Prepubertal growth in children with long-term parenteral nutrition.

In children who depend on long-term parenteral nutrition (PN), a major goal is to obtain optimal growth. The aim of this retrospective study was to analyze growth in children on long-term cyclic nocturnal home PN, over at least 8 years before puberty. Nine boys and 7 girls were studied.

Urological manifestations associated with chronic intestinal pseudo-obstructions in children.

Purpose: Chronic intestinal pseudo-obstruction syndrome is a rare pathological condition characterized by chronic intestinal occlusion without any anatomical or mechanical lesion. Recent progress in the treatment of these children has led to improved survival and development of associated urological complications. We define urological manifestations of chronic intestinal pseudo-obstruction to optimize evaluation and treatment.

[Nutritional treatment of acute diarrhea in an infant and young child].

This paper written by the Comité de nutrition de la Société française de pédiatrie is specially devoted to the nutritional treatment of infant and child acute diarrhea, i.e. oral rehydration with salts solution and feeding.

Indications and results of surgery in patients with Crohn's disease with onset under 10 years of age: a series of 18 patients.

In order to examine the presentation and course of Crohn's disease (CD) with an onset of CD before the age of 10 and to assess indications and effects of surgery, a retrospective study was performed in 18 children. One hundred and twenty-two children with CD have been followed over the last 22 years in our institution. Thirty of them had the first onset of disease before 10 years of age.

[Study of psychological repercussions of 2 modes of treatment of adolescents with Crohn's disease].

Unlabelled: No study has yet compared the respective psychological impact of corticotherapy and enteral nutrition in the treatment of Crohn's disease, and especially, the psychological problems linked to the wearing of a nasogastric tube 24 hours a day. The goal of this study was to collect comparative information regarding the real-life experience and the feeling of these two treatments.

Patients And Methods: From September 1997 to February 1998 at the clinic of inflammatory bowel diseases of the hospital Necker-Enfants malades, 51 patients aged 12 to 18 (average 15) participated in this study.

Centrilobular necrosis in children after combined liver and small bowel transplantation.

Background: Centrilobular necrosis is not an uncommon finding after isolated liver transplantation. In this study, we sought to describe hepatic centrilobular necrosis in children after combined liver and small bowel transplantation (LSBT), and to assess the predictive factors, possible causes, and prognosis.

Methods: Six children aged 4 to 11 years, in whom liver biopsy showed centrilobular necrosis at least once, 3 weeks to 2 years after LSBT, were compared with nine children without this pathology.

[Pediatric gastrointestinal endoscopy: which sedation?].

Through an analysis of the French experience of digestive endoscopists in adult patients and of their own 25 years practice of pediatric digestive endoscopy, the authors militate in favour of anesthetic sedation in order to reduce painfulness and to obtain better acceptation of these procedures by children and their parents.

[Infant formulas and soy protein-based formulas: current data].

For many years soy bean-based formulas (SBBF) were the only dietary product used for infants with cow's milk intolerance. At the present time, their place in infant nutrition is reduced as a result of the availability of new dietary products without lactose and/or cow's milk proteins and the recognition of soy bean protein allergy. There is no evidence that SBBF have any efficiency in infant colic.

Apolipoprotein B Arg3500Gln mutation prevalence in children with hypercholesterolemia: a French multicenter study.

Background: Familial defective apolipoprotein B-100, a dominantly inherited form of hypercholesterolemia caused by a single Arg3500Gln mutation, is silent in childhood but may confer a high risk of cardiovascular disease in adulthood. The objective was to determine the prevalence of familial defective apolipoprotein B-100 in hypercholesterolemic French children and to provide a basis for targeting screening efforts in this population.

Methods: One hundred ninety children attending 13 pediatric clinics distributed throughout France were included based on the presence of type IIa hypercholesterolemia with a plasma low-density lipoprotein-cholesterol level of more than 130 mg/dL.

A novel syndrome with dwarfism, poorly muscled build, absent clavicles, humeroradial fusion, slender bones, oligodactyly and micrognathia.

We report on a 15-year-old girl with severe pre- and post-natal growth retardation, poorly muscled build, micrognathia, ulnar ray oligodactyly, absent clavicles, abnormal scapulae, humeroradial fusion, hip dislocation, small iliac wings, slender tubular bones and normal intelligence. An extensive search has failed to ascribe this association to a known condition. This child shared some clinical and radiological features with the Yunis-Varon syndrome but the normal intelligence and the ulnar ray oligodactyly of our patient does not support this diagnosis.

Saccharomyces boulardii does not stimulate mucosal hyperplasia after intestinal resection in the rat.

Background: Polyamines have been shown to be important regulators of the intestinal adaptation process after massive bowel resection. Saccharomyces boulardii is yeast that has the ability to synthesize polyamines. Therefore.

Indications for pediatric intestinal transplantation: a position paper of the American Society of Transplantation.

Parenteral nutrition represents standard therapy for children with short bowel syndrome and other causes of intestinal failure. Most infants with short bowel syndrome eventually wean from parenteral nutrition, and most of those who do not wean tolerate parenteral nutrition for protracted periods. However, a subset of children with intestinal failure remaining dependent on parenteral nutrition will develop life-threatening complications arising from therapy.

Intestinal transplantation: indications, results and strategy.

The term 'intestinal failure' is now often used to describe gastrointestinal function insufficient to satisfy body nutrient and fluid requirements. The first recognized condition of intestinal failure was short bowel syndrome. Severe motility disorders such as chronic intestinal pseudo-obstruction syndrome in children as well as congenital intractable intestinal mucosa disorders are also forms of intestinal failure, because no curative treatment for these diseases is yet available.

X-linked neonatal diabetes mellitus, enteropathy and endocrinopathy syndrome is the human equivalent of mouse scurfy.

To determine whether human X-linked neonatal diabetes mellitus, enteropathy and endocrinopathy syndrome (IPEX; MIM 304930) is the genetic equivalent of the scurfy (sf) mouse, we sequenced the human ortholog (FOXP3) of the gene mutated in scurfy mice (Foxp3), in IPEX patients. We found four non-polymorphic mutations. Each mutation affects the forkhead