Pancreas divisum in a family with hereditary pancreatitis.

Hereditary pancreatitis is characterized by an autosomal-dominant mode of inheritance with incomplete penetrance, onset of symptoms in childhood or early adolescence (mean age of onset approximately 13 years), and an approximately equal sex incidence. Pancreas divisum is a congenital variant of pancreatic ductal anatomy in which the ventral and dorsal pancreatic ductal systems fail to fuse, so that two functional papillae drain the exocrine secretions of the pancreas. In recent years, several reports of pancreatitis associated with pancreas divisum in children have appeared. We now report a family in which the mother, son, and daughter all had presented with recurrent pancreatitis from an early age. Both the mother and son have endoscopic retrograde cholangiopancreatography-documented pancreas divisum, whereas the daughter has a stricture in her distal pancreatic duct. To our knowledge, this is the first such report of "familial" pancreas divisum. The implications of these findings in the setting of hereditary pancreatitis highlight the controversial issues of the clinical significance of pancreas divisum and the appropriateness of surgical therapy.

Immunoglobulin studies in children with inflammatory bowel disease.

Serum immunoglobulin A, and G subclasses were measured in patients with ulcerative colitis, Crohn's disease, and in normal controls. Significant differences were noted in elevated total gamma globulin in both disease groups: in elevated IgG1 in ulcerative colitis and elevated IgG2 in patients with Crohn's disease. These differences were not significantly related to disease activity in either disease although higher levels were observed in those with moderate and severe disease.

Crohn's disease in children 10 years old and younger: comparison with ulcerative colitis.

In order to examine the presentation and course of Crohn's disease (CD) versus those of ulcerative colitis (UC) in children < or = 10 years of age, a retrospective review of children < or = 10 years old with inflammatory bowel disease singled out 40 patients and compared their findings with those of 38 children with UC. The mean age at onset was 7.5 years for CD, as compared with 5.9 years for UC. A family history of inflammatory bowel disease was present in 13 patients (32%). Abdominal pain (97%), diarrhea (78%), and weight loss (88%) were the major initial complaints, with growth retardation present in 12 (30%) children. At onset, four children had diffuse small-bowel disease, nine had terminal ileal disease, 15 had ileocolitis, and 12 had colitis; at the end of the study two had diffuse small-bowel disease, four had terminal ileal disease, 25 had ileocolitis, and seven had colitis. Extra-intestinal manifestations increased with duration of disease. Although the number of recurrences did not differ greatly between groups, those with ileocolitis and colitis needed longer steroid therapy and more days in hospital than did those with only small-bowel disease. Operation was required in 42.5% of children with CD, as compared with 5% of those with UC, with six CD children (35%) requiring later reoperation for recurrent disease or fistula and abscess. Two children died from causes unrelated to their disease (gastric volvulus, carcinoma of the breast).(ABSTRACT TRUNCATED AT 250 WORDS)

Ulcerative colitis in children 10 years old or younger.

The onset and course of ulcerative colitis diagnosed in 38 children at or before 10 years of age were reviewed. The mean age at onset was 5.9 years. A family history of inflammatory bowel disease was present in 24% of patients, and 13% had a history of cow milk allergy in infancy. Initially, by radiologic or colonoscopic studies, 71% had total colonic disease, 13% had left-sided colitis, and 6% had proctitis; extensive examination was not performed in 4 patients. Four patients (11%) presented with severe colitis, 14 (37%) with moderate colitis, and 20 (53%) with mild colitis. The most frequent symptoms were abdominal pain (94%), diarrhea (84%), and rectal bleeding (84%). Between 2 and 10 years after diagnosis, 89% of children had total colonic disease and 11% had left-sided disease. All four patients with severe disease at onset responded to medical therapy with one having a colectomy 15 years later with pathology consistent with Crohn's disease. Of those with moderate disease, half had infrequent moderate recurrences and half had intermittent mild disease. One patient had colectomy at 21 years for intractable disease. Of the 20 with mild disease, 16 continued to have intermittent mild recurrences, 1 had chronic mild disease, 2 had moderate recurrent disease, and 1 has remained asymptomatic for 5 years. Psychiatric disturbances requiring therapy were identified in 5 (13%) children. Results are encouraging: after the first 2 years of illness, two thirds of the children have had subsequent mild colitis with infrequent relapses and three quarters consider their life to be of good quality.

Eating disorders in inflammatory bowel disease.

Before or after their diagnosis, each of three patients with inflammatory bowel disease had an established eating disorder. Two had bulimia and one was presumed to have had anorexia/bulimia. In addition to the usual modes of weight control, such as vomiting and fasting, two of the patients, all of whom were lactose intolerant, used milk ingestion as a purgative.

Gastric emptying in childhood inflammatory bowel disease: nutritional and pathologic correlates.

To determine the incidence of gastric emptying abnormalities in children with inflammatory bowel disease, we performed dual liquid/solid-emptying studies on 25 children with ulcerative colitis (UC) and on 45 with Crohn's disease (CD) over a 6-yr period. Nutritional parameters were evaluated initially and at the time of repeat study in those with abnormal emptying after a period of nutritional rehabilitation. All UC patients and 30 of 45 with CD (67%) had normal emptying of both liquid and solid components of the meal. Fifteen children with CD had delayed emptying of the solid liquid. Fourteen of these had preceding weight loss and one had no weight gain for 3 months prior to the study. Twelve complained of upper gastrointestinal symptoms (nausea, early satiety, postprandial epigastric pain, and anorexia), and five had evidence of growth retardation. The group averaged 4.8 abnormal nutritional parameters, compared with an average of 2.2 in those with CD and normal gastric emptying. Twelve of the 15 with abnormal emptying had abnormal gastric and/or duodenal biopsies: 10 were treated with sulfasalazine and prednisone, whereas five were taking only sulfasalazine. Studies repeated 6-15 months later after establishment of weight gain by caloric supplementation showed significant improvements in gastric emptying, nutritional status, and disease activity for the group, despite persistence of upper gastrointestinal disease documented in eight patients who underwent reexamination.

Relationship of common laboratory parameters to the activity of Crohn's disease in children.

The Pediatric Crohn's Disease Activity Index (PCDAI) has been proposed as a simple instrument to aid in the classification of patients by disease severity. The PCDAI includes subjective patient reporting of symptoms, physical examination, nutritional parameters, and several common laboratory tests (hematocrit, erythrocyte sedimentation rate, albumin). In this report we examine the relationship of each of the laboratory parameters to the PCDAI, as well as to a modified Harvey-Bradshaw Index score and physician global assessment of disease activity. Data were gathered from the clinical and laboratory observations from 133 children and adolescents at 12 pediatric gastroenterology centers in North America. A statistically significant relationship (p less than 0.05) was noted between each of the laboratory tests and the PCDAI for patients with either disease limited to the small bowel or in those with colonic involvement. For patients with disease limited to the small bowel, a statistically significant (p less than 0.05) relationship was also noted between the three laboratory parameters and the modified Harvey-Bradshaw Index and global assessment. For patients with large-bowel involvement, the erythrocyte sedimentation rate was statistically related to the modified Harvey-Bradshaw Index and global assessment (p less than 0.01), as was hematocrit to global assessment (p less than 0.01). Although the laboratory parameters used in the PCDAI appear to generally reflect disease activity in most patients, no single laboratory test is adequate to reflect disease activity in all patients. Future work will need to identify additional laboratory measures to reflect the inflammatory process and serve as important adjuncts in the assessment of disease activity.

Peptic ulcer disease in children.

Primary duodenal ulcer disease occurs in children of all ages, but is most often seen in those over 10 years. As in the adult, it often pursues a chronic course. Primary gastric ulcer is seen in children under 6 years, is more unusual, and does not tend to recur. Stress ulcers are seen most often in infants and in critically ill children and are asymptomatic until the complications of hemorrhage or perforation appear. Drug-related ulcers are being seen more frequently as the use of nonsteroidal anti-inflammatory agents increases. With the use of new therapeutic agents, management has been simplified and surgical intervention has become a rarity. Helicobacter pylori is now a recognized cause of antral gastritis and ulceration in the child.

Clostridium difficile in inflammatory bowel disease relapse.

Stools of 65 patients with exacerbation of symptoms of inflammatory bowel disease were examined for the presence of enteric pathogens and Clostridium difficile. Ten (16%) had C. difficile toxin. Symptoms in all patients cleared after therapy, with improvement correlating with elimination of toxin from the stool.

Positive role of Clostridium difficile infection in diarrhea in infants and children.

A retrospective review of children with Clostridium difficile infection and diarrhea identified 43 patients. Fifteen (35%) had immunoglobulins below the normal range for age, and typified those with transient hypogammaglobulinemia of infancy, because all increased their levels over the following 12 months. The age of those with hypogammaglobulinemia was significantly younger (p less than 0.01), averaging 18.8 months, than those with normal immunoglobulins who had a mean age of 4.6 yr. The number with recurrent C. difficile infection was significantly greater (p less than 0.001) in the hypogammaglobulinemic group than in those with normal immunoglobulins (46% vs 18%). Eleven children were identified who had C. difficile toxin in the stool after antibiotic therapy, but who had no diarrhea at the time of study. All had normal immunoglobulins. Control patients (20 without and 40 with diarrhea) had no evidence of C. difficile infection, and all but three had normal immunoglobulins. Three (7%) of those with diarrhea had IgA deficiency, and all had a diagnosis of milk protein allergy. These data suggest that immunoglobulin values be obtained in infants and children who have problematic C. difficile diarrhea in order to identify disorders of immunoglobulin synthesis which may be the underlying cause of repeated upper respiratory disease requiring antibiotic usage.

Development and validation of a pediatric Crohn's disease activity index.

Clinical and laboratory observations of 133 children and adolescents with Crohn's disease were used to validate an index of severity of illness previously developed by a group of senior pediatric gastroenterologists at a research forum in April 1990. This pediatric Crohn's disease activity index (PCDAI) included (a) subjective reporting of the degree of abdominal pain, stool pattern, and general well-being; (b) presence of extraintestinal manifestations, such as fever, arthritis, rash, and uveitis; (c) physical examination findings; (d) weight and height; and (e) hematocrit, erythrocyte sedimentation rate, and serum albumin. Independent evaluation of each patient by two physician-observers was performed at the time of a visit, and each physician completed a PCDAI index and a modified Harvey-Bradshaw index and made a "global assessment" of disease activity as none, mild, moderate, or severe. Excellent interobserver agreement was noted for the PCDAI, modified Harvey-Bradshaw index, and global assessment. There was a strong correlation between global assessment and both the PCDAI or modified Harvey-Bradshaw. Increasing PCDAI scores were noted with increasing disease severity, and significant differences in scores were noted between the severity groups. We propose that the PCDAI could be used in multicenter projects to facilitate patient stratification by disease severity and that longitudinal PCDAI scores might provide a numerical measure of response to therapeutic regimens.

Peptic ulcer disease in children.

Chronic gastroduodenal ulceration is the end product of an imbalance between acid levels, peptic hostile factors, and mucosal defenses. This condition differs significantly from stress ulceration, in which the primary factor is decreased mucosal blood flow, and from nonsteroidal anti-inflammatory drug-induced injury, in which there is local vascular injury and inhibition of prostaglandin synthesis. The identification of H pylori as a cause of chronic gastritis, duodenitis, and peptic ulcer is required for specific antibacterial therapy.

Cow's milk protein sensitivity during infancy in patients with inflammatory bowel disease.

