MTB - Pediatrics Flashcards Preview

Step 3 > MTB - Pediatrics > Flashcards

Flashcards in MTB - Pediatrics Deck (242):
1

Routine newborn screening tests

PKU
Galactosemia
Hypothyroidism

2

erythema toxicum

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

3

Sturge Weber syndrome

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

4

Tx. port wine stain

pulsed laser therapy

5

Tx. hemangioma

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

6

what are pre-auricular tags/pits associated with?

Hearing loss
Genitourinary abnormalities

7

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

Hearing Test
Kidney USG

8

Coloboma of the Iris

defect in the iris; assoc. with CHARGE

9

CHARGE

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

10

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

Absence of Iris
Assoc. with Wilms tumor

11

Screening for Wilms tumor

abdominal USG Q3 months until age 8

12

Dx. neck mass, lateral to the midline

Branchial Cleft Cyst
- remnant of embryonic development

13

Mngmt. Branchial Cleft Cyst

Infected? abx
Large? surgery

14

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

thyroglossal duct cyst

15

Tx. thyroglossal duct cyst

1. Surgery
2. Get thyroid scans and TFTs pre-op

16

what is an umbilical hernia in a newborn assoc. with

congenital hypothyroidism

17

what screening test should be done if baby has umbilical hernia

TSH

18

Management: Cryptorchidism

No tx until age 1
1. hormones - bhcg or testosterone
2. orchiopexy

19

what is hypospadias assoc. with

undescended testes
inguinal hernias

20

Mngmt. Epispadias

Surgical eval. for bladder exstrophy

21

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?

Infant of Diabetic mother

22

Lab abnormalities in an Infant of a Diabetic Mother

hypoglycemia
hypocalcemia
hypomagnesemia
hyperbilirubinemia
polycythemia

23

IODM is assoc. with

1. cardiac abnormalities (VSD, ASD, truncus)
2. small left colon syndrome

24

best initial test for resp. distress in newborn

CXR

25

best initial treatment for resp. distress in newborn

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

26

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

congenital heart defects --> cardiac causes

27

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

transient tachypnea of newborn

28

Dx. transient tachypnea of newborn

CXR

29

Signs of TTN on CXR

1. air trapping
2. fluid in fissures
3. perihilar streaking

30

Tx, TTN

min. O2

31

Tx. Meconium aspiration

PPV
High frequency ventilation
Nitric oxide therapy
ECMO

32

what dz's can cause meconium plugs

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

33

When is hyperbilirubinemia pathologic? (5)

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

34

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

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

35

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

cholestasis
- get LFTS and ultrasound

36

when do you use phototherapy for treatment of hyperbilirubinemia?

if bili is > 10-12 mg/dl

37

signs of kernicterus

hypotonia
seizures
opisthotonos
delayed motor skills
choreoathetosis
sensorineural hearing loss

38

what entails a sepsis work-up?

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

39

MCC of early onset sepsis (first 24 hours)

pneumonia
- Group B strep
- E.coli
- H.flu
- Listeria

40

MCC of late onset sepsis (> 7 days)

meningitis and bacteremia
- Staph aureus
- E.coli
- Klebsiella
- Pseudomonas

41

Tx. neonatal sepsis

ampicillin + gentamicin until 48-72 hr cultures negative
if meningitis, add cefotaxime

42

MC extraneural complication of myelomeningocele

involvement of genitourinary system (bladder dysfunction)

43

extraneural complications of myelomeningocele

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

44

genera diagnostic test for all TORCHES infections

elevated total cord blood IgM

45

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?

seizures

46

Diagnostic Workup in Neonatal Seizures

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

47

Tx. acute neonatal seizures

lorazepam or diazepam rectally

48

which drugs cause respiratory and CNS depression in the newborn?

Anesthetics
Barbiturates
Mg sulfate (resp)

49

Effect of phenobarbital on neonate

vitamin K deficiency

50

Effect of sulfonamides on neonate

displace bilirubin from albumin = hyperbili

51

Effect of NSAIDs on neonate

premature closure of PDA

52

Effect of ACEI on neonate

craniofacial abnormalities

53

Effect of isotretnoin on neonate

facial /ear anomalies
CHD

54

Effect of Phenytoin on neonate

Hypoplastic nails
Typical Facies
IUGR

55

Effect of warfarin on neonate

facial dysmorphism
chondrodysplasia

56

Diagnostic W/U in baby born with trisomy 21

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

57

Dx. W/U in baby born with trisomy 18

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

58

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

1. ECHO
2. Renal USG - polycystic kidneys

59

WAGR syndrome

Wilms tumor
Aniridia
GU anomalies
Retardation

60

Dx. W/U in baby born with Klinefelters

Testosterone levels - hypogonadism and hypogenitalism
- may require testosterone replacement at age 11-12

61

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

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

62

what psych disorder are patients with Fragile X prone to?

