GI, Nephro, Hema

Question 1

Gold standard for biliary atresia

Answer 1

Direct Cholangiography

Question 2

cone-shaped fibrotic mass cranial to the bifurcation of the portal vein, may be seen in patients with biliary atresia

Answer 2

Triangular cord sign

Question 3

Extrahepatic location
Triangular cord sign
Acholic stool
+ intact basic hepatic lobular archi`

Answer 3

Biliary atresia
definitive: liver transplant

Question 4

MCC of viral AGE in infants

Answer 4

Rotavirus

Question 5

how many losses in acute diarrhea in infants, and older children

Answer 5

excessively loose stools of >10 mL/kg/day in infants
>200 g/24 hr in older children

lasts < 14 days

Question 6

WHO recommendation for all patients with bloody diarrhea
irrespective of age

Answer 6

Ciprofloxacin 30 mg/kg/day in 2 divided doses

Question 7

first clinical evidence of HBV infection is

Answer 7

elevation of ALT levels (6-7 wks after exposure)

Question 8

most valuable single serologic marker of acute HBV infection because it is present as early as HbsAg and continues to be present later in the course of the disease when HBsAg has disappeared

Answer 8

Anti-HBcAg

Question 9

serves as a marker of active viral replication;
identification of infected people at increased risk of transmitting
HBV

Answer 9

HBeAg

Question 10

first serologic marker in Hepatitis to appear & its rise coincides with the onset of symptoms;

Answer 10

HBsAg

Question 11

identification of people who have resolved infections with HBV; determination of immunity after immunization

Answer 11

Anti-HBs

Question 12

dentification of infected people with lower risk of transmitting HBV

Answer 12

Anti-HBe

Question 13

identification of people with acute, resolved, or chronic HBV infection

Answer 13

Anti-HBc

Question 14

identification of people with acute or recent HBV infections (including HBsAg-negative people during the “window” phase of infection)

Answer 14

IgM anti-HBc

Question 15

MCC of gross hematuria in pediatric population

Answer 15

Urinary Tract Infection

Question 16

best single antibody titer that document skin infection in GN

Answer 16

DNAse B

Question 17

these are the only causes of Renal insufficiency that cause decrease in C3

Answer 17

PSGN
MPGN
Lupus Nephritis

Question 18

Bilateral sensorineural hearing loss
Ocular abnormalities – anterior lenticonus
macular flecks, & corneal erosions
Family History

Answer 18

ALPORT SYNDROME (HEREDITARY NEPHRITIS

ESRD occurs before 30 y/o in 75%

Question 19

Major complication of nephrotic syndrome

Answer 19

Infection

most frequent type of infection: bacterial peritonitis
MC organism causing peritonitis: pneumococcus
Tx: 3G cephalosporin

Question 20

Nephro: MC serious bacterial infection in younger 24 mos

Answer 20

Pyelonephritis
Fever - may be only manifestation

Question 21

TRIAD of HUS

Answer 21

RAT
Renal failure
Anemia (microangiopathic HA)
Thrombocytopenia

Microvascular injury with endothelial cell damage is
characteristic of all forms of HUS.
MC in <4y/o

Question 22

PBS of HUS

Answer 22

Peripheral smear: helmet cells, burr cells, fragmented RBCs

Question 23

Criteria of TTP

Answer 23

Renal failure
Anemia (microangiopathic HA)
Thrombocytopenia
CNS involvement
Fever

Question 24

KDIGO definition of AKI

Answer 24

increase in crea by >/= 0.3mg/dl from baseline within 48 hr increase in crea to >/= 1.5 times within the prior 7 days
urine volume

Question 25

MC hematologic disease of infancy and childhood

Answer 25

IDA MC in 9-24mons MCC poor dietary intake Most important sign: Pallor

Question 26

micro, hypochromic RBC dec RC N RDW PBS: target and Heinz cells

Answer 26

Thalassemia tx: frequent transfusions, chelation therapy with Deferoxamine as needed, splenectomy

Question 27

micro, hypochromic RBC dec RC inc RDW dec Fe inc TIBC dec ferritin

Answer 27

IDA

Question 28

dec Fe decTIBC inc ferritin

Answer 28

Anemia of Chronic disease

Question 29

gram negative organism that EATS up the FREE IRON

Answer 29

Yersinia enterocolitica

Question 30

blood test for confirming the presence of fragile sphere shaped RBC

Answer 30

Osmotic fragility test (Spherocytosis) increase MCHC high RC inc indirect bilirubin level

Question 31

XRay: crew cut/hair on end Howell Jolly Bodies single missense mutation in B-globin gene of Hgb

Answer 31

Sickle cell disease

Question 32

MC bleeding disorder?

Answer 32

von Willebrand Disease ↓ vWF levels,↓ Factor VIII prolonged bleeding time abnormal platelet adhesion,↑ PTT tx: mild - Desmopressin Severe: Factor VIII concentrate

Question 33

most common and most serious congenital coagulation factor deficiencies

Answer 33

hemophilia A (VIII) HM: prolonged PiTT

Question 34

most common hereditary hypercoagulable disorder?

Answer 34

Factor V Leiden

Question 35

MC childhood malignancy

Answer 35

Acute Lymphocytic Leukemia pancytopenia + hypercellularity (blast) PE: + signs of infiltration

Question 36

single most prognostic factor in ALL

Answer 36

Treatment 3 most predicting factor: age, initial leukocyte, speed of response to treatment

Question 37

2nd MC malignant abdominal tumor in childhood

Answer 37

Wilms tumor 2-5 y/o Assoc with: Neurofibromatosis Beckwith-Wiedemann WAGR syndrome

Question 38

embryonal tumor of neural crests cell origin 3rd MC pediatric cancer

Answer 38

Neuroblastoma Non-tender, abdominal mass which may cross the midline, Horner syndrome, hypertension, cord compression mets: periorbital bruising (“raccoon eyes”), subcutaneous tumor nodules, opsoclonus /myoclonus (“dancing eyes, dancing feet”)

Question 39

Neuroblastoma is associated with

Answer 39

N-myc oncogene - tuberous sclerosis Neurofibromatosis – Pheochromocytoma Hirschsprung disease

Question 40

Most common solid tumor

Answer 40

Brain Tumor

Question 41

Most common solid tumor outside CNS?

Answer 41

Neuroblastoma

Question 42

Most common soft tissue tumor?

Answer 42

Rhadomyosarcoma

Question 43

Malignancy with highest mortality

Answer 43

Brain (PNET)

Question 44

MC congenital cause of AA

Answer 44

Fanconi Anemia