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Anu Cards > AAP Question > Flashcards

AAP Question Flashcards

1
Q

HSV

A

Enveloped ds-dna viruses
3 categories
skin-eye-mucosa (25%)
disseminated (45%)
central nervous system (30%)

Treatment is IV acyclovir for 21 days for CNS disease and 14 days for isolated

Also recommended that they receive oral suppressive treatment for 6 months, should be monitored for acyclovir associated neutropenia

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2
Q

Inhaled nitric is contraindicated in

A

Left ventricular dysfunction

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3
Q

Pharmakokinetics
Pharmakodynamics

A

Zero order constant amount eliminated does not change with the volume of drug
First order constant percentage eliminated, elimination amount changes with volume of drug

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4
Q

Hyponatermia leads to poor growth by inhibiting the Na/H channel

A
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5
Q

Epinephrine role

A

system vasoconstricion
increases SVR and diastolic blood pressure
improving coronary perfusion
0.1-03ml/kg of 0.1ml/mg concentration

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6
Q

GALD

A

Immune mediated, sporadic occurrence
Maternal antibodies crossing the placenta and binding to hepatocytes inducing fetal liver injury
If previous child, risk of recurrence is 80-90% mother can get weekly IVIG 14 weeks, 16 weeks and then weekly at 18 weeks to lower clinical manifestations
liver biopsy will show hepatic necrosis
Diagnosis: Liver injury and can have extra hepatic source of siderosis (iron deposition) and can do salivary gland biopsy.
Clinical symptoms: Manifests as hypoglycemia and coaugulopathy a few hours after birth. Elevated Ferritin, Elevated AFP. Low AST/ALT.
Persistent ductus venous on ultrasound.

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7
Q

omphalocele

A

1:4000-7000 live births
Giant is greater than 5cm in diameter

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8
Q

Surgical nec mangement

A

No significant difference between peritoneal drainage compared to laparotomy

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9
Q

Alaagille syndrome

A

1 in 70,000 live births
neonatal cholestatis with bile duct paucity
congenital hear disease with peripheral pulmonary stenosis being the most common
dysmorphic facies
ocular posterior embryotoxin
renal and intracranial lesions

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10
Q

Glutamine

A

Glutamine is a primary substrate for enterocytes and is a primitive growth factor for intestinal cells.

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11
Q

CDH

A

With use of ultrasound most cases identified 16-24 weeks gestation
LHR is most reliable between 22-26 weeks gestation and observed to expected LHR < 25% is associated with less than 30% survival whereas >46% with more than 85% survival
fluid filled stomach in thorax

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12
Q

Disorders of abnormal UGT

A

Gilbert is decreased activity
Crigler is absent UGT

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13
Q

liver biopsy showing
Multinucleated giant cells

A

Neonatal idiopathic hepatitis

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14
Q

liver biopsy showing
Bile duct proliferation

A

Biliary Atresia

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15
Q

liver biopsy showing
necrotizing duct lesions

A

sclerosis cholangitits

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16
Q

liver biopsy showing intrahepatocytic globules

A

Alpha-1 antitrypsin deficiency
neonates can have severe liver disease due to protein misfiling and accumulation in the liver, this may resolve spontaneously
17% require transplantation

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17
Q

Biliary Atresia

A

Normal ultrasound does not rule out, needs intraoperative
50% need liver transplant before the age of 2
total bilirubin 3 months after Kasai is a reliable marker for transplant
10% may have other malformations

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18
Q

Choledochal cyst

A

Most are diagnosed in childhood jaundice and palpable abdominal mass
After resection risk of malignancy remains elevated
At risk for cholangitits after treatment

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19
Q

Hemaphogocytic Lyphohistocytosis

A

Low NK cell activity
Increase IL 2
Persistent febrile, splenomegaly, cytopenia, hypofibrinogen, hypertriglycerides, hyperferritin,hemophagocytosis,
Treatment is stem cell transplant

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20
Q

Hirshprung’s Disease

A

Rectosigmoid ration <1 (normal >1 )
Failure of caudal migration of neural crest cells at 8-10 weeks
No ganglion cells in rectum on suction biopsy.

