Hypereosinophilic syndrome in childhood appearing as inflammatory bowel disease

A 7-year-old girl with hypereosinophilic syndrome is described, the first such patient to present with inflammatory bowel disease. Hepatosplenomegaly, anaemia, and hypergammaglobulinaemia were also prominent features. Colonoscopy revealed a frank colitis of nonspecific histologic appearance. An elemental diet was unhelpful, but there was a prompt clinical and partial haematological response to systemic steroids. She has remained well receiving a small maintenance dose of prednisolone for 3 years.     

Nephrolithiasis in childhood inflammatory bowel disease

Six children with inflammatory bowel disease and nephrolithiasis are reported. Their mean age at the passage of the first stone was 12.5 years and the mean duration of active inflammatory bowel disease was 34.5 months. Four had ulcerative colitis and two had Crohn's disease. In three patients, the onset of stone disease was associated with a flare in the bowel disease. Stone passage in four patients was accompanied by an increase in abdominal pain; three experienced gross hematuria. Stones from four of the patients were composed primarily of calcium phosphate; stones from the remaining patients contained uric acid and/or calcium oxalate. The pathogenesis of nephrolithiasis as it relates to inflammatory bowel disease is considered and an approach to therapy offered.     

Fecal alpha 1-antitrypsin clearance in patients with inflammatory bowel disease

We evaluated fecal clearance of alpha 1-antitrypsin (alpha 1-AT) as a method of detecting and quantitating intestinal protein loss in patients with inflammatory bowel disease. We investigated alpha 1-AT clearance (C alpha 1-AT) in 14 patients (seven with Crohn's disease, seven with ulcerative colitis) and in 10 children with gastrointestinal disorders and normal serum albumin values who served as controls. The inflammatory bowel disease patients were analyzed for nutritional status, intestinal absorption, disease activity and distribution, and presence or absence of rectal bleeding. alpha 1-AT was measured in stool (72-h collections) and serum by radial immunodiffusion, and the clearance was calculated. The mean C alpha 1-AT in patients with inflammatory bowel disease was significantly (p less than 0.05) higher than that of the controls. C alpha 1-AT in the former patients was inversely related to the serum albumin level (p less than 0.001), but not to disease activity, medications, absorption, nutritional status, or moderate rectal bleeding. In the patients with Crohn's disease there was a trend to increased C alpha 1-AT from only ileal to diffuse small intestinal disease involvement. We conclude that in patients with inflammatory bowel disease, fecal clearance of alpha 1-AT is a useful method for quantitating intestinal protein loss, and that moderate rectal bleeding does not affect the C alpha 1-AT determination.     

The incidence of childhood inflammatory bowel disease in Wales

A prospective study was undertaken throughout Wales over the period from 1 January 1995 to 30 March 1997, to determine the incidence of childhood inflammatory bowel disease (IBD). Thirty-eight (24 male) new cases of IBD were reported. Twenty (16 male) of the reported cases had Crohn's disease, 11 (5 male) had ulcerative colitis (UC) and 7 (3 male) indeterminate colitis. Crohn's disease occurred four times more often in boys than girls, whereas there was no sex difference in the incidence for UC and indeterminate colitis. The median age at presentation was 12 (range 1.5–16) years and there was no difference in the age of presentation or the duration of symptoms prior to diagnosis in any of the types of IBD. Conclusion The overall incidence (95% confidence intervals) for IBD in Wales was 2.6 (1.87–3.48) cases per 100,000 per year. The incidence for Crohn's disease was 1.36 (0.86–2.04) cases per 100,000 per year, for UC 0.75 (0.39–1.28) cases per 100,000 per year and for indeterminate colitis 0.48 (0.2–0.92) cases per 100,000 per year. Received: 14 June 1999 / Accepted in revised form: 23 September 1999     

Neurovascular and thromboembolic complications of inflammatory bowel disease in childhood

Vascular complications occurred in 3.3% (6 of 180) of children with chronic inflammatory bowel disease. Clinical disease activity was severe in four of six patients who presented with these symptoms. In contrast to adults, in whom deep-vein thrombotic complications predominate, the majority of children had involvement of the CNS including encephalopathy, arteritis, arterial occlusion, and thromboembolism. One patient died 3 1/2 years later from progressive intestinal and neurological deficits: morbidity included blindness, epilepsy, and developmental delay. Hematological parameters varied widely. Prevention is not always possible, but risk factors include: (a) family history of collagenosis (83% incidence), (b) severe inanition and immobility, (c) delayed hospitalization, and (d) clinical presentation with cutaneous vasculitis. Therapy must be individualized.     

