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Pediatrics > Gastroenterology > Flashcards

Gastroenterology Flashcards

0
Q

Liver failure after introduction of juices

A

Fructosemia

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1
Q

Reducing substances in urine

A

Galactosemia

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2
Q

Succinylacetone in urine

A

Tyrosinemia

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3
Q

Antibody that appears early and then fades off

A

IgM

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4
Q

Positive HB surface antigen +/- positive HB envelope antigen

A

Acute Hep B infection

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5
Q

Positive HB surface antibody

A

Previous Hep B infection

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6
Q

May be the only positive marker in a patient infected with Hep B who is going to clear the virus but has not yet developed the protective antibody (anti-Hep B surface antigen)

A

IgM anti-Hep B core antibody

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7
Q

Occurs only with co-infection with hepatitis B

A

Delta Hepatitis

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8
Q

Type of hepatitis associated with fetal loss and higher risk of fulminant liver failure in pregnant woman

A

Hepatitis E

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9
Q

TB drug that can cause acute hepatitis

A

INH

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10
Q

Anticonvulsant that can cause acute hepatitis

A

Valproate

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11
Q

Tx for acetaminophen OD

A

N-acetyl cysteine

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12
Q

Most common mushroom to cause acute hepatitis

A

Amanita phalloides

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13
Q

Negative Hep B surface antibody

A

Hep B chronic carrier state

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14
Q

Indicates active Hep B replication

A

Positive Hep B envelope antigen

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15
Q

Low serum copper and ceruloplasmin. Elevated 24 hr urine copper and liver copper content

A

Wilson’s disease

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16
Q

Kayser-Fleischer rings

A

Wilson’s disease

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17
Q

Conjugated hyperbilirubinemia in first 4 weeks of life (MUST evaluate any baby jaundiced beyond two weeks of life)

A

Biliary atresia

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18
Q

Paucity of the intrahepatic bile ducts

A

Alagille’s syndrome

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19
Q

Syndrome associated with congenital heart disease (especially pulmonary stenosis), short statute, triangular facies, butterfly vertebrae, xanthomas

A

Alagille’s syndrome

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20
Q

Most common infections in calculus cholecystitis

A

E. Coli & enterococcus

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21
Q

85 to 90% of kids with this will have a soy allergy

A

Cows milk allergy

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22
Q

If fructose to glucose ratio is increased, there is a higher chance of developing

A

Watery diarrhea

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23
Q

Present in fruit juices, excessive consumption causes diarrhea

A

Sorbitol

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24
Q

The HLA class 1 molecule associated with Celiac

A

B8

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25
Q

The HLA class II molecules associated with celiac

A

DR3 and DR7

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26
Q

Foamy macrophages, caused by tropherma whipplii

A

Whipple’s Disease

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27
Q

Pancreatic insufficiency from deficiency in pancreatic enzyme production + bone marrow failure, cyclic neutropenia

A

Schwachman-Diamond Syndrome

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28
Q

Of the three, which has greatest likelihood of steatorrhea:

1) cystic fibrosis
2) celiac sprue
3) Schwachman Diamond

A

Cystic fibrosis

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29
Q

1) absence of ganglion cells

2) hypertrophied nerve fibers

A

Hirschsprungs Disease

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30
Q

% body water in infants

A

70 to 75%

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31
Q

% body water in young adults

A

50 to 60%

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32
Q

Basal energy needs are most accurately calculated by:

A

Body surface area

33
Q

Premature infants who receive inadequate linoleic acid are at high risk for what? (E.g children receiving long term TPN but no lipids)

A

Essential fatty acid deficiency

34
Q

60% whey, 40% casein

A

Mature breast milk

35
Q

40% whey, 60% casein

A

Cow’s milk formula

36
Q

Best formula to use in premature infants

A

One containing medium chain triglycerides (preterm infants have decreased amount of intraluminal bile acids and decreased absorption of LCFA and fat soluble vitamins)

37
Q

Vitamin deficiency that causes macrocytic anemia (hyper segmented neutrophils) and CNS changes

A

B12 deficiency

38
Q

Breast fed infants of vegan mothers are at high risk for

A

Cyanocobalamin deficiency

39
Q

Patients a/p distal small bowel resection are at increased risk for what deficiency

A

B12 (it is absorbed in the illeum)

40
Q

Goat’s milk is deficient in?