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21
Q

Fat soluble vitamins should be supplemented in neonates with cholestatis

A
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22
Q

Role of citruilline measure in short bowel

A

Citrulline is a marker of enterocyte hass and short bowel function

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23
Q

Probiotics

A

Good ones are Lactobacillus and Bifidobacterium

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24
Q

Protein Digestion

A

PROTEIN
-pepsin
- trypsin and chemotrypsin (decrease in PT 6% and FT 29%)
PEPTIDES
- peptidases, dipeptidase (well developed)
A.A, dipeptides, small peptides (transport well develeped)

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25
Q

Fat Digestion

A

PT and FT have reduced bile salts and lipase
short chain and medium chain are directly absorbed and travel in portal vein

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26
Q

Ladd’s

A

Reducing the volvulus by turning the bowel in a counterclockwise fashion
Ladds bands
Appendectomy
Situating the bowel in the abdomen with the cecum in the left lower quadrant and the duodenum on the right

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27
Q

Silver-Russell Syndrome

A

Imprinting

IUGR
fifth finger clinodactyly
triangular face
micrognathia
Len length asymmetry

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28
Q

Trisomy 18

A

Second most common trisomy
1 in 7,900 live births
Low growth
microcephaly
short sternum
camptodatyly
nail hypoplasia
clenched hand posture
congenital heat disease (90%) septal defects, pda, poly valves
Renal and GU

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29
Q

Eye finding in Alagille

A

ocular posterior embryotoxin

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30
Q

anterior fontanelle

A

completely closes by 2 years

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31
Q

posterior fontanella

A

can be closed at birth

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32
Q

Mobius syndrome

A

Bilateral facial paresis
expresionless face

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33
Q

Early pre-eclampsia is due to

A

placental abnormalities that affects the fetus

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34
Q

Late preterm are at the highest risk for

A

jaundice

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35
Q

Listeria

A

small parenchyma yellow white pustules that correlate with mircoabscesses and necrosis

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36
Q

Quintero staging

A

TTTS
Stage I- oligo/poly with visible bladder
Stage II- oligo/poly without visible bladder
Stage III- oligo/poly with abnormal dopplers
Stage IV-oligo/poly with hydrops
Stage V- oligo/poly

Regardless of the type of procedure performed to treat, echocardiographic evidence of cardiomyopathy is greatest risk factor

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37
Q

Screening for DM

A

24-28 weeks

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38
Q

Progesterone role during pregnancy

A

assisting with quiescence of uterine myocardium, decreasing oxytocin receptors, increasing the stimulation threshold for contractions
there is decrease in progesterone levels prior to the labor

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39
Q

When to perform echocardiogram for PVC

A

-high frequency of PVC’s >60 PVC/hr
-persistence of PVC beyond 1-2 weeks of life
- cardiomegaly on CXR, or other cardiomyopathy

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40
Q

Neonatal Long QT

A

Calculate Qtc in leads II and V5
Qtc < 470 ms, but can be up to 0.49ms < 6 months of age

In long QT atria can contract but due to the long repol they will not be conducted

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41
Q

Causes of prolonged QT

A

Electrolytes: hypoMg, hypoCa, hypoK
Channelopathies genes include KCNQ1,KCNQ2, SCN5A, if found the family should be screened

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42
Q

Torsades

A

A malignant ventricular arrhythmia resulting from cell depolarization while the myocardium is still repol, treat with Mg sulfate.

