Aabha Nagral

A novel Global Assessment Scale for Wilson's Disease (GAS for WD)

Wilsons disease (WD) is an inherited disorder of copper metabolism. Despite being treatable, patients with WD suffer severe disabilities due to delay in initiation and difficulty in monitoring treatment. We propose a two tier, Global Assessment Scale for Wilsons Disease (GAS for WD) that grades the multisystemic manifestations of the disease. Tier 1 scores the global disability in four domains: Liver, Cognition and behavior, Motor, and Osseomuscular. Tier 2 is multidimensional scale for a fine grained evaluation of the neurological dysfunction. We prospectively validated this scale in 30 patients with WD. Both tiers had a high inter‐rater reliability (Intraclass correlation coefficient ICC (A, 2) = 0.96–1.0). Tier 2 items were internally consistent (Cronbachs α = 0.89) and factorial analysis showed that 90.3% of the Tier 2 total score variance was determined by seven factors. Scores of both tiers were commensurate with the disease burden as assessed by standard disability scales (Child Pugh, UPDRS, SS3, and CGI) and satisfied criteria for validity. Longitudinal follow‐up over 1.5 years showed that the scale was sensitive to clinical change. This suggests that GAS for WD is a practical tool with potential applications in management of patients, and in testing and comparison of treatment regimens.

Budd-Chiari syndrome in children: experience with therapeutic radiological intervention.

Objectives: Budd-Chiari syndrome (BCS) in children is not uncommon. Published literature on therapy for this condition is scarce. We therefore attempted radiological interventions in these patients to determine their efficacy and safety. Patients and Methods: Fourteen of 16 children with a median age of 22 months diagnosed as having BCS were subjected to an inferior vena cava/hepatic venogram with the aim to establish a normal antegrade flow in at least 1 hepatic vein (HV). Results: A normal antegrade flow in at least 1 of the HVs could be established in 11 children. Three patients had angioplasty of the HV (vein size ≤4 mm), 2 underwent HV stent placements (vein size ≥5 mm), and 6 had transjugular intrahepatic porta systemic shunt ([TIPSS] total occlusion of all 3 HVs or veno-occlusive disease). The youngest child undergoing a successful stenting was 7 months of age and the child undergoing TIPSS was 3 years of age. One patient had reversal of fulminant liver failure following a successful TIPSS. Postprocedure, 2 patients developed reversible encephalopathy and 1 had a neck hematoma. There was no procedure-related mortality. The procedure was successful in both patients with stenting (100%), 5 of the 6 patients with TIPSS (80%), and only 1 of the 4 patients (25%) with angioplasty. The median follow-up was 31 months. Conclusions: Radiological therapeutic intervention is feasible and safe in children with BCS. The overall results of stenting/TIPSS are better than with angioplasty; however, long-term results of these interventions need to be evaluated.

Consensus Statement of HCV Task Force of the Indian National Association for Study of the Liver (INASL). Part I: Status Report of HCV Infection in India

Globally, around 150 million people are infected with hepatitis C virus (HCV). India contributes a large proportion of this HCV burden. The prevalence of HCV infection in India is estimated at between 0.5% and 1.5%. It is higher in the northeastern part, tribal populations and Punjab, areas which may represent HCV hotspots, and is lower in western and eastern parts of the country. The predominant modes of HCV transmission in India are blood transfusion and unsafe therapeutic injections. There is a need for large field studies to better understand HCV epidemiology and identify high-prevalence areas, and to identify and spread awareness about the modes of transmission of this infection in an attempt to prevent disease transmission.

Consensus Statement of HCV Task Force of the Indian National Association for Study of the Liver (INASL). Part II: INASL Recommendations for Management of HCV in India.

