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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). One transplanted and 1 nontransplanted patient were lost to follow-up. We estimated 100, 100, and 86% for 5-,10-, and 20-year survival, respectively, with only 1 death. Of the 22 patients alive at the time of study (11 transplanted, 11 nontransplanted), median age was 9.2 years (range 2.7–22.9 years). Longest posttransplant follow-up was 16 years. Seventeen patients had available prenatal imaging reports; all showed distended bladder. Eight had genetic testing (5, ACTG2; 2, MYH11; 1, MYL9). Almost all patients had normal growth with median weight z-score −0.77 (interquartile range −1.39 to 0.26), height z score −1.2 (−2.04 to −0.48) and body mass index z-score 0.23 (−0.37 to 0.93) with no statistical difference between transplanted and nontransplanted patients. All nontransplanted patients were on parenteral nutrition with minimal/no feeds, and all except 1 of the transplanted patients were on full enteral feeds. Recent average bilirubin, INR, albumin, and creatinine fell within the reference ranges. CONCLUSIONS: This is the largest single-center case series with the longest duration of follow-up for MMIHS patients. In the current era of improved intestinal rehabilitation and transplantation, MMIHS patients have excellent outcomes in survival, growth, and liver function. This observation contradicts previous reports and should alter counselling and management decisions in these patients at diagnosis.

The placement of a nasogastric tube (NGT) in a pediatric patient is a common practice that is generally perceived as a benign bedside procedure. There is potential risk for NGT misplacement with each insertion. A misplaced NGT compromises patient safety, increasing the risk for serious and even fatal complications. There is no standardized method for verification of the initial NGT placement or reverification assessment of NGT location prior to use. Measurement of the acidity or pH of the gastric aspirate is the most frequently used evidence-based method to verify NGT placement. The radiograph, when properly obtained and interpreted, is considered the gold standard to verify NGT location. However, the uncertainty regarding cumulative radiation exposure related to radiographs in pediatric patients is a concern. To minimize risk and improve patient safety, there is a need to identify best practice and to standardize care for initial and ongoing NGT location verification. This article provides consensus recommendations for best practice related to NGT location verification in pediatric patients. These consensus recommendations are not intended as absolute policy statements; instead, they are intended to supplement but not replace professional training and judgment. These consensus recommendations have been approved by the American Society for Parental and Enteral Nutrition (ASPEN) Board of Directors.

Enteral nutrition is delivered via the gastrointestinal tract when oral intake is not sufficient to maintain nutrition status. There is evidence that long-term home enteral nutrition (HEN) can be advantageous to pediatric patients by improving quality of life and function. Data from pediatric patients receiving either nasogastric tube or gastrostomy tube feeds indicate good outcomes in terms of maintaining and improving nutrition status as evidenced by anthropometric measurements. As the number of pediatric patients requiring HEN increases, development of an effective program to allow smooth transition to the home is necessary. The formation of a multidisciplinary HEN management team can achieve the important task of taking care of children requiring HEN who are technology dependent. Members of a multidisciplinary HEN team can include care coordinators, nurses, physicians, dietitians, and speech therapists. Planning for patient discharge to home should start well before the actual discharge, with timely identification of home caregivers, the family's learning style, and completion of other requirements for safe administration of HEN. Education should be provided in a continuum, starting at the identification of nutrition support while still in the hospital and continuing after discharge to address concerns and long-term complications of HEN.

The placement of a nasogastric tube (NGT) in a pediatric patient is a common practice that is generally perceived as a benign bedside procedure. There is potential risk for NGT misplacement with each insertion. A misplaced NGT compromises patient safety, increasing the risk for serious and even fatal complications. There is no standardized method for verification of the initial NGT placement or reverification assessment of NGT location prior to use. Measurement of the acidity or pH of the gastric aspirate is the most frequently used evidence-based method to verify NGT placement. The radiograph, when properly obtained and interpreted, is considered the gold standard to verify NGT location. However, the uncertainty regarding cumulative radiation exposure related to radiographs in pediatric patients is a concern. To minimize risk and improve patient safety, there is a need to identify best practice and to standardize care for initial and ongoing NGT location verification. This article provides consensus recommendations for best practice related to NGT location verification in pediatric patients. These consensus recommendations are not intended as absolute policy statements; instead, they are intended to supplement but not replace professional training and judgment. These consensus recommendations have been approved by the American Society for Parental and Enteral Nutrition (ASPEN) Board of Directors.

