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. METHODS: This was an observational epidemiological study, cross-sectional in design, conducted in a tertiary-care setting over a 2-year period. RESULTS: Thirty-seven cases were enrolled, with a mean ± SD age of 8 ± 4.1 years and a male:female ratio of 1.8:1. Etiologies noted were Wilson disease (n = 8), autoimmune hepatitis (n = 4), secondary hemochromatosis (n = 4), chronic hepatitis B (n = 3), chronic hepatitis C (n = 2), non-alcoholic steatohepatitis (n = 2), progressive familial intrahepatic cholestasis (n = 2), extrahepatic biliary atresia (n = 2), Alagille syndrome (n = 1), galactosemia (n = 1), Gaucher disease (n = 1), Niemann-Pick disease (n = 1), and Budd-Chiari syndrome (n = 1), with an inconclusive diagnosis in five children. Relevant investigations were ordered more frequently by the specialist consultant (SC) and super specialist (SS) combined in comparison with the senior resident (SR) and junior resident (JR) together. (P = 0.0013). Irrelevance of the tests ordered was significantly higher in the junior tier (JR and SR; SR > JR) in contrast to the senior tier of doctors (SC and SS) (P < 0.01). The clinicohistological correlation of an etiological diagnosis significantly differed between the junior and senior ranks of physicians. We noted that an ideal clinical acumen could help to avoid liver biopsy for etiological diagnosis in 78.3% (29/37) of the study population. CONCLUSION: Interpretation of clinical presentation by the senior set of doctors is preferable, which could obviate the need for liver biopsy regarding diagnosis in a proportion of pediatric CLD patients.

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. MATERIALS AND METHODS: The work was carried out in two medical institutes, over a span of 3 years. Consecutively enrolled patients below 18 years of age, with compensated cirrhosis confirmed by liver biopsy, were evaluated for related blood parameters, transient elastography (TE) and esophagogastroduodenoscopy. RESULTS: Out of the 33 recruited patients, five (15.15%) met the criteria for VNT. The sensitivity, specificity, PPV and NPV of Baveno VI and Expanded Baveno VI were observed as 60%, 92.3%, 60% and 92.3%, and 20%, 100%, 100% and 88%, respectively. We found that the Revised Baveno VI criteria with TE <19 kPa and platelet count of >175×109 cells/L, with sensitivity 100%, specificity 79%, PPV 45%, NPV 100% and accuracy of 82%, are more appropriate for this age group. CONCLUSION: We propose that further multicentrer studies with a larger sample size should be conducted before incorporating Revised Baveno VI criteria for high-risk varices in patients below 18 years in future guidelines.

The prevalence of behavioral disorders among children under parental care and out of parental care: A comparative study in India.

BACKGROUND: Children may suffer from a wide range of behavioral problems, but children living under conditions of perpetual stress and trauma and experiencing breakdown of family structures are tremendously affected. This study was adopted to identify the prevalence of behavioral disorders among children under parental care and out of parental care. METHODS: A cross-sectional comparative descriptive study was conducted among 300 children of age 6-12 years from a pediatric outpatient department of a selected hospital and 300 children from selected orphanages in Kolkata to compare the prevalence of behavioral disorders in children under parental care and out of parental care using Strengths and Difficulties Questionnaire (SDQ). RESULTS: Total difficulty was more prevalent in children out of parental care (220 children) than in children under parental care (128 children). Conduct problem was the most prevalent behavioral disorder among all the subscales of SDQ with 48.70% and 84.30% of children, respectively. This was followed by peer problem (44.60% and 48.30%), emotional problem (33.70% and 55.60%), and hyperactivity problem (26.70% and 32.30%), respectively. There was significant difference in total difficulty, all subscales, externalizing score, internalizing score, and impact score between the two groups. Regression analysis showed significant relationship of selected variables such as the child's favorite hobbies and number of close friends the child has using total difficulty score in both the groups. Significant correlation was found among subscales in both the groups. CONCLUSION: Family-based care and improvised institutional care should be emphasized to reduce the burden of behavioral problem in children.

Assessment of normal portal vein diameter in children.

