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Background: Any disease and its treatment has an important impact on health-related quality of life for affected individuals. There have been few previous studies on the quality of life for children with inflammatory bowel disease (IBD). Methods: A cross-cultural comparison was performed to determine whether the concerns of children with IBD in the United Kingdom are ranked similarly to those of children with IBD in Canada. An item reduction questionnaire, developed from interviews with Canadian children with IBD, was scored by 53 British children with IBD for importance and frequency, as a questionnaire had been scored previously by 117 Canadian children. Results: There was a significant correlation between the mean scores (r = 0.831, P < 0.001) and ranks (r = 0.801, P < 0.001) for the 96 questions, and 43 of the 50 highest-ranking concerns corresponded for both populations. Confidence interval analysis showed a significant difference between the mean values for 21 of the 96 items; 20 of these 21 were ranked higher in the United Kingdom than they had been in Canada, suggesting that the frequency and/or degree of concern was greater for the British children with IBD. Conclusions: Health-related concerns of British children with Crohn disease and ulcerative colitis correlate closely with those of Canadian children with those diseases. Further studies are needed to determine the sensitivity of individual questions, the most appropriate wording of these questions, and the optimal length for a proposed instrument to assess quality of life in children with IBD.

Glutamine is traditionally considered a nonessential amino acid but may be conditionally essential in patients with catabolic conditions. Glutamine-supplemented foods in these patients have been shown to prevent deterioration of gut permeability, protect against the development of intestinal mucosal atrophy, and improve nitrogen balance. Animal models of inflammatory bowel disease suggest that glutamine-enriched enteral diets may lead to less severe intestinal damage, less weight loss, improved nitrogen balance, and reduced disease activity. The purpose of the current study was to compare the efficacy of a glutamine-enriched polymeric diet with a standard low-glutamine polymeric diet in the treatment of active Crohn's disease.Eighteen children with active Crohn's disease were randomly assigned to receive a 4-week course of either a standard polymeric diet with a low glutamine content (4% of amino acid composition; group S) or a glutamine-enriched polymeric diet (42% of amino acid composition; Group G). The two diets were isocaloric and isonitrogenous with an identical essential amino acid profile. Remission rates were analysed on an intent-to-treat basis. Changes in clinical and laboratory parameters of disease activity were also compared after 4 weeks of nutritional treatment.Two of the children, both in group G, were withdrawn from the trial because of nontolerance of the diet. There was no difference between the two groups in proportion of patients achieving remission (intent-to-treat basis): 5 (55.5%) of 9 in group S versus 4 (44.4%) of 9 in group G (p = 0.5). Improvement in mean paediatric Crohn's disease activity index (PCDAI) was significantly more in group S (p = 0.002) but changes in orosomucoid level, platelet count, and weight were not different between the groups.The findings suggest that a glutamine-enriched polymeric diet offers no advantage over a standard low-glutamine polymeric diet in the treatment of active Crohn's disease. Rather, it appears to be less effective in improving PCDAI. The reported beneficial effects of glutamine seen in many catabolic states must be viewed with caution when extrapolating to the management of Crohn's disease.

In a 10-week randomised cross-sectional study we used an 88-item questionnaire to assess the quality of life in 16 children (ages 8-17 years) with Crohn's disease and their families. The questionnaire covered six domains of health-related quality of life, including disease and its treatment, social, emotional, family, education, and future aspects. Crohn's disease affected education, with absenteeism in 12 and distraction during school work in six. Three children had had a home tutor, and five stated their need for one. Engaging in sports was a problem for eight children, mainly because of a lack of energy in five and the presence of a stoma in three children. Three children had missed every PE lesson in 1 year. Five children cited the social problem of being unable to stay over at friends' houses. Bullying concerned parents more than the children. Holiday difficulties included long distance traveling or lack of toilet facilities during school trips. Elemental diet was the preferred treatment, although the majority complained about the taste. Surgery was the most effective method of symptom control, though the resulting stoma was upsetting and restricted sports activities. Children on steroids had more depressive symptoms. Using the Rutter A Questionnaire, five children were designated "neurotic." Parents' views of the severity of symptoms significantly correlated with their children's views regarding rectal bleeding, poor growth, lack of energy, and poor appetite (p0.01). The main parental concerns were the side effects of medications and issues concerning their children's future, including schooling, job prospects, and marriage. The parents of 11 children cited problems with children's behaviour. The parents of seven cited disruption of work, and those of six named taking holidays. Crohn's disease in children, in addition to being a symptomatically disabling condition, has a great impact on the health-related quality of life of both sufferers and their parents. The questionnaire was a useful instrument, and with some adjustment it can be used again in large group studies.