A

Folate

41
Q

Methotrexate impairs the absorption of

A

Folate

42
Q

Vitamin K dependent coagulation factors

A

2, 7, 9, 10, protein C & S

43
Q

Syndrome with:

  • Enlargement of costochondral junctions
  • Widening of epiphyses
  • Thickening of wrists
  • Chostochondral beading
  • Bowing of long bones
  • Softening of skull (craniotabes)
A

Rickets

44
Q
  • dry skin
  • alopecia
  • carotenemia
A

Vitamin A Toxicity

45
Q

Paresthesias, foot and wrist drop, Wernicke encephalopathy (opthalmaplegia, ataxia, confusion)

A

Beriberi (Thiamin/B1 deficiency)

46
Q

Cheilosis, sore tongue

A

Riboflavin B2 deficiency

47
Q

Pellagra - dermatitis, dementia, diarrhea

A

Niacin B3 deficiency

48
Q

Seizures

A

Pyridoxine B6 deficiency

49
Q

Megaloblastic anemia

A

Cobalamin B12 and folate deficiency

50
Q

Scurvy

A

Vitamin C deficiency

51
Q

Neural tube defects

A

B12 deficiency

52
Q

Spina bifida

A

Folate deficiency

53
Q

Painful brittle bones, loose teeth, bleeding gums

A

Vitamin C deficiency (needed for collagen, affects vessels and bone junctions)

54
Q

Decreased reflexes, weakness, limited ability to look upward

A

Vitamin E deficiency

55
Q

Hemolysis, reticulocytes, nerve signs

A

Vitamin E deficiency

56
Q

Vitamin K is necessary for which factors

A

2, 7, 9, 10

57
Q

Measures extrinsic pathway (factor VII)

A

Pat

58
Q

Night blindness, immune dysfunction

A

Vitamin A deficiency

59
Q

Scaly skin, pseudotumor cerebri, hepatomegaly

A

Vitamin A excess

60
Q

Macrocephaly, pigemented penile lesions, hamartomatous polyps

A

Ruvalcaba Myhre Smith

61
Q

STK11 gene, hamartomatous polyps, mucho cutaneous hyper pigmentation, associated with other cancers

A

Peutz Jeghers

62
Q

Rectal prolapse can be associated with which disorder

A

CF

63
Q

Pancreatic insufficiency, AR, skeletal abnormalities, neutropenia

A

Shwachman Diamond

64
Q

Arteriohepatic dysplasia

A

Alagille Syndrome

65
Q

AD, paucity of bile ducts, triangular face, peripheral pulmonary stenosis, posterior embryotoxon in eyes, butterfly vertebrae

A

Alagille Syndrome

66
Q

Requires coexistent Hep B infection

A

Hepatitis D

67
Q

20% fatality rate from this fulminate hepatitis in third trimester of pregnancy

A

Hepatitis E

68
Q

Jaundice when ill, defect in promoter for UDP-GT (which conjugated bilirubin) so less enzyme is made to conjugate with. Result = increased level of indirect bili. No tx needed.

A

Gilbert Syndrome

69
Q

Complete absence of UDP-GT so they don’t make any conjugated bilirubin. Tx: lifetime PTX or liver transplant

A

Crigler Najjar Type 1

70
Q

Defect in gene for conjugated bilirubin transporter resulting in mild conjugated hyper bili, no hepatocyte injury

A

Dubin Johnson

71
Q

Partial activity of UDP-GT, does not require therapy

A

Crigler Najjar Syndrome Type 2

72
Q

Disease of copper accumulation

A

Wilson Disease

73
Q

Kayseri Fleischer rings, liver disease, hemolysis, psych illness, behavior changes

A

Wilson Disease

74
Q

Tx: penicillamine

A

Wilson Disease

75
Q

Oral contraceptives associated with hepatic vein thrombosis and liver tumors

A

Buds Chiari Syndrome

76
Q

AR, liver disease in Norwegian newborns, severe cholestasis with lower extremity lymphedema

A

Aagenaes Syndrome

77
Q

Jaundice at one month of life, clay colored stools, dark urine, no gallbladder on ultrasound

A

Biliary atresia

78
Q

Definitive test for biliary atresia

A

Intraoperative cholangiogram

79
Q

Tx for biliary atresia

A

Kasai procedure

80
Q

Gallbladder hydrops seen in:

A 
Kawasaki 
Strep pharyngitis
Prolonged fasting
TPN
HSP
81
Q

Second most common cause of exocrine pancreatic insufficiency (after CF)

A

Schwachman Diamond syndrome. AR

Pediatrics (20 decks)

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