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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Niemann-Pick variant lipidosis presenting as "neonatal hepatitis".

Neonatal hepatitis is a nonspecific term that may include a variety of disease entities. Two patients are presented who developed jaundice in the neonatal period and progressive hepatosplenomegaly. The infants were initially felt to have "neonatal hepatitis" but were subsequently found to have Niemann-Pick disease.

Obesity in celiac sprue.

Celiac sprue usually results in biochemical and clinical signs of malabsorption, nutrient loss, and resulting growth failure. We report a child with celiac sprue diagnosed at 1 year of age who was initially cachectic but who eventually developed obesity while taking a gluten-containing diet.