Midgut Flashcards

1
Q

Monitoring test in fulminant hepatic failure

A

PT/INR the best lab to monitor status (up to four times per day)

  • Extensive and complete lab evaluation
  • Doppler ultrasonography of the liver
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2
Q

Transplant criteria for fulminant hepatic failure

A

Acetaminophen induced liver failure
* Arterial pH <7.30
OR
* Grade 3 or 4 encephalopathy with PT>100seconds and Cr>340 mg/L

Non-acetaminophen induced liver failure
* PT>100 seconds
OR
* Any three of :
* Age <10 or >40 years
* Non-A and non-B viral hepatitis, idiosyncratic drug reaction, Wilson
* Jaundice >7 days prior to encephalopathy
* PT>50 seconds
* Bilirubin >180 mg/L

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3
Q

Reservoir for Hep A

A

only in humans

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4
Q

hep A % -> fulminant liver failure?

A

< 1% (usually if already have liver disease)

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5
Q

hep B % -> fulminant liver failure?

A

0.1-0.5% -due to massive immune-mediated lysis of infected hepatocytes (often DNA negative)

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6
Q

HBV extra-hepatic manifestations

A

in 20%
* Polyarteritis nodosa
* Glomerular disease (membranous nephropathy, MPGN, nephrotic syndrome)
* Serum sickness (arthritis, rash)

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7
Q

HBV treatment criteria

A
  • Anyone with cirrhosis
  • Immune active chronic HBV

Maybe (guidelines changing)
* HBV DNA >2000 IU/ml (HBeAg neg) with ALT 2x ULN
* HBV DNA >20,000 IU/ml (HBeAg pos) with ALT 2x ULN

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8
Q

HBV that doesn’t need treatment

A
  • Immune tolerant phase (HBeAg positive, High DNA, normal transaminases)
  • Inactive carrier phase (low or no DNA, normal transaminases)
  • Latent HBV infection (DNA without HBsAg)
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9
Q

Which HBV treatment safe if preggers?

A

Lamivudine

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10
Q

HCV viral halflife?

A

~ 45 mins

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11
Q

Percent of acute HCV -> Chronic -> Cirrhosis

A

60-85% to chronic
20-30% with chronic to cirrhosis (over 20-30 years)

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12
Q

Extra-hep manifestations HCV

A

Cognitive impairment independent of liver disease stage

Lichen planus
* 19% with lichen planus will have HCV
* Six fold rise in risk for HCV in persons with lichen planus
* a T-cell mediated autoimmune disorder in which inflammatory cells attack an unknown protein within the skin and mucosal keratinocytes.

Essential Mixed Cryoglobulinemia
- Half of persons with HCV will have cryoglobulins
- Leukocytoclastic vasculitis
- Arthralgias
- Membranoproliferative glomerulonephritis
- Neurologic disease, peripheral neuropathy

Porphyria Cutanea Tarda
(a rare disorder characterized by painful, blistering skin lesions that develop on sun-exposed skin (photosensitivity))

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13
Q
A

Porphyria Cutanea Tarda
* Decreased activity of uroporphyrinogen decarboxylase
* May be inherited or acquired
* Sun exposed skin changes
* Elevated urine uroporphyrin levels
* 50% of those with PCT have HCV

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14
Q

HEV transmisison

A
  • RNA virus transmitted in water/feces
  • Highest incidence in Asia, Africa, Middle East, and Central America
  • Animal reservoir (rodents, deer, boar)
  • Acute disease in nonimmunocompromized patients
  • Chronic disease in those with transplants
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15
Q

Fulminant HEV?

A
  • Fulminant hepatic failure in 15-25 % in women who are pregnant
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16
Q

Most common meds -> DILD?

A
  • Paracetamol / acetaminophen most common
  • Amoxacillin/clavulanate most common antibiotic
17
Q

Treatment for DILD

A

Drug withdrawal

Specific therapies
* N-acetylcysteine for paracetamol
* L-carnitine for valproic acid overdose

Glucocorticoids - may have a role in hypersensitivity reactions

Transplant if needed

18
Q

Treatment in alcoholic hepatitis

A

Rule out other stuff

Prednisone
- 40 mg daily for 28 days, followed by a 16 day taper
- Lille score can be used to determine response to tx

