Vikrant Sood

Profile and Outcome of first 109 cases of Pediatric Acute liver Failure at a Specialized Pediatric Liver Unit in India

The outcome of paediatric acute liver failure largely depends on age and aetiology. The aim of this work was to study the aetiological spectrum and outcome of the paediatric acute liver failure cases.

Cholemic or Bile Cast Nephropathy in a Child with Liver Failure

Cholemic or bile cast nephropathy is an under-reported entity characterized by acute renal dysfunction in patients with hepatic insult. Limited literature is available regarding its clinical presentation, pathogenesis and prognosis. We hereby present a pediatric case who presented with acute on chronic liver failure with renal dysfunction secondary to cholemic nephropathy.

Non-cirrhotic Portal Fibrosis in Pediatric Population.

Objectives: Noncirrhotic portal fibrosis (NCPF) has been classically described as a disease of young to middle age with limited literature regarding its occurrence, onset, or clinical presentation in children. We hereby present a series of 19 patients diagnosed and managed as NCPF in pediatric age group. Methods: A retrospective review of all the patients presenting to the pediatric hepatology department (age <18 years) and diagnosed as NCPF was done and data were evaluated. Results: A total of 19 patients were diagnosed as NCPF with median age at onset of symptoms and diagnosis as 10 years and 13.8 years respectively. Majority presented with left upper quadrant discomfort or mass. Laboratory parameters showed hypersplenism in majority with preserved liver synthetic functions. Median values for hepatic venous pressure gradient and liver stiffness measurement were 13.5 mmHg and 10.6 kPa, respectively. Classical hepatic histopathological features seen were maintained lobular architecture, atretic portal tracts, approximation of portal-portal and portal-central areas, and aberrant peripheral portal channels. During follow-up, majority of the patients did not show disease progression. Conclusions: NCPF is not an uncommon entity in pediatric population with age of onset in early second decade. Hepatic histopathology must be used to exclude cirrhosis and to confirm the diagnosis. Hepatic venous pressure gradient and liver stiffness measurement values, in some cases, may overlap with those in patients with cirrhosis and may not be diagnostic in isolation. Any patient presenting with evidence of portal hypertension with preserved hepatic functions, irrespective of the age, should be evaluated for possible NCPF.

Metabolic Liver Disease: When to Suspect and How to Diagnose?

Metabolic liver diseases are still considered by many as a ‘rare’ diagnosis, though scenario has definitely changed in recent times. With recent advances and wider availablility of newer techniques, many of these are now amenable to diagnosis and optimum management. Though the logistics involved are still out of reach of a significant proportion of our population, a stepwise and methodological approach with simple diagnostic tests can help point towards a probable diagnosis (with resultant directed investigations), helping to avoid unnecessary and costly workup. This review focuses on diagnostic protocol-based approach to common metabolic liver diseases encountered frequently in pediatric hepatology.

Profile, risk factors and outcome of acute kidney injury in paediatric acute-on-chronic liver failure

There are no studies on acute kidney injury in paediatric acute‐on‐chronic liver failure. This study was planned with aim to describe the clinical presentation and outcome of acute kidney injury among paediatric acute‐on‐chronic liver failure patients.

Clinical Spectrum and Outcome of Pediatric Drug Induced Liver Injury

Limited literature is available in pediatric population regarding drug-induced liver injury (DILI) making it a diagnostic challenge. This study was thus planned to determine the clinical spectrum and the outcome of DILI in children. All patients with DILI under 18 y of age were retrospectively reviewed and details regarding clinical presentation, Roussel Uclaf Causality Assessment Method (RUCAM) scale, drugs implicated, biochemical abnormalities and outcome were noted. DILI constituted 3.7% of all children with liver disease. Cases were divided into the hepatocellular (18, 50%), cholestatic (10, 27.8%), and mixed pattern (8, 22.2%). Complementary and alternative medicines (CAM) and antitubercular (ATT) drugs accounted for three-fourth cases of total DILI (39% and 33% cases respectively). Overall, 4 (11%) patients died and 5 (14%) patients progressed to chronic DILI. Presence of ascites, non-hepatocellular injury pattern and high serum total IgG levels were significantly associated with unfavourable outcome (death or chronicity).

Response to Profile and outcome of first 109 cases of paediatric acute liver failure at a specialized paediatric liver unit in India: Methodological issues

In the present study, we have considered both these points before preparing the multivariate model. We first listed all the variables with P<.2, but then considered the more significantly associated variables, that is those with smaller P values, for logistic reason. Being aware of limited sample size, we could not include more than nine variables. As for the multicollinearity, all the variables considered were relating to liver failure and indices were calculated from the same parameters, hence all variables are multicollinear. Despite multicollinearity, we have found the best model but by adding the variable one by one and/or doing exploratory analysis. In the exploratory analysis there was no improvement in the prediction of outcome from 87.3%.These details may have been deleted in trying to limit the manuscript in terms of length. We did not perform formal sample size calculation, but followed the rule of thumb which states that we should have at least 510 events2 per variable included in the multivariate model in our study we have not exceeded the minimum requirement. Our centre is unique by being the only public sector paediatric liver transplant centre in India, hence the single centre data report. But we feel this data is important and can be usefully used in a metaanalysis later. Finally we must thank Ayubi et al. for improving our knowledge about measures to be taken to handle sparse data. We need time to understand the concept before we can use it on any data.

Imaging in ductal plate malformations

Ductal plate malformations are a heterogenous group of congenital fibrocystic liver diseases resulting from insult to the ductal plate at various stages of embryogenesis. As a result various biliary malformations, cysts, hamartomas and congenital hepatic fibrosis may be seen. We present a radiological pictorial of ductal plate malformations, accurate diagnosis of which is important for clinical management.

Caudal regression syndrome with bilateral popliteal webbing without maternal diabetes: a rare entity

Caudal regression syndrome refers to a constellation of congenital caudal anomalies affecting the caudal spine and spinal cord, the hindgut, the urogenital system, and the lower limbs. About 15–25 % of mothers of children with caudal regression syndrome have insulin-dependent diabetes mellitus. We hereby present a 3-year-old child with caudal regression syndrome, with popliteal webbing and ectopic right kidney, born to a nondiabetic mother.

Hepatic Dysfunction in a Child Post Bone Marrow Transplantation

A 10-year-old female child, a known case of Beta-Thalassemia major with superimposed chronic Hepatitis C Viral (HCV) infection, underwent bone marrow transplantation and presented, 7 months later, with worsening hepatic functions and skin rash. Considering the wide variety of possible etiologies, she was evaluated and later confirmed as a case of hepatic dysfunction secondary to HCV related hepatic injury with concomitant chronic graft versus host disease (dermatological involvement only).