Deficiency of 3beta-hydroxy-delta5-C27-steroid dehydrogenase (3beta-HSDH), the enzyme that catalyses the second reaction in the principal pathway for the synthesis of bile acids, has been reported to present with prolonged neonatal jaundice with the biopsy features of neonatal hepatitis. It has also been shown to present between the ages of 4 and 46 months with jaundice, hepatosplenomegaly, and steatorrhoea (a clinical picture resembling progressive familial intrahepatic cholestasis). This paper reports two children with 3beta-HSDH deficiency who developed rickets during infancy and did not develop clinically evident liver disease until the age of 3 years. Bile acid replacement resulted in considerable clinical and biochemical improvement. The importance of thorough investigation of fat soluble vitamin deficiencies in infancy is emphasised.

Background: Focus group meetings were held with two groups, each containing 12 children with Crohn's disease aged 8 to 12 and 12 to 17 years. The children were asked broad questions about how Crohn's disease and its treatment affected their lives. To explore these areas in more detail, an 88-item questionnaire was developed and read to an additional 16 of 20 children with Crohn's disease selected at random from outpatients. At first, many of the children denied that Crohn's disease affected their lives at all, but it soon became apparent that many were frustrated or angry about physical symptoms, lack of understanding about Crohn's disease, unpleasant investigations. treatment, and hospitalisation. Methods: The questionnaire covered six domains of health-related quality of life including symptoms and treatment, social, emotional, family, educational, and future aspects. Results: Elemental diet was the preferred treatment, although surgery was more effective in controlling symptoms. Children receiving steroids had more depressive symptoms. Absenteeism from school and inability to engage in school sports, swimming, and running were frequent problems. There were also difficulties with taking holidays and staying at friends' houses. Worry was reported in 14 of 16 children, anger and frustration and feeling fed up in 12 of 16. Conclusions: In addition to being a symptomatically disabling condition, Crohn's disease has a great impact on the health-related quality of life of affected children. Future studies of treatment in children with inflammatory bowel disease should include an attempt to assess the impact on the child's health-related quality of life.

Background: Few investigators have reported on the quality of life of family members of children with inflammatory bowel disease. Psychological symptoms have been reported in parents and siblings, but the problems that which give rise to these symptoms are seldom examined. In this pilot study, some of the issues that affect the quality of life of parents and siblings of children with inflammatory bowel disease were examined. Methods: Focus group meetings were held separately with 20 parents and 7 siblings of children with inflammatory bowel disease. The participants were encouraged to identify voluntarily problems related to the disease that affected their lives, and the discussions were tape recorded. Each participant subsequently wrote down the three most important concerns. Results: Thirteen (65%) parents were very concerned about the effect of the disease on the child's future (jobs, marriage, independence) and 11 (55%) were worried about problems the ill child was encountering at school. Other issues of most concern to parents included side effects of medication (n = 7), limited job prospects (n = 3), persistent feelings of guilt (n = 3), and restricted family lifestyle (n = I ). Most siblings (57%) were concerned about their parents' keeping information about the illness from them, and three (43%) of them were also concerned about others bullying the ill child at school. Other issues of concern to siblings included fear about the disease and treatment (n = 3), parents overprotecting the ill child, and feelings of jealousy (n = 1 ). Conclusion: Families of children with inflammatory bowel disease experience many problems that may affect their quality of life. Resources should be made available to help families with these concerns.

Seven children with intractable diarrhoea of infancy seen between 1990 and 1993 developed endoscopically established inflammatory bowel disease unrelated to bacterial infection and not responsive to whole protein exclusion. Mouth ulcers were present in two patients, endoscopic upper-gastrointestinal-tract inflammation in two, villous atrophy in six, and chronic nonspecific colitis in all seven. There was considerable clinical and histological heterogeneity. All patients required parenteral nutrition. Steroid therapy was completely successful in only one child, while two underwent colectomy, and two died. Previously described cases other than those due to infection or milk intolerance have been rare and sporadic. The prevalence of inflammatory bowel disease in infancy is unknown but appears to be increasing.

The mitotic activity of epithelial lymphocytes (expressed as percentage mitotic figures/3,000 lymphocytes/mucosal biopsy) was determined in a random sample of jejunal biopsies performed on 44 children with malabsorption, diarrhoea, or failure to thrive. The mitotic index (MI) exceeded 0.2% in 19 biopsies obtained from children with untreated celiac sprue (CS); there were no false positives. The remaining 25 biopsies (MI of less than 0.2%) were considered to be "nonceliac" in origin, among which were several with a severe degree of villous flattening. Conditions in this latter category excluded by a low MI included cow's milk protein enteropathy, selective immunoglobulin A deficiency, combined variable immunodeficiency, Crohn's jejunitis, and intractable diarrhoea of infancy. A high MI (greater than 0.2%) prospectively distinguishes mucosal lesions due to untreated CS from other causes of malabsorption, particularly those associated with villous flattening, but in which the MI is less than 0.2%. This index is therefore proposed as a simple, reliable, and prospective histological marker of CS, and one that could: reduce the need to perform multiple biopsies during a gluten-free diet; and avoid the necessity for follow-up "diagnostic" gluten challenges, especially in very young children.