BACKGROUND AND AIM: The internal diameter of the portal vein varies with age and anthropometric parameters. The caliber of the normal portal vein in adults has been extensively studied but little is known about portal vein dimensions in the growing child. This study was conducted to establish standards of portal vein diameter by ultrasonography in healthy Indian children based on age, gender and anthropometric parameters. METHODS: Total 306 healthy children between the age of < 1 month and 12 years, visiting our outpatient departmentor accompanying their siblings were enrolled in the study. The children were distributed into ten age-groups. Each group was further divided in two sub-groups based on gender. Anthropometric parameters including weight, height and chest circumference were measured.Portal vein diameter was assessed by ultrasonography. RESULTS: The portal vein diameter increases with age, height, weight and chestcircumference. But the values are similar in boys and girls. Multiple logistic regression (adjusted R- square: 0.922) revealed age (p = 0.002), height/length (p < 0.0001), weight (p = 0.011), and chest circumference (p < 0.0001), as independent determinants of portal vein diameter. However, height/length emerged as the most consistent determinant (coefficient of regression: 1.536; p < 0.001; 95% confidence interval: 0.066-0.092). CONCLUSION: Our results provide a normal range of portal vein diameter according to age, gender and anthropometric parameters. We conclude that portal vein diameter strongly correlates with age and anthropometric variables like height, weight and chestcircumference,with height being the strongest determinant.

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. METHODOLOGY: All children aged 0 - 12 years having characteristic clinical features along with diagnostic hallmark of any MLDs were included in this study and data were collected on a pre-designed proforma. After appropriate management and discharge, all patients were followed-up for next 6 months. RESULTS: Fifty one children with mean age 4.34 ± 3.78 years (range 2 days - 12 years), male: female ratio 1.55:1, were studied. The etiologies were Wilson's disease (33.33%, n = 17); glycogen storage disorder (23.53%, n = 12); galactosemia (19.61%, n = 10); non-alcoholic fatty liver disease (11.76%, n = 6); Gaucher disease (5.88%, n = 3); mucopolysaccharidoses (3.92%, n = 2) and familial hyperlipoproteinemia type-I (1.96%, n = 1). Jaundice (n = 24) and hepatomegaly (n = 47), was the commonest symptom and sign respectively. Of the 17 non-responders, most were Wilson's disease (n = 7) cases. There was statistical difference in outcome with respect to INR > 1.3 at diagnosis (p = 0.026). CONCLUSION: High index of suspicion, early detection and screening, simple dietary modification and cost effective drugs along with good compliance are sufficient to treat and even prevent evolution of most causes of the MLDs.

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.

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. Relevant investigations were done and guardians of all the PHT patients were interviewed with a predesigned pretested questionnaire. Among the total 134 cases of PHT, 29 (21.64%) were due to NCPF and the remaining 105 (78.36%) cases were due to other causes of PHT other than NCPF. Among the sociodemographic variables studied significant association was found with residency in arsenic affected areas. The study highlights the need of extensive studies to validate the association in the paediatric population.

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). RESULTS: At the end of 2 years study period, 3 children (4.83%) had breakthrough bleeding. A decrease, increase and no alteration in grade of oesophageal varices was seen in 40, 9 and 13 cases respectively. Of the 9 children with associated gastroeosophageal varices (GOV), the severity of lesions was reduced in 8 of them. Both the drugs had efficacious outcome in sinusoidal as well as presinusoidal cases, having a significant coefficient of correlation (r > 0.5) with time. Carvedilol was more effective than propranolol statistically (p = 0.035 and p = 0.034 respectively), only at 4 and 5 month follow-up period. CONCLUSION: Beta blockers are effective in preventing variceal bleed in children with portal hypertension. Long-term efficacy of carvedilol and propranolol was similar.

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. Liver histology was suggestive of chronic liver disease in six children with protracted jaundice. Patients with atypical presentations were older (7.7 [1.6] years vs. 6.5 [2.6] years; p=0.012) and had higher total serum bilirubin (13.7 [8.1] mg/dL vs. 7.2 [4.0] mg/dL; p=<0.001) than those with typical presentation. Approximately 15% of children with acute hepatitis A infection have atypical presentation which is associated with increase in morbidity.

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. High index of suspicion is important as management initiated at an early stage is rewarding.

Efficacy of high-dose Lactobacillus rhamnosus GG in controlling acute watery diarrhea in Indian children: a randomized controlled trial.

AIM: To evaluate the effective dose of Lactobacillus rhamnosus GG (LGG) as probiotic in acute watery diarrhea (AWD) in Indian children. SETTING: Hospital-based study. DESIGN: Randomized, controlled, blinded trial. METHODS: All patients of AWD admitted over 1 year were included in the study. They were randomized into 3 groups to receive either only oral rehydration solution (ORS) (group A/control), ORS+LGG powder containing 10(10) colony forming units (CFU) (group B), or ORS+LGG powder containing 10(12) CFU (group C) twice daily for a minimum period of 7 days or until diarrhea stopped along with correction of dehydration. None of them received any other drug such as antibiotic or antidiarrheal medication. The duration and frequency of diarrhea and vomiting were studied. Data were analyzed by SPSS-10 software. RESULTS: The study comprised of 559 patients, group A/controls (n=185), group B (n=188), and group C (n=186). All the groups were similar with respect to age, number of breastfed infants, presentation with dehydration, degree of protein energy malnutrition, and rotavirus infection. The frequency and duration of diarrhea, requirement for intravenous therapy, and hospital stay were significantly lower in both the intervention groups compared with the controls. There was no significant difference between the 2 intervention groups. No complication was observed from the doses of LGG used. CONCLUSIONS: Both the doses of LGG (10(10) and 10(12) CFU) were equally effective to decrease the frequency and duration of diarrhea and reduction in hospital stay in patients of AWD.

Myelomeningocele with hydrocephalus and agenesis of corpus callosum.

A 45-day-old male baby presented with a leaking myelomeningocele and signs of meningitis. A computerised tomographic scan of brain revealed gross supratentorial ventriculomegaly with inflammatory exudates suggestive of intracranial infection. An unexpected association of complete agenesis of corpus callosum was discovered, however it is an incidental finding, a rare combination indeed.

Klebsiella pneumoniae brain abscess in two neonates.

We report two premature infants who developed multiple brain abscesses following Klebsiella pneumoniae infection. Both the cases were diagnosed by ultrasonogram (USG) and cranial tomography. Abscess had intraventricular communication in one case. One infant was managed conservatively while the other required surgical drainage.

Efficacy of Lactobacillus rhamnosus GG in acute watery diarrhoea of Indian children: a randomised controlled trial.

AIM: To evaluate the role of Lactobacillus rhamnosus GG (LGG) as probiotic in acute watery diarrhoea (AWD). SETTING: Hospital-based study. DESIGN: Randomised, controlled, blinded trial. PATIENTS AND METHOD: All patients of AWD (n = 684) admitted over 1-year period were invited to participate in the study as per predefined inclusion and exclusion criteria and were randomised to intervention and control groups. After adequate rehydration the intervention group (n = 330) received ORS with probiotic powder containing 60 million cells of LGG, while the control group (n = 332) received ORS alone twice daily for a minimum period of 7 days or till diarrhoea ceased. During the study period all patients received ORS and/or IV fluids for ongoing losses, and nutritional supplementation. None of them received any antibiotic or antidiarrhoeal medication. After exclusion of 16 patients, 646 (323 in each arm) patients completed the study. The daily frequency and total duration of diarrhoea and vomiting and the length of hospital stay were studied. Data were analysed by SPSS-10 software. Statistical significance was calculated by Student's t-test and chi2-test. RESULTS: Rotavirus was isolated in 75.85%. There was no significant difference between treatment groups in the daily frequency or duration of diarrhoea or vomiting or in the length of hospital stay. No complication was observed from the use of LGG. CONCLUSION: LGG supplementation does not decrease the frequency and duration of diarrhoea and vomiting in children with AWD, and does not reduce hospital stay in these patients.

Effect of Lactobacillus rhamnosus GG in persistent diarrhea in Indian children: a randomized controlled trial.

AIM: To evaluate the role of Lactobacillus rhamnosus GG (LGG) as probiotic in persistent diarrhea (PD) in children of North Bengal, India. SETTING: Hospital-based study. DESIGN: Randomized, double-blind controlled trial. PATIENTS AND METHODS: All patients of PD admitted over a period of 2 years were included in the study as per predefined inclusion criteria. They were randomized to receive oral rehydration solution (ORS) alone, or ORS plus LGG powder containing 60 million cells, twice daily for a minimum period of 7 days or till diarrhea has stopped along with correction of dehydration with ORS and/or intravenous fluids as per WHO protocol and antibiotics in culture positive patients. The duration and frequency of purge and vomiting were studied. Data were analyzed by SPSS-10 software. Statistical significance was calculated by Student t test and chi2 test. RESULTS: The study comprised of 235 patients randomized into 2 groups, cases (117) and controls (118). Both the groups were similar with respect to age, number of breastfed infants, presentation with dehydration, degree of protein energy malnutrition, and distribution of infections. Stool culture was positive in 90 (38.3%) patients, Escherichia coli being the commonest organism followed by Shigella spp. and Clostridium difficile. The mean duration of diarrhea was significantly lower in the cases than in controls (5.3 vs. 9.2 d). The average duration of hospital stay was also significantly lesser in cases. No complication was observed from the dose of LGG used. CONCLUSIONS: LGG (dose of 60 million cells) could decrease the frequency and duration of diarrhea and vomiting and reduced hospital stay in patients of PD.

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. RESULTS: Of the 45 patients in our study, a majority was from the southern part of West Bengal. Their mean age was 7.12 +/- 0.37 years. The male: female ratio was 1:1.25. It was possible to determine the aetiology in 35 of the 45 patients (77.7%) admitted. Of these 35, a diagnosis of hepatitis due to hepatotropic viruses was made in 30 patients. The hepatitis A virus was responsible for 16 of the 30 cases (53.3%), 9 cases attributed to HAV only. Following this was the hepatitis E virus causing ALF in 14 cases (46.6%), 7 singularly so. Hepatitis B virus caused 8 cases (26.6%), 6 singly. The survival rate during hospital stay was 51.1%. Prodrome, decreased liver span, ascites, cerebral oedema, coagulopathy, renal failure, spontaneous bacterial peritonitis, signs and symptoms of clinical sepsis (corroborated by laboratory data), severe hypoalbuminaemia and electrolyte imbalance were significantly more in patients who died. The mean age, prothrombin time, serum bilirubin level and stage of encephalopathy differed significantly between survivors and non-survivors. CONCLUSION: In southern Bengal, hepatotropic viruses are the predominant cause of acute liver failure in children. Of these, the hepatitis viruses A and E transmitted via the enteric route dominate (24 of 30 cases).

Risk factors for mortality from neonatal tetanus: 7 years experience in North Bengal, India.

BACKGROUND: Neonatal tetanus (NNT), a preventable disease, remains an important cause of infant mortality in developing countries such as India. OBJECTIVES: To evaluate the demographic and clinical data of NNT in North Bengal, India and determine the risk factors for mortality. METHODS: Hospital records of all cases of NNT admitted over a 7-year period (1997-2003) were analysed. Demographic data, clinical presentation, progression and outcome were evaluated and comparisons made between the group who survived and those who died. RESULTS: The overall mortality was 66.3%. The group who survived had a significantly greater mean bodyweight on admission, later onset of the disease and were hospitalised early. Risus sardonicus, generalised rigidity, fever, intercurrent infections and respiratory arrest were significantly more common in the fatal group. CONCLUSION: Although over the 7 years improvement was observed in admissions for NNT, maternal literacy and the economic status of families, there is a persisting lack of awareness regarding antenatal care, and deliveries are still conducted unhygienically by untrained persons.

Sialidosis type I (cherry red spot-myoclonus syndrome).

Sialidosis type 1 or the cherry red spot-myoclonus syndrome (CRSM) is an autosomal recessive disorder with the onset in adolescence of myoclonus and gradual visual failure. Here, a case of CRSM in a 12-year-old Bengali Muslim girl with the history of myoclonic jerks of limbs and the body since last 2 years and gradual impairment of vision since last one year is presented with a brief review of the literature.