Megacystis Microcolon Intestinal Hypoperistalsis Syndrome: A Case Series With Long-term Follow-up and Prolonged Survival.

Objectives: Describe clinical characteristics, management, and outcome in a cohort of megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) patients.

Methods: We conducted a retrospective chart review of MMIHS patients followed at a large transplant and intestinal rehabilitation center over a period of 17 years.

Results: We identified 25 patients with MMIHS (68% girls, 13 transplanted).

Use of Temporary Enteral Access Devices in Hospitalized Neonatal and Pediatric Patients in the United States.

Background: Temporary enteral access devices (EADs), such as nasogastric (NG), orogastric (OG), and postpyloric (PP), are used in pediatric and neonatal patients to administer nutrition, fluids, and medications. While the use of these temporary EADs is common in pediatric care, it is not known how often these devices are used, what inpatient locations have the highest usage, what size tube is used for a given weight or age of patient, and how placement is verified per hospital policy.

Materials And Methods: This was a multicenter 1-day prevalence study.

Interdisciplinary management of pediatric intestinal failure: a 10-year review of rehabilitation and transplantation.

Management of children with intestinal failure is optimized by interdisciplinary coordination of parenteral and enteral nutrition support, medical management of associated complications, surgical lengthening procedures, and intestinal transplantation. Three hundred eighty-nine pediatric patients have been referred to our center for interdisciplinary assessment of intestinal failure since 1996 (median age=1 year; range 1 day-28.8 years).

The Bianchi procedure: a 20-year single institution experience.

Background/purpose: The emergence of improved outcomes for small bowel (SB) transplantation has raised questions regarding the utility of autologous intestinal lengthening for patients with short bowel syndrome (SBS). Chronic immunosuppression, multiple hospitalizations, and posttransplant lymphoproliferative disease are significant adverse factors. The purpose of this study is to evaluate the 20-year single institution experience with the Bianchi procedure and analyze its role in the management of pediatric SBS.

Enteral formula use in children after small bowel transplant.

The ultimate goal of intestinal transplantation (ITx) is the maintenance of nutritional status enterally/orally. We retrospectively identified children who had received ITx since the inception of our Intestinal Care Center in December 1996 (n = 24; median age, 2.6 years).

Results of growth trophic therapy in children with short bowel syndrome.

Purpose: In addition to the structural, ultrastructural, and functional changes that occur after extensive enterectomy or in utero bowel loss that results in short bowel syndrome (SBS), a complex array of humoral responses take place that may also affect adaptation of the remaining small intestine as well as nutritional status or growth. These include alterations in the levels of circulating hormones and trophic substances such as growth hormone (GH) and insulinlike growth factors (IGF-1 and IGFBP-3). The purpose of this investigation is to report on the management/treatment of 3 children with SBS (>4 years in duration) and growth failure.

Serum growth factors and growth indices pre- and post-pediatric intestinal transplantation.

Background/purpose: Although intestinal transplantation (ITx) has succeeded in liberating children with intestinal failure from total parenteral nutrition (TPN), positive growth has yet to be achieved in the majority of patients. This investigation aims to evaluate levels of serum growth factors as they relate to growth parameters and nutritional outcomes.

Methods: Serum measures of insulin-like growth factor 1 (IGF-1) and insulin-like growth factor binding protein 3 (IGFBP-3) that had been obtained before and after transplantation were reviewed (with Institutional Review Board approval) in a subset of pediatric ITx recipients.

Long-term nutritional outcome after pediatric intestinal transplantation.

Background/purpose: The aim of this study was to describe the long-term nutritional status of a large population of children after intestinal transplantation and to identify factors associated with nutritional outcomes.

Methods: Longitudinal anthropometric data are maintained in a database registry for all patients referred to our Intestinal Care Center (ICC). Z-scores for weight and height were calculated biannually over a maximum of 2 years, and associations between baseline and follow-up laboratory measures and growth were evaluated for patients greater than 6 months post intestinal transplant.