William M. Michener

Prognosis of Crohn's disease with onset in childhood or adolescence

A long-term follow-up study of 522 patients (1955–1974) with onset of Crohns disease under age 21 was conducted. Follow-up information was obtained for 513 (98.4%) of the patients. The mean follow-up for the entire series was 7.7 years with a greater than five-year follow-up for 67% of patients. Nearly 60% of the patients were ages 16–20 at onset of disease. Operation had been performed for 69% of the patients and 13 (2.4%) had died. Among the survivors 67% considered themselves to be in suboptimal health. Although clinical features and complications varied considerably with anatomic disease location, colonic disease generally caused more disability than small intestine location of disease. Age at onset did not correlate with clinical severity except for presence of growth retardation. Thus, this long-term follow-up study demonstrated the chronic nature of Crohns disease with onset under age 21. It also emphasized that the disease more commonly affected adolescents than children, that operation was required in about two thirds of the patients, and that, while mortality was low, morbidity from the disease continued for many years.

Long-term prognosis of ulcerative colitis with onset in childhood or adolescence.

From 1955 through 1974, 336 patients with ulcerative colitis diagnosed before age 21 years were studied. In 93 patients (29%), a blood relative had ulcerative colitis, one case of Crohns disease being found. The total colon was involved in 63% of patients; the entire colon or all but the rectal stump was removed in 35%. Eighteen patients died, nine of carcinoma of the colon. Sixty-five percent of patients had symptoms for longer than 6 months before the diagnosis of ulcerative colitis. If the diagnosis was delayed more than 24 months, there was a statistically significant correlation with increased rate of operations and complications and less good quality of life. When the 20-year study period was divided into two 10-year periods, the operative and complication rates were significantly different. Early diagnosis and treatment appear to improve the long-term prognosis of young patients with ulcerative colitis.

High Prevalence of Antibodies to Intestinal Epithelial Antigens in Patients with Inflammatory Bowel Disease and Their Relatives

STUDY OBJECTIVE To assess whether healthy members of families of patients with inflammatory bowel disease share an immune reactivity to gut epithelial cell antigens. DESIGN Assessment of immune reactivity against epithelial-cell-associated components (ECAC). METHODS Detection of specific anti-ECAC serum antibodies by antibody-dependent cellular cytotoxicity (percent specific lysis) and by immunoblotting (Western blots). PATIENTS Index cases (131) and first-degree relatives in 17 families with 2 or more affected members, and 13 with only 1 member affected. MAIN RESULTS Compared with a gastrointestinal disease control group (0.5% +/- 0.8%), specific lysis against ECAC-C (colon-derived) among patients with inflammatory bowel disease was significantly greater in both multiply affected (8.4% +/- 8.2%; P less than 0.01) and singly affected (5.2% +/- 5.4%; P less than 0.05) families. In contrast, specific lysis by patients with other inflammatory processes of the small and large bowel (1.1% +/- 1.4%) or autoimmune disease (0.7% +/- 1.0%) did not differ from that of the gastrointestinal disease control group. Among relatives of patients with inflammatory bowel disease (index cases), specific lysis was also significantly higher than in the control group (4.8% +/- 5.5% for multiply affected, P less than 0.01, and 4.3% +/- 5.5% for singly affected, P less than 0.05). Relatives of patients with chronic inflammatory liver disease had a level of lysis (0.6% +/- 0.9%) similar to that of controls. The prevalence of antibodies to ECAC-C was 69.7% among patients with chronic inflammatory bowel disease, and 55.7% among relatives; both prevalences were significantly higher than that of the control group (8.0%, P less than 0.001). Using small-bowel-derived ECAC, the prevalence of antibodies among patients with inflammatory bowel disease and relatives was also significantly higher than that of controls. Reactivity of sera was directed to a 160- and a 137-kilodalton macromolecule. CONCLUSIONS Immune sensitization to intestinal epithelial antigens is common in families with chronic inflammatory bowel disease; its high frequency among asymptomatic relatives suggests it may represent a primary phenomenon, perhaps predisposing individuals to gut tissue injury.

