Peds GI

Question 1

Swenson procedure

Answer 1

rectosigmoidectomy for HD

Question 2

Management suspected retained segment in HD

Answer 2

full thickness biopsy

Question 3

Enteric ganglion cells enable ___ relaxation of the smooth muscle in the intestinal wall

Answer 3

Parasympathetic

Question 4

Enteric ganglia, nerves, and glial cells develop from what?

Answer 4

enteric neural crest–derived cells (ENCDCs)

Question 5

Inactivating mutations in this gene –> 15% of sporadic and 50% of familial forms of HD?

Answer 5

RET (tyrosine kinase receptor for glial cell line–derived neurotrophic factor)

Question 6

Assesses the rectoanal inhibitory reflex.

Answer 6

Anorectal manometry (ARM)

Question 7

Increased rectal distention -> IAS relaxation

Answer 7

rectoanal inhibitory reflex

Question 8

Mutated gene a/w HD I/s/o Waardenburg syndrome

Answer 8

Endothelin receptor type B (EDNRB)

Question 9

Rectoanal inhibitory reflex in HD s/p pull through

Answer 9

Abnormal or absent (some ganglionic rectum in place)

Question 10

Enteric neural crest–derived cells progress in what direction?

Answer 10

Rostrocaudal

Question 11

Aganglionosis in retained bowel after surgical resection for HD –>

Answer 11

obstructive symptoms and enterocolitis

Question 12

Acute radiation enteropathy is due to?

Answer 12

Interruption of normal enterocyte replacement and villous atrophy

Question 13

MMCs are ____ in radiation enteropathy

Answer 13

Normal

Question 14

laxative that can cause skin desquamation and blistering

Answer 14

Senna

Question 15

Stimulant laxatives

Answer 15

Senna, bisacodyl

Question 16

delayed gastric emptying in the absence of mechanical obstruction

Answer 16

gastroparesis

Question 17

D/c motility meds __ before gastric emptying study

Answer 17

2 days

Question 18

Imaging at __ hours after ingestion in adult gastric emptying study

Answer 18

1,2,4

Question 19

Adult delayed gastric emptying is __ at 2 hours or >10% at 4 hours

Answer 19

> 60%

Question 20

Adult delayed gastric emptying is >60% at 2 hours or __ at 4 hours

Answer 20

> 10%

Question 21

FNRFI

Answer 21

functional nonretentive fecal incontinence

Question 22

FIC1 deficiency

Answer 22

PFIC1

Question 23

PFIC1

Answer 23

FIC1 deficiency

Question 24

PFIC2

Answer 24

BSEP deficiency

Question 25

PFIC3

Answer 25

MDR3 deficiency

Question 26

PFIC4

Answer 26

TJP2 deficiency

Question 27

BSEP

Answer 27

bile salt excretory pump

Question 28

PFIC w/ recurrent disease in allograft liver

Answer 28

PFIC2 (BSEP deficiency)

Question 29

Treatment of BSEP recurrence

Answer 29

IVIG, ritux, plasmapheresis

Question 30

PFIC w/ high GGT cholestasis

Answer 30

PFIC3

Question 31

PFIC w/ low GGT cholestasis

Answer 31

PFIC1, PFIC2, and PFIC4

Question 32

Canalicular membrane protein that transports bile acids from hepatocyte to canalicular space

Answer 32

BSEP

Question 33

Initial tx BSEP deficiency

Answer 33

external or internal biliary diversion

Question 34

Systemic PFIC

Answer 34

PFIC1 (chronic diarrhea, hearing loss, pancreatitis, and asthma-like symptoms)

Question 35

Tx PFIC3

Answer 35

ursodeoxycholic acid

Question 36

Bowel lengthening procedures used in short gut

Answer 36

serial transverse enteroplasty (STEP)

Question 37

Bowel diameter requirement for STEP in short gut

Answer 37

>4cm

Question 38

Lactulose decreases ammonia by

Answer 38

acidifying colon (NH4+ not absorbed as well) and osmotic catharsis (expelling urease-producing bacteria)

Question 39

Tx hepatic encephalopathy

Answer 39

enteral lactulose, enteral rifaxamin

Question 40

Tx giardia

Answer 40

metronidazole

Question 41

GERD w/ symptoms without significant evidence of esophageal damage

Answer 41

Nonerosive reflux disease (NERD)

Question 42

GERD w/ symptoms are related to acid exposure damage to the esophageal epithelium

Answer 42

Erosive reflux disease (ERD)

Question 43

GERD symptoms w/o acid exposure, reflux correlate, or esophageal motility disorder

Answer 43

functional heartburn

Question 44

prolonged pH/MII testing

Answer 44

24-hour combined esophageal impedance and pH study

Question 45

colic tx

Answer 45

lactobacillus reuteri or fennel extract; reassurance

Question 46

Risk factors associated with CFLD

Answer 46

severe mutation of the CFTR gene, meconium ileus, male sex, and pancreatic insufficiency

Question 47

inspissated bile and diminished bile flow in apical membrane of cholangiocytes -->

Answer 47

focal biliary cirrhosis in CF

Question 48

Patients with FBC and large intrahepatic bile duct disease with cirrhosis are at risk for

Answer 48

HCC and cholangiocarcinoma

Question 49

Patients with CFLD at risk for development of

Answer 49

hepatopulmonary syndrome and portopulmonary hypertension

Question 50

Prophylaxis for viral gastro

Answer 50

lactobacillus rhamnosus GG

Question 51

Infants with cholestasis can be at risk for what trace element deficiency?

Answer 51

Copper

Question 52

Cobalamin (b12) bound to intrinsic factor is absorbed where?

Answer 52

Ileum

Question 53

Tx esophageal diverticulum 2/2 stricture

Answer 53

dilation

Question 54

Polyps plus mucocutaneous pigmentation

Answer 54

Peutz-Jeghers syndrome

Question 55

Peutz-Jeghers gene

Answer 55

STK11

Question 56

FAP gene

Answer 56

APC (or MUTYH)

Question 57

JPS gene

Answer 57

SMAD4

Question 58

WCE approved in older than _ yo

Answer 58

2

Question 59

In Crohn disease, giving __ w/ infliximab increases duration of response

Answer 59

MTX

Question 60

Hepatosplenic T-cell lymphoma is a/w

Answer 60

thiopurines

Question 61

___ phenotype in Crohn disease a/w favorable response to infliximab

Answer 61

inflammatory

Question 62

the presence of __ or more juvenile polyps suggests polyposis syndrome

Answer 62

5

Question 63

Thyroid ultrasonography is indicated in children with FAP because of the increased risk of ___

Answer 63

papillary thyroid cancer

Question 64

incidental malro

Answer 64

surgical consultation for ?Ladd

Question 65

preferred diagnostic modality for suspected cholelithiasis, cholecystitis, or choledocholithiasis

Answer 65

US

Question 66

RUQ pain, fever, jaundice, acholic stools, and dark urine

Answer 66

choledocholithiasis

Question 67

Meckel diverticulum arises from incomplete obliteration of the

Answer 67

omphalomesenteric duct

Question 68

Conjugates unconjugated bilirubin bound to albumin

Answer 68

UGT 1A1 (uridine diphosphate glucuronosyltransferase)