The estimated prevalence of hepatitis C virus (HCV) infection in India is between 0.5 and 1.5% with hotspots showing much higher prevalence in some areas of northeast India, in some tribal populations and in certain parts of Punjab. Genotype 3 is the most prevalent type of infection. Recent years have seen development of a large number of new molecules that are revolutionizing the treatment of hepatitis C. Some of the new directly acting agents (DAAs) like sofosbuvir have been called game-changers because they offer the prospect of interferon-free regimens for the treatment of HCV infection. These new drugs have not yet been approved in India and their cost and availability is uncertain at present. Till these drugs become available at an affordable cost, the treatment that was standard of care for the whole world before these newer drugs were approved should continue to be recommended. For India, cheaper options, which are as effective as the standard-of-care (SOC) in carefully selected patients, are also explored to bring treatment within reach of poorer patients. It may be prudent to withhold treatment at present for selected patients with genotype 1 or 4 infection and low levels of fibrosis (F1 or F2), and for patients who are non-responders to initial therapy, interferon intolerant, those with decompensated liver disease, and patients in special populations such as stable patients after liver and kidney transplantation, HIV co-infected patients and those with cirrhosis of liver.

Surgery in a patient with liver disease.

Surgery is often needed in patients with concurrent liver disease. The multiple physiological roles of the liver places these patients at an increased risk of morbidity and mortality. Diseases necessitating surgery like gallstones and hernia are more common in patients with cirrhosis. Assessment of severity of liver dysfunction before surgery is important and the risk benefit of the procedure needs to be carefully assessed. The disease severity may vary from mild transaminase rise to decompensated cirrhosis. Surgery should be avoided if possible in the emergency setting, in the setting of acute and alcoholic hepatitis, in a patient of cirrhosis who is child class C or has a MELD score more than 15 or any patient with significant extrahepatic organ dysfunction. In this subset of patients, all possible means to manage these patients conservatively should be attempted. Modified Child-Pugh scores and model for end-stage liver disease (MELD) scores can predict mortality after surgery fairly reliably including nonhepatic abdominal surgery. Pre-operative optimization would include control of ascites, correction of electrolyte imbalance, improving renal dysfunction, cardiorespiratory assessment, and correction of coagulation. Tests of global hemostasis like thromboelastography and thrombin generation time may be more predictive of the risk of bleeding compared with the conventional tests of coagulation in patients with cirrhosis. Correction of international normalized ratio with fresh frozen plasma does not necessarily mean reduction of bleeding risk and may increase the risk of volume overload and lung injury. International normalized ratio liver may better reflect the coagulation status. Recombinant factor VIIa in patients with cirrhosis needing surgery needs further study. Intra-operatively, safe anesthetic agents like isoflurane and propofol with avoidance of hypotension are advised. In general, nonsteroidal anti-inflammatory drug (NSAIDs) and benzodiazepines should not be used. Intra-abdominal surgery in a patient with cirrhosis becomes more challenging in the presence of ascites, portal hypertension, and hepatomegaly. Uncontrolled hemorrhage due to coagulopathy and portal hypertension, sepsis, renal dysfunction, and worsening of liver failure contribute to the morbidity and mortality in these patients. Steps to reduce ascitic leaks and infections need to be taken. Any patient with cirrhosis undergoing major surgery should be referred to a specialist center with experience in managing liver disease.

Long-term clinical outcome of Budd-Chiari syndrome in children after radiological intervention.

