase in the levels of gonadotrophins and testosterone levels; on exam, enlargement of the testes

113
Q

constitutional pubertal delay

A

delayed puberty (>14 yo)
retarded bone age
positive family hx
absence of any systemic disorders

114
Q

slipped capital femoral epiphysis - in children younger than age 10 is associated with what other disorders?

A

metabolic disorders, including hypothyroidism, panhypopituitarism, hypogonadism, renal osteodystrophy, GH abnormalities

115
Q

treatment: SCFE

A

immediate internal fixation with a single screw

116
Q

concurrent otitis media and purulent conjunctivitis

A

nontypeable H.flu infection

117
Q

adenovirus infection

A

upper respiratory sx
nonpurulent conjunctivitis
gastroenteritis
otitis media

118
Q

MCC of acute otitis externa

A

pseudomonas

119
Q

treatment: AOM

A

first line: 10 days, high dose amoxicillin

second line: if recurrence w/in same month - amoxicillin-clavulanic acid

120
Q

diagnostic criteria for NF1 (2 or more of):

A
  1. first degree relative with NF1
  2. > 6 CALS of 5 mm in greatest diameter (children) or 15 mm(adult)
  3. presence of >2 neurofibromas, Lisch nodules, optic glioma, bone dysplasia or axillary freckling
121
Q

if you suspect a diagnosis of NF1 in a child - what should you order next?

A

immediate ophthalmologic evaluation

122
Q

C/I to DTaP vaccine

A
  1. history of anaphylaxis to previous DTaP

2. history of encephalopathy within 7 days of admin of previous DTaP vaccine

123
Q

goal of treating strep pharyngitis with antibiotics

A

to reduce symptom duration and severity
decrease spread to close contacts
prevent acute rheumatic fever

124
Q

clinical manifestations of refeeding syndrome

A

arrhythmias
congestive heart failure (pulm edema, peripheral edema)
seizures
Wernicke encephalopathy

125
Q

what electrolyte abnormalities are common in refeeding syndrome

A
  1. decreased phosphorus, potassium and Mg
  2. decreased serum thiamine
  3. increased sodium and water retention
126
Q

max percentage of Hb A level in sickle cell beta (+) thalassemia

A

25% (vs. 60% in sickle cell trait)

127
Q

varicella vaccination of household contacts of transplant recipients

A

safe and recommended

- monitor for development of rash in patient - if a rash develops, isolate the patient

128
Q

preschool age pt presents with symptoms of pneumonia; CXR shows focal lung findings. MCC (1)? Treatment (2)

A

MCC - strep pneumo

Tx. high dose amoxicillin

129
Q

older child presents with symptoms of pneumonia. CXR shows bilateral interstitial infiltrates. MCC (1) Tx?

A

MCC - mycoplasma

Tx. azithromycin

130
Q

proper management of child < 24 mo with first UTI

A

renal and bladder USG to evaluate for anatomic abnormalities; tx. with 10-14 days of abx and monitor closely for recurrence

131
Q

indications for voiding cystourethrogram

A

1) abnormal findings on USG i.e. hydronephrosis, VUR, renal scarring, obstructive uropathy
2) recurrent UTIs (>2 febrile UTIs)
3) first febrile UTI is any of these are present: fam hx of renal dz, poor growth, HTN or organism other than E.coli

132
Q

Tx. bacterial conjunctivitis

A

erythromycin ointment
sulfa drops
polymyxin/trimethoprim
FQs should be reserved for contact lens wearers

133
Q

Tx. cryptorchidism

A

orchiopexy before age 1

- if not descending by 6 months unlikely to descend

134
Q

complications of cryptorchidism

A

inguinal hernias
testicular torsion
subfertility
testicular cancer

135
Q

Tx. Croup

A
  1. humidified oxygen

2. nebulized epinephrine and corticosteroids

136
Q

Management Epiglotitis

A
  1. transfer to hospital/OR
  2. consult ENT /anesthesia
  3. Intubate
  4. Antibiotics –> Ceftriaxone and Steroids
  5. Consider rifampin prophylaxis for household contacts
137
Q

DIff. between tracheitis and epiglotitis

A

tracheitis does not have sx of drooling or dysphagia but does have brassy cough with fever and respiratory distress; epiglotitis does not have a cough

138
Q

MCC of tracheitis and treatment

A

Staph aureus

Tx. anti-staph abx

139
Q

Tx. angioedema

A

steroids, epinephrine

140
Q

MC sites of foreign body aspiration

  1. children < 1 yo
  2. children > 1 yo
A
  1. trachea, right mainstem bronchus

2. larynx

141
Q

next step in management in suspected foreign body aspiration in pt with respiratory distress

A

bronchoscopy

142
Q

recurrent pulm infections in a young child should raise suspicion for…

A

previously undiagnosed aspiration

- get a CXR to look for post-obstruction atelectasis or visualize foreign body

143
Q

best prevention against bronchiolitis

A

breast feeding

- colustrum particularily

144
Q

who should receive pavalizumab prophylaxis

A

pts with bronchopulmonary dysplasia

preterm infants

145
Q

2 month old infant presents with insiduous onset of nasal congestion and staccato cough. PMHX is positive for conjunctivitis at birth. Labs show peripheral eosinophilia - Dx? Tx?