Desquamative interstitial pneumonitis complicating inflammatory bowel disease of childhood

Sulfasalazine has been associated with bronchopulmonary complications of inflammatory bowel disease (IBD) in adults. We describe a 12-year-old boy who developed desquamative interstitial pneumonitis and hepatic cirrhosis several years following the onset of ulcerative colitis. The restrictive lung disease progressed despite cessation of sulfasalazine and initiation of corticosteroid therapy. We discuss a variety of bronchopulmonary complications of IBD and their association with sulfasalazine.     

Potential uses for quality-of-life measures in childhood inflammatory bowel disease

Health-related quality of life (HRQOL) in children with inflammatory bowel disease is clearly reduced in the physical as well as in the psychological and social functioning domains. However, very few studies of these children have been performed from a quality-of-life perspective. Multicenter studies on HRQOL are needed to answer questions about the impact of Crohn's disease and ulcerative colitis and their treatment and complications on HRQOL in children and adolescents and their association with outcome. This article describes perspectives from which quality of life studies may be performed, potential uses of HRQOL measures, and issues that should be accounted for in designing multicenter clinical studies on this topic.     

Colonic inflammation in pediatric inflammatory bowel disease: detection with magnetic resonance enterography

Background

Colonic involvement in pediatric inflammatory bowel disease is common. Magnetic resonance (MR) enterography is considered the best imaging modality for pediatric inflammatory bowel disease evaluation. It is unclear whether the lack of a dedicated large bowel preparation prevents a reliable colonic assessment.

Objective

To determine the diagnostic performance of standard MR enterography in detecting and grading colonic inflammatory activity.

Materials and methods

We retrospectively evaluated children who underwent both MR enterography and ileocolonoscopy with biopsies <4 weeks apart. Two radiologists independently reviewed MR examinations and quantified inflammation in each of the five colonic segments using a standardized MR score system. Findings were compared with histological examination of the corresponding segment. Mann-Whitney, Kruskal-Wallis, Jonckheere-Terpstra and Bland-Altman statistics were used.

Results

One hundred seventy-five segments from 37 examinations were included. MR enterography diagnostic performance for inflammation was as follows: sensitivity 94% (95% confidence interval [CI]: 90-97%), specificity: 64% (95% CI: 57-71%). A significant positive correlation was found between MR score and inflammatory activity histologically graded (P<0.001, Jonckheere-Terpstra test). The interobserver agreement was good (mean difference between MR enterography scores was -0.03; limits of agreement -2.8 to 2.7).

Conclusion

Standard MR enterography is sensitive for the detection of actively inflamed colonic segments. MR enterography might provide useful information for guiding biopsies and its role as an alternative to ileocolonoscopy in monitoring colonic disease activity in children should be further investigated.

     

The IMPACT questionnaire: a valid measure of health-related quality of life in pediatric inflammatory bowel disease

BACKGROUND AND AIMS IMPACT: is a disease-specific health-related quality-of-life questionnaire developed for use in pediatric inflammatory bowel disease through a process of patient interviews and analysis of patient responses to an item-reduction questionnaire. This study sought to assess the feasibility, reliability, and validity of the instrument. METHODS: The readability statistics and number of unanswered questions were assessed among 147 patients (97 CD, 50 UC) with mean age 14.4 +/- 2.2 years (range 9.2-18.0 years) using the self-administered questionnaire. Internal consistency was assessed with Cronbach's alpha and test-retest reliability using intraclass correlation coefficient (ICC). Construct validity was based on a priori hypotheses. Mean total scores were compared by ANOVA among patients grouped according to disease activity and disease severity within the past year. RESULTS: The readability statistic showed a Flesch-Kincaid Grade level of 4.5. Only 0.68% of questions were left blank. Reliability was excellent with Cronbach's alpha = 0.96, and an ICC of 0.90 in patients with stable disease over a two-week period (n = 32). The mean total IMPACT score for patients with quiescent disease (180 +/- 32) was significantly higher (better QOL) than for those with active disease (146 +/- 31 for mild, 133 +/- 34 for moderate/severe) (P < 0.005). CONCLUSIONS: The IMPACT questionnaire is a valid and reliable reflection of health-related quality of life of older children and adolescents with both ulcerative colitis and Crohn's disease.     

