Erratum to 'Indian National Association for the Study of Liver Consensus Statement on Acute Liver Failure (Part-2): Management of Acute Liver Failure' [J Clin Exp Hepatol 10 (2020) 477-517].

[This corrects the article DOI: 10.1016/j.jceh.2020.04.011.].

Indian National Association for the Study of Liver Consensus Statement on Acute Liver Failure (Part-2): Management of Acute Liver Failure.

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 encephalopathy and cerebral edema in ALF is unique and multifactorial. Ammonia plays a crucial role in the pathogenesis, and several therapies aim to correct this abnormality. The role of newer ammonia-lowering agents is still evolving. These patients are best managed at a tertiary care hospital with facility for liver transplantation (LT). Aggressive intensive medical management has been documented to salvage a substantial proportion of patients. In those with poor prognostic factors, LT is the only effective therapy that has been shown to improve survival. However, recognizing suitable patients with poor prognosis has remained a challenge. Close monitoring, early identification and treatment of complications, and couseling for transplant form the first-line approach to manage such patients. Recent research shows that use of dynamic prognostic models is better for selecting patients undergoing liver transplantation and timely transplant can save life of patients with ALF with poor prognostic factors.

Indian National Association for the Study of the Liver Consensus Statement on Acute Liver Failure (Part 1): Epidemiology, Pathogenesis, Presentation and Prognosis.

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.

Laparoscopic surgery in pancreatic diseases: Pushing the boundaries.

BACKGROUND: Laparoscopic surgery has expanded exponentially in the last two decades but, somehow it is limited in pancreatic surgery by virtue of the pancreas being a friable, retroperitoneal organ with difficult access and adjacent major vessels risking torrential bleed. It is thought to be unforgiving if not handled well. However, improvements in technology and surgeon's expertise have pushed the boundaries of minimal access surgery (MAS) to include pancreas in its domain. We present our series of laparoscopic pancreatic surgery (LPS) with an aim to look at the feasibility and outcomes. METHODS: This is a retrospective review of all LPS done at the Gastrointestinal Surgery (GIS) centre of a tertiary care Armed Forces Hospital over a period of 3 years. RESULTS: A total of 24 LPS were done during this period. The median age of the patients was 46 years (range; 13-81). There were 14 male and 10 female patients. Nine patients had at least one co-morbidity. Three patients underwent laparoscopic lateral pancreaticojejunostomy, 4 distal pancreatectomy, 4 laparoscopic Whipples pancreaticoduodenectomy, 6 laparoscopic pancreatic necrosectomy, 6 laparoscopic cystogastrostomy and 1 roux en y cystojejunostomy. CONCLUSION: LPS can be performed for almost all open pancreatic surgeries and can be done with reasonable outcomes. However, it has a steep learning curve and therefore, a hybrid approach leading to a totally laparoscopic approach may be the way forward.

A 37-year-old male with extensive cerebral venous thrombosis: Clinicopathological correlation of a rare case.

We present the autopsy findings and differential diagnosis of a 37-year-old immunocompetent male patient who presented primarily with extensive cerebral vein thrombosis and was found to have a rare association with JAK2V617F mutation positivity.

A 54-year-old male with rapidly progressive neurologic syndrome: Clinicopathologic correlation of a rare diagnosis.

Diagnosis of systemic lupus erythematosus (SLE) as primary presentation with central nervous system involvement as a rapidly progressive neurologic syndrome is extremely rare. We present a rare case of a 54-year-old hypertensive male patient, who presented with a fulminant neurologic syndrome. He presented with cerebellar and meningeal signs, aseptic meningitis and had a rapid downhill course following admission. A postmortem revealed feature of systemic connective tissue fulfilling diagnostic criteria of SLE with lupus cerebritis.

A 36-year-old man with vomiting, pain abdomen, significant weight loss, hyponatremia, and hypoglycemia.

Diagnosis of Strongyloides stercoralis hyperinfection can be a challenge. The key to a timely diagnosis is to have a high index of suspicion. We present a rare case of a 36-year-old human immunodeficiency virus negative male patient, who was on multidrug therapy for lepromatous leprosy and was treated for type 2 lepra reactions with steroids in the past. The patient presented with vomiting and pain abdomen, persistent hyponatremia, and terminal hypoglycemia. He had features of malnutrition and had a rapid downhill course following admission. A diagnosis of S. stercoralis hyperinfection with sepsis and multiorgan failure, adrenal hemorrhage, and syndrome of inappropriate antidiuretic hormone secretion was established on a postmortem examination.

Assessment of adrenal function in liver diseases.

