Anupam Sibal

VSL#3 improves symptoms in children with irritable bowel syndrome: a multicenter, randomized, placebo-controlled, double-blind, crossover study.

Background and Objectives: Irritable bowel syndrome (IBS) is a common problem in pediatrics, for which no safe and effective treatment is available. Probiotics have shown some promising results in adult studies, but no positive study has been published on pediatric age. We aimed at investigating the efficacy of VSL#3 in a population of children and teenagers affected by IBS, in a randomized, double-blind, placebo-controlled, crossover study conducted in 7 pediatric gastroenterology divisions. Patients and Methods: Children 4 to 18 years of age, meeting eligibility criteria, were enrolled. The patients were assessed by a questionnaire for a 2-week baseline period. They were then randomized to receive either VSL#3 or a placebo for 6 weeks, with controls every 2 weeks. At the end, after a “wash-out” period of 2 weeks, each patient was switched to the other group and followed for a further 6 weeks. Results: A total of 59 children completed the study. Although placebo was effective in some of the parameters and in as many as half of the patients, VSL#3 was significantly superior to it (P < 0.05) in the primary endpoint, the subjective assessment of relief of symptoms; as well as in 3 of 4 secondary endpoints: abdominal pain/discomfort (P < 0.05), abdominal bloating/gassiness (P < 0.05), and family assessment of life disruption (P < 0.01). No significant difference was found (P = 0.06) in the stool pattern. No untoward adverse effect was recorded in any of the patients. Conclusions: VSL#3 is safe and more effective than placebo in ameliorating symptoms and improving the quality of life in children affected by IBS.

Management of neonatal cholestasis: Consensus statement of the pediatric gastroenterology chapter of Indian academy of pediatrics

JustificationNeonatal cholestasis is an important cause of chronic liver disease in young children. Late referral and lack of precise etiological diagnosis are reasons for poor outcome in substantial number of cases in India. There is a need to create better awareness among the pediatricians, obstetricians and primary care physicians on early recognition, prompt evaluation and referral to regional centers.ProcessEminent national faculty members were invited to participate in the process of forming a consensus statement. Selected members were requested to prepare guidelines on specific issues, which were reviewed by two other members. These guidelines were then incorporated into a draft statement, which was circulated to all members. A round table conference was organized; presentations, ensuing discussions, and opinions expressed by the participants were incorporated into the final draft.ObjectivesTo review available published data on the subject from India and the West, to discuss current diagnostic and management practices in major centers in India, and to identify various problems in effective diagnosis and ways to improve the overall outcome. Current problems faced in different areas were discussed and possible remedial measures were identified. The ultimate aim would be to achieve results comparable to the West.RecommendationsEarly recognition, prompt evaluation and algorithm-based management will improve outcome in neonatal cholestasis. Inclusion of stool/urine color charts in well baby cards and sensitizing pediatricians about differentiating conjugated from the more common unconjugated hyperbilirubinemia are possible effective steps. Considering the need for specific expertise and the poor outcome in suboptimally managed cases, referral to regional centers is warranted.

Experience of 100 solid organ transplants over a five-yr period from the first successful pediatric multi-organ transplant program in India

To analyze the clinical profile and outcome of pediatric patients who had undergone a liver and/or RT at our center over a five yr period, case records of all the patients who had undergone a liver or RT were analyzed retrospectively. One hundred solid organ transplants were performed at our center between January 2007 and January 2012. These included 50 liver, 44 renal, one sequential liver and renal, and two CLKT. BA was the most common indication for an LT (38%). At a median follow‐up of two yr three months, the patient survival was 88%. The most common indication for an RT was chronic glomerulonephritis (54.5%). At a median follow‐up of three yr, the survival was 91%. The CLKT were performed for hyperoxaluria. Two yr post LT, a sequential RT was performed for ESRD resulting from transplant associated microangiopathy. All patients received a living related graft. The common post‐operative complications were infections, vascular complications, and graft dysfunction. Survival rates for liver and RT at our center are comparable to those in the established centers in the West.

Are fathers catching up with mothers in liver donation

abnormalities [1-3]. Fluorescence in situ hybridization (FISH) analysis was carried out using TUPLE region probe (from Kreatech Diagnostics, Netherland) on metaphase and interphase cells. Presence of two intact signals on chromosome 22 ruled out 22q11.2 deletion. Thus, chromosomal analysis was carried out using the GTG-banding technique and the patient was found to be tetrasomy for sex chromosome-X i.e. 48,XXXX.

Indiana pouch in children: A 15-year experience

OBJECTIVE We report our experience with the Indiana pouch (continent urinary reservoir) in 12 consecutive children over the last 15 years and report their follow-up. MATERIAL AND METHODS Twelve consecutive children, who underwent the continent urinary reservoir procedure in the form of an Indiana pouch, were prospectively enrolled in the study. All consecutive children who were referred to our service with multiple failed surgeries for exstrophy-epispadias repair, cloacal exstrophy, genitourinary rhabdomyosarcoma with residual disease in the trigonal area not amenable to partial cystectomy, and neuropathic bladder with nephrogenic metaplasia were included over the period 1997-2012. All these children were offered the same form of bladder substitution (Indiana pouch) as described by the Indiana group many years ago. Postoperative care was on a fixed protocol, and follow-up details recorded over the years. They were followed up for dry interval with clean intermittent catheterisation, social acceptance, and early and late complications. RESULTS Out of these 12 patients (7 males and 5 females), eight patients had exstrophy-epispadias with multiple failed operations carried out elsewhere, cloacal exstrophy (2), residual rhabdomyosarcoma in the trigonal area with incontinence following chemotherapy (1), and neuropathic bladder with recurrent diffuse neoplastic polyposis (1). In the follow-up period of 1-15 years (median 24 months) all the patients had a dry interval of 4 h or more with clean intermittent catheterisation. One patient had wound dehiscence during the postoperative period and another required stomal revision 1 year after initial surgery. CONCLUSIONS The Indiana pouch is a reliable, safe, and effective form of bladder substitution. It can be reconstructed in a wide range of lower urinary tract disorders. In the vast majority of children with multiple failed surgical procedures for exstrophy-epispadias, the Indiana pouch is a safe, reliable, and reproducible procedure to provide a socially acceptable quality of life with a dry interval of 4 h.

Incidentally Detected Hepatocellular Carcinoma in Cirrhotic Children

To the Editor: Unlike hepatoblastoma, hepatocellular carcino-ma (HCC) in children is rare and generally develops in thepresence of some underlying liver disease and/or cirrhosis. Inchildrenitisusuallyassociatedw ithviralhepatitis,tyrosinemia,progressive familial intrahepatic cholestasis (PFIC) or rarely inbiliary atresia [1]. Although, regular surveillance done by ul-trasoundexaminationandalpha-fetoprotein(AFP)testingleadsto early detection of HCC, it can be missed and only detectedincidentally during the histopathological analysis of explantedliver tissue [2–4].We decided to look at the explanted liver histopathologyof the last 35 children, without increased AFP or nodules onimaging, who had undergone a liver transplant at our center.The underlying etiology was biliary atresia (n=16), PFIC(n=8), Wilson disease (n=4), cryptogenic cirrhosis (n=6)and tyrosinemia (n=1). All patients had been screened forbiochemical markers like alpha-fetoprotein, viral markers(hepatitis B and C) and radiological imaging including ul-trasonography and CT abdomen. The explanted livers wereexamined at intervals of 0.5 cm. Two specimens (5.7 %)were detected to have an underlying HCC. Both the caseshadsinglefocusofthelesion.ThediagnosiswasPFICinonecase and cryptogenic cirrhosis in the other. Both the caseshad no clinical features suggestive of HCC either during thesurgery or on follow-up. The AFP levels were 6.7 and8.5 mg/dL respectively. Both the children have remainedwell and have not shown any sign/symptom of HCC.The rate of transition from established cirrhosis to HCCoccurs at a rate of 1–4 % per year [5]. The proportion ofincidentally detected HCC in our series is 5.7 % whereas inother studies it ranges from 9.74 % to 63.3 % [2–4].Both ourcases were unifocal whereas in reports from adults the inci-dence of multinodular HCC varies from 23.3 to 60 %. Thiscouldpossiblybeduetothelesserdurationforwhichtheliverremains cirrhotic in children. Incidental finding of hepatocel-lular carcinoma on explanted liver pathology is uncommon,but not rare [2–4]. Our study shows that biochemical andradiologicalscreeningmaynotalwaysbehelpfulfordetectionof the malignant change in spite of extensive pre-transplantwork up in cirrhotic children. Serum alpha-fetoprotein levelsand ultrasound screening though helpful but are not absolutemarkers of the malignant change. If HCC is detected inexplanted liver histopathology, the patient needs to be evalu-ated for the recurrence of HCC in follow-up.References

Antituberculosis Therapy–Associated Cutaneous Leukocytoclastic Vasculitis

Antituberculosis therapy-associated cutaneous leukocytoclastic vasculitis (CLV) has been rarely reported. We describe a case of CLV induced by rifampicin and pyrazinamide. A 14-year-old male diagnosed with disseminated tuberculosis developed purpuric lesions after 1.5 months of treatment. Histopathology was consistent with leukocytoclastic vasculitis. Skin lesion improved after cessation of the two drugs and treatment with corticosteroids.

Ureterocele in newborns, infants and children: Ten year prospective study with primary endoscopic deroofing and double J (DJ) stenting.

AIM To evaluate the success of endoscopic deroofing with double J (DJ) stenting as a primary treatment modality of uncomplicated ureterocele. PATIENTS AND METHODS All babies with uncomplicated ureterocele referred to us between 2005 and 2015 were to be prospectively recruited into the study without any exception. The preoperative management would include ultrasound KUB on a fixed protocol, micturating cystourethrogram, DTPA scan and MRU (magnetic resonance urography) or IVP (intravenous pyelography). Those who had non-obstructing ureterocele were to be followed conservatively. The indications for operative intervention were to be obstructed ureterocele, bladder outlet obstruction, recurrent UTI or progressive renal scarring. They were then to be taken for cystoscopy in diuretic phase for endoscopic deroofing and DJ stenting with 9F resectoscope. Four weeks later, the DJ stent was to be removed after examining the deroofing in each patient. They were then followed up for future development of symptoms and need for secondary surgery with anatomical and functional imaging as necessary. RESULTS Forty three consecutive patients (47 ureterocele units) with ureterocele were recruited into the study between 2005 and 2015. Thirty four patients were managed by endoscopic first approach with ureterocele deroofing and DJ stenting. Four babies were asymptomatic and did not show obstructed drainage pattern on DTPA and hence were managed conservatively and kept on follow-up. Four patients underwent primary open surgery and reconstruction because of presentation with complications. ECTOPIC URETEROCELE There were 17 patients with ectopic ureterocele. Thirteen underwent primary endoscopic deroofing. Three underwent open surgery and one underwent robotic bilateral ureterocele excision and ureteric reimplantation. Eight children out of thirteen are completely asymptomatic after the endoscopic incision. On ultrasound evaluation 8/13 have resolution of the hydronephrosis and 5/13 have stable hydronephrosis with no increase in dilatation. None of these 13 children have required a second endoscopic procedure for inadequate decompression of the ureterocele. None of the 13 patients have any voiding difficulty or incontinence. 2/13 cases with recurrent UTI and persistent symptoms, required secondary surgery. This constituted 15% secondary surgery in the ectopic ureterocele category. ORTHOTOPIC URETEROCELE There were 21 patients out of 22 with orthotopic ureterocele who underwent endoscopic deroofing. Six out of these 21 patients had episodes of UTI, of which three had resolution of the reflux and two had downgrading of reflux. One child continued to have recurrent UTI and needed a laparoscopic nephroureterectomy. Eighteen children are completely asymptomatic after the initial endoscopic deroofing. The rate of secondary surgery was 4.7% in orthotopic ureterocele group, where nearly 50% have more than five years follow-up. None of our patients who are more than five years, have had any urinary incontinence or voiding dysfunction. All patients remain on follow-up from 6months to 10years (median 5years). CONCLUSION At this stage of our study with median follow-up of 5years (6months-10years), we suggest that primary endoscopic deroofing with DJ stenting for obstructing ureterocele is the best initial approach for adequate decompression and reducing the rate of secondary surgery.

