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Peds GI > Peds GI > Flashcards

Peds GI Flashcards

1
Q

Swenson procedure

A

rectosigmoidectomy for HD

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2
Q

Management suspected retained segment in HD

A

full thickness biopsy

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3
Q

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

A

Parasympathetic

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4
Q

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

A

enteric neural crest–derived cells (ENCDCs)

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5
Q

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

A

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

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6
Q

Assesses the rectoanal inhibitory reflex.

A

Anorectal manometry (ARM)

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7
Q

Increased rectal distention -> IAS relaxation

A

rectoanal inhibitory reflex

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8
Q

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

A

Endothelin receptor type B (EDNRB)

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9
Q

Rectoanal inhibitory reflex in HD s/p pull through

A

Abnormal or absent (some ganglionic rectum in place)

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10
Q

Enteric neural crest–derived cells progress in what direction?

A

Rostrocaudal

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11
Q

Aganglionosis in retained bowel after surgical resection for HD –>

A

obstructive symptoms and enterocolitis

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12
Q

Acute radiation enteropathy is due to?

A

Interruption of normal enterocyte replacement and villous atrophy

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13
Q

MMCs are ____ in radiation enteropathy

A

Normal

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14
Q

laxative that can cause skin desquamation and blistering

A

Senna

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15
Q

Stimulant laxatives

A

Senna, bisacodyl

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16
Q

delayed gastric emptying in the absence of mechanical obstruction

A

gastroparesis

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17
Q

D/c motility meds __ before gastric emptying study

A

2 days

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18
Q

Imaging at __ hours after ingestion in adult gastric emptying study

A

1,2,4

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19
Q

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

A

> 60%

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20
Q

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

A

> 10%

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21
Q

FNRFI

A

functional nonretentive fecal incontinence

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22
Q

FIC1 deficiency

A

PFIC1

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23
Q

PFIC1

A

FIC1 deficiency

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24
Q

PFIC2

A

BSEP deficiency

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25
Q

PFIC3

A

MDR3 deficiency

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26
Q

PFIC4

A

TJP2 deficiency

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27
Q

BSEP

A

bile salt excretory pump

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28
Q

PFIC w/ recurrent disease in allograft liver

A

PFIC2 (BSEP deficiency)

29
Q

Treatment of BSEP recurrence

A

IVIG, ritux, plasmapheresis

30
Q

PFIC w/ high GGT cholestasis

A

PFIC3

31
Q

PFIC w/ low GGT cholestasis

A

PFIC1, PFIC2, and PFIC4

32
Q

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

A

BSEP

33
Q

Initial tx BSEP deficiency

A

external or internal biliary diversion

34
Q

Systemic PFIC

A

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

35
Q

Tx PFIC3

A

ursodeoxycholic acid

36
Q

Bowel lengthening procedures used in short gut

A

serial transverse enteroplasty (STEP)

37
Q

Bowel diameter requirement for STEP in short gut

A

> 4cm

38
Q

Lactulose decreases ammonia by

A

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

39
Q

Tx hepatic encephalopathy

A

enteral lactulose, enteral rifaxamin

40
Q

Tx giardia

A

metronidazole

41
Q

GERD w/ symptoms without significant evidence of esophageal damage

A

Nonerosive reflux disease (NERD)

42
Q

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

A

Erosive reflux disease (ERD)

43
Q

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

A

functional heartburn

44
Q

prolonged pH/MII testing

A

24-hour combined esophageal impedance and pH study

45
Q

colic tx

A

lactobacillus reuteri or fennel extract; reassurance

46
Q

Risk factors associated with CFLD

A

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

47
Q

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

A

focal biliary cirrhosis in CF

48
Q

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

A

HCC and cholangiocarcinoma

49
Q

Patients with CFLD at risk for development of

A

hepatopulmonary syndrome and portopulmonary hypertension

50
Q

Prophylaxis for viral gastro

A

lactobacillus rhamnosus GG

51
Q

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

A

Copper

52
Q

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

A

Ileum

53
Q

Tx esophageal diverticulum 2/2 stricture

A

dilation

54
Q

Polyps plus mucocutaneous pigmentation

A

Peutz-Jeghers syndrome

55
Q

Peutz-Jeghers gene

A

STK11

56
Q

FAP gene

A

APC (or MUTYH)

57
Q

JPS gene

A

SMAD4

58
Q

WCE approved in older than _ yo

A

2

59
Q

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

A

MTX

60
Q

Hepatosplenic T-cell lymphoma is a/w

A

thiopurines

61
Q

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

A

inflammatory

62
Q

the presence of __ or more juvenile polyps suggests polyposis syndrome

A

5

63
Q

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

A

papillary thyroid cancer

64
Q

incidental malro

A

surgical consultation for ?Ladd

65
Q

preferred diagnostic modality for suspected cholelithiasis, cholecystitis, or choledocholithiasis

A

US

66
Q

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

A

choledocholithiasis

67
Q

Meckel diverticulum arises from incomplete obliteration of the

A

omphalomesenteric duct

68
Q

Conjugates unconjugated bilirubin bound to albumin

A

UGT 1A1 (uridine diphosphate glucuronosyltransferase)

Peds GI (1 decks)

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