Incidence, clinical characteristics, and natural history of pediatric IBD in Wisconsin: a population-based epidemiological study.

BACKGROUND: Epidemiological studies of pediatric inflammatory bowel diseases (IBD) are needed to generate etiological hypotheses and inform public policy; yet, rigorous population-based studies of the incidence and natural history of Crohn's disease (CD) and ulcerative colitis (UC) in the United States are limited. METHODS: We developed a field-tested prospective system for identifying all new cases of IBD among Wisconsin children over an 8-year period (2000-2007). Subsequently, at the end of the study period, we retrospectively reconfirmed each case and characterized the clinical course of this incident cohort. RESULTS: The annual incidence of IBD among Wisconsin children was 9.5 per 100,000 (6.6 per 100,000 for CD and 2.4 per 100,000 for UC). Approximately 19% of incident cases occurred in the first decade of life. Over the 8-year study period, the incidence of both CD and UC remained relatively stable. Additionally, (1) childhood IBD affected all racial groups equally, (2) over a follow-up of 4 years, 17% of patients with CD and 13% of patients with patients with UC required surgery, and (3) 85% and 40% of children with CD were treated with immunosuppressives and biologics, respectively, compared with 62% and 30% of patients with UC. CONCLUSIONS: As in other North American populations, these data confirm a high incidence of pediatric-onset IBD. Importantly, in this Midwestern U.S. population, the incidence of CD and UC seems to be relatively stable over the last decade. The proportions of children requiring surgery and undergoing treatment with immunosuppressive and biological medications underscore the burden of these conditions.

Dumping syndrome, a cause of acquired glycogenic hepatopathy.

A 2-year-old boy, having undergone fundoplication for gastroesophageal reflux disease and fed by gastrostomy, presented with recurrent emesis, syncope with hypoglycemia, and persistently elevated serum liver transaminase levels. Liver biopsy revealed hepatocellular glycogenosis by light and electron microscopy. Further evaluation showed no evidence of diabetes mellitus, glycogen storage disease, or corticosteroid use. Since the hyperglycemic-hyperinsulinemic state of dumping syndrome would provide a mechanism for hepatocellular glycogenosis, the biopsy findings prompted consideration of dumping syndrome. Metabolic evaluation confirmed the diagnosis of dumping syndrome, and appropriate dietary management led to sustained resolution of symptomatology and hypertransaminasemia. Dumping syndrome is proposed to be a cause of hepatocellular glycogenosis, the latter representing a form of acquired glycogenic hepatopathy.

Fecal calprotectin is useful in predicting disease relapse in pediatric inflammatory bowel disease.

BACKGROUND: Fecal calprotectin (FC) has been proposed as a noninvasive surrogate marker to determine the degree of intestinal inflammation and predicting relapse in patients with inflammatory bowel disease (IBD). The aim was to compare FC levels in IBD and healthy controls, to correlate FC levels with clinical disease activity, and to assess whether FC levels can be used to predict clinical relapse in children with IBD. METHODS: Enzyme-linked immunosorbent assay (ELISA) determined levels of FC were measured in more than 1 stool samples (n) from 32 IBD patients (n = 97) and from 34 healthy controls (n = 37). Disease activity was assessed by the Harvey-Bradshaw index in Crohn's disease (CD) and by Physician's Global Assessment (PGA) in both CD and ulcerative colitis (UC). Clinical events were recorded up to 9 months following stool collection in CD patients. Wilcoxon rank sum test and Fisher's exact tests were used to compare FC levels in IBD patients and in control. Kaplan-Meyer analysis was used to determine a risk of clinical relapse in relation to FC levels. RESULTS: The IBD group had higher FC levels (range 17-7500 g/g) compared with control (16-750 g/g, P < 0.0001). FC levels were higher during relapse (CD, 3214 +/- 2186; UC, 2819 +/- 1610) compared to remission (CD, 1373 +/- 1630; UC, 764 +/- 869; P < 0.0001). Among those with clinical relapse, 90% had FC levels more than 400 mug/g in CD. Eighty-nine percent of CD encounters with FC levels less than 400 mug/g remained in clinical remission. CONCLUSIONS: FC levels differentiate active IBD from controls. Among children with CD and in remission, FC levels may be useful in predicting impending clinical relapse.

The spectrum of valproic acid-associated pancreatitis.

OBJECTIVE: Our goal was to characterize valproic acid-associated pancreatitis in children. PATIENTS AND METHODS: The charts of all patients with pancreatitis (diagnosed by using strict criteria) associated with valproic acid during a 10-year period were reviewed. Clinical and laboratory results were abstracted. RESULTS: Twenty-two patients with valproic acid-associated pancreatitis were seen during the study period. Symptoms were similar to those of patients with pancreatitis from other etiologies and included abdominal pain/tenderness (83%), vomiting/retching (74%), abdominal distention (30%), and fever/chills (26%). Valproic acid levels were in the therapeutic range in all but 1 patient. The mean duration of therapy before the onset of pancreatitis was 32 months. The serum lipase level was >3 times the reference value in all patients, but the serum amylase level was not significantly elevated in 31% of the patients tested. Imaging studies altered clinical management in only 1 patient. The length of stay was generally brief (mean: 8 days). Two patients died. Of the 5 patients who were rechallenged, 4 had relapses. CONCLUSIONS: Valproic acid-associated pancreatitis does not depend on valproic acid serum level and may occur any time after the onset of therapy. The serum lipase level is more sensitive than the serum amylase level and should be obtained when pancreatitis is suspected. Early imaging studies did not change clinical management. Rechallenge with valproic acid is dangerous and should be avoided.

Inflammatory bowel disease.

Crohn's disease (CD) and ulcerative colitis (UC), collectively known as inflammatory bowel disease (IBD) are idiopathic, life-long, destructive chronic inflammatory conditions of the gastrointestinal tract that typically manifest during late childhood and adolescence. These chronic relapsing diseases may have devastating effects on patients. New medical progress in IBD includes genetics, gut ecology and microflora, immune mechanisms, and targeted biologic therapies. This article reviews the current understanding of the etiopathogenesis of IBD, the emerging epidemiologic data in pediatric IBD, clinical presentations, diagnostic evaluation, distinctions between adult and pediatric-onset disease, and a comprehensive review of both conventional and new therapies, highlighting age-specific issues such as growth, sexual delay, and psychological and behavioral health.