A

Chlamydia trachomatis

Tx. erythromycin 14 days

146
Q

outpatient management of pneumonia in children

A

amoxicillin

alt. cefuroxime, amoxi-clav

147
Q

inpatient management of pneumonia in children

A

IV cefuroxime

  • if staph aureus: add Vanco
  • if complicated (empyema, abscess): add Clinda
148
Q

criteria for hospitalization for pneumonia in children

A 
O2 sat < 92%
dehydration
RR > 50 /min
toxic appearance
complications - empyema, effusion
failure of outpatient therapy
149
Q

best initial test and most specific to diagnose CF

A

sweat chloride test

- 2 elevated levels (>60) on seperate days

150
Q

signs and symptoms that may warrant a CF work-up

A
  1. meconium ileus
  2. failure to thrive (malabsorption)
  3. rectal prolapse
  4. persistent cough in first year of life w/ copious purulent mucus
  5. undescended testes, absent vas deferens (infertility)
  6. allergic bronchopulmonary aspergillosis
151
Q

newborn screening for CF

A

determines level of immunoreactive trypsinogen

152
Q

Ivacaftor

A

approved for patients with CF who are greater than 6 yo and carry atleast one copy of G551D mutation

153
Q

what treatments in CF have been shown to improve survivial

A
  1. Ibuprofen - reduces inflammatory lung response
  2. azithromycin - slows rate of decline of FEV1 in pts < 13 yo
  3. antibiotics during exacerbations
154
Q

antibiotics to treat mild CF

A

macrolides
TMP-SMX
Ciprofloxacin

155
Q

antibiotics to treat documented infection with pseudomonas in CF pt

A

Zosyn

Ceftazidime

156
Q

antibiotics used to treat resistant pathogens in CF

A

inhaled tobramycin

157
Q

MC symptoms of acyanotic CHD

A

congestive heart failure

158
Q

MC acyanotic CHD

A

VSD, ASD, PDA, AS, coarctation, AV canal, PS

159
Q

when will CHDs that rely on a patent ductus typically present

A

within 1 month of life

160
Q

when will infants with L–>R shunting lesions typically present

A

at 2-6 months

161
Q

Pt presents with shock, tachypnea and cyanosis. His cyanosis and hypoxemia do NOT respond to oxygen delivery - what should you be considering?

A

congenital heart disease

162
Q

signs of CHD in infants

A

feeding difficulty
sweating while feeding
rapid respirations
easy fatigue

163
Q

what abnormalities can be seen on exam in a pt with CHD

A
  1. UE HTN or LE hypotension
  2. decreased femoral pulses (L sided lesions)
  3. facial edema, hepatomegaly
  4. heart sounds
164
Q

murmurs that may suggest CHD

A 
pansystolic murmur
any grade > 3/6
PMI at upper left sternal border
harsh murmur
early midsystolic click
abnormal S2
165
Q

features of an innocent murmur

A
  1. fever, anxiety or infection in history
  2. systolic only
  3. grade < 2/6
166
Q

best initial test for dx. CHD

A

CXR and EKG

167
Q

most specific test for dx CHD

168
Q

increased pulmonary vascular markings are seen in which CHD?

A

transposition of great arteries
hypoplastic left heart syndrome
truncus arteriosus

169
Q

harsh, holosystolic murmur over LLSB +/- thrill

loud pulmonic S2

A

VSD

- >50% of cases close on their own w/in first 6 months

170
Q

surgical repair of VSD - indications

A

failure to thrive
pulmonary HTN
R–>L shunt > 2:1

171
Q

loud S1, wide fixed splitting of S2

systolic ejection murmur along LUSB

A

ASD

- majority are asx, secundum type and close by age 4

172
Q

which types of ASD require surgery

A

primary and sinus types of ASD

PFO if paradoxical embolus gone through

173
Q

Tx. pulmonary stenosis

A

PGE1 infusion after birth

Attempt balloon valvuloplasty

174
Q

who should receive abx prophylaxis in endocarditis

A
  1. prosthetic valves
  2. previous endocarditis
  3. CHD - unrepaired or persistent defect
  4. cardiac transplant pts with valve abnormalities
175
Q

in every pediatric patient presenting with HTN - what should you consider?

