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Flashcards in liver 2 Deck (43):
1

Inability to bind copper normally in ceruloplasmin due to genetic defect

Wilson's disease

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Wilson's dz Pathophysiology:

failure to bind copper to ceruloplasmin = accumulation of copper in liver

eventually backup into brain, cornea and kidney

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Wilson's MC diagnosed populations

liver dz in children 9-13 y/o OR neurologic disease in young adults 15-21 yrs

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consider Wilson's in which pt?

young w/:

Hepatitis or splenomegaly

Portal hypertension

Neurologic or psychiatric abnormalities

Coombs negative Hemolytic anemia

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Wilsons manifestations - Liver:

transaminase elevation,
steatosis,
cirrhosis,
portal HTN,

+/- fulminant liver failure

6

Wilsons manifestations -- Neurologic:

dysarthria
ataxic gait,
tremor,
dystonia

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Wilsons manifestations -Psychiatric

personality and behavior changes,
emotional lability,
socially inappropriate behavior

8

Wilsons - Pathognomonic sign

Kayser-Fleisher ring

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how is Wilson's diagnosed? LAB values

increased urinary copper excretion

low serum ceruloplasmin levels

low serum Cu levels

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tx of Wilson's disease

Chelation therapy and lifestyle changes (diet restriction and zinc supplementation)

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Hemochromatosis
Pathophysiology:

increased small absorption of iron = accumulation of hemosiderin in the liver, pancreas, heart, adrenals, testes, and kidneys

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excess iron causes what issues

production of free radicals =

DNA cleavage
impaired protein synthesis
impairment of cell integrity
cell proliferation

13

Hemochromatosis

epidemiology

autosomal recessive

MC in Caucasian,

>50, earlier in men (women menstruate)

14

Hemochromatosis s/s

hepatomegaly, elevated transaminases, fibrosis, cirrhosis

DM, impotence, Dilated Cardiomyopathy, conduction deficits, susceptibility to infection and skin bronzing

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w/u Hemochromatosis

serum transferrin >45% or serum ferritin >200,

genetic testing

Imaging: MRI/CT show signs of iron overload

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Hemochromatosis tx

dietary restriction
weekly phlebotomy

17

AATD patho

genetic defect that does not allow AAT to be released, therefore they accumulate in the liver

18

AATD diagnosis

low serum AAT level

special staining of tissue from liver biopsy

19

autoimmune hepatitis

epidemiology

young/middle aged 40-50 years, women > men

20

s/s of autoimmune hepatitis (general)

arthritis, Sjogren’s syndrome, thyroiditis, nephritis, UC, Coombs + hemolytic anemia

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liver specific s/s of autoimmune hepatitis (6)

Hepatomegaly,
jaundice,
splenomegaly,
spider angiomata,
ascites,
encephalopathy

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lab work up autoimmune hepatitis

Transaminases > 1000
autoantibody testing (ANA, Anti-SM), liver biopsy

23

tx autoimmune hepatitis

mild elevation

low dose prednisone

24

tx autoimmune hepatitis

mod-severe

prednisone and azathioprine

25

NAFLD/NASH patho

obesity and overconsumption of calories causes deposition of fat in liver causes oxidative stress and inflammation

26

NAFLD/NASH epidemiology

Diagnosed in 40s-50s, MC Hispanics > Caucasians > Blacks

Associated with obesity, hypertension, dyslipidemia, insulin resistance/diabetes

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NAFLD/NASH labs

Mild elevations in transaminases (100s) or Alk phos (2-3x ULN, bilirubin normal)

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NAFLD/NASH Causes:

obesity
DM
medications (corticosteroids, amiodarone, tamoxifen, diltiazem, HAART)

29

NAFLD/NASH tx

diet, weight loss, exercise

30

alcoholic liver dz in women

more likely to develop alcoholic hepatitis after consumption of less alcohol and after shorter time, progresses much more quickly

31

alcoholic liver dz
increased likelihood

nutritional deficiencies
concurrent HBV/HCV
other liver toxin ingestion

32

clinical manifestations of alcoholic liver dz

non-specific (malaise, nausea, low grade fever)

UGIB, confusion, AMS, ascites

Alcohol withdrawal

33

alcoholic liver dz Lab Findings:

AST elevated 2x ALT
macrocytic anemia
thrombocytopenia
L shift leukocytosis
prolonged PTT

34

mc cause of mushroom toxicity

amateur mushroom foragers picking one of 100 species that cause harm

35

timeline of mild mushroom toxicity

Milder poisonings typically become symptomatic early (w/in 5 hrs)

36

timeline of severe toxicity

Mushrooms can produce hepatic and renal failure, typically do not produce symptoms for 6-24 hrs

37

clinical manifestations of mushroom toxicity

Fulminant liver failure

Renal failure

GI toxicity

Hemolytic anemia

Rhabdomyolysis

Electrolyte abnormalities

38

End result of hepatocellular injury (fibrosis and nodular regeneration thru out the liver)

Cirrhosis

39

Cirrhosis symp

Abdominal pain
Impotence
Gynecomastia

insidious onset of weakness, fatigability, disturbed sleep, muscle cramps, and weight loss

40

Cirrhosis signs

Rectal varices (hemorrhoids)

Palmar erythema

Dupuytren’s contractures

Muscle wasting

Ascites, pleural effusions, peripheral edema

Spider nevi

Caput medusa

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cirrhosis lab findings

Thrombocytopenia (decreased production and splenomegaly)

Anemia (multifactorial)

Leukopenia/neutropenia

Coagulopathy (increased INR)

Moderately elevated liver enzymes

42

tx of cirrhosis

confirmation of diagnosis and staging, avoidance of alcohol, healthy diet, balanced protein intake

Sodium restriction if patient has fluid retention

Protein restriction if hepatic encephalopathy

Ultrasound screening q 6 months

43

cirrhosis complications

Ascites
Spontaneous Bacterial Peritonitis
Hepatic Encephalopathy
Esophageal Varices