ADHD

63

MCC of mental retardation in boys

Fragile X syndrome

64

CF: Beckwith Wiedemann syndrome

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

65

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

increased risk of abdominal tumors
--> US and serum AFP every 6 months through 6 yo to look for Wilms tumor and hepatoblastoma

66

CF. Angelmann syndrome

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

67

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

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

68

best indicator for acute malnutrition

height and weight < 5th percentile

69

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

GH or thyroid hormone deficiency
excessive cortisol secretion
skeletal dysplasias

70

C/I to breast feeding

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

71

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

12 months

72

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

18 months

73

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

36 months (3 yo)

74

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

9 months

75

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

15 months

76

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

24 months

77

up to what age is bedwetting "normal"

age 5

78

Enuresis

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

79

diurnal enuresis

MC among girls; higher rate of UTIs
MCC - UTI, DI, seizure, constipation, abuse

80

best initial test for enuresis

urinalysis

81

best initial therapy for enuresis

behavioral therapy
- if this fails: imipramie, desmopressin

82

Encopresis

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

83

best initial test: encopresis

AXR
- distinguishes between retentive and nonretentive

84

best initial therapy encopresis

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

85

C/I to MMR vaccine

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

86

egg allergy contraindicates to what vaccine

yellow fever only
- MMR no
- influenza: give inactivated vaccine

87

Measles exposure prophylaxis in:
1) 0-6 month old
2) 6-12 month old
3) > 12 months old

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

88

hep b prophylaxis in infant born to hep b positive mom

HBIG + vaccine at birth, 1 month and 6 months

89

details about DTap Vaccine

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

90

meningococcal conjugate vaccine

given at age 11-12 or at age 15

91

preferred method of identifying small joint effusions

USG

92

transient synovitis of the hip

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

93

what antibiotic is linked to pyloric stenosis

erythromycin
- also usage of macrolides in breast feeding women

94

fatigued child with difficulty breathing and apneic spells

bronchiolitis

95

precocious pseudo-puberty

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

96

sequence of events in precocious puberty due to hypothalamic dysfunction

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

97

manifestation of 47 XYY karyotype

severe acne

98

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

gag reflex --> frequently impaired

99

abx active against pseudomonas

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

100

neonatal polycythemia

peripheral venous Htc > 65%

101

tx. neonatal polycythemia

adequate hydration
partial exchange transfusion

102

pathogenesis of infant botulism

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

Tx. infant botulism

human derived botulism immune globulin

104

when do you use equine-derived botulinum antitoxic

foodborne or wound botulism; usually avoided in infants

105

management of bronchiolitis case

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

106

standard of diagnosis for bronchiolitis

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

complications of RSV bronchiolitis

apnea (esp. < 2 mos old)
respiratory failure

108

RSV bronchiolitis associated with development of what disease(s)

AOM in 20% of children
reactive airway disease in 30% of children

109

idiopathic thrombocytopenic purpura in children - management

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

110

Tx. lead poisoning with levels between 45-69

DMSA (Succimer) - PO
if they cannot tolerate PO --> IV EDTA

111

Tx. lead poisoning with levels > 70

IM Dimercaprol PLUS IV EDTA

112

Earliest sign of puberty in males

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

constitutional pubertal delay

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

114

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

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

115

treatment: SCFE

immediate internal fixation with a single screw

116

concurrent otitis media and purulent conjunctivitis

nontypeable H.flu infection

117

adenovirus infection

upper respiratory sx
nonpurulent conjunctivitis
gastroenteritis
otitis media

118

MCC of acute otitis externa

pseudomonas

119

treatment: AOM

first line: 10 days, high dose amoxicillin
second line: if recurrence w/in same month - amoxicillin-clavulanic acid

120

diagnostic criteria for NF1 (2 or more of):

