Clinicohistological correlation of etiological spectrum of chronic liver disease diagnosed during noncirrhotic stages in children: Can need of liver biopsy be obviated?

Background And Aim: Limited data exist regarding the etiological spectrum of the subset of chronic liver diseases (CLDs) diagnosed in noncirrhotic states in children. Our primary objective was to study the clinicoetiological profile of CLDs detected in noncirrhotic stages in children younger than 12 years of age. The secondary objective was to find the hepatic histological correlation of provisional diagnosis by different ranks of doctors.

Validation of Revised Baveno VI Criteria for Screening of Varices Needing Treatment in Children with Cirrhosis.

Background: Baveno VI criteria for screening varices needing treatment (VNT) have not yet been validated in an exclusive pediatric and adolescent set of the population, in whom baseline parameters differ in relation to adults. Therefore, our primary objective was to validate Baveno VI and its expanded form in children below 18 years of age. The secondary aim was to elicit whether any revision of the above criteria with a target of not missing more than 5% VNT could be more accurate for this age group.

Varadi Papp syndrome, an unusual variant of oral-facial-digital syndrome: Report of a rare case.

Varadi Papp syndrome or oral-facial-digital syndrome type VI (OFDS VI) is a rare autosomal-recessive disorder distinguished from other OFDSs by metacarpal abnormalities with central polydactyly and by cerebellar abnormalities. Our patient had a broad forehead, arched eyebrows, left-sided squint, hypertelorism, epicanthic folds, fleshy nodular tongue, midline upper lip cleft, high arched palate, both pre-axial and post-axial polydactyly of limbs, hypotonia and cerebellar hypoplasia with molar tooth sign consistent with the diagnosis of Varadi Papp syndrome.

Profile of childhood cancers: a multicentric study.

The incidence of cancer has been increasing steadily in the developing world including India. Childhood cancers are a special entity with different genetic, environmental factors playing a role in their aetiology. The profiles of cancer incidence reflect the racial, cultural and geographical diversity within populations.

Rheumatic valvular insufficiency in Bardet-Biedl syndrome: a case report.

Bardet-Biedl syndrome (BBS) is an autosomal recessive disorder with multisystem affection. Cardiac Involvement, when present is usually in the form of congenital heart disease. However, valvular involvement due to acquired heart disease is extremely unusual.

Etiology, clinical spectrum and outcome of metabolic liver diseases in children.

Objective: To determine the etiology, clinical spectrum and outcome of metabolic liver diseases (MLD) in children admitted in a tertiary care hospital of Eastern India.

Study Design: An observational study.

Place And Duration Of Study: Paediatric Liver Clinic and Paediatrics Inpatient Department of Nilratan Sircar Medical College and Hospital, Kolkata, Eastern India, from April 2009 to March 2011.

Effectiveness of beta blockers in primary prophylaxis of variceal bleeding in children with portal hypertension.

Aim: The primary aim of our study was to assess the effectiveness of beta blockers in non bleeding portal hypertensive children. The secondary objective was to evaluate whether the newer generation beta blockers were superior compared to conventional ones.

Methods: Conventional propranolol and newer generation carvedilol were administered to 31 subjects each, after stratifying them into nearly equal subgroups according to etiology (sinusoidal or presinusoidal).

Overlap of sturge-weber syndrome and klippel-trenaunay syndrome.

Sturge-Weber syndrome is a rare sporadic condition of mesodermal phakomatosis, characterized by purple-colored flat cutaneous cranial (face) hemangiomas (most commonly along the trigeminal nerve), glaucoma and vascular lesions in the ipsilateral brain and meninges. Klippel-Trenaunay syndrome is also an uncommon mesodermal phakomatosis characterized by a triad of cutaneous and visceral hemangiomas, venous varicosities and soft tissue or bone hypertrophy. Sturge-Weber syndrome in combination with Klippel-Trenaunay syndrome is unusual.

Non-cirrhotic portal fibrosis among children admitted in a tertiary care hospital of Kolkata: a search for possible aetiologies.

Non-cirrhotic portal fibrosis (NCPF) is reported to be a very infrequent cause of portal hypertension (PHT) in paediatric population, but a number of cases of NCPF were found in the paediatric ward of NRS Medical College and Hospital, Kolkata which prompted us to do a study with objectives to find out the proportion of NCPF cases among the study population (the total number of PHT cases admitted in the study period) and their sociodemographic profile with special reference to residence in arsenic affected areas. It was a clinic-based retrospective study. All children below 12 years during the study period (August 2005 to July 2008) with clinical features suggestive of PHT were screened for NCPF.

Neurocutaneous syndrome: a prospective study.

Background: Neurocutaneous syndromes (NCS) are a group of genetic disorders that produce a variety of developmental abnormalities of the skin along with an increased risk of neurological complications. Cutaneous manifestations usually appear early in life and progress with time, but neurological features generally present at a later age. There is a paucity of data regarding the evolution of skin lesions and their correlation with the central nervous system involvement in children.

Eligibility criteria of Doctorate in Medicine (Medical Gastroenterology) - is the change justified?

Eligibility criteria of Doctorate in Medicine (DM) in Medical Gastroenterology had been changed recently by Medical Council of India from MD/DNB in General Medicine and Pediatrics to only that of General Medicine. As DM (Pediatric Gastroenterology) has not come up in a big way in India, this will only lead to shortage of skilled manpower in near future in this emerging field. Enthusiastic pediatricians will also be reluctant to take the discipline, as DM degrees in other broad (adult) superspecialities are open to them.

Unusual associations of necrotizing fascitis: a case series report from a tertiary care hospital.

Necrotizing fasciitis is a rapidly progressive, potentially fatal infection of the superficial fascia and subcutaneous tissue. It is rare in children. We report three such cases in which differentiating from common soft tissue infection was challenging.

Profile of hepatitis A infection with atypical manifestations in children.

We assessed the clinical course and biochemical profile of symptomatic children with viral hepatitis A who had atypical manifestations. Of 229 children with hepatitis A, atypical manifestations were found in 32 (14%) subjects. Prolonged cholestasis (n = 14), acute liver failure (9), relapse (9), ascites (8), and hematological problems (8) were the common presentations.

Clinical profile, prognostic indicators and outcome of Wilson's disease in children: a hospital based study.

Background: Wilson's disease is a common metabolic disease of the tropics, which is treatable, if diagnosed early. In the paediatric group, the manifestations are mainly hepatic.

Aims: The objective was to study the varied presentations of the disease and to evaluate the diagnostic values of conventional tests in children.

Aetiology, clinical profile and prognostic indicators for children with acute liver failure admitted in a teaching hospital in Kolkata.

Objective: To estimate the prevalence of hepatotropic viruses in the causation of acute liver failure in children admitted to a tertiary hospital in Kolkata.

Design: Analysis of clinical and laboratory parameters (including viral markers) of children with acute liver failure using a predesigned, structured proforma.

Subjects And Settings: Admitted patients aged from 1 through 12 years who met the criteria of acute liver failure were included in the study.