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Inherited hyperbilirubinemia - Unconjugated?
• Criggler-Najjar and Gilbert’s syndromeQ
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Inherited hyperbilirubinemia – Conjugated?
- 1. Dubin-Johnson syndromeQ
- 2. Rotor’s syndromeQ
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Most common surgical cause of obstructive jaundice?
CBD stones
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What is Courvoisier’s Law?
In obstruction of the common bile duct due to a stone, distention of gall bladder seldom occurs; the organ usually is shriveledQ.
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Exceptions to Courvoisier’s Law?
- • Double impaction of stonesQ i.e. one in the cystic duct and other in CBD.
- • Oriental cholangiohepatitisQ
- • Pancreatic calculus obstructing the ampulla of VaterQ
- • Mucocele due to stone in the cystic ductQ
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Enzymes elevatedin Cholestasis?
- • ALPQ
- • 5’-NucleotidaseQ
- • Gamma glutamyl transpeptidaseQ
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Liver biopsy is done through which intercoastal space?
8th ICS in midaxillary line to avoid Lung injury Q
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Size of biopsy specimen for grading of chronic viral hepatitis?
2 cm Q in length or longer containing at least 11 complete portal tractsQ
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DOC of choice for Actinomycosis of liver? Q.
Penicillin
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What is Peliosis Hepatitis?
- Multiple, small, blood-filled sinusesQ
- Commonly occurs in immunocompromised posttransplant patients, AIDS patients, and patients taking long term steroidsQ
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Treatment of Peliosis hepatitis?
Angiographic embolizationQ.
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Manifestation of Hemochromatosis?
- Liver: Hepatomegaly, cirrhosis, HCCQ
- Skin: HyperpigmentationQ (bronzing of skin)
- Pancreas: Diabetes mellitusQ
- Joints: ArthropathyQ
- Heart: CHF, cardiomyopathyQ
- HypogonadismQ
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What is Wilson’s Disease?
Autosomal recessiveQ inherited disorder of copper metabolism, characterized by excessive deposition of copper in the liver, brain, and other tissuesQ.
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Physiological defect in Wilson disease?
Excessive absorption of copper from the small intestine and decreased excretion of copper by the liver Q.
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Chromosome defect in Wilson disease?
13q (ATP7B gene)Q
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Clinical manifestation in Wilson disease?
- Liver disease - First decade of life
- Neuropsychiatric illness - Third decadeQ.
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What are Kayser-Fleischer rings?
- Deposition of copper in the Descemet’s membrane in the limbus of the cornea.
- Color - Greenish gold to brownQ
- Rings - initially appear at the superior poleQ
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Diagnosis of Wilson disease?
- Ceruloplasmin <20 mg/dQ (reference range, 20-40 mg/dL)
- Urinary copper excretion rate is >100 mg/dQ (reference range, <40 mg/d)
- A liver biopsy with sufficient tissue reveals levels of >250 μg/g of dry weightQ even in asymptomatic patients
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Treatment of Wilson’s disease?
ZincQ
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Liver-specific contrast media?
- Mangofodipir trisodiumQ (taken up by hepatocytes) and
- Ferrumoxides (taken up by Kupffer cells)Q
- These two contrast agents are also useful in characterizing specific liver tumors, such as FNH, hepatic adenoma and HCCQ.
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