Benny Kerzner

Extraintestinal manifestations of pediatric inflammatory bowel disease and their relation to disease type and severity.

Objectives: Although it is known that extraintestinal manifestations (EIMs) commonly occur in pediatric inflammatory bowel disease (IBD), little research has examined rates of EIMs and their relation to other disease-related factors in this population. The purpose of this study was to determine the rates of EIMs in pediatric IBD and examine correlations with age, sex, diagnosis, disease severity, and distribution. Patients and Methods: Data were prospectively collected as part of the Pediatric IBD Collaborative Research Group Registry, an observational database enrolling newly diagnosed IBD patients <16 years old since 2002. Rates of EIM (occurring anytime during the period of enrollment) and the aforementioned variables (at baseline) were examined. Patients with indeterminate colitis were excluded from the analysis given the relatively small number of patients. Results: One thousand nine patients were enrolled (mean age 11.6 ± 3.1 years, 57.5% boys, mean follow-up 26.2 ± 18.2 months). Two hundred eighty-five (28.2%) patients experienced 1 or more EIMs. Eighty-seven percent of EIM occurred within the first year. Increased disease severity at baseline (mild vs moderate/severe) was associated with the occurrence of any EIM (P < 0.001), arthralgia (P = 0.024), aphthous stomatitis (P = 0.001), and erythema nodosum (P = 0.009) for both Crohn disease (CD) and ulcerative colitis (UC) during the period of follow-up. Statistically significant differences in the rates of EIMs between CD and UC were seen for aphthous stomatitis, erythema nodosum, and sclerosing cholangitis. Conclusions: EIMs as defined in this study occur in approximately one quarter of pediatric patients with IBD. Disease type and disease severity were commonly associated with the occurrence of EIMs.

Factors That Determine Risk for Surgery in Pediatric Patients With Crohn's Disease

BACKGROUND & AIMS We examined the incidence of Crohns disease (CD)-related surgery in a multi-center, inception cohort of pediatric patients with CD. We also examined the effect of starting immunomodulator therapy within 30 days of diagnosis. METHODS Data from 854 children with CD from the Pediatric Inflammatory Bowel Disease Collaborative Research Group who were diagnosed with CD between 2002 and 2008 were analyzed. RESULTS Overall, 76 (9%) underwent a first CD-related surgery, 57 (7%) underwent a first bowel surgery (bowel resection, ostomy, strictureplasty, or appendectomy), and 19 (2%) underwent a first non-bowel surgery (abscess drainage or fistulotomy). The cumulative risks for bowel surgery, non-bowel surgery, and all CD-related surgeries were 3.4%, 1.4%, and 4.8%, respectively, at 1 year after diagnosis and 13.8%, 4.5%, and 17.7%, respectively, at 5 years after diagnosis. Older age at diagnosis, greater disease severity, and stricturing or penetrating disease increased the risk of bowel surgery. Disease between the transverse colon and rectum decreased the risk. Initiation of immunomodulator therapy within 30 days of diagnosis, sex, race, and family history of inflammatory bowel disease did not influence the risk of bowel surgery. CONCLUSIONS In an analysis of pediatric patients with CD, the 5-year cumulative risk of bowel surgery was lower than that reported in recent studies of adult and pediatric patients but similar to that of a recent retrospective pediatric study. Initiation of immunomodulator therapy at diagnosis did not alter the risk of surgery within 5 years of diagnosis.

Clinical Investigation of Feeding Difficulties in Young Children: A Practical Approach

1 manuscript offers a practical guide to help the pediatrician screen for organic disease and provide constructive advice for the full range of children with feeding problems—namely, those with underlying pathology and the many others whose functional feeding difficulties range from mild to severe. In employing this approach, which is outlined in Figure 1, pediatricians are encouraged to pursue red flags implicating organic disease and identify behavioral and environmental factors that will help them to classify the specific type of feeding difficulty and tailor treatment to it. Because children often have more than 1 type of problematic feeding behavior evident, this guide for clinicians focuses on the behaviors that require attention rather than specific diagnoses.

