Overlap, common features, and essential differences in pediatric granulomatous inflammatory bowel disease.

Overlap in the clinical presentation of pediatric granulomatous inflammatory bowel disease may be substantial, depending on the mode of presentation. Chronic granulomatous disease (CGD) may present with granulomatous colitis, perianal abscesses, hepatic abscesses or granulomas, failure to thrive, and obstruction of the gastrointestinal tract (including esophageal strictures and dysmotility, delayed gastric emptying, and small bowel obstruction). Anemia, thrombocytosis, elevated C-reactive protein and erythrocyte sedimentation rate, and hypoalbuminemia are nonspecific and may occur in any of the granulomatous inflammatory bowel diseases. In histology, macrophages with cytoplasmic inclusions will be rather specific for CGD. Sarcoidosis may present with abdominal pain or discomfort, diarrhea, weight loss, growth failure, delayed puberty, erythema nodosum, arthritis, uveitis, and hepatic granulomata. Only in 55% of the patients will angiotensin-converting enzyme be elevated. The noncaseating epithelioid granulomata will be unspecific. Bronchoalveolar lymphocytosis and abnormalities in pulmonary function are reported in sarcoidosis and in Crohn disease (CD) and CGD. Importantly, patients with CD may present with granulomatous lung disease, fibrosing alveolitis, and drug-induced pneumonitis. Sarcoidosis and concomitant gastrointestinal CD have been reported in patients, as well as coexistence of CD and sarcoidosis in siblings. Common susceptibility loci have been identified in CD and sarcoidosis. CD and CGD share defects in the defense mechanisms against different microbes. In the present review, common features and essential differences are discussed in clinical presentation and diagnostics--including histology--in CGD, sarcoidosis, and CD, together with 2 other granulomatous inflammatory bowel diseases, namely abdominal tuberculosis and Hermansky-Pudlak syndrome. Instructions for specific diagnosis and respective treatments are provided.

A dietary fiber mixture versus lactulose in the treatment of childhood constipation: a double-blind randomized controlled trial.

BACKGROUND: Constipation is a common problem in children. As first-line treatment, increased dietary fiber is often advocated. To our knowledge, however, no large studies evaluating the effect of dietary fibers in childhood constipation have been published. PATIENTS AND METHODS: A randomized, double-blind, prospective controlled study was performed. Patients received either a fiber mixture or lactulose in a yogurt drink. After a baseline period of 1 week, patients were treated for 8 weeks followed by 4 weeks of weaning. Polyethylene glycol 3350 was added if no clinical improvement was observed after 3 weeks. Using a standardized bowel diary, parents recorded defecation frequency during the treatment period. In addition, incontinence frequency, stool consistency, presence of abdominal pain and flatulence, necessity for step-up medication, and dry weight of feces were recorded, as were adverse effects. RESULTS: A total of 147 children were eligible; 12 children wished not to participate. Of the remaining children, 65 were randomized to treatment with fiber mixture and 70 to treatment with lactulose. In all, 97 children completed the study. No difference was found between the groups after the treatment period concerning defecation frequency (P = 0.481) and fecal incontinence frequency (P = 0.084). However, consistency of stools was softer in the lactulose group (P = 0.01). Abdominal pain and flatulence scores were comparable (P = 0.395 and P = 0.739, respectively). The necessity of step-up medication during the treatment period was comparable (P = 0.996), as were taste scores (P = 0.657). No serious adverse effects were registered. CONCLUSIONS: A fluid fiber mixture and lactulose give comparable results in the treatment of childhood constipation.

Bifidogenic effects of solid weaning foods with added prebiotic oligosaccharides: a randomised controlled clinical trial.

OBJECTIVES: Breast-fed infants have relatively high proportions of faecal bifidobacteria. This bifidogenic microbiota is at least partly explained by the high levels of prebiotic oligosaccharides in human milk. The present study aimed at testing the effect of solid foods with added prebiotic galacto- and fructo-oligosaccharides (GOS/FOS) on the composition of the intestinal microbiota of fully formula-fed infants during the weaning period. METHODS: The study was a double-blind, randomised trial with an intervention period of 6 weeks. Infants aged 4 to 6 months who were about to start consuming solid foods were randomised to receive either weaning products with a mixture of GOS/FOS or control weaning products with maltodextrin. After an adjustment period, the presented daily dose of GOS/FOS was 4.5 g. Total numbers of bacteria and bifidobacteria in faecal samples were analysed with fluorescent in situ hybridization. RESULTS: Thirty-five infants were included in the study, and 20 infants were included in a per-protocol analysis. In the GOS/FOS group, the faecal percentage of bifidobacteria significantly increased from 43% to 57% (P = 0.031) from week 0 to week 6, but did not significantly change in the control group (36% and 32%, respectively; P = 0.387). The change in the percentage of bifidobacteria between week 0 and week 6 in the GOS/FOS was significantly different from this change in the control group (P = 0.026). CONCLUSIONS: We conclude that addition of GOS/FOS to solid foods induces an increase in the faecal proportion of bifidobacteria in the intestinal microbiota of fully formula-fed infants with an established, mixed-type microbiota in the weaning period.

Pediatric median neuropathy due to pruritus in Alagille syndrome.