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

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

immediate ophthalmologic evaluation

122

C/I to DTaP vaccine

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

123

goal of treating strep pharyngitis with antibiotics

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

124

clinical manifestations of refeeding syndrome

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

125

what electrolyte abnormalities are common in refeeding syndrome

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

126

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

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

127

varicella vaccination of household contacts of transplant recipients

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

128

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

MCC - strep pneumo
Tx. high dose amoxicillin

129

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

MCC - mycoplasma
Tx. azithromycin

130

proper management of child < 24 mo with first UTI

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

131

indications for voiding cystourethrogram

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

Tx. bacterial conjunctivitis

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

133

Tx. cryptorchidism

orchiopexy before age 1
- if not descending by 6 months unlikely to descend

134

complications of cryptorchidism

inguinal hernias
testicular torsion
subfertility
testicular cancer

135

Tx. Croup

1. humidified oxygen
2. nebulized epinephrine and corticosteroids

136

Management Epiglotitis

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

137

DIff. between tracheitis and epiglotitis

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

MCC of tracheitis and treatment

Staph aureus
Tx. anti-staph abx

139

Tx. angioedema

steroids, epinephrine

140

MC sites of foreign body aspiration
1. children < 1 yo
2. children > 1 yo

1. trachea, right mainstem bronchus
2. larynx

141

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

bronchoscopy

142

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

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

143

best prevention against bronchiolitis

breast feeding
- colustrum particularily

144

who should receive pavalizumab prophylaxis

pts with bronchopulmonary dysplasia
preterm infants

145

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?

Chlamydia trachomatis
Tx. erythromycin 14 days

146

outpatient management of pneumonia in children

amoxicillin
alt. cefuroxime, amoxi-clav

147

inpatient management of pneumonia in children

IV cefuroxime
- if staph aureus: add Vanco
- if complicated (empyema, abscess): add Clinda

148

criteria for hospitalization for pneumonia in children

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

149

best initial test and most specific to diagnose CF

sweat chloride test
- 2 elevated levels (>60) on seperate days

150

signs and symptoms that may warrant a CF work-up

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

newborn screening for CF

determines level of immunoreactive trypsinogen

152

Ivacaftor

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

153

what treatments in CF have been shown to improve survivial

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

154

antibiotics to treat mild CF

macrolides
TMP-SMX
Ciprofloxacin

155

antibiotics to treat documented infection with pseudomonas in CF pt

Zosyn
Ceftazidime

156

antibiotics used to treat resistant pathogens in CF

inhaled tobramycin

157

MC symptoms of acyanotic CHD

congestive heart failure

158

MC acyanotic CHD

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

159

when will CHDs that rely on a patent ductus typically present

within 1 month of life

160

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

at 2-6 months

161

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

congenital heart disease

162

signs of CHD in infants

feeding difficulty
sweating while feeding
rapid respirations
easy fatigue

163

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

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

164

murmurs that may suggest CHD

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

165

features of an innocent murmur

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

166

best initial test for dx. CHD

CXR and EKG

167

most specific test for dx CHD

echo

168

increased pulmonary vascular markings are seen in which CHD?

transposition of great arteries
hypoplastic left heart syndrome
truncus arteriosus

169

harsh, holosystolic murmur over LLSB +/- thrill
loud pulmonic S2

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

170

surgical repair of VSD - indications

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

171

loud S1, wide fixed splitting of S2
systolic ejection murmur along LUSB

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

172

which types of ASD require surgery

primary and sinus types of ASD
PFO if paradoxical embolus gone through

173

Tx. pulmonary stenosis

PGE1 infusion after birth
Attempt balloon valvuloplasty

174

who should receive abx prophylaxis in endocarditis

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

175

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

renal etiology

176

best initial anti-hypertensive meds in children

diuretic or BB

177

MCC of acute diarrhea in infancy

rotavirus

178

MCC of bloody diarrhea in children

campylobacter
ameoba (E.histolytica)
Shigella
E.coli
Salmonella

179

Best initial test in acute diarrhea

Stool examination for:
- cultures with blood and leukocytes
- C.diff toxin
- ova/parasites