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43
Q

Auto immune mediated congenital heart block

A

fetal HR < 55 bpm is associated with fetal hydrops

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44
Q

Which cardiac structural disease is most commonly seen with heart block

A

L-TGA

45
Q

SVT

A

Re-enterant tachycardia between A+V are WPW and AVNRT
Re-enterant tachycardia within the atria which is atrial flutter
Autonomic tachycardias

46
Q

Most common SVT in neonates

A

WPW
Second most common is atrial flutter

47
Q

Which congenital heart defect is most commonly associated with an accessory pathway

A

Ebstein

48
Q

Pathopnemoic ECG for Ebstein

A

Tall p waves and RsR prime showing RBBB

49
Q

Thyroid hormone

A

TSH DOES NOT CROSS PLACENTA
Fetal rT3 high
Thyroid gland mostly secreted T4 90%
IV dopamine can decrease TSH levels transiently

50
Q

Maternal treatment of Graves in first trimester

A

PTU, it is associated with pre auricular sinus/fistuala, urinary tract anomalies, low fetal weight
Methimazole use after first trimester because of risks of cutis aplasia

51
Q

Total calcium falls by 0.8mg/dl for every 1g/dl decrease in serum albumin

A
52
Q

ca gluconate vs ca chloride

A

ca chloride does not require metabolism by liver so can be used if liver not working

53
Q

Hypophosphatemia

A

Mild < 4mg/dl

recommended phosphate is 31-93 mg/kd/day, close to 1 mmol/kg/day= 33 mg/kg/day.

54
Q

Enteral goals for VLBW

A

Calcium 120-220 mg/kg/day
Phosphorus 70-120 mg/kg/day
Magnesium 8-15 mg/kg/day

55
Q

Fetal mineral amounts

A

Calcium 90-120 mg/kg/day
Phosphorus 60-75 mg/lg/day
Magnesium 3-5 mg/kg/day

56
Q

Abnormal Thyroid

A

TSH >40 and low T4 need to work up right away and treat

57
Q

cryo components

A

Factor XIII and VIII
Fibrinogen and Fibronectin
von wilebrand factor

58
Q

FFP

A

All clotting factors
Fibrinogen and Fibronectin
Gamma-Globulins
Albumin
Plasma Proteins

59
Q

Aicardi Syndrome

A

agenesisof corpus callousum

60
Q

Cranial Nerves

A

1 olfaction
2 optic
3 oculomotor pupils
4 extra ocular movements
5 trigeminal facial sensation and mastications
6 extra ocular movements
7 facial
8 auditory
9 glossopharyngeal
10 vagus
11 sternocleidomastoid functioon
12 tongue

61
Q

Abnormal MRI findings in hypoglycemia

A

bilateral posterior occipital cortex

62
Q

Infants with full recovery from brachial plexus injury show improvement by

A

2 weeks and recover by age 6 months
If residual impairment is observed at 15 months, it usually persists

63
Q

Antenatal steroids

A

Administered before 34 weeks decrease IVH, RDS, and mortality

Increased risk of hypoglycemia if administered for late preterm and decreased need for respiratory support in the first 3 days

64
Q

Noonan Syndrome

A

pulmonic stenosis
RASopathies
increased nuchal transulcency and hydrops

65
Q

Pyriform aperture stenosis

A

presents similarly as bilateral choanal atresia, pyriform aperture <11mm, first line treatment is with conservative measurements

66
Q

RhD

A

Is AD

67
Q

Biliary Atresia

A

Matrix metalloprotease 7

68
Q

Prolonged use of Prostaglandins

A

hypperostosis or cortical proliferation of the long bones

69
Q

Transpyloric or jejunal feeds

A

Need to monitor zinc, copper, selenium, and iron

70
Q

Absorption

A

Duodenum: Calcium and Iron
Terminal ileum: B12, bile salts, zinc

71
Q

Hereditary Spherocytosis

A

AD
Index of MCHC/MCV >0.36 detected Hereditary Spherocytosis

72
Q

Obstructive Hydrocephalus

A

Aqueductal stenosis can be caused by
X linked hydrocephalus, large hydrocephalus, ageneis of corpus callous, bilaterally adducted thumbs
1:30,000

73
Q

Aicardi Syndrome

A

Agenisis of the corpus callosum
Sporadic mutation
ocular abnormalities of chorioretinal lacunae
hemivertabrae and
intractable seizures

74
Q

NEC major risk factors

A

prolonged infusion of PGE

75
Q

Congenital hemangioma

A

Grow in utero, do not proliferate after birth
stain negative for glucose transporter protein1

76
Q

Ortalani and Barlow

A

Barlow is adduction and posterior force, if hear clunk bad
Ortalani is abduction and to assess for dislocation following the Barlow maneuver

77
Q

Neonatal Hypertension

A

Common causes in UAC and BPD/lung disease

78
Q

Preterm infants have a lower creatine clearance because of impaired GFR and they have greater absorption of filtered creatine in renal tubule.