Background In pediatric short bowel syndrome (SBS), adding fiber to enteral feedings is 1 treatment method to manage increased stool output. However, there are no standardized recommendations on the use of fiber in this setting, including type, dosage, titration strategies, etc. OBJECTIVE: The aim of this study is to determine current prevailing practices on the use of fiber in the treatment of chronic high stool output in the pediatric SBS population. Methods An anonymous electronic survey with 13 questions was sent through health professional electronic mailing lists. The survey was completed by healthcare professionals including physicians (primary care, subspecialists, and surgeons), nurse practitioners, and registered dietitians. Results A total of 94 responses were received. The most common supplemental fiber used was pectin (62.8%). The 2 major factors considered when initiating fiber therapy were consistency of stool (74.5%) and volume of stool output (85.1%). The major factor that determined discontinuation of fiber was abdominal distention (67%). A majority of providers waited 2 weeks or less to see improvement following fiber initiation before discontinuing it. Conclusions The goal of the survey was to gather more information with regard to fiber use in the management of SBS patients. The data collected can be used to provide future direction on determining best practices for fiber use in SBS patients.

1. Wednesday Marie A. Sevilla, MD, MPH, CNSC* 1. *Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children’s Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA * BMI: : body mass index CF: : cystic fibrosis CHD: : congenital heart disease CKD: : chronic kidney disease DRI: : daily required intake ESLD: : end-stage liver disease GH: : growth hormone GI: : gastrointestinal IBD: : inflammatory bowel disease MCT: : medium-chain triglycerides 1. Clinicians should be knowledgeable of the multiple factors that affect nutritional status and cause growth failure in children with chronic disease. 2. Clinicians should be knowledgeable of the basic elements of nutritional assessment in children who need nutritional support for growth failure. 3. Clinicians should be aware of the effects of limited dietary intake on nutritional status and growth. After completing the article, readers should be able to: 1. Determine the pathophysiological factors in childhood chronic disease that lead to growth failure. 2. Describe components of clinical evaluation and basic anthropometric measurements that are used to monitor nutritional status in children with chronic disease. 3. Recognize the effects of a restricted diet on nutritional intake adequacy. Growth failure, defined as the inability to reach expected growth potential based on standard anthropometric measurements for age regarding weight and height, is common in chronic childhood disease. Growth failure in chronic childhood disease is multifactorial. Nutritional outcome is affected by baseline nutritional status, disease state, and, rarely, an inherent genetic abnormality. (1) A sign of growth failure is stunting, defined as length or stature below the minimum cutoff for age, usually the fifth percentile. (2) It represents systemic dysfunction during an important phase of physical development and is the hallmark of chronic malnutrition. Related to this is the definition of short stature—a height measurement 2 standard deviations (SD) scores below the mean for age and sex. Wasting, on the other hand, is an indicator of acute malnutrition and is defined as weight-for-height or -length 2 SD scores or more below the mean for age and sex. It usually signals more recent weight loss. (2) The measurement of height and …

Patients with chronic pancreatitis (CP) frequently have genetic risk factors for disease. Many of the identified genes have been connected to trypsinogen activation or trypsin inactivation. The description of CP in patients with mutations in the variable number of tandem repeat (VNTR) domain of carboxyl ester lipase (CEL) presents an opportunity to study the pathogenesis of CP independently of trypsin pathways. We tested the hypothesis that a deletion and frameshift mutation (C563fsX673) in the CEL VNTR causes CP through proteotoxic gain-of-function activation of maladaptive cell signaling pathways including cell death pathways. HEK293 or AR42J cells were transfected with constructs expressing CEL with 14 repeats in the VNTR (CEL14R) or C563fsX673 CEL (CEL maturity onset diabetes of youth with a deletion mutation in the VNTR (MODY)). In both cell types, CEL MODY formed intracellular aggregates. Secretion of CEL MODY was decreased compared with that of CEL14R. Expression of CEL MODY increased endoplasmic reticulum stress, activated the unfolded protein response, and caused cell death by apoptosis. Our results demonstrate that disorders of protein homeostasis can lead to CP and suggest that novel therapies to decrease the intracellular accumulation of misfolded protein may be successful in some patients with CP.