Endoscopic esophageal substitution for pure esophageal atresia and wide gap esophageal atresia: A report of five cases with minimum follow-up of twelve months.

AIM The aim of the study is to report feasibility and safety of endoscopic esophageal substitution in infants with pure esophageal atresia and wide gap tracheoesophageal fistula with a minimum one year follow-up. MATERIALS AND METHODS This prospective study was conducted from January 2012 for twenty four consecutive months at Apollo Hospital, New Delhi. All babies either followed up or referred for esophageal substitution without any history of mediastinitis or associated major congenital anomaly and weighing greater than 6kg were to be included in the study. The indication, intraoperative details, operative approach, conversion to open, esophageal substitute, postoperative ventilation, ICU and hospital stay, time to solid foods, morbidity and mortality were recorded. Informed consent was obtained from all the parents and ethical clearance was obtained for the study from the hospital ethical committee. Postoperatively babies were followed up monthly for first six months, 3 monthly for next six months and annually thereafter. RESULTS Between January 2012 and December 2013, in the two year period six infants were admitted for laparoscopic gastric transposition. In five patients the procedure was completed by the laparoscopic approach and one required conversion to open surgery owing to dense adhesions. The age range at the time of surgery was from 8months to 12months with a mean age of 10months. Four patients had pure esophageal atresia (type A) and two had wide gap esophageal atresia with distal tracheoesophageal atresia (type C). Five had primary esophagostomy and gastrostomy as a newborn, the sixth had postoperative anastomotic leak and required subsequent diversion. The mean operating time was 194minutes (range 170-210minutes). The mean stay in ICU was 7days with a range of 4-12days. All patients were ventilated in the postoperative period for an average of 5days with a range of 4-7days. One patient had prolonged gastric ileus which delayed the oral feeds by 14days. The mean time to start the oral feeds was 8days with a range of 6-14days. The mean hospital stay was 19.6days (range 16-23days). Early complications were pneumonia and pleural effusion in one patient. One patient developed anastomotic stricture which was amenable to dilatation. One patient had leak from esophagogastric anastomosis which healed spontaneously. All children are now orally fed, swallow without difficulty, and parents report an excellent cosmetic outcome. The follow-up ranges from 12 to 36months. CONCLUSION The initial results of endoscopic esophageal substitution are encouraging and easily comparable to the outcome of open surgery with all the attendant benefits of minimally invasive approach.

Pediatric inflammatory bowel disease

BackgroundThe incidence of inflammatory bowel disease is increasing in the pediatric population worldwide.Need and purpose of reviewThere is paucity of high quality scientific data regarding pediatric inflammatory bowel disease. Most of the guidelines are offshoots of work done in adults, which have been adapted over time to diagnose and treat pediatric patients. This is in part related to the small numbers in pediatric inflammatory bowel disease and less extensive collaboration for multicentric trials both nationally and internationally.MethodsA literature search was performed using electronic databases i.e. Pubmed and OVID, using keywords: pediatric, inflammatory bowel disease, Crohn’s disease, Ulcerative colitis, epidemiology and guidelines. This article amalgamates the broad principles of diagnosing and managing a child with suspected inflammatory bowel disease.Main conclusions25% of the patients with inflammatory bowel disease are children and and young adolescents. The primary concern is its impact on growth velocity, puberty and quality of life, including psychosocial issues. Treatment guidelines are being re-defined as the drug armamentarium is increasing. The emphasis will be to achieve mucosal healing and normal growth velocity with minimal drug toxicity.