A
79
Q

ARPKD and multicystic dysplastic kidneys

A

ARPKD is associated with congenital hepatic fibrosis and some degree of biliary dysgenesis, PKHD1 gene.
Multicystic kidney disease have large echogenic kidneys with thin-walled cysts that are visible at 20 weeks gestation.

80
Q

Renal Vein Thrombosis

A

Hypertension, hematuria, low platelets
Increased risk if mother is diabetic or infant with coagulopathy disorder
Palpable renal mass may be seen

81
Q

In preterm neonates the serum cr will normally increase during the first days after birth before gradually decreasing

A
82
Q

Trisomy 21

A

Obtain Karyotype
Recurrence will happen more often if balanced translocation

Fish or microarray can diagnose but can not tell if it is due to a balanced translocation that is why get a karyotype

83
Q

45 X

A

Obtain Karyotype

84
Q

Karyotype

A

Number of chromosomes or Aneuploidy

85
Q

22q11

A

FISH for microdeletion or duplication only looks at one chromosomal area

You go fishing for one deletion
Does not give information about other areas of the chromosome

86
Q

Microarray

A

microarray is the first line test recommended by the American College of Genetics for multiple congenital anomalies that do not fit a specific pattern of diagnosis.

comparative genomic hybridization
100-150k oligonucleotide probes
detects deletions or duplications copy number variants
Does not tell information about translocation

87
Q

Whole exam sequencing

A

sequence of protein coding regions of genes

88
Q

Fetal hydrops seen in which metabolic disorder

A

Storage disorders

89
Q

PKU

A

microcephaly
10% cardiac malformations DORV, aortic arch abnormalities, VSD

90
Q

After you give transfusion you can not detect galactosemia or thalassemia which is why you do it before.

A
91
Q

Pompe Disease

A

Acid alpha glucosidase deficiency GSD II
Infantile Onset
EKG short PR
Enzyme replacement therapy

92
Q

Variables influencing phototherapy

A

440-460 nm

93
Q
A
94
Q

Top trial

A

No difference in survival in high rbc threshold vs low rbc threshold

95
Q

Leukoreduction

A

CMV

96
Q

Irradiation

A

Graft vs host

97
Q

Early Decel

A

Head Compression

98
Q

Late Decel

A

Uteroplacental insufficiency

99
Q

Variable Decal

A

Cord Compression

100
Q

Club Foot

A

If diagnosed prenatally 60% will have associated anomaly
If diagnosed at birth 10% will have associated anomaly

101
Q

Moasic turners

A

concern for gonadoblastoma

102
Q

Legius Syndrome

A

AD
cafe au lait
macrocephaly
overlap with NF1

103
Q

Listeria

A

Early- skin lesions after birth and white plaques on umbilical cord
Late- no skin findings

104
Q

Hardy Weinberg genetics

A

p+q=1
example: homozygous recessive is 1/2500=1/50=0.02
frequency of recessive allele is 0.02
frequency of dominant allele is 0.98
Carrier status is 2(p)(q) which is 2(0.02)(0.98)=0.04.

105
Q

Epidermoylsis Bullosa 3 types

A

EB simplex AD
EB Junctional AR
EB dystrophic AD and AR

106
Q

GI embryology

A

6th week midgut herniates through the umbilical ring
270 degree counterclockwise rotation around the SMA
10th week returns to abdominal cavity

107
Q

Meningitis

A

1/3-1/2 with neurological sequelae
E.coli with K1 antigen
Seizures are not predictive of outcome

108
Q
A

Anu Cards (6 decks)

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