Step 3 Hepatology

• The disproportionate elevation of AST levels is the most typical biochemical pattern in these patients.
• At the same time, an elevated AST/ALT ratio (more than 2.0) is quite specific because it is rarely seen in other forms of liver disease (e.g., viral hepatitis or ischemic liver injury).
• The relatively lower elevation of serum ALT levels has been ascribed to hepatic deficiency of pyridoxal 6-phosphate in alcoholics, which is a cofactor for the enzymatic activity of AL T; therefore, the increased AST/ALT ratio is generally attributed to failure to appropriately increase the ALT levels, rather than a disproportionate elevation in AST levels.

• It causes progressive parenchymal liver damage.
• Its course is variable, and severe cases can progress to cirrhosis and liver failure in 6 months.
• Most cases occur in young to middle-aged women. Autoimmune manifestations such as arthritis, erythema nodosum, thyroiditis, pleurisy, pericarditis, anemia and sicca syndrome are common.

• Common findings include elevated AST and ALT levels, a normal or near-normal alkaline phosphatase level, and a normal bilirubin level.
• Autoantibodies are common, and the most typical are antinuclear antibodies (in a homogeneous staining pattern, hence the term "lupoid hepatitis") and anti smooth muscle antibodies (against actin).

• It is a chronic and progressive autoimmune liver disease .
• Most patients (90%) are women, and disease onset is usually between 30 and 65 years of age.
• Its major pathological feature is the destruction of small- and mid-sized bile ducts. There is progressive fibrosis, and end-stage liver disease can supervene 5 to 10 years after the diagnosis.

Signs and symptoms include progressive jaundice, steatorrhea, fatigue (an early and sometimes debilitating symptom), hyperlipidemia with formation of xanthomas, bone disease (osteoporosis and/or osteomalacia), hyperpigmentation of exposed areas and autoimmune manifestations such as keratoconjunctivitis sicca, CREST syndrome, type 1 diabetes, rheumatoid arthritis and antithyroid antibodies.

• Antimitochondrial antibodies (AMA) have high sensitivity (more than 90%) and 98% specificity for PBC.
• Diagnostic confirmation requires liver biopsy.
• This procedure can also give information about disease stage and prognosis.

• It include ursodeoxycholic acid and liver transplantation.
• Steroids and immunosuppressive drugs aren't useful, despite the disease's apparently autoimmune nature.
• Ursodeoxycholic acid can slow the progression of PBC, improve overall survival and maybe transplantation-free survival.

• Osteomalacia and osteoporosis are important complications of long-standing cholestatic disease, and of PBC in particular.
• Screening with bone densitometry, calcium supplementation and eventual treatment with vitamin D and/or bisphosphonates are essential in these patients follow-up.

• It is frequently asymptomatic but can present with malaise, nausea, right upper quadrant pain, and liver function test abnormalities.
• It is important to maintain a high degree of suspicion for hepatitis C in patients with risk factors.
• Because anti-HCV antibodies are frequently negative during the acute phase of infection, testing for HCV RNA may be necessary.
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• The Model for End-stage Liver Disease (MELD) scoring system is preferred to the Child-Turcotte Pugh scoring system for determining prognosis in patients with chronic liver disease.
• It is also the scoring system used to determine allocation of liver transplants and whether or not TIPS should be performed.
• The MELD score can be calculated using the following formula: 3.8[ serum bilirubin (mg/dL)] + 11.2[INR] +9.6 [serum creatinine (mg/dL)] +6.4.

• Hepatitis A vaccine should be given to all unimmunized patients with underlying chronic liver disease.
• Hepatitis B vaccine is recommended in all unimmunized patients with chronic liver disease, including chronic hepatitis C.

Patients with chronic hepatitis C having HCV RNA positive, consistently elevated ALT, and at least moderate inflammation on liver biopsy should be treated with interferon and ribavirin.

• It is a autosomal recessive genetic disorders.
• It results in the excessive accumulation of iron in parenchymal organs.
• It can ultimately manifest as liver disease, skin pigmentation, diabetes mellitus, arthropathy, impotence, or cardiac enlargement.
• Symptoms are often present for ten years or more before the diagnosis is made.
• The classic triad of cirrhosis, diabetes mellitus, and skin pigmentation ("bronze diabetes") is a late finding that occurs when the total body iron content is 20 grams or more.

• The preferred means of treating hemochromatosis is therapeutic phlebotomy, which entails the removal of approximately one unit of blood each week until iron stores normalize.
• Patients with a normal ALT level and/or mild inflammation without fibrosis on liver biopsy can be observed closely.

• Small (less than 5cm) and asymptomatic lesions are usually managed with discontinuation of OCPs.
• Malignancy should be suspected if the tumor increases in size even after discontinuation of OCPs, or if the AFP level is increased.

Main complications of hepatic adenoma are sudden rupture with intra-abdominal bleeding and malignant transformation.

• Patients with an isolated elevated level of anti HBc antibody should have a repeat Hepatitis B panel checked to rule out a false positive result, followed by a measurement of anti HBc lgM and liver function tests to determine the acuity of the infection.
• These tests are helpful since anti HBc may be the only positive result in the Hepatitis B panel in a patient with acute infection during the "window period."