[Bile lake post Kasai hepatoportoenterostomy in biliary atresia].

In this case report we describe a boy with extrahepatic biliary atresia who underwent a Kasai hepatoportoenterostomy at six weeks of age. Beginning several weeks post-op, he had recurrent cholangitis inadequately controlled by various antibiotic prophylaxis regimens. Imaging revealed the development of several bile lakes in the liver hilum. Due to the recurrent nature of his cholangitis, and some evidence of acutely impaired biliary drainage, he underwent a refashioning of his portoenterostomy with resultant improved drainage. However, shortly thereafter, the patient developed ongoing fever and anemia. Culture of the bile lake aspirate grew multiresistant Klebsiella and a 6 week course of parenteral meropenume controlled his fever and his anemia improved. Following treatment cessation his fever and anemia returned. A biliary drain was inserted into his larger bile lake and following another course of parenteral antibiotics he has remained free of clinically detected cholangitis despite ongoing contamination of drained bile fluid. The development of bile lakes after Kasai hepatoportoenterostomy is not an uncommon finding. This have been associated with worse prognosis including increased incidence of cholangitis. Often, conservative treatment with prophylactic antibiotics suffices, however, in rare cases, more aggressive intervention may be considered including percutaneous bile drainage or surgical management. The benefit of these management strategies must be balanced with the potential gain regarding quality of life and delaying transplant, on an individual basis.

Scalloping is a reliable endoscopic marker for celiac disease.

BACKGROUND: Scalloping of duodenal folds noted on esophagogastroduodenoscopy (EGD) has been associated with various illnesses including celiac disease (CD). The aim of the present study was to examine the frequency of scalloping in pediatric patients undergoing EGD and to assess its significance in the diagnosis of CD. We also evaluated the association of scalloping with the histopathology and celiac serology in the subgroup of celiac patients. PATIENTS AND METHODS: All children (0-18 years) who underwent EGD at Shaare Zedek Medical Center for any reason during a 2.5-year period were retrospectively included, yielding a consecutive cohort without selection bias. Relevant data were obtained from the patient files. RESULTS: During the study period, 623 children underwent EGD of whom 149 (24%) were eventually diagnosed with CD. In 74/623children (12%), scalloping was seen and had a sensitivity of 48% (95% CI 0.40-0.57), specificity of 99% (0.98-0.99) and positive predictive value of 97% (0.9-0.99) to diagnose CD. The prevalence of scalloping increased with advancing stage of the Marsh classification from 33% (7/21) in Marsh 1 to 63% (34/54) in Marsh 3c (P < 0.001). Scalloping was associated with a significantly higher median tissue transglutaminase level (153 [IQR 98-168] versus 49 [IQR 11-143]; P = 0.011). CONCLUSION: The results suggest that the diagnosis of CD is almost certain if isolated scalloping is observed during EGD done to rule out CD. Thus, attention to this finding may serve as an additional tool in the diagnosis of CD.

Methacholine challenge test results in children are season dependent.

BACKGROUND: It is known that several parameters influence the positivity of a methacholine challenge (MCH), including a recent viral disease, allergies, and air pollution. Reports regarding the influence of the season upon the positivity of MCH are scarce. The aim of this study was to assess the percentage of positive MCH tests per season. METHODS: We retrospectively evaluated all MCH tests performed in children and adults in a single center over a 30-month period. The percentage of positive tests for summer was compared with that of other seasons. RESULTS: A total of 155 challenges were performed in children (under 20 years old) and 527 in adults. Thirty-eight percent of the tests were positive in adults and 71 % in children. The percentage of positive tests in the summer was significantly lower than the percentage of positive results during the rest of the year in children (58.5 vs. 75.4 %, respectively; p = 0.046). By contrast, there was no difference between the seasons in adults (39 vs. 38 %, respectively; p = 0.92). CONCLUSIONS: There is a difference of 22.4 % in the percentage of positive tests in the summer months compared to the rest of the year in children, suggesting a reduction in the sensitivity of the MCH test in the hot season. We suggest that in cases where asthma is strongly suspected in a child and the MCH test was negative in the summer, one should consider repeating the MCH test in another season.

Role of surgery in severe ulcerative colitis in the era of medical rescue therapy.

Despite the growing use of medical salvage therapy, colectomy has remained a cornerstone in managing acute severe ulcerative colitis (ASC) both in children and in adults. Colectomy should be regarded as a life saving procedure in ASC, and must be seriously considered in any steroid-refractory patient. However, colectomy is not a cure for the disease but rather the substitution of a large problem with smaller problems, including fecal incontinence, pouchitis, irritable pouch syndrome, cuffitis, anastomotic ulcer and stenosis, missed or de-novo Crohn's disease and, in young females, reduced fecundity. This notion has led to the widespread practice of offering medical salvage therapy before colectomy in most patients without surgical abdomen or toxic megacolon. Medical salvage therapies which have proved effective in the clinical trial setting include cyclosporine, tacrolimus and infliximab, which seem equally effective in the short term. Validated predictive rules can identify a subset of patients who will eventually fail corticosteroid therapy after only 3-5 d of steroid therapy with an accuracy of 85%-95%. This accuracy is sufficiently high for initiating medical therapy, but usually not colectomy, early in the admission without delaying colectomy if required. This approach has reduced the colectomy rate in ASC from 30%-70% in the past to 10%-20% nowadays, and the mortality rate from over 70% in the 1930s to about 1%. In general, restorative proctocolectomy (ileoanal pouch or ileal pouch-anal anastomosis), especially the J-pouch, is preferred over straight pull-through (ileo-anal) or ileo-rectal anastomosis, which may still be considered in young females concerned about infertility. Colectomy in the acute severe colitis setting, is usually performed in three steps due to the severity of the inflammation, concurrent steroid treatment and the generally reduced clinical condition. The first surgical step involves colectomy and constructing an ileal stoma, the second - constructing the pouch and the third - closing the stoma. This review focuses on the role of surgical treatment in ulcerative colitis in the era of medical rescue therapy.

Retrospective cohort study of a new infant formula during the first 6 months of life: reflections on growth curves, human milk and formula feeding.

BACKGROUND: Optimil is an infant formula, manufactured in Israel and introduced to the market in May 2008. OBJECTIVES: To assess the effect of this formula on infant growth. METHOD: The study group comprised 52 infants who for the first 6 months of life consumed Optimil, which constituted at least 25% of their total daily intake. Anthropometric data were collected from the records of the well-baby clinics. Weight, length and head circumference at baseline and 3 months thereafter were converted to gender and age-matched standard deviation Z-scores. As an exploratory uncontrolled analysis, questionnaires were sent to the caregivers to assess satisfaction with the formula and to note the rate of constipation, irritability and vomiting as well as apparent palatability. RESULTS: The baseline Z-scores of all three parameters were below zero but increased significantly after 3 months (-0.2 +/- 0.88 to 0.12 +/- 0.88, P= 0.013 for weight; -0.44 +/- 0.87 to 0.10 +/- 0.72, P< 0.001 for length; and -0.58 +/- 0.78 to -0.1 +/- 0.76, P< 0.001 for head circumference). There was a significant dose-response effect of the formula with weight gain. The formula was generally well accepted, with 8% constipation, 8% vomiting and 6% significant irritability. CONCLUSIONS: This study provides the first evidence that infants consuming Optimil under age 6 months have adequate growth. Nonetheless, breastfeeding during this period should be preferred in almost all cases.