Hepatic encephalopathy (HE) is a major complication that develops in some form and at some stage in a majority of patients with liver cirrhosis. Overt HE occurs in approximately 30-45% of cirrhotic patients. Minimal HE (MHE), the mildest form of HE, is characterized by subtle motor and cognitive deficits and impairs health-related quality of life. The Indian National Association for Study of the Liver (INASL) set up a Working Party on MHE in 2008 with a mandate to develop consensus guidelines on various aspects of MHE relevant to clinical practice. Questions related to the definition of MHE, its prevalence, diagnosis, clinical characteristics, pathogenesis, natural history and treatment were addressed by the members of the Working Party.Key words critical clicker frequency, health-related quality of life, hepatic encephalopathy, lactulose, minimal hepatic encephalopathy, psychometric hepatic encephalopathy score, psychometry.

Porphyrias are a heterogenous group of diseases that may result in disabling or life threatening neurovisceral symptoms and/or cutaneous photosensitivity. In acute intermittent porphyria, the clinical features, particularly neurological symptoms, may be life-threatening and disabling. Conventional treatment with human hemin, though effective in reducing symptoms, does not reverse neuropathy when structural nerve damage has occurred and may cause intense phlebitis. Liver transplantation (LT) may be considered as treatment for those with repeated life-threatening acute attacks resulting in poor quality of life, requirement of ventilatory support, and progressive loss of venous access due to hemin infusion. Patients with variegate porphyria (VP) present after puberty with neurovisceral symptoms and skin manifestations. LT resolved VP in the 1 patient reported in the literature. Aminolaevulinic acid dehydratase deficient porphyria is a rare autosomal recessive disorder and a child who presented with failure to thrive and required transfusions and parenteral nutrition did not improve with LT. In erythropoietic protoporphyria (EPP), there is excessive production of protoporphyrin in the bone marrow. Protoporphyrin is hepatotoxic and pigment loading of hepatocytes and bile canalicular sludging may result in progressive cholestasis and cirrhosis. LT is beneficial for such patients with end-stage liver disease. Perioperative management includes use of filters on operative lights to prevent skin burns and intestinal perforation. Other concerns include development of neuropathy, biliary complications, and recurrent liver disease. This review addresses the rationale, patient selection, evaluation, management issues, and technique of performing LT in various types of porphyria.

Extended stay at HAA may be a risk factor for development of symptomatic portal system thrombosis.

Organ donation following brain stem death is infrequent in India. There is no prospective study on prevalence of brain stem death and causes of non-donation. Consecutive patients admitted to intensive care unit from Sep 2006 to Sep 2008 were studied prospectively. Families of those with brain stem death were approached for organ donation by transplant coordinator. Extensive awareness drive was launched. Reasons for non-donation, if any, were documented. Of 2820 patients admitted, 994 (35%) were on mechanical ventilator and 657 (23%) died. Brain stem death could be diagnosed in 55, 37 males, median age 46 years (range 7 to 87 years) i.e., 1.9% of all admissions and 8.3% of all deaths. Among neurology and neurosurgery patients brain stem death was seen in 45 of 1037 (4.3%) admissions and 45 of 161 (27.9%) deaths. Complications of brain stem death were hypotension in 49, diabetes insipidus in 17 and hypertension in 5 patients. Of 33 families counselled, 16(48%) consented to organ donation. In 14(