Background: Postabdominal intestinal surgery (PAIS) infants pose many complex management issues. Utilization of feeding guidelines has been shown to improve outcomes in preterm and low-birth-weight infants. We propose that standardization of feeding for PAIS infants is safe. Methods: We identified 163 PAIS infants: 93 prior to and 70 after implementation of a feeding guideline. The primary outcome was time to full enteral nutrition (EN). A propensity score–matched analysis was performed. Results: The preimplementation and postimplementation PAIS infants were similar at baseline. No significant differences were seen in matched groups for time to full EN, parenteral nutrition days, or time to discharge, but cholestasis was less severe in the postimplementation group and breast milk use increased. Good compliance (67%) to daily guideline use was achieved during the initial 2 years. Conclusions: Utilization of a feeding guideline is safe and standardizes care within an institution, improving compliance to evi...

Efficient dietary fat digestion is essential for newborns who consume more dietary fat per body weight than at any other time of life. In many mammalian newborns, pancreatic lipase related protein 2 (PLRP2) is the predominant duodenal lipase. Pigs may be an exception since PLRP2 expression has been documented in the intestine but not in the pancreas. Because of the differences in tissue-specific expression, we hypothesized that the kinetic properties of porcine PLRP2 would differ from those of other mammals. To characterize its properties, recombinant porcine PLRP2 was expressed in HEK293T cells and purified to homogeneity. Porcine PLRP2 had activity against tributyrin, trioctanoin and triolein. The activity was not inhibited by bile salts and colipase, which is required for the activity of pancreatic triglyceride lipase (PTL), minimally stimulated PLRP2 activity. Similar to PLRP2 from other species, PLRP2 from pigs had activity against galactolipids and phospholipids. Importantly, porcine PLRP2 hydrolyzed a variety of dietary substrates including pasteurized human mother's milk and infant formula and its activity was comparable to that of PTL. In conclusion, porcine PLRP2 has broad substrate specificity and has high triglyceride lipase activity even in the absence of colipase. The data suggest that porcine PLRP2 would be a suitable lipase for inclusion in recombinant preparations for pancreatic enzyme replacement therapy.

Correspondence: Mark E Lowe Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children’s Hospital of Pittsburgh of UPMC, 4401 Penn Avenue, Pittsburgh, PA, 15224, USA Tel +1 412 692 5180 Fax +1 412 692 7355 Email loweme2@upmc.edu Abstract: Pancreatitis is inflammation of the pancreas that can be acute and self-limiting or, in a small percentage of patients, recurrent. Patients with recurrent episodes of acute pancreatitis (RAP) often progress to chronic pancreatitis. Pancreatitis in all forms causes significant economic and social burdens. Prevention of RAP may decrease those burdens and halt progression to chronic disease. Unfortunately, no therapy has proven effective at altering the course of RAP. While enteral nutritional therapy plays an important role in the treatment of acute pancreatitis during episodes, nutritional advice provided to patients in an attempt to prevent recurrent episodes has not proven effective in most cases. Discontinuing alcohol consumption and treating dyslipidemia with diet and medication can help patients with these issues. In patients whose pancreatitis is associated with celiac disease or eosinophilic gastroenteritis, a gluten-free diet and avoidance of food allergens can be effective in stopping RAP. Advice to take pancreatic enzyme replacement therapy, lose weight, control diabetes, decrease dietary sucrose, decrease dietary fat or avoid monosodium glutamate has little to no evidence of efficacy. Some studies suggest that an antioxidant cocktail may decrease the frequency of RAP and the intensity of chronic pain, but the evidence is weak. Nutritional therapy may have a role in the treatment of patients with RAP. At present, there are no clear guidelines for nutritional advice to give these patients. More studies are needed to identify nutritional interventions that will benefit patients with RAP.