MTB - Pediatrics Flashcards
1
Q
Routine newborn screening tests
A
PKU
Galactosemia
Hypothyroidism
2
Q
erythema toxicum
A
firm, white-yellow pustules with erythematous base, peak on 2nd DOL; self-limited
3
Q
Sturge Weber syndrome
A
AV malformation causing port wine stain along with seizures, MR and glaucoma
4
Q
Tx. port wine stain
A
pulsed laser therapy
5
Q
Tx. hemangioma
A
steroids or pulsed laser therapy if large or interferes with organ function; normally involutes by age 9
6
Q
what are pre-auricular tags/pits associated with?
A
Hearing loss
Genitourinary abnormalities
7
Q
what tests should be ordered if baby has preauricular tags/pits?
A
Hearing Test
Kidney USG
8
Q
Coloboma of the Iris
A
defect in the iris; assoc. with CHARGE
9
Q
CHARGE
A
Coloboma Heart Defects Atresia of nasal coanae Retarded growth Genitourinary abnormalities Ear abnormalities
10
Q
Aniridia - what is it and what is it assoc. with
A
Absence of Iris
Assoc. with Wilms tumor
11
Q
Screening for Wilms tumor
A
abdominal USG Q3 months until age 8
12
Q
Dx. neck mass, lateral to the midline
A
Branchial Cleft Cyst
- remnant of embryonic development
13
Q
Mngmt. Branchial Cleft Cyst
A
Infected? abx
Large? surgery
14
Q
Dx. neck mass in the midline with moves with swallowing or tongue protrusion
A
thyroglossal duct cyst
15
Q
Tx. thyroglossal duct cyst
A
- Surgery
2. Get thyroid scans and TFTs pre-op
16
Q
what is an umbilical hernia in a newborn assoc. with
A
congenital hypothyroidism
17
Q
what screening test should be done if baby has umbilical hernia
A
TSH
18
Q
Management: Cryptorchidism
A
No tx until age 1
- hormones - bhcg or testosterone
- orchiopexy
19
Q
what is hypospadias assoc. with
A
undescended testes
inguinal hernias
20
Q
Mngmt. Epispadias
A
Surgical eval. for bladder exstrophy
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
22
Q
Lab abnormalities in an Infant of a Diabetic Mother
A
hypoglycemia hypocalcemia hypomagnesemia hyperbilirubinemia polycythemia
23
Q
IODM is assoc. with
A
- cardiac abnormalities (VSD, ASD, truncus)
2. small left colon syndrome
24
Q
best initial test for resp. distress in newborn
A
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)
