Profile of extrahepatic portal venous obstruction among children in Central India.

INTRODUCTION: There is a paucity of data regarding the clinical profile of children with extrahepatic portal venous obstruction (EHPVO) from Central India. MATERIAL AND METHODS: Retrospective analysis of 30 children with EHPVO treated between 2002 and 2012. RESULTS: The median age of presentation was 12.5 years (range 5-14 years) and median duration of symptoms was 3.2 years (range 0.3-10 years). Home delivery was noted in 30 cases (100%), umbilical sepsis in 8 (27%) and history of cow dung application over the umbilical cord in 2 cases (6.7%). Twenty-two cases (73.4%) presented with upper gastrointestinal (GI) bleeding while 18 (60%) were referred for pancytopenia and/or hypersplenism. The patients underwent a median of 3.2 sessions of endoscopic therapy for varices. Most patients (19/30, 63.4%) dropped out of serial endotherapy due to the paucity of funds. Shunt surgery was performed in 7 patients. CONCLUSIONS: Home delivery and umbilical sepsis are significant risk factors for EHPVO. Most patients present with GI bleeding and do well on endoscopic treatment/shunt surgery.

Neonatal Cholestasis - Single Centre Experience in Central India.

BACKGROUND: Neonatal cholestasis syndrome (NCS) is a major cause of morbidity and mortality in infants. The disorder has rarely been studied in centers from Central India. OBJECTIVES: To study the prevalence, clinical presentation and etiology of NCS at a tertiary referral center in Central India. MATERIALS AND METHODS: The study was carried out at a tertiary referral center in Central India. The study is a descriptive study. The records of all patients with suspected NCS treated in the Department of Pediatrics from 2007-2012 were analyzed. RESULTS: One hundred and sixty-eight children had a provisional diagnosis of NCS. The complete records of 100 children were available for the study. The median age of presentation was 78 days (range 15-270 days). The male: female ratio was 1.17:1. The clinical features noted were- jaundice (100/100,100%), failure to thrive (73,73%), organomegaly (68, 68%), acholic stools (38,38%), abdominal distention (52,52%) and poor feeding (29, 29%). The etiology as confirmed by investigations is as follows- neonatal hepatitis (20,20%), idiopathic neonatal hepatitis (18,18%), biliary atresia (41,41%), sepsis (14,14%) and others (7,7%). CONCLUSIONS: The proportion of NCS in our group of patients was 1.2 per 1000 patients. Jaundice, organomegaly and failure to thrive are the common presentations. Biliary atresia, neonatal hepatitis and idiopathic neonatal hepatitis were the common etiological factors at our center.

Unusual clustering of diseases and manifestations in pediatric admissions of a tertiary care center.

BACKGROUND: Cluster investigations has been an important tool in investigations of diseases. While clustering of diseases or a manifestation in community can cause great public alarm, similar unexplained clustering in hospital admissions has baffled clinicians. OBJECTIVES: (a) To determine whether perceived unexplained clustering of diseases and manifestations among hospital admissions is real or significant. (b) To look for possible explanations of such clustering. DESIGN: One-year prospective observational and two-year retrospective study. MATERIAL AND METHODS: Cases admitted under pediatric and neonatal services (excluding rooming in babies) from September 2002 to August 2003 formed material for prospective and cases admitted for 2 years prior for retrospective study. Whenever an unexplained cluster of disease/manifestation was perceived, case definition was finalized and cases recorded. Retrospective cluster analysis was done for some significant unexplained clusters detected prospectively. These clusters case rate were analyzed in relation to age, sex, climatic conditions, etiology of case, etc. to detect any correlation. Pearson correlation coefficient, chi-square test, centroid method and Z test of proportion were used for statistical significance. RESULTS: Eight unexplained clusters were perceived in prospective study. 3 (hyperbilirubinemia in exchange range hypocalcemic seizures and vesicoureteric reflux) proved statistically not significant. 3 were small clusters of uncommon diseases / manifestations (biliary atresia, cardiomyopathy, and acral gangrene). Two large significant clusters, major non-traumatic bleeding manifestations (MNTBM) and acute renal failure (ARF) occurred to which retrospective study was extended. Tmax and Tmin (average maximum and minimum daily temperature) had statistically significant positive correlation with ARF (gamma = + 0.83, P <0.001 for Tmax and gamma =+ 0.56, P = 0.002 for Tmin) and negative correlation with MNTBM (gamma = - 0.34, P = 0.040 for Tmax and gamma = - 0.59, P <0.001 for Tmin). Barometric pressure has significant negative and positive correlation with MNTBM and ARF respectively (gamma =+ 0.57, P <0.001 for MNTBM and gamma = -0.45,P = 0.006 for ARF). The clusters also had significant positive correlations with female sex and age under 1 year (Z = 2.48, P <0.001,chi2=13.83, P<0.001 for sex and age of MNTBM and Z =3.11, P <0.001, chi2 =10.85, P <0.001 forage and sex of ARF cases respectively). Three small clusters and a small subgroup of MNTBM(subcutaneous nodules as manifestations of bleeding disorders) occurred predominantly under one year and different sexes were involved. CONCLUSIONS: Several significant unexplained clustering were noted among hospital admissions. There was significant correlation with climatic conditions, age and sex. Larger, longer and multicentric studies in different geographical areas are required to investigate more plausible but complex biological phenomenon and associations related to diseases or manifestations. Cluster awareness has diagnostic and management implications for clinician as it also helps in early recognition of disease outbreak and dissemination of information and hospital staff to be prepared to handle increased number of cases and its treatment.