MICI-MINOTS: Linguistic and metric validation of a pediatric questionnaire on knowledge about inflammatory bowel disease

BackgroundTherapeutic education is an essential part of the treatment of chronic diseases, such as inflammatory bowel disease (IBD). The IBD-KID, developed in Canada in English, assesses children's and adolescents’ acquired knowledge about their condition and has been validated in Canadian and Australian populations. However, there is no pediatric questionnaire in French to assess patients’ knowledge about IBD.ObjectiveTo report the linguistic validation process and metric validity of the MICI-MINOTS, the French version of the IBD-KID.MethodThe translation process consisted of three consecutive steps: forward–backward translation, acceptability testing, and cognitive interviews. The IBD-KID consists of 23 questions, but a 24th question about immunomodulatory therapy was added in the MICI-MINOTS. Psychometric testing was conducted with five groups: children with IBD, their parents, children in a control group, their parents, and health workers recruited from the Timone Pediatric Hospital and the Saint-Sébastien Maternal and Child Protection Center, Marseille, France. A total of 15 individuals completed the tool twice, with a 15-day interval. Internal consistency, reliability, external validity, reproducibility, and sensitivity to change were tested.ResultsA total of 38 children with IBD (sex: 20 boys, 18 girls; age: 13.90 [± 2.88] years; 25 with Crohn's disease), 20 children in the control group, 58 parents (every child was included with one parent), and 62 health workers were included in the analysis. Intraclass correlation was 0.94 (95% confidence interval 0.83–0.98) for test–retest assessment. Readability using the Scolarius score corresponded to elementary school level. Among the children with IBD, 89.5% answered all 24 questions. For 23 questions, the mean score of children with IBD was higher than among children in the control group: 9.58 (± 3.01) versus 5.47 (± 3.56), respectively (P < 0.01). Parents of children with IBD scored higher than parents of children in the control group: 10.63 (± 3.16) versus 8.4 (± 3.07), respectively (P = 0.012). In the health workers’ group, pediatric residents (17.82 ± 3.46) scored higher than nurses 11.75 (± 3.4) and ward clerks (8.67 ± 2.40; P < 0.01). Patients’ knowledge score was significantly related to their parents’ knowledge score (r = 0.402, P = 0.012) for 23 questions.ConclusionThe French version of the IBD-KID showed satisfactory psychometric properties to assess knowledge about the disease in French-speaking children.     

Epidemiology of inflammatory bowel diseases in childhood

Inflammatory Bowel Disease (IBD) is common in most industrialised countries and childhood IBD accounts for nearly 30% of total cases. Various studies, mostly from Europe and USA have reported epidemiological characteristics of childhood IBD. The incidence figures vary greatly from region to region and within a region over time. Almost all reported studies have documented an increase in the incidence, mainly of Crohn’s disease over the last few decades. The reasons for the increase are not clear but epidemiological observations have led to many postulates. Incidence in developing countries is perceived to be low, but limited data suggest that it may not be as uncommon as previously thought. IBD can occur at any age but is rare in infancy. Among childhood IBD, early onset IBD appears to be different epidemiologically and is characterised by predominance of colonic involvement and high positive family history. It has become apparent that only about 25% of childhood Crohns disease presents with classical triad of abdominal pain, diarrhoea and weight loss. Pediatricians should be aware of atypical manifestations and should maintain high index of suspicion. Though epidemiological studies of childhood IBD done so far have contributed towards understanding of IBD, they have differed in study design, population, time period, age group and case definitions. Unfortunately there are no uniform, clear diagnostic criteria which are evidence based. To address this problem, recently the IBD working group of European Society of Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) has published “The Porto Criteria” which details a consensus based diagnostic criteria for the diagnosis of childhood IBD. This should bring uniformity in ascertainment of newly diagnosed IBD cases. An European multicentre prospective database has also been established to facilitate future epidemiological studies.     

Acute inflammatory bowel disease in childbood: a new disease?

Three children aged between 7 and 11 years, after an acute onset of diarrhoea and vomiting, developed protracted diarrhoea and severe loss of weight. None had been abroad. No significant aetiological agent was found. There was evidence of acute inflammatory disease on proximal small intestinal biopsy, and some evidence of more widespread gut involvement--of the rectum in Cases 1 and 2, and the terminal ileum in Cases 2 and 3. The disease resolved spontaneously and without relapse.