liver 2 Flashcards by Sara Fieger

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

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Wilsons manifestations – Neurologic:

dysarthria
ataxic gait,
tremor,
dystonia

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Wilsons manifestations -Psychiatric

personality and behavior changes,
emotional lability,
socially inappropriate behavior

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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

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Hemochromatosis

epidemiology

autosomal recessive

MC in Caucasian,

> 50, earlier in men (women menstruate)

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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

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AATD patho

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

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AATD diagnosis

low serum AAT level

special staining of tissue from liver biopsy

autoimmune hepatitis

epidemiology

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

s/s of autoimmune hepatitis (general)

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

liver specific s/s of autoimmune hepatitis (6)

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

lab work up autoimmune hepatitis

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

tx autoimmune hepatitis

mild elevation

low dose prednisone

tx autoimmune hepatitis

mod-severe

prednisone and azathioprine

NAFLD/NASH patho

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

NAFLD/NASH epidemiology

Diagnosed in 40s-50s, MC Hispanics > Caucasians > Blacks Associated with obesity, hypertension, dyslipidemia, insulin resistance/diabetes

NAFLD/NASH labs

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

NAFLD/NASH Causes:

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

NAFLD/NASH tx

diet, weight loss, exercise

alcoholic liver dz in women

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

alcoholic liver dz | increased likelihood

nutritional deficiencies concurrent HBV/HCV other liver toxin ingestion

clinical manifestations of alcoholic liver dz

non-specific (malaise, nausea, low grade fever) UGIB, confusion, AMS, ascites Alcohol withdrawal

alcoholic liver dz Lab Findings:

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

mc cause of mushroom toxicity

amateur mushroom foragers picking one of 100 species that cause harm

timeline of mild mushroom toxicity

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

timeline of severe toxicity

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

clinical manifestations of mushroom toxicity

Fulminant liver failure Renal failure GI toxicity Hemolytic anemia Rhabdomyolysis Electrolyte abnormalities

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

Cirrhosis

Cirrhosis symp

Abdominal pain Impotence Gynecomastia insidious onset of weakness, fatigability, disturbed sleep, muscle cramps, and weight loss

Cirrhosis signs

Rectal varices (hemorrhoids) Palmar erythema Dupuytren’s contractures Muscle wasting Ascites, pleural effusions, peripheral edema Spider nevi Caput medusa

cirrhosis lab findings

Thrombocytopenia (decreased production and splenomegaly) Anemia (multifactorial) Leukopenia/neutropenia Coagulopathy (increased INR) Moderately elevated liver enzymes

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

cirrhosis complications

Ascites Spontaneous Bacterial Peritonitis Hepatic Encephalopathy Esophageal Varices