Acute liver failure (ALF) is an infrequent, unpredictable, potentially fatal complication of acute liver injury (ALI) consequent to varied etiologies. Etiologies of ALF as reported in the literature have regional differences, which affects the clinical presentation and natural course. In this part of the consensus article designed to reflect the clinical practices in India, disease burden, epidemiology, clinical presentation, monitoring, and prognostication have been discussed. In India, viral hepatitis is the most frequent cause of ALF, with drug-induced hepatitis due to antituberculosis drugs being the second most frequent cause. The clinical presentation of ALF is characterized by jaundice, coagulopathy, and encephalopathy. It is important to differentiate ALF from other causes of liver failure, including acute on chronic liver failure, subacute liver failure, as well as certain tropical infections which can mimic this presentation. The disease often has a fulminant clinical course with high short-term mortality. Death is usually attributable to cerebral complications, infections, and resultant multiorgan failure. Timely liver transplantation (LT) can change the outcome, and hence, it is vital to provide intensive care to patients until LT can be arranged. It is equally important to assess prognosis to select patients who are suitable for LT. Several prognostic scores have been proposed, and their comparisons show that indigenously developed dynamic scores have an edge over scores described from the Western world. Management of ALF will be described in part 2 of this document.
Acute liver failure (ALF) is not an uncommon complication of a common disease such as acute hepatitis. Viral hepatitis followed by antituberculosis drug-induced hepatotoxicity are the commonest causes of ALF in India. Clinically, such patients present with appearance of jaundice, encephalopathy, and coagulopathy. Hepatic encephalopathy (HE) and cerebral edema are central and most important clinical event in the course of ALF, followed by superadded infections, and determine the outcome in these patients. The pathogenesis of
Hereditary haemorrhagic telangiectasia (HHT) is a rare autosomal dominant disorder characterized by a variety of abnormalities of the vasculature. This study investigated by questionnaire an unselected group of 58 patients suffering from HHT to identify and diagnose any headache history. These were compared with a control group comprising 40 patients with an unrelated autosomal dominant disorder, familial adenomatous polyposis. Fifty percent of the HHT patients reported attacks of headache which fulfilled current diagnostic criteria for migraine with aura. This was over four times the figure for the control group and at least 10 times the expected level for a normal population. The auras described by all the affected patients were classic visual disturbances preceding the headaches. Two patients also described typical visual auras unassociated with headache symptoms. The reason for such a clear association between migraine with aura and HHT is uncertain but cerebral arteriovenous malformations seem to be the likely link. A greater understanding of the reasons for the relationship may provide an insight into the pathophysiology of both conditions.
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