180

Tx. Shigella

Trimethoprim/Sulfamethoxazole

181

Tx. campylobacter

self limiting
- erythromycin may speed recovery and reduce carrier state

182

Tx. Salmonella

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

183

Tx, E. Histolytica or Giardia

Metronidazole

184

HUS

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

Tx. HUS

supportive
HTN -> acute = CCB, chronic = ACEi

186

when do you give a transfusion of PRBCs in HUS

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

187

indications for dialysis in HUS

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

188

Dx. giardiasis as a cause of chronic malabsorption

duodenal aspirate/ biopsy or immunoassay

189

Fat malabsorption
- best initial screen
- best confirmatory test

1. Sudan black stain
2. 72 hour fecal stool fat (gold standard)

190

best initial test for protein malabsorption

spot stool alpha 1 anti-trypsin level

191

best initial diagnostic test for celiac disease

antiendomysial and antigliadin antibodies

192

most specific test for celiac disease

histology on biopsy

193

best initial test in malrotation/volvulus
next step in management?

1. Abdominal USG or barium enema
2. emergent surgery

194

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

Tc 99m pertechnetate scan

195

Intussusception
1. best initial test
2. confirmatory/exclusion test
3. diagnostic/treatment test

1. plain XR
2. abdominal US
3. air enema

196

Tx. cystitis (pediatrics)

TMP-SMX
Amoxicillin

197

Tx. pyelonephritis

IV ceftriazone or
ampicillin + gentamycin

198

antibiotic prophylaxis for VUR

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

199

post-traumatic hematuria

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

200

DOC in acute post-strep GN

penicillin
supportive care

201

Dx. tests to order in acute post-strep GN

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

202

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

IgA Nephropathy

203

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

Alport's disease

204

MC form of persistent proteinuria in children

orthostatic proteinuria
- rule this out before any other evaluation is done

205

CF: Nephrotic syndrome

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

206

Tx. Nephrotic syndrome

1. supportive care: Na and fluid restriction
2. Oral prednisone

207

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

cyclophosphamide
cyclosporine
high dose pulsed methylpred

208

MC complications of nephrotic syndrome

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

209

CF: CAH in an infant

ambiguous female genitalia
hyperkalemia
hyponatremia
hypoglycemia
dehydration
vomiting

210

CAH
1) best initial test
2) definitive diagnostic test

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

211

Tx. CAH

hydrocortisone
fludrocortisone if salt losing

212

Management: Kawasaki Dz

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

HSP is associated with..

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

214

Diagnostic Testing in HSP

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

Tx. HSP

supportive
1. if intestinal or renal complications --> steroids
2. if anticardiolipin or antiphospholipids +, give ASA

216

physiologic anemia in newborn

Term infants, nadir at 12 weeks (Hb 9-11)
Preterm infants, nadir is at 3-6 weeks (Hb 7-9)

217

therapy duration when treating iron deficiency anemia with ferrous sulfate

continue for 8 weeks after blood value normalizes

218

when do you transfuse in cases of sickle cell anemia

symptomatic anemia --> SOB or chest pain

219

when do you do an exchange transfusion in sickle cell

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

220

Indications for Hydroxyurea

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

221

Routine care in Sickle Cell patients

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

222

CF: beta thalassemia major

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

223

Best initial and most specific test for beta thalassemia major

Hemoglobin electrophoresis

224

Lab features in beta thalassemia major

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

225

Tx. Beta thalassemia major

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

226

Routine care in beta thalassemia

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

227

MCC of bleeding in children

Thrombocytopenia - platelet level should be first test in children

228

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

Deficiency of clotting factor

229

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

An inhibitor is present
- MC inhibitor within in hospital patients is heparin

230

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

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

231

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

Lupus anticoagulant is present

232

Tx. Hemophilia A

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

233

Tx. Hemophilia B

Major or minor bleeding tx with factor IX concentrates

234

Tx. Von willebrands disease

Minor bleeding: desmopressin
Major bleeding: plasma derived vWF concentrates with factor VIII

235

Tx. Of ITP in children

If skin sx only - no treatment, observe only
If bleeding - 1) prednisone 2) IVIG

236

Chronic ITP

Rituximab
Splenectomy - refractory cases only

237

Dx. Criteria febrile seizure

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

238

Long term prognosis in febrile seizures

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

239

Alternative to ethosuximide in tx of absence seizures

Valproic acid

240

Juvenile myoclonic epilepsy

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

241

West syndrome

Infantile spasms during first year
Hypsarrhythmia - high voltage slow waves, irregularly interspersed with spikes and sharp waves

242

Tx. west syndrome

ACTH
Prednisone
Vigabatrin
Vit B6. (Pyridoxine)