Two-year-old twins refuse to eat : a multidisciplinary approach to diagnosis and treatment

This round table addresses the diagnosis and treatment of feeding disorders and failure to thrive. It describes a family-centered approach by a multidisciplinary feeding disorders team, beginning with the screening evaluation by the team coordinator at intake, through the evaluations by various team members, the team meeting with the parents, the development of a treatment plan, to a 1-year follow-up after treatment. Both twins refused to eat textured food and were found to have a posttraumatic feeding disorder secondary to repeated gagging and choking on cereal and lumpy baby food. However, only one twin was engaged in a battle with the parents over eating and developed infantile anorexia associated with failure to thrive. The differential diagnosis and the specific treatment plans of these two feeding disorders characterized by food refusal are discussed.

Oxygen saturation during esophagogastroduodenoscopy in children: general anesthesia versus intravenous sedation.

To the Editor: Lamireau et al. (1) have identified hypoxia as a frequent occurrence in children undergoing upper digestive endoscopy under sedation with midazolam. In their small series (n = 18), 50% of patients experienced oxygen desaturation during the procedure. They conclude that general anesthe

Do some adolescents with rumination syndrome have "supragastric vomiting"?

JPGN Volume 50, ome is defined by the R umination syndr Rome III criteria as repeated painless regurgitation and rechewing or expulsion of food within minutes of starting a meal (1). It is a phenomenon considered normal in ruminant animals, but its occurrence in humans is always pathologic. This syndrome had been reported in the past as being prevalent among infants and adults with developmental disabilities (2,3), but more recently its recognition has increased among children, adolescents, and adults with normal intelligence (4,5). The pathophysiology of rumination remains incompletely understood. The aim of this article is to describe 2 cases of adolescent girls with rumination syndrome in which we identified a new pathophysiologic mechanism similar to what has been described in supragastric belching (6). We termed it ‘‘supragastric vomiting’’ and to our knowledge it has not been described before.

Primary sclerosing cholangitis in Wiskott-Aldrich syndrome.

Wiskott–Aldrich syndrome (WAS) is a rare X-linked immunodeficiency syndrome characterized by the clinical triad of infection, thrombocytopenia, and eczema, with onset of symptoms in infancy (1). When first described by Wiskott in 1937, it was frequently fatal in early childhood. Life expectancy has increased only modestly until the past 20 years (2). Survival has improved dramatically with splenectomy and allogenic bone marrow transplantation (BMT) (3). Identification of the WAS gene in 1994 (4) and subsequently the gene product, the Wiskott–Aldrich syndrome protein (WASP), has led to improved understanding of the pathogenesis of WAS (5) and perhaps the development of specific therapies in the future, including gene therapy (6). Primary sclerosing cholangitis (PSC) is characterized by inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts resulting in bile duct obstruction and eventually cirrhosis. Although the pathogenesis of PSC is unknown, it has been associated with a variety of diseases including chronic ulcerative colitis (CUC), Crohn’s disease of the colon, and Langerhan’s cell histiocytosis X (7). Sclerosing cholangitis is described in several immunodeficiency syndromes including acquired immune deficiency syndrome (AIDS)(8). There are no documented cases of PSC in the WAS population, although CUC has been reported in WAS (9). We describe two patients with WAS, one of whom exhibited clear evidence of development of PSC. The other had clinical and radiographic features of sclerosing cholangitis and abnormal liver histology, as well as CUC.

CASE REPORT: Application of a Self-Expandable Metal Stent (SEMS) in a Child with Complete Malignant Large Bowel Obstruction

Self-expandable metal stents (SEMS) are used by adult gastroenterologists as a palliative treatment for strictures resulting from esophageal, gastric, and colorectal malignancies. However, this method has never been tried in children. This is the first such report in a pediatric patient who was rescued from an acute intractable bowel obstruction by an intracolonic stent placement. The child presented with life-threatening colonic obstruction resulting from a metastatic desmoplastic tumor of the posterior abdominal wall. He had not passed any stool for more than 1 month and was on the verge of perforation before the stent was placed endoscopically. The obstruction was relieved and the child had normal bowel movements after the procedure. The stent worked well for 6 weeks until the tumor invaded farther into the proximal colon and the child eventually underwent a semielective colostomy. A SEMS can be a suitable method for bypassing the rarely seen acute malignant intestinal obstructions in children.