Median entrapment neuropathy or carpal tunnel syndrome is uncommon in children. The majority of cases are related to genetic conditions which result in skeletal dysplasia or altered connective tissue characteristics, direct injury to the median nerve caused by intensive sports or trauma, or hereditary neuropathy with liability to pressure palsies. This report describes a 10-year-old patient with Alagille syndrome who presented with poor fine motor skills because of an entrapment neuropathy of the median nerve at the wrist. This condition was probably caused by intermittent external compression at the wrists due to years of rubbing both wrists and hands to relieve pruritus. To our knowledge, median neuropathy has never been associated with Alagille syndrome, although severe pruritus is considered a major symptom and many patients exhibit widespread scratching and rubbing.

Effects of infant formula containing a mixture of galacto- and fructo-oligosaccharides or viable Bifidobacterium animalis on the intestinal microflora during the first 4 months of life.

Adding prebiotics or probiotics to infant formula to improve the intestinal flora of formula-fed infants is considered to be a major innovation. Several companies have brought relevant formulations onto the market. However, comparative data on the effects of pre- and probiotics on the intestinal microflora of infants are not available. The present study aimed to compare the effects of infant formula containing a mixture of galacto- and fructo-oligosaccharides or viable Bifidobacterium animalis on the composition and metabolic activity of the intestinal microflora. Before birth, infants were randomised and double blindly allocated to one of three formulas. The prebiotic (GOS/FOS) group (n 19) received regular infant formula supplemented with a mixture of galacto-oligosaccharides and fructo-oligosaccharides (6 g/l). The probiotic (Bb-12) group (n 19) received the same formula supplemented with 6.0x10(10) viable cells of B. animalis per litre. The standard group (n 19) received non-supplemented regular formula. A group of sixty-three breast-fed infants was included as a reference group. Faecal samples were taken at postnatal day 5 and 10, and week 4, 8, 12 and 16. Compared with the groups fed Bb-12 and standard formula, the GOS/FOS formula group showed higher faecal acetate ratio (69.7 % (sem 2.7), 69.9 % (sem 3.9) and 82.2 % (sem 5.3); P<0.05) and lactate concentration (11.3 (sem 7.9), 3.1 (sem 2.3) and 34.7 (sem 10.7) mmol/kg faeces) and lower pH (6.6 (sem 0.2), 7.1 (sem 0.2) and 5.6 (sem 0.2); P<0.05) at 16 weeks. Differences in percentage of bifidobacteria between the GOS/FOS (59.2 % (sem 7.7)), Bb-12 (52.7 % (sem 8.0)) and the standard (51.8 % (sem 6.4)) groups were not statistically significant at 16 weeks. Feeding infants GOS/FOS formula resulted in a similar effect on metabolic activity of the flora as in breast-fed infants. In the Bb-12 group, composition and metabolic activity of the flora were more similar to those of the standard group.

Allergic colitis presenting within the first hours of premature life.

UNLABELLED: A prematurely born infant developed rectal blood loss several hours after birth, after his first formula feeding. Discontinuing the feeding resolved symptoms, but after resuming feeding rectal blood loss reappeared. There were no signs of necrotizing enterocolitis. Suspecting cow's milk allergy, the feeding was changed to a casein-based protein hydrolysate, without effect. Meanwhile, laboratory tests indicated cow's milk allergy. Symptoms only resolved after introducing an amino acid-based formula supporting a definite diagnosis of cow's milk-induced allergic colitis. This is the first description of a premature infant with symptoms of allergic colitis, appearing within hours after birth, suggestive of intrauterine sensitization. The exact mechanisms of sensitization remain obscure. CONCLUSION: Cow's milk-induced allergic colitis can occur after the first feed, even in a prematurely born neonate. This is most probably due to intrauterine sensitization, and should be included in the differential diagnosis of rectal blood loss.

Infliximab therapy in 30 patients with refractory pediatric crohn disease with and without fistulas in The Netherlands.

OBJECTIVE: The purpose of this study was to describe the clinical experience with the anti-tumor necrosis factor chimeric monoclonal antibody, infliximab, in pediatric patients with Crohn disease in The Netherlands. DESIGN: Descriptive. METHODS: Clinical response and adverse effects of infliximab were recorded for pediatric patients with Crohn disease treated from October 1992 to January 2003. RESULTS: Thirty patients (aged 7-18 years) with refractory Crohn disease (with or without severe fistulas) were treated with infliximab. Patients were treated with up to 30 infusions. Mean follow-up was 25.3 months. A total of 212 infusions were administered. Thirteen patients had refractory Crohn disease without fistulas. Six patients showed good long-term response to infliximab treatment (defined as clinical index < or =10 points). Sixteen patients had refractory Crohn disease with draining fistulas. Nine showed good long-term response (closure or nonproductiveness of fistulas). One patient with metastatic Crohn disease in the skin had a good long-term response. Six patients developed an allergic reaction during infusion. In one patient, the allergic reaction occurred after an infliximab-free interval of 9 years. One patient died of sepsis. CONCLUSIONS: Infliximab was an effective therapy in 53% of patients with refractory pediatric Crohn disease, with or without fistulas. Approximately half of the patients become unresponsive to infliximab therapy. Randomized controlled studies are mandatory to assess long-term efficacy and safety to define the optimal therapeutic strategy of infliximab therapy in children with Crohn disease.