Liver dz Flashcards

1
Q

Which combo of mom and baby can get AFLP?

A

Mother heterozygous for long chain hydroxyacyl CoA DH def, fetus homozygous

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2
Q

Sxs of AFLP?

A

Abd pain, HPM, liver fx

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3
Q

What is AFLP histo?

A

microvesicular steatosis

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4
Q

Which liver pts have the best post TP survival?

A

Cholestatic liver dz like PSC, alc second

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5
Q

Which post TP pts have worst survival

A

HCV

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6
Q

Liver bx in sarcoidosis shows what?

A

Noncaseating granulomas

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7
Q

What LFT is typically abnl in sarcoid of the liver? What other lab test can be helpful?

A

Alk phos; ACE

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8
Q

T/F: Pt with hepatic sarcoid should undergo pulm workup

A

True

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9
Q

What common drug can cause SOS?

A

Azathioprine, chemotx

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10
Q

What do pts with SOS present with?

A

Abd pain, distention, ascites

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11
Q

Which AI conditions more likely in pts with PBC?

A

Sjogrens, CREST, Raynauds, Thyroid Dz

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12
Q

T/F: UDCA can be used for pruritis in PBC?

A

False, no effect on itching

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13
Q

What % of normal ppl have +AMA?

A

<1%

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14
Q

What % of PBC pts are AMA neg?

A

5%

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15
Q

Type 1 GSD (Von Gierkes) has an inc risk of what?

A

Hepatic adenomas which can become HCC

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16
Q

T/F: GSD 1 is AW cirrhosis.

A

False, not AW cirrhosis

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17
Q

Which GSD AW cirrhosis?

A

Type 3 (Forbes-Cori) - debranching enzyme def and abnl prots accumulate in liver, hrt, skel muscle

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18
Q

What is the defect of GSD1?

A

G6Pase def

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19
Q

What should pts with GSD1 avoid and why?

A

Glc, galactose, frc, and Fasting - lead to hypoglycemia which can cause lactic acidosis, HPM, FTT, etc

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20
Q

What foods should be taken in more in GSD 1?

A

Complex carbs such as starches

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21
Q

Why should lipid emulsifications not be added in GSD 1 pts?

A

Already high risk of HLD

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22
Q

Erlenmeyer flask deformity of distal femur in what?

A

Gaucher dz

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23
Q

What is abnl in gaucher?

A

Glucocerebroside

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24
Q

Gaucher dz presentation

A

TCTopenia, bone pain, fx, HSM, anemia

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25
Q

Gaucher dz bx

A

Crumpled paper cells (Gaucher cells)

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26
Q

Name 4 bx findings in NASH.

A

Hepatocyte ballooning, MD bodies, acidophil bodies, hepatic steatosis

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27
Q

Preg < 1 yr after liver TP has higher risk of what?

A

ACR

28
Q

If small varices, how long to wait for repeat EGD?

A

If cause removed, 2 yrs. If cause not removed, 1 yr.

29
Q

If no varices and ongoing liver injury, when to repeat exam?

A

2 yrs

30
Q

T/F: Higher prevalence of LBW babies after liver TP?

A

True - 17-57% but no more deaths or birth defects

31
Q

T/F: Higher rates of birth defects to mothers on mTOR inhibitors or MFM?

A

True; mTOR inhibs include everolimus, sirolimus

32
Q

What drug class is tacrolimus?

A

IL-2 inhibitor

33
Q

What is 5 yr survival of PSC s/p TP?

A

85%

34
Q

After liver TP, what is dz recurrence rate of PSC after 5-10 yrs?

A

20-25%

35
Q

What size to consider TP in CCA pts?

A

< 3 cm after radiation

36
Q

Typical age of presentation of juvenile hered hemochromatosis? What defect involved?

A

Age 15-20, Hemojuvelin defect

37
Q

Besides liver, what does Juv HH affect?

A

heart, amenorrhea

38
Q

Which HH type has very elevated hepcidin whereas the rest have low hepcidin?

A

Ferroportin dz

39
Q

Ferroportin dz iron studies

A

Elevated ferritin, normal Tf sat

40
Q

Which HH has endocrine involevement?

A

TR2 HCTosis

41
Q

How do pts with AFLP present?

A

In third trimester with epigastric pain, HPM, ALF

42
Q

Which gene and mother/baby combo of genes leads to AFLP?

A

LCHAD gene - mother is heterozygous for defect and baby is homozygous for defect

43
Q

AFLP on biopsy has what?

A

microvesicular steatosis

44
Q

List causes of microvesicular steatosis

A

AFLP
Reyes syndrome
Valproic acid tox
Tetracycline tox

45
Q

List conditions with best and worst post TP survival?

A

Best - PBC

Worst - HCV

46
Q

List tx algorithm for budd chiari

A

Anticoag + thrombolysis or stenting if possible.
If no TL/stent, then do TIPS.
If TIPS fx, liver TP

47
Q

T/F: Pts with HCTosis should get liver bx if ferritin >1000 to stage degree of fibrosis

A

True

48
Q
Name if hyperbili is conj or unconj.
Rotor
Gilberts
Dubin Johnson
Crigler-Najjar
A

Unconj - Gilberts and CN

Conj - Rotor and DJ

49
Q

WHich AI DOs is PBC AW

A

CREST, Rayndauds, thyroid dz

50
Q

T/F: Close HLA matching of recipient and donor in liver transplant is a RF for GVHD?

A

True

51
Q

T/F: Donor and recipient HLA mismatching is a RF for GVHD in AHSCT pt?

A

True

52
Q

What is the MC genetic cause of liver dz in kids?

A

A1AT

53
Q

What type of scenario would make A1AT testing inaccurate?

A

Needing transfusions - genetic testing will be off as well as blood A1AT level

54
Q

T/F: GSD1 (von Gierkes) has no inc risk of HCC.

A

False, has adenomas of liver which inc risk of HCC

55
Q

Which GSD has risk for cirrhosis?

A

GSD3 (Forbes-Cori)

56
Q

What dietary changes should someone with GSD1 make?

A

Eat complex carbs like starch every 3-4 hours - otherwise will become hypoglycemic. Fruits should be avoided as no G6Pase and so fructose and galactose cannot be broken down

57
Q

Erlenmeyer flask deformity

A

Gaucher’s dz - defect in glucocerebroside

58
Q

What tx may be helpful in histo in NASH?

A

Vit E

59
Q

Name 4 NASH histo findings.

A

MD bodies, HC ballooning, acidophil bodies, hepatic steatosis

60
Q

T/F: Total serum copper is not a reliable marker of copper status in Wilsons dz.

A

True

61
Q

What is the cutoff for urinary copper for WIlsons dz?

A

> 100 mcg/24 hrs

62
Q

What is the copper quant cutoff on liver bx for Wilsons dz? What is the cutoff below which dx is excluded?

A

> 250 mcg/g dry weight diagnostic

< 50 mcg/g dry weight rules it out

63
Q

Initial rec tx for Wilsons.

A

Trientene

64
Q

MC presentation of NCPH?

A

Variceal bleeding

65
Q

Which HIV med inc risk of NCPH?

A

Didanosine

66
Q

T/F: Pts with NCPH AW HIV are at high risk of PVT

A

True