Seventy-eight patients with inflammatory bowel disease (35 with Crohn's disease and 43 with ulcerative colitis) and a control population of 36 children without organic disease were surveyed to determine the frequency of symptoms compatible with cow's milk-protein sensitivity during infancy. The incidence of a history compatible with cow's milk sensitivity was 8.5% (3/35) in patients with Crohn's disease and 2.8% (1/36) in controls. Patients with ulcerative colitis had a significantly greater prevalence of symptoms, compared with the other patient groups (20.9%, 9/43; p less than 0.03). In addition, patients with a history of cow's milk allergy, who subsequently developed ulcerative colitis, did so at an earlier age (6.68 +/- 2.05 yr vs. 10.62 +/- 0.74 yr: p less than 0.02) than those without a history of cow's milk sensitivity. Thus, there appears to be a potential relationship between early cow's milk sensitivity and the development of ulcerative colitis.

Effect of bismuth subsalicylate on chronic diarrhea in childhood: a preliminary report.

Stools collected from 39 infants and children with chronic nonspecific diarrhea and from 10 age-matched controls were examined for bile acid content with use of gas-liquid chromatography. Values were correlated with stool color. Mean concentration of bile acids in green stools (7.7 +/- 1.52 mg/g dry weight) was significantly higher than that in brown stools (3.76 +/- 0.7 mg/g dry weight) and in control stools (1.42 +/- 0.35 mg/g dry weight). In patients with green diarrhea, treatments with cholestyramine and bismuth subsalicylate (Pepto-Bismol) were equally effective in decreasing stool frequency, with the latter being more effective in decreasing water content. Patients with brown stools had an insignificant response to therapy.

Cerebral thromboembolic disease in pediatric and adult inflammatory bowel disease: case report and review of the literature.

Cerebral and retinal vascular disease are rare complications of inflammatory bowel disease. Most reports are of adult patients, with only seven instances described in children. The eighth case, a 14-year-old boy with ulcerative colitis and cerebral venous thrombosis is reported in whom the diagnosis was confirmed by magnetic resonance imaging. The adult and pediatric literature is also reviewed.

Perioperative considerations in esophageal replacement for epidermolysis bullosa: report of two cases successfully treated by colon interposition.

Esophageal stricture commonly occurs in patients with epidermolysis bullosa dystrophica recessive (EBDR), but esophageal replacement is considered a high risk procedure because of limited exposure of the airway, malnutrition, and postoperative skin bullae to secondary infection. Recent innovations in care, including preoperative parenteral nutrition, topical care for bullae and skin ulceration, fiberoptic tracheal intubation, electrocardiogram monitoring with metallic pacer leads, and an overall concern to protect the fragile intact skin, have improved the results of esophageal replacement. Utilizing these adjunctive measures, ileocolonic substernal interposition has been successfully performed in a 26-yr-old male and a 19-yr-old female at our institution. Despite cervical anastomotic stricture requiring resection in one, and an obstructive cervical esophageal bullous developing acutely 5 yr after operation in the second, both patients now gum or chew a solid diet. The first patient also benefited from total esophagectomy for squamous dysplasia detected at the time of esophageal replacement. Multidisciplinary management by the surgeon, gastroenterologist, anesthesiologist, and dermatologist makes esophageal replacement available for younger patients with epidermolysis bullosa dystrophica recessive and esophageal strictures.

Gastrointestinal manifestations of epidermolysis bullosa in children.

The medical and surgical management of the chronic and recurrent esophageal and anal lesions of recessive dystrophic epidermolysis bullosa pose challenging problems for the physician. Various therapeutic approaches are discussed, and the case histories of four problem patients are reviewed.

Juvenile polyps and ulcerative colitis.

Four children with ulcerative colitis, three of whom were in clinical remission of their disease, experienced rectal bleeding due to solitary juvenile polyps. Only one of the four polyps was detected radiologically. Two children passed their polyps spontaneously, one was identified at colonoscopy, and the fourth caused colocolic intussusception and was removed during surgery.

Munchausen syndrome by proxy: considerations in diagnosis and confirmation by video surveillance.

Munchausen syndrome by proxy is a form of abuse in which the child suffers from a factitious illness induced by a parent. A case report of an 18-month-old boy who suffered from intractable diarrhea because of the surreptitious administration of laxatives by his mother is presented. The evolution of this case is discussed, as are the legal and ethical considerations in the diagnosis of Munchausen syndrome by proxy.