Inherited hyperbilirubinemia - Unconjugated?
• Criggler-Najjar and Gilbert’s syndromeQ
Inherited hyperbilirubinemia – Conjugated?
- 1. Dubin-Johnson syndromeQ
- 2. Rotor’s syndromeQ
Most common surgical cause of obstructive jaundice?
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.
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
Enzymes elevatedin Cholestasis?
- • ALPQ
- • 5’-NucleotidaseQ
- • Gamma glutamyl transpeptidaseQ
Liver biopsy is done through which intercoastal space?
8th ICS in midaxillary line to avoid Lung injury Q
Size of biopsy specimen for grading of chronic viral hepatitis?
2 cm Q in length or longer containing at least 11 complete portal tractsQ
DOC of choice for Actinomycosis of liver? Q.
What is Peliosis Hepatitis?
- Multiple, small, blood-filled sinusesQ
- Commonly occurs in immunocompromised posttransplant patients, AIDS patients, and patients taking long term steroidsQ
Treatment of Peliosis hepatitis?
Manifestation of Hemochromatosis?
- Liver: Hepatomegaly, cirrhosis, HCCQ
- Skin: HyperpigmentationQ (bronzing of skin)
- Pancreas: Diabetes mellitusQ
- Joints: ArthropathyQ
- Heart: CHF, cardiomyopathyQ
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.
Physiological defect in Wilson disease?
Excessive absorption of copper from the small intestine and decreased excretion of copper by the liver Q.
Chromosome defect in Wilson disease?
13q (ATP7B gene)Q
Clinical manifestation in Wilson disease?
- Liver disease - First decade of life
- Neuropsychiatric illness - Third decadeQ.
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
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
Treatment of Wilson’s disease?
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.