Course and Prognosis of Idiopathic Ulcerative Proctosigmoiditis in Young Patients

The course and prognosis of idiopathic ulcerative proctosigmoiditis were studied in 85 young patients whose symptoms had begun before the age of 21 (mean age at onset, 16 years), and the results were compared with those in adults with proctosigmoiditis. Data regarding extension of disease, available in 66 patients, allowed us to identify two groups of patients. Presenting symptoms were the same in both groups. In Group I (41 patients, 62%), the disease remained stable or did not extend beyond the descending colon. In Group II (25 patients, 38%), there was evidence of extension to the hepatic flexure or beyond. Extension was unpredictable in individual patients, but generally occurred within 5 years from the onset of symptoms (73%). The clinical course and prognosis were different in each group. In Group I, there were more females, the disease ran a milder course, and there were few complications. In Group II, there were relatively more males, the disease was more active and severe, and a high incidence of intestinal and extraintestinal complications was observed. Fifteen patients, all from Group II, required colectomy. The natural history of proctosigmoiditis in young patients is somewhat different from that in adults, being characterized by a greater tendency to proximal extension (38% vs. 10%). However, when the disease remains confined to the rectosigmoid region (or does not spread beyond the splenic flexure), the course and prognosis are no different than in adults.

The Spectrum of Eosinophilic Gastroenteritis Report of Six Pediatric Cases and Review of the Literature

Eosinophilic gastroenteritis is an inflammatory disease of unknown etiology characterized by infiltration of the gastrointestinal tract with eosinophilic leukocytes, accompanied by varying abdominal symptoms and usually by peripheral blood eosinophilia. We report our experience with six pediatric cases presenting to the Cleveland Clinic Foundation over the past eight years. Unusual findings in our patients included ascitic fluid without eosinophilia and eosinophilic pericarditis (one patient), and eosinophilic cholecystitis (one patient). Endoscopic examination and biopsy helped to establish the diagnosis in all patients. Bone marrow aspiration supported the diagnosis by demonstrating eosinophilia and identifying reactivation of the disease, even in cases without peripheral eosinophilia. All six patients responded promptly to prednisone. Diagnosis is challenging and eosinophilic gastroenteritis may be more common than is recognized. This series of cases significantly expands the spectrum of the disease in children, and documents the usefulness of diagnostic endoscopy in this condition.

Dumping syndrome in children

Dumping syndrome developed in seven children after gastric surgery, (Nissen fundoplication in six, Whipple procedure in one). The patients ranged from age 10 months to 13 years, and four of the seven were neurologically impaired. The diagnosis was made by demonstrating an abnormal response to an orally administered glucose challenge. The pediatric literature records only eight cases, but we believe that dumping syndrome is more common in children than once believed. Dietary management can often dramatically diminish the associated symptoms.

Colonoscopy in the pediatric patient

In 1963 the description 1 of the first workable fiberoptic panendoscope heralded a new era of accurate gastrointes- tinal tract diagnosis and therapeutic procedures. Since the introduction of the flexible fiberoptic colonoscope in the early 1970s. colonoscopy has become an established proce- dure for the diagnosis, evaluation, and treatment of large bowel disease in pediatric patients. The number of proce- dures has increased dramatically during the past 10 years. The use and importance of colonoscopy have continued to increase as instruments specifically designed for children come into routine use. Differences between pediatric and adult patients not only influence the approach to the patient but also modify therapeutic decisions when abnormalities are suspected or identified. We review here the value of the procedure, patient selection, instrumentation, and compli- cations. Advances in technique, premedication, and appli-

Nutritional support of the critically ill child.

New techniques for meeting the special metabolic requirements of the pediatric patient and the specific nutritional needs created by different disease states have become available both in enteral feeding and parenteral nutrition. Assessment of nutritional status and requirements of critically ill children are outlined, and the indications, techniques of administration, and complications of the available nutritional modalities are discussed.

Management of Children and Adolescents with Inflammatory Bowel Disease

The management of children and adolescents with inflammatory bowel disease requires all the skills offered by the health care team. This article reviews the principles of therapy, the specifics of therapy, and the attitudes of the authors relating to long-term management of these patients. Specific recommendations relating to nutritional support for patients are also made. A pertinent updated bibliography is also given.

Studies of Family History in Inflammatory Bowel Disease

Although it has been known for many years that inflammatory bowel disease [IBD] can occur in family clusters (1,2), in recent years there has been an increasing number of reports of familial occurrences of IBD. A study from the University of Chicago described a familial occurrence rate of 17.5% with 113 of 646 patients reporting a positive family history (3). Other studies have emphasized occurrence of IBD in multiple family members and the predilection for familial aggregation of patients with ankylosing spondylitis in Crohn’s disease [CD] (4,5). In a progress report in 1976, Lewkonia and McConnell (6), stated that “study of the families of patients with IBD leaves no doubt that ulcerative colitis and Crohn’s disease are closely associated. This association within families may be due to a common environmental aetiology, but more probably it is due to a shared genetic background.”