BACKGROUND: In recent times, there are reports of adrenal dysfunction in whole spectrum of liver disease. Adrenal insufficiency (AI) has been shown to correlate with progression of liver disease. Hence this study was conducted to assess adrenal function in subjects with acute liver disease (ALD), chronic liver disease (CLD) and post liver transplantation (LT). MATERIAL AND METHODS: This study included 25 healthy controls, 25 patients of ALD, 20 subjects of CLD with Child-Pugh stage A (CLD-1) and 30 with Child-Pugh stage B or C (CLD-2), and 10 subjects with LT. All subjects were assessed clinically, biochemically and for adrenal functions. RESULTS: AI was present in 9 (34.6%) patients with ALD, 20 (40%) patients with CLD and 4 (40%) in subjects with LT. AI was more common in CLD-2 (18 patients - 60%) than CLD-1 (2 patients - 10%). All patients with chronic liver disease had significantly lower basal cortisol (8.8±4.8, P=0.01), stimulated cortisol (18.2±6.3, P <0.00001) and incremental cortisol (9.4±4.6, P <0.00001) as compared to controls. There was increase in percentage of subjects with adrenal dysfunction with progression of liver disease as assessed by Child-Pugh staging. AI was predicted by lower levels of serum protein, serum albumin, total cholesterol and HDL cholesterol and higher levels of serum bilirubin and INR. Adrenal functions showed recovery following liver transplantation. CONCLUSIONS: AI forms important part of spectrum of acute and chronic liver disease. Deterioration of synthetic functions of liver disease predicts presence of AI, and these patients should be evaluated for adrenal dysfunction periodically.

Liver transplantation in Indian Armed Forces-initial experience.

BACKGROUND: This study retrospectively analyses the initial experience of liver transplantation (LT) in the Indian Armed Forces. METHODS: Fifty-three patients underwent LT at Army Hospital (R&R) Delhi Cantt. between March 2007 and March 2011. Of these 35 patients underwent deceased donor liver transplantation (DDLT) and living donor liver transplantation (LDLT) was carried out in 18 patients. The surgical techniques, complications and mortality were analysed. RESULTS: A high consent rate of 35.9% for organ donation was achieved by the Armed Forces Organ Retrieval and Transplantation Authority (AORTA). Biliary complications occurred in five patients (9.4%). However, most of them could be managed by endoscopic interventions. Hepatic artery thrombosis (HAT) occurred in five patients (9.4%). Of these, two DDLT grafts were revascularised following HAT, by creating extra-anatomic arterial conduits with excellent outcome. The overall mortality was 18.8% (n = 10). There was no significant difference in the overall complications or mortality in patients undergoing DDLT or LDLT. CONCLUSION: The overall survival and morbidity in this study is comparable to those from other centres. Urgent revascularisation of grafts following HAT should be attempted as it can salvage grafts with satisfactory outcome. There is a reduction in the incidence of biliary complications with refinements in surgical techniques.

Tuberculous pseudoaneurysm of gastroduodenal artery.

We report a patient with Gastroduodenal artery (GDA) pseudoaneurysm of tuberculous aetiology, who presented with massive hematemesis and who was successfully managed with transarterial steel coil embolization. Pseudoaneurysms are a rare but potentially fatal complication of tuberculosis and hence early recognition and management of this complication is important. To the best of our knowledge this is the first report of a GDA pseudoaneurysm resulting from tuberculosis.

Intussusception due to gastrointestinal stromal tumour of the colon: diagnosis by colonoscopy.

Intussusception of the intestine is rare in adults. We report a case of a 45-year-old male who presented with long-standing pyrexia of unknown origin, abdominal pain, gastrointestinal bleeding and constitutional symptoms. Colonoscopic examination revealed a large invaginated mass suggestive of colonic intussusception due to gastrointestinal stromal tumour of the colon. His symptoms disappeared after surgical removal of the tumour.

Plummer Vinson syndrome: unusual features.

Plummer Vinson syndrome is a constellation of postcricoid esophageal webs, iron deficiency anemia, dysphagia and koilonychia. We describe some unusual manifestations in three patients with this syndrome; these were clubbing instead of koilonychia, tortuous esophagus in addition to presence of esophageal webs, and celiac disease.

A CLINICAL STUDY OF FALCIPARUM MALARIA - 302 CASES.

Three hundred and two cases of falciparum malaria including 30 cases of severe falciparum malaria admitted between Jan 93 - Dec 93 were studied for their clinical features and response to treatment. Intermittent or remittent fever with chills and headache were the commonest presentation noted (99.3%). Cough (46.4%), hyperbilirubinemia (31.8%), and pyuria (52.9%) were the other notable features. Anaemia was uncommon (16.6%). Hypoglycacmia was detected in none of the cases. Quinine for 3 days along with tetracycline and sulfadoxine - pyrimethamine achieved a 100 per cent cure rate in uncomplicated chloroquine resistant falciparum malaria. Amodiaquine along with tetracycline and sulfadoxine - pyrimethamine was effective in 76.2 per cent of uncomplicated cases.