Paul B. Pencharz

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BACKGROUND & AIMS Congenital tufting enteropathy (CTE) is a rare autosomal recessive diarrheal disorder presenting in the neonatal period. CTE is characterized by intestinal epithelial cell dysplasia leading to severe malabsorption and significant morbidity and mortality. The pathogenesis and genetics of this disorder are not well understood. The objective(More)
The Codex Alimentarius Commission of the Food and Agriculture Organization of the United Nations (FAO) and the World Health Organization (WHO) develops food standards, guidelines and related texts for protecting consumer health and ensuring fair trade practices globally. The major part of the world's population lives in more than 160 countries that are(More)
Whole body oxidation of dietary stearic, oleic, and linoleic acid was measured in males consuming a test diet of normal foods at a level commensurate with energy requirements for 16 days. Labeled stearic, oleic or linoleic acid was consumed with the breakfast meal on either day 8, 11, or 14. Breath samples were analyzed for total CO2 content and 13CO2(More)
BACKGROUND Liquid diets given enterally combined with "bowel rest' are efficacious in the treatment of active Crohn's disease, but rapid recrudescence of gastrointestinal symptoms after resumption of a normal diet is common. AIMS This study examined whether continuation of enteral nutrition as a nocturnal supplement to an ad libitum daytime intake of a(More)
PURPOSE Treatment for acute lymphoblastic leukemia (ALL) in childhood results in a reduction in bone mineral density (BMD). Whether there is a recovery of this lost bone mass in survivors of ALL is not known. We sought to determine if changes in BMD are common long-term sequelae in children with ALL. METHODS Bone mineral densitometry of the lumbar spine(More)
Altered use of different dietary fatty acids may contribute to several chronic diseases, including obesity, noninsulin-dependent diabetes mellitus, and cardiovascular disease. However, few comparative data are available to support this link, so the goal of the present study was to compare the metabolism of [(13)C]oleate, [(13)C]alpha-linolenate,(More)
Television viewing (TVV) is considered a contributing factor to the development of childhood obesity yet it is unclear whether obesity results, in part, from increased energy intake during TVV. The objective of this study was to determine the effect of TVV on food intake (FI) of boys at a meal and its effect on caloric compensation at the test meal after a(More)
This study assessed brain function in 20 adolescent females with anorexia nervosa (AN) and 20 controls using event-related potentials (ERPs) and a battery of neuropsychological tests. In the AN group, N4 latencies for a nonverbal memory task were significantly longer than for a verbal task, and P3 latencies for the verbal task were significantly longer(More)
RATIONALE Asymmetric dimethylarginine (ADMA) is an endogenous nitric oxide synthase (NOS) inhibitor that competes with L-arginine for binding to NOS. It has been suggested that ADMA contributes to inflammation, collagen deposition, nitrosative stress, and lung function in murine models. OBJECTIVES To test the hypothesis that ADMA is increased in asthma(More)
BACKGROUND Parenteral omega-3 fatty acids, such as Omegaven, may benefit patients with pediatric short bowel syndrome (SBS) who develop parenteral nutrition-associated liver disease (PNALD). PATIENTS AND METHODS Retrospective cohort describing the outcome of all 12 children with SBS and advanced PNALD who were treated with Omegaven (target omega-6 to(More)