Pentoxifylline
- 400 mg three times per day as an alternative

Inhibitor of TNF (Controversial) Discontinue nonselective beta-blockers

19
Q

Risk scores in Alc Hep

A

Maddrey Discriminant Function
* DF= (4.6 x PT elevation)+ bilirubin mg/dl
* If >= 32, high short-term mortality, consider steroids

MELD
* If >11, high mortality

Glasgow alcoholic hepatitis score

20
Q

Type 1 vs type 2 AI hepatitis

A

Type 1 AIH
* SMA and ANA
* Sens 43%, Spec 99%
* Anti-actin Ab

Type 2 AIH
* Anti-LKM1
* Spec 99%

21
Q

Simplified diagnostic criteria for AI hep

A
22
Q

Autoimmune hepatitis treatment

A
  • Treatment not required in asymptomatic patients with normal or minimally elevated transaminases and gamma globulin levels and minimal necroinflammatory activity on biopsy
  • Monotherapy
  • prednisone 60, 40, 30, 30, 20 indefinitely
  • Combination
  • Prednisone 30, 20, 15,15,10, … 5…..?
  • Azathioprine 50 mg daily (or 1-2 mg/kg/day)

Investigational
* Budesonide (9mg/day) and azathioprine an option if no cirrhosis

23
Q

Haemachromatosis genes

A

C282Y or H63D

  • C282Y homozygosity in 1 in 250 white northern European descendents
  • Compound heterozygotes (C282Y/H63D) in 10%
24
Q

what upregulates hepcidin?

A

Interleukin-6 upregulates Hepcidin Ç Anemia of chronic disease

25
Q

Hepcidin main actions

A
  • reducing transfer across the basolateral membrane of enterocytes by binding ferroportin
  • induces macrophages to store iron
26
Q

Haemachromatosis diagnostic criteria

A
  • Transferrin saturation >45%
  • Ferritin >200 ng/ml in men or >150 ng/ml in women
  • Liver biopsy is only needed in select cases
27
Q

Haemachromatosis extra-hepatic manifestations

A

No Improvement with phlebotomy
* Diabetes in 50%
* Arthropathy (pseudogout, chondrocalcinosis) Second and Third MCP

Improvement
* Cardiomyopathy
* Hypogonadism (secondary)

28
Q

ceruloplasmin in Wilson’s disease

A
  • Low serum ceruloplasmin - due to instability from lack of Cu incorporation
  • Binds and transports the copper from the cytoplasm to the late endosomal lumen
29
Q

Clinical manifestations Wilson’s disease

A

Liver disease – 18-84%
* Steatosis, fulminant hepatic failure with Coombs-negative hemolytic anemia, cirrhosis
* Acute liver failure – alkaline phosphatase is typically normal or subnormal
* Only 50% have Kayser-Fleischer rings
Eye signs

Neurologic Disease – 18-73%
* Behavior deterioration, difficulty writing, tremor, speech change, Parkinsonlike, dysarthria, dystonia, ataxia, drooling
* MR may show basal ganglia hyperintensity on T2-weighted images
* 98% will have Kayser-Fleischer rings, Sunflower cataracts
* Elevated copper in CSF

Psychiatric – 10-100%
* psychosis, depression, anxiety

Most diagnosed between 5 and 35 years

30
Q

Treatment for Wilson’s?

A

Copper removal
1. Chelators - usually 6 -12 months to remove the excess copper
* D-penicillamine – 30% cannot tolerate
* Trientine – Lower AE, expensive
2. Tetrathiomolybdate – Being studied

Low Copper diet
– Avoid liver, kidney, shellfish, nuts, dried fruits or beans, peas, unprocessed wheat, chocolate, cocoa, and mushrooms

  • Oral Zinc- interferes with Cu absorption
31
Q

Which patients should D-penicillamine not be used in

A

– Not ideal for neurologic disease – may worsen neuro symptoms

in general ~ 30% cannot tolerate

32
Q

What is the number one cause of death in individuals with nonalcoholic steatoheapatitis (NASH)?

A

Cardiovascular disease

33
Q

MELD score needed for liver transplant

A

15 and above are approaching it

34
Q

Leading cause of death in a person who has had a liver transplant?

A

Infection

35
Q

Liver transplantation complications?

A

Infection
- leading cause for mortality after transplant
- Highest risk in the first three months
CMV (arthralgias, leukopenia, recent treatment for rejection)

Biliary obstruction or hepatic artery thrombus if cholestatic

Hypertension
- develops in approximately 70% post-transplant

Diabetes

Dyslipidemia

Metabolic bone disease

Malignancy