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Gastro Review > Liver dz > Flashcards

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
Gaucher dz bx
Crumpled paper cells (Gaucher cells)
26
Name 4 bx findings in NASH.
Hepatocyte ballooning, MD bodies, acidophil bodies, hepatic steatosis
27
Preg < 1 yr after liver TP has higher risk of what?
ACR
28
If small varices, how long to wait for repeat EGD?
If cause removed, 2 yrs. If cause not removed, 1 yr.
29
If no varices and ongoing liver injury, when to repeat exam?
2 yrs
30
T/F: Higher prevalence of LBW babies after liver TP?
True - 17-57% but no more deaths or birth defects
31
T/F: Higher rates of birth defects to mothers on mTOR inhibitors or MFM?
True; mTOR inhibs include everolimus, sirolimus
32
What drug class is tacrolimus?
IL-2 inhibitor
33
What is 5 yr survival of PSC s/p TP?
85%
34
After liver TP, what is dz recurrence rate of PSC after 5-10 yrs?
20-25%
35
What size to consider TP in CCA pts?
< 3 cm after radiation
36
Typical age of presentation of juvenile hered hemochromatosis? What defect involved?
Age 15-20, Hemojuvelin defect
37
Besides liver, what does Juv HH affect?
heart, amenorrhea
38
Which HH type has very elevated hepcidin whereas the rest have low hepcidin?
Ferroportin dz
39
Ferroportin dz iron studies
Elevated ferritin, normal Tf sat
40
Which HH has endocrine involevement?
TR2 HCTosis
41
How do pts with AFLP present?
In third trimester with epigastric pain, HPM, ALF
42
Which gene and mother/baby combo of genes leads to AFLP?
LCHAD gene - mother is heterozygous for defect and baby is homozygous for defect
43
AFLP on biopsy has what?
microvesicular steatosis
44
List causes of microvesicular steatosis
AFLP Reyes syndrome Valproic acid tox Tetracycline tox
45
List conditions with best and worst post TP survival?
Best - PBC | Worst - HCV
46
List tx algorithm for budd chiari
Anticoag + thrombolysis or stenting if possible. If no TL/stent, then do TIPS. If TIPS fx, liver TP
47
T/F: Pts with HCTosis should get liver bx if ferritin >1000 to stage degree of fibrosis
True
48
``` Name if hyperbili is conj or unconj. Rotor Gilberts Dubin Johnson Crigler-Najjar ```
Unconj - Gilberts and CN | Conj - Rotor and DJ
49
WHich AI DOs is PBC AW
CREST, Rayndauds, thyroid dz
50
T/F: Close HLA matching of recipient and donor in liver transplant is a RF for GVHD?
True
51
T/F: Donor and recipient HLA mismatching is a RF for GVHD in AHSCT pt?
True
52
What is the MC genetic cause of liver dz in kids?
A1AT
53
What type of scenario would make A1AT testing inaccurate?
Needing transfusions - genetic testing will be off as well as blood A1AT level
54
T/F: GSD1 (von Gierkes) has no inc risk of HCC.
False, has adenomas of liver which inc risk of HCC
55
Which GSD has risk for cirrhosis?
GSD3 (Forbes-Cori)
56
What dietary changes should someone with GSD1 make?
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
Erlenmeyer flask deformity
Gaucher's dz - defect in glucocerebroside
58
What tx may be helpful in histo in NASH?
Vit E
59
Name 4 NASH histo findings.
MD bodies, HC ballooning, acidophil bodies, hepatic steatosis
60
T/F: Total serum copper is not a reliable marker of copper status in Wilsons dz.
True
61
What is the cutoff for urinary copper for WIlsons dz?
> 100 mcg/24 hrs
62
What is the copper quant cutoff on liver bx for Wilsons dz? What is the cutoff below which dx is excluded?
> 250 mcg/g dry weight diagnostic | < 50 mcg/g dry weight rules it out
63
Initial rec tx for Wilsons.
Trientene
64
MC presentation of NCPH?
Variceal bleeding
65
Which HIV med inc risk of NCPH?
Didanosine
66
T/F: Pts with NCPH AW HIV are at high risk of PVT
True

Gastro Review (15 decks)

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