Objectives Budd–Chiari syndrome (BCS) is an uncommon cause of chronic liver disease in children. The literature on the management of pediatric BCS is scarce. Our aim was to determine the long-term outcome of patients undergoing a radiological intervention for the treatment of BCS. Methods Thirty-two children diagnosed with BCS between 2004 and 2014 were included. Data on the course of disease, medical management, response, and complications related to radiological interventions and outcome were collected. Main results Twenty-five patients who were on regular follow-up were analyzed. The median age of the patients at presentation was 9 months (4.5–214). Sixteen patients initially received anticoagulation alone. This was associated with a high failure rate of 66%. Twenty patients underwent a radiological intervention in the form of angioplasty (n=7), hepatic vein stenting (n=3) or transjugular intrahepatic portosystemic shunt (TIPS) (n=14). Success with angioplasty was achieved in 43% of cases. Hepatic vein stenting was successful in 66%, whereas TIPS was successful in 72% of cases. TIPS was feasible in all patients. The median follow-up duration was 44 months (5–132). Four patients developed hepatopulmonary syndrome after a median period of 3 years (1.5–5.25) and one patient developed hepatocellular carcinoma. Conclusion BCS commonly presents during infancy. Anticoagulation alone and angioplasty of the hepatic veins are associated with a high failure rate. Hepatic vein stenting or TIPS is feasible and efficacious in improving liver function, portal hypertension, and growth. It is associated with good long-term outcome and delays the need for liver transplantation, but may not prevent complications such as hepatopulmonary syndrome and hepatocellular carcinoma.

Primary Liver Sinusoidal Non-Hodgkin's Lymphoma Presenting as Acute Liver Failure

We describe a case of a middle-aged woman, who presented to us with fever, anorexia, abdominal distension from a massive hepatomegaly, low hemoglobin, and acute liver failure. A liver biopsy revealed B cell non-Hodgkins lymphoma predominantly in the sinusoids with CD10, CD20, and Bcl-2 positive on immunohistochemistry. She initially responded well to chemotherapy but succumbed 6 months later to the recurrence of disease. Sinusoidal non-Hodgkins lymphoma of the liver should be considered in the differential diagnosis of a patient with large hepatomegaly presenting with acute liver failure.

Liver Transplantation in India: At the Crossroads

As the liver transplant journey in India reaches substantial numbers and suggests quality technical expertise, it is time to dispassionately look at the big picture, identify problems, and consider corrective measures for the future. Several features characterize the current scenario. Although the proportion of deceased donor liver transplants is increasing, besides major regional imbalances, the activity is heavily loaded in favor of the private sector and live donor transplants. The high costs of the procedure, the poor participation of public hospitals, the lack of a national registry, and outcomes reporting are issues of concern. Organ sharing protocols currently based on chronology or institutional rotation need to move to a more justiciable severity-based system. Several measures can expand the deceased donor pool. The safety of the living donor continues to need close scrutiny and focus. Multiple medical challenges unique to the Indian situation are also being thrown up. Although many of the deficits demand state intervention and policy changes the transplant community needs to take notice and highlight them. The future of liver transplantation in India should move toward a more accountable, equitable, and accessible form. We owe this to our citizens who have shown tremendous faith in us by volunteering to be living donors as well as consenting for deceased donation.

An unusual cause of vomiting in a child

The authors report the case of a 13-month-old male child who presented with sudden onset vomiting and refusal to take solid food. Barium swallow and CT scan of the chest were inconclusive. The cause of symptoms was only evident more than a month later on endoscopy, which revealed an unusual flower-shaped foreign body impacted in the cervical oesophagus. This was successfully removed, and review of imaging made the diagnosis obvious in retrospect. This report is to emphasise the importance of considering the possibility of foreign body ingestion with unusual sudden onset symptoms and vigilant interpretation of findings on imaging studies, especially in the paediatric population.

An unusual surgical site infection in a liver transplant recipient

Mycobacterium abscessus is a rare cause of human infection and is difficult to diagnose unless it is suspected. A 45-year-old woman underwent deceased donor liver transplantation following which she developed non-healing surgical site infection, which did not resolve with routine antibiotics for 2 months. The scraping of the wound revealed M abscessus infection. Definitive identification of this species of mycobacterium was possible by its growth characteristics on culture and reverse line blot hybridisation assay. She was treated with clarithromycin and cotrimaxazole as per sensitivity and showed complete recovery from the infection within 6 weeks of starting the drugs, which were continued for 6 months. We believe that this is the first documented case of surgical site infection by M abscessus in a liver transplant recipient.