A

renal etiology

176
Q

best initial anti-hypertensive meds in children

A

diuretic or BB

177
Q

MCC of acute diarrhea in infancy

178
Q

MCC of bloody diarrhea in children

A 
campylobacter
ameoba (E.histolytica)
Shigella
E.coli
Salmonella
179
Q

Best initial test in acute diarrhea

A

Stool examination for:

  • cultures with blood and leukocytes
  • C.diff toxin
  • ova/parasites
180
Q

Tx. Shigella

A

Trimethoprim/Sulfamethoxazole

181
Q

Tx. campylobacter

A

self limiting

- erythromycin may speed recovery and reduce carrier state

182
Q

Tx. Salmonella

A

only if pt is < 3 months old, has disseminated disease or if patient has S.typhi

183
Q

Tx, E. Histolytica or Giardia

A

Metronidazole

184
Q

HUS

A

young child presents 5-10 days after E.coli diarrhea with pallor (anemia), weakness, oliguria and acute renal insufficiency. Labs show anemia, leukocytosis, negative Coomb’s, low platelets, hematuria and proteinuria

185
Q

Tx. HUS

A

supportive

HTN -> acute = CCB, chronic = ACEi

186
Q

when do you give a transfusion of PRBCs in HUS

A

if Hb < 6 and Hct < 18%; transfuse until Hb is between 8-9

187
Q

indications for dialysis in HUS

A
  1. sx of uremia
  2. BUN > 80
  3. fluid overload that cannot be managed medically
  4. hyperkalemia + acidosis
188
Q

Dx. giardiasis as a cause of chronic malabsorption

A

duodenal aspirate/ biopsy or immunoassay

189
Q

Fat malabsorption

  • best initial screen
  • best confirmatory test
A
  1. Sudan black stain

2. 72 hour fecal stool fat (gold standard)

190
Q

best initial test for protein malabsorption

A

spot stool alpha 1 anti-trypsin level

191
Q

best initial diagnostic test for celiac disease

A

antiendomysial and antigliadin antibodies

192
Q

most specific test for celiac disease

A

histology on biopsy

193
Q

best initial test in malrotation/volvulus

next step in management?

A
  1. Abdominal USG or barium enema

2. emergent surgery

194
Q

best initial test for intermittent, painless rectal bleeding in a toddler

A

Tc 99m pertechnetate scan

195
Q

Intussusception

  1. best initial test
  2. confirmatory/exclusion test
  3. diagnostic/treatment test
A
  1. plain XR
  2. abdominal US
  3. air enema
196
Q

Tx. cystitis (pediatrics)

A

TMP-SMX

Amoxicillin

197
Q

Tx. pyelonephritis

A

IV ceftriazone or

ampicillin + gentamycin

198
Q

antibiotic prophylaxis for VUR

A

TMP-SMX or nitrofurantoin

- use for first year following diagnosis of any grade VUR, esp. in younger infants to prevent kidney scarring

199
Q

post-traumatic hematuria

A

in a child, any level should be investigated with a CT scan

200
Q

DOC in acute post-strep GN

A

penicillin

supportive care

201
Q

Dx. tests to order in acute post-strep GN

A

UA: RBC, RBC casts, protein, PMNs
low C3
positive throat culture or ASO titre
most specific test: anti-DNase antigen

202
Q

adolescent patient presents with gross hematuria after URI or GI illness; W/u shows: mild proteinuria, HTN, normal C3 - DX?

A

IgA Nephropathy

203
Q

hearing difficulties and asymptomatic microscopic hematuria following URI; strong family history

A

Alport’s disease

204
Q

MC form of persistent proteinuria in children

A

orthostatic proteinuria

- rule this out before any other evaluation is done

205
Q

CF: Nephrotic syndrome

A 
Proteinuria (>40 mg/m2/hour)
Hypoalbuminemia (<2.5)
Edema
Hyperlipidemia
C3/C4 normal
206
Q

Tx. Nephrotic syndrome

A
  1. supportive care: Na and fluid restriction

2. Oral prednisone

207
Q

Tx. if a patient with nephrotic syndrome relapses after oral steroids

A

cyclophosphamide
cyclosporine
high dose pulsed methylpred

208
Q

MC complications of nephrotic syndrome

A
  1. infection - SBP; immunize against pneumococcus and varicella
  2. increased risk of thromboembolism
209
Q

CF: CAH in an infant

A 
ambiguous female genitalia
hyperkalemia
hyponatremia
hypoglycemia
dehydration
vomiting
210
Q

CAH

1) best initial test
2) definitive diagnostic test

A

1) increased 17OH progesterone

2) 17OH progesterone before and after IV bolus of ACTH

211
Q

Tx. CAH

A

hydrocortisone

fludrocortisone if salt losing

212
Q

Management: Kawasaki Dz

A
  1. IVIG + high dose ASA
  2. EKG, ECHO - get at baseline, at 2-3 weeks and 6-8 weeks
  3. add warfarin if platelets extremely high (1 million)
213
Q

HSP is associated with..

A

intussusception
arthritis
GN/nephrosis - monitor BP and UA weekly for first two months

214
Q

Diagnostic Testing in HSP

A
  1. increased plts, WBCs, ESR, IgA and IgM
  2. anemia
  3. anticardiolipin or antiphospholipid abs
  4. urine: RBCs, WBCs, casts, albumin
  5. definite dx: skin biopsy
215
Q

Tx. HSP

A

supportive

  1. if intestinal or renal complications –> steroids
  2. if anticardiolipin or antiphospholipids +, give ASA
216
Q

physiologic anemia in newborn

A

Term infants, nadir at 12 weeks (Hb 9-11)

Preterm infants, nadir is at 3-6 weeks (Hb 7-9)

217
Q

therapy duration when treating iron deficiency anemia with ferrous sulfate

A

continue for 8 weeks after blood value normalizes

218
Q

when do you transfuse in cases of sickle cell anemia

A

symptomatic anemia –> SOB or chest pain

219
Q

when do you do an exchange transfusion in sickle cell

A

life threatening complications i.e. stroke, acute chest syndrome, splenic crisis
before high risk surgeries

220
Q

Indications for Hydroxyurea

A
  1. > 3 episodes/crises per year
  2. symptomatic anemia
  3. life threatening complications
221
Q

Routine care in Sickle Cell patients

A
  1. Penicillin prophylaxis from 3 mos - 5 yo
  2. Immunizations
  3. Daily folate supplementation
222
Q

CF: beta thalassemia major

A

Progressive anemia, hypersplenism, cardiac decompensation
Expanded extra medullary spaces due to extra medullary hematopoiesis
Hepatosplenomegaly

223
Q

Best initial and most specific test for beta thalassemia major

A

Hemoglobin electrophoresis

224
Q

Lab features in beta thalassemia major

A

Severe microcytic anemia with markers of hemolysis (LDH, bilirubin, increased serum ferritin and transferrin)

225
Q

Tx. Beta thalassemia major

A
  1. Transfusion therapy to maintain Hb > 9g/dL
  2. Iron chelation therapy with deforaxamine plus vitamin C
  3. Splenectomy
  4. BMT is curative
226
Q

Routine care in beta thalassemia

A

Folate supplementation
Vaccinations
Growth hormone - excess iron is related to GH deficiency

227
Q

MCC of bleeding in children

A

Thrombocytopenia - platelet level should be first test in children

228
Q

In a mixing study, if lab prolongation is corrected….

A

Deficiency of clotting factor

229
Q

In mixing studies, if lab prolongation is not corrected….

A

An inhibitor is present

- MC inhibitor within in hospital patients is heparin

230
Q

In mixing studies, if it is more prolonged with clinical bleeding ….

A

Antibody against a clotting factor is present ESP factor VIII, IX OR XI

231
Q

In mixing study, if there is no clinical bleeding but both PTT and mixing study are prolonged…

A

Lupus anticoagulant is present

232
Q

Tx. Hemophilia A

A 
Minor bleeding: Desmopressin + aminocaproic acid or tranexamic acid
Major bleeding (into joints): replace factor VIII
233
Q

Tx. Hemophilia B

A

Major or minor bleeding tx with factor IX concentrates

234
Q

Tx. Von willebrands disease

A

Minor bleeding: desmopressin

Major bleeding: plasma derived vWF concentrates with factor VIII

235
Q

Tx. Of ITP in children

A

If skin sx only - no treatment, observe only

If bleeding - 1) prednisone 2) IVIG

236
Q

Chronic ITP

A

Rituximab

Splenectomy - refractory cases only

237
Q

Dx. Criteria febrile seizure

A 
6 mo - 6 yrs
Tmax > 38
No history of seizures
No CNS infection
No acute systemic metabolic cause
238
Q

Long term prognosis in febrile seizures

A

30-50% recurrence rate
<5% change of developing epilepsy
Normal development and intelligence

239
Q

Alternative to ethosuximide in tx of absence seizures

A

Valproic acid

240
Q

Juvenile myoclonic epilepsy

A

Jerky movements occuring in the morning in an adolescent
Irregular spike and wave pattern
Tx. Valproic acid

241
Q

West syndrome

A

Infantile spasms during first year

Hypsarrhythmia - high voltage slow waves, irregularly interspersed with spikes and sharp waves

242
Q

Tx. west syndrome

A

ACTH
Prednisone
Vigabatrin
Vit B6. (Pyridoxine)

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