Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Anu Cards > AAP Question > Flashcards

AAP Question Flashcards

(108 cards)

Start Studying
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Inhaled nitric is contraindicated in

A

Left ventricular dysfunction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

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

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

omphalocele

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Surgical nec mangement

A

No significant difference between peritoneal drainage compared to laparotomy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Glutamine

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Disorders of abnormal UGT

A

Gilbert is decreased activity
Crigler is absent UGT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

liver biopsy showing
Multinucleated giant cells

A

Neonatal idiopathic hepatitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

liver biopsy showing
Bile duct proliferation

A

Biliary Atresia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

liver biopsy showing
necrotizing duct lesions

A

sclerosis cholangitits

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Fat soluble vitamins should be supplemented in neonates with cholestatis

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Role of citruilline measure in short bowel

A

Citrulline is a marker of enterocyte hass and short bowel function

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Probiotics

A

Good ones are Lactobacillus and Bifidobacterium

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Fat Digestion
PT and FT have reduced bile salts and lipase short chain and medium chain are directly absorbed and travel in portal vein
26
Ladd's
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
27
Silver-Russell Syndrome
Imprinting IUGR fifth finger clinodactyly triangular face micrognathia Len length asymmetry
28
Trisomy 18
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
29
Eye finding in Alagille
ocular posterior embryotoxin
30
anterior fontanelle
completely closes by 2 years
31
posterior fontanella
can be closed at birth
32
Mobius syndrome
Bilateral facial paresis expresionless face
33
Early pre-eclampsia is due to
placental abnormalities that affects the fetus
34
Late preterm are at the highest risk for
jaundice
35
Listeria
small parenchyma yellow white pustules that correlate with mircoabscesses and necrosis
36
Quintero staging
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
37
Screening for DM
24-28 weeks
38
Progesterone role during pregnancy
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
39
When to perform echocardiogram for PVC
-high frequency of PVC's >60 PVC/hr -persistence of PVC beyond 1-2 weeks of life - cardiomegaly on CXR, or other cardiomyopathy
40
Neonatal Long QT
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
41
Causes of prolonged QT
Electrolytes: hypoMg, hypoCa, hypoK Channelopathies genes include KCNQ1,KCNQ2, SCN5A, if found the family should be screened
42
Torsades
A malignant ventricular arrhythmia resulting from cell depolarization while the myocardium is still repol, treat with Mg sulfate.
43
Auto immune mediated congenital heart block
fetal HR < 55 bpm is associated with fetal hydrops
44
Which cardiac structural disease is most commonly seen with heart block
L-TGA
45
SVT
Re-enterant tachycardia between A+V are WPW and AVNRT Re-enterant tachycardia within the atria which is atrial flutter Autonomic tachycardias
46
Most common SVT in neonates
WPW Second most common is atrial flutter
47
Which congenital heart defect is most commonly associated with an accessory pathway
Ebstein
48
Pathopnemoic ECG for Ebstein
Tall p waves and RsR prime showing RBBB
49
Thyroid hormone
TSH DOES NOT CROSS PLACENTA Fetal rT3 high Thyroid gland mostly secreted T4 90% IV dopamine can decrease TSH levels transiently
50
Maternal treatment of Graves in first trimester
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
Total calcium falls by 0.8mg/dl for every 1g/dl decrease in serum albumin
52
ca gluconate vs ca chloride
ca chloride does not require metabolism by liver so can be used if liver not working
53
Hypophosphatemia
Mild < 4mg/dl recommended phosphate is 31-93 mg/kd/day, close to 1 mmol/kg/day= 33 mg/kg/day.
54
Enteral goals for VLBW
Calcium 120-220 mg/kg/day Phosphorus 70-120 mg/kg/day Magnesium 8-15 mg/kg/day
55
Fetal mineral amounts
Calcium 90-120 mg/kg/day Phosphorus 60-75 mg/lg/day Magnesium 3-5 mg/kg/day
56
Abnormal Thyroid
TSH >40 and low T4 need to work up right away and treat
57
cryo components
Factor XIII and VIII Fibrinogen and Fibronectin von wilebrand factor
58
FFP
All clotting factors Fibrinogen and Fibronectin Gamma-Globulins Albumin Plasma Proteins
59
Aicardi Syndrome
agenesisof corpus callousum
60
Cranial Nerves
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
Abnormal MRI findings in hypoglycemia
bilateral posterior occipital cortex
62
Infants with full recovery from brachial plexus injury show improvement by
2 weeks and recover by age 6 months If residual impairment is observed at 15 months, it usually persists
63
Antenatal steroids
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
Noonan Syndrome
pulmonic stenosis RASopathies increased nuchal transulcency and hydrops
65
Pyriform aperture stenosis
presents similarly as bilateral choanal atresia, pyriform aperture <11mm, first line treatment is with conservative measurements
66
RhD
Is AD
67
Biliary Atresia
Matrix metalloprotease 7
68
Prolonged use of Prostaglandins
hypperostosis or cortical proliferation of the long bones
69
Transpyloric or jejunal feeds
Need to monitor zinc, copper, selenium, and iron
70
Absorption
Duodenum: Calcium and Iron Terminal ileum: B12, bile salts, zinc
71
Hereditary Spherocytosis
AD Index of MCHC/MCV >0.36 detected Hereditary Spherocytosis
72
Obstructive Hydrocephalus
Aqueductal stenosis can be caused by X linked hydrocephalus, large hydrocephalus, ageneis of corpus callous, bilaterally adducted thumbs 1:30,000
73
Aicardi Syndrome
Agenisis of the corpus callosum Sporadic mutation ocular abnormalities of chorioretinal lacunae hemivertabrae and intractable seizures
74
NEC major risk factors
prolonged infusion of PGE
75
Congenital hemangioma
Grow in utero, do not proliferate after birth stain negative for glucose transporter protein1
76
Ortalani and Barlow
Barlow is adduction and posterior force, if hear clunk bad Ortalani is abduction and to assess for dislocation following the Barlow maneuver
77
Neonatal Hypertension
Common causes in UAC and BPD/lung disease
78
Preterm infants have a lower creatine clearance because of impaired GFR and they have greater absorption of filtered creatine in renal tubule.
79
ARPKD and multicystic dysplastic kidneys
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
Renal Vein Thrombosis
Hypertension, hematuria, low platelets Increased risk if mother is diabetic or infant with coagulopathy disorder Palpable renal mass may be seen
81
In preterm neonates the serum cr will normally increase during the first days after birth before gradually decreasing
82
Trisomy 21
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
45 X
Obtain Karyotype
84
Karyotype
Number of chromosomes or Aneuploidy
85
22q11
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
Microarray
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
Whole exam sequencing
sequence of protein coding regions of genes
88
Fetal hydrops seen in which metabolic disorder
Storage disorders
89
PKU
microcephaly 10% cardiac malformations DORV, aortic arch abnormalities, VSD
90
After you give transfusion you can not detect galactosemia or thalassemia which is why you do it before.
91
Pompe Disease
Acid alpha glucosidase deficiency GSD II Infantile Onset EKG short PR Enzyme replacement therapy
92
Variables influencing phototherapy
440-460 nm
93
94
Top trial
No difference in survival in high rbc threshold vs low rbc threshold
95
Leukoreduction
CMV
96
Irradiation
Graft vs host
97
Early Decel
Head Compression
98
Late Decel
Uteroplacental insufficiency
99
Variable Decal
Cord Compression
100
Club Foot
If diagnosed prenatally 60% will have associated anomaly If diagnosed at birth 10% will have associated anomaly
101
Moasic turners
concern for gonadoblastoma
102
Legius Syndrome
AD cafe au lait macrocephaly overlap with NF1
103
Listeria
Early- skin lesions after birth and white plaques on umbilical cord Late- no skin findings
104
Hardy Weinberg genetics
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
Epidermoylsis Bullosa 3 types
EB simplex AD EB Junctional AR EB dystrophic AD and AR
106
GI embryology
6th week midgut herniates through the umbilical ring 270 degree counterclockwise rotation around the SMA 10th week returns to abdominal cavity
107
Meningitis
1/3-1/2 with neurological sequelae E.coli with K1 antigen Seizures are not predictive of outcome
108

Anu Cards (6 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions