liver 2 Flashcards

1
Q

Inability to bind copper normally in ceruloplasmin due to genetic defect

A

Wilson’s disease

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2
Q

Wilson’s dz Pathophysiology:

A

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

eventually backup into brain, cornea and kidney

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3
Q

Wilson’s MC diagnosed populations

A

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

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4
Q

consider Wilson’s in which pt?

A

young w/:

Hepatitis or splenomegaly

Portal hypertension

Neurologic or psychiatric abnormalities

Coombs negative Hemolytic anemia

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5
Q

Wilsons manifestations - Liver:

A

transaminase elevation,
steatosis,
cirrhosis,
portal HTN,

+/- fulminant liver failure

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6
Q

Wilsons manifestations – Neurologic:

A

dysarthria
ataxic gait,
tremor,
dystonia

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7
Q

Wilsons manifestations -Psychiatric

A

personality and behavior changes,
emotional lability,
socially inappropriate behavior

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8
Q

Wilsons - Pathognomonic sign

A

Kayser-Fleisher ring

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9
Q

how is Wilson’s diagnosed? LAB values

A

increased urinary copper excretion

low serum ceruloplasmin levels

low serum Cu levels

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10
Q

tx of Wilson’s disease

A

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

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11
Q

Hemochromatosis

Pathophysiology:

A

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

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12
Q

excess iron causes what issues

A

production of free radicals =

DNA cleavage
impaired protein synthesis
impairment of cell integrity
cell proliferation

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13
Q

Hemochromatosis

epidemiology

A

autosomal recessive

MC in Caucasian,

> 50, earlier in men (women menstruate)

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14
Q

Hemochromatosis s/s

A

hepatomegaly, elevated transaminases, fibrosis, cirrhosis

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

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15
Q

w/u Hemochromatosis

A

serum transferrin >45% or serum ferritin >200,

genetic testing

Imaging: MRI/CT show signs of iron overload

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16
Q

Hemochromatosis tx

A

dietary restriction

weekly phlebotomy

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17
Q

AATD patho

A

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

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18
Q

AATD diagnosis

A

low serum AAT level

special staining of tissue from liver biopsy

19
Q

autoimmune hepatitis

epidemiology

A

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

20
Q

s/s of autoimmune hepatitis (general)

A

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

21
Q

liver specific s/s of autoimmune hepatitis (6)

A
Hepatomegaly, 
jaundice, 
splenomegaly, 
spider angiomata, 
ascites, 
encephalopathy
22
Q

lab work up autoimmune hepatitis

A
Transaminases > 1000
autoantibody testing (ANA, Anti-SM), liver biopsy
23
Q

tx autoimmune hepatitis

mild elevation

A

low dose prednisone

24
Q

tx autoimmune hepatitis

mod-severe

A

prednisone and azathioprine

25
Q

NAFLD/NASH patho

A

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

26
Q

NAFLD/NASH epidemiology

A

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

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

27
Q

NAFLD/NASH labs

A

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

28
Q

NAFLD/NASH Causes:

A

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

29
Q

NAFLD/NASH tx

A

diet, weight loss, exercise

30
Q

alcoholic liver dz in women

A

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

31
Q

alcoholic liver dz

increased likelihood

A

nutritional deficiencies
concurrent HBV/HCV
other liver toxin ingestion

32
Q

clinical manifestations of alcoholic liver dz

A

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

UGIB, confusion, AMS, ascites

Alcohol withdrawal

33
Q

alcoholic liver dz Lab Findings:

A
AST elevated 2x ALT
macrocytic anemia
thrombocytopenia
L shift leukocytosis
prolonged PTT
34
Q

mc cause of mushroom toxicity

A

amateur mushroom foragers picking one of 100 species that cause harm

35
Q

timeline of mild mushroom toxicity

A

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

36
Q

timeline of severe toxicity

A

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

37
Q

clinical manifestations of mushroom toxicity

A

Fulminant liver failure

Renal failure

GI toxicity

Hemolytic anemia

Rhabdomyolysis

Electrolyte abnormalities

38
Q

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

A

Cirrhosis

39
Q

Cirrhosis symp

A

Abdominal pain
Impotence
Gynecomastia

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

40
Q

Cirrhosis signs

A

Rectal varices (hemorrhoids)

Palmar erythema

Dupuytren’s contractures

Muscle wasting

Ascites, pleural effusions, peripheral edema

Spider nevi

Caput medusa

41
Q

cirrhosis lab findings

A

Thrombocytopenia (decreased production and splenomegaly)

Anemia (multifactorial)

Leukopenia/neutropenia

Coagulopathy (increased INR)

Moderately elevated liver enzymes

42
Q

tx of cirrhosis

A

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
Q

cirrhosis complications

A

Ascites
Spontaneous Bacterial Peritonitis
Hepatic Encephalopathy
Esophageal Varices