GI

Question 1

malnutrition as a result of near starvation from protein and non preteen deficiency, very thin from loss of muscle and body fat

Answer 1

marasmus

Question 2

seen in parts of the world where starch is main diet, protein deficient state

Answer 2

kwashiorkor

Question 3

stool ph below 5.6 suggests?

Answer 3

carbohydrate malabsorption

Question 4

a positive clinitest reaction shows?

Answer 4

watery and acidic stool resulting detected as reducing substances

Question 5

what are 2 causes of carbohydrate malabsorption?

Answer 5

isolated congenital enzyme deficiency

mucosal atrophy

Question 6

what can be used to document enteric protein losses?

Answer 6

fecal a1-antitrypsin levels

Question 7

decreased _____ activity results in steatorrhea?

Answer 7

lipase actvity

Question 8

abetalipoproteinemia is suggested by what?

Answer 8

acanthocytosis

Question 9

macrocytic blood smear suggests what?

Answer 9

folic acid or vitamin b 12 deficiency

Question 10

what majorly causes protein intolerance?

Answer 10

cows milk protein

Question 11

what is the management of protein intolerance?

Answer 11

withdrawal and will resolve by 1-2 years of age

Question 12

celiac disease location?

Answer 12

proximal small intestine

Question 13

etiology of celiacs?

Answer 13

intolerance to gluten

Question 14

what is the gold standard for celiac disease?

Answer 14

small bowel biopsy

Question 15

what will small bowel biopsy show?

Answer 15

short/flat villi, deep crypts and vacuolated epithelium

Question 16

what are specific in screening in celiac disease?

Answer 16

serum IgA - endomysial and serum tissue transglutaminase antibody testing

Question 17

this occurs from small bowel resection as a result of volvulus, gastroschises?

Answer 17

short bowel syndrome

Question 18

whats important in management of short bowel syndrome?

Answer 18

early TPN and parental feedings

Question 19

this is characteristic torticollis with arching of the back caused by painful esophagitis

Answer 19

sandifer syndrome

Question 20

this is the gold standard for GERD diagnosis

Answer 20

pH probe measurement

Question 21

this is a motility agent used to increase LES tone or increase gastric emptying?

Answer 21

metoclopramide

Question 22

palpable olive mass, projectile vomitting, and peristaltic waves

Answer 22

pyloric stenosis

Question 23

metabolic profile of pyloric stenosis

Answer 23

hypochloremic, hypokalemic, metabolic alkalosis

Question 24

what is the diagnostic method of choice for pyloric stenosis

Answer 24

ultrasound

Question 25

what is the management of pyloric stenosis?

Answer 25

correct electrolyte abnormalities and then correct w/ partial pyloromyotomy

Question 26

diagnostic tool for volvulus?

Answer 26

upper go contrast imaging

Question 27

is volvulus a surgical emergency?

Answer 27

yes

Question 28

what is the most common location of intussusception?

Answer 28

ileocolic

Question 29

what is generally the etiology of intussception?

Answer 29

lead point

Question 30

how do an infants legs present w/ intussception?

Answer 30

draw legs towards chest

Question 31

currant jelly stools, sausage shaped mass and coil spring sign on radiograph?

Answer 31

intussecption

Question 32

gold standard for intussception

Answer 32

contrast enema w/ air

Question 33

first line therapy for intussception?

Answer 33

contrast enema w/ air or hydrostatic pressure

Question 34

if intessucpetion w/ peritonitis or pneumoperitoneum?

Answer 34

operative reduction

Question 35

bluish discoloration of flanks

Answer 35

gray turner sign

Question 36

bluish discoloration of periumbilical area

Answer 36

cullen sign

Question 37

what do gray turner sign and cullen sign indicate?

Answer 37

pancreatitits

Question 38

what is elevated in acute pancreatitis?

Answer 38

serum amylase and serum lipase

Question 39

imaging for pancreatitis?

Answer 39

abdominal US

Question 40

imaging for pseudocyst in pancreatitis?

Answer 40

abdominal CT

Question 41

acute a calculous cholecystitis is caused by what infections?

Answer 41

salmonella, shigella and e coli

Question 42

what condition may cause acute chile in infants?

Answer 42

sickle cell disease

Question 43

what is used to soften the stool in constipation?

Answer 43

mineral oil

Question 44

painless acute rectal bleeding in a otherwise healthy child?

Answer 44

meckels diverticulum

Question 45

how does jaundice generally occur?

Answer 45

begins cranially and extends caudally

Question 46

this syndrome associatiated with hemolysis or with a very large hematoma, first starts as increased indirect to increased direct bill

Answer 46

inspissated bile syndrome

Question 47

mild unconjugated biliburinemia occurs w/ stress or poor nutrition

Answer 47

UDP-glucuronyl transferase deficiency

Question 48

autosomal recessive disorder 100% of udp transferase is absent causing kernicterus

Answer 48

criggler najar type i

Question 49

autosomal dominant 90% udp gluorynl transferase absent

Answer 49

criggler najar type II

Question 50

most common cause of cholestasis in new born

Answer 50

neonatal hepatitis

Question 51

management of neonatal hepatitis

Answer 51

supportive nutritional support ursodeoxycholic acid livertransplantation

Question 52

hepatosplenomegaly, polyspenia syndrome, increased bilirubin levels?

Answer 52

biliary atresia

Question 53

progressive fibroscleortic disease that affects the extra hepatic biliary tree

Answer 53

biliary atresia

Question 54

intraop cholangiogram w/ laparotomy to examine biliary tree

Answer 54

dx. of biliary atresia

Question 55

management of biliary atresia

Answer 55

kasaiportoenterstomy (roux-en-y intestinal loop)

Question 56

paucity of intrahepatic bile ducts and multi organ involvement?

Answer 56

alagille syndrome

Question 57

clinical features of alagille syndrome

Answer 57

``` debilitating pruritus broad forehead saddle nose pulm outflow obstruction renal disease posterior embryotoxon butterfly vertebrae and broad thumbs ```

Question 58

management of alagille syndrome

Answer 58

supportive

Question 59

picornovirus hepatitis caused by fecal oral route

Answer 59

hep A

Question 60

a DNA virus heptitis?

Answer 60

hepatitis B

Question 61

transmission of hep B

Answer 61

perinatal vertical exposure, infected blood products, body secretions

Question 62

HBsAg

Answer 62

active disease ( used in hep B vaccine)

Question 63

HBsAB

Answer 63

protective results from vaccination or natural infection

Question 64

HBcAb

Answer 64

natural infection and persists life long

Question 65

HBeAg

Answer 65

infectious, acute

Question 66

RNA flavivirus family

Answer 66

hep C infection

Question 67

those with hepatitis B have an increased risk of what?

Answer 67

hepatocellular carcinoma

Question 68

this virus requires HBsAg for replication

Answer 68

heptatis D

Question 69

elevated serum transaminatses, hypergammaglobulinema and circulating autoantibodies + family history of autoimmune disease

Answer 69

autoimmune hepatitis

Question 70

management for autoimmune hepatitis

Answer 70

corticosteroids and immunosuppresive agents (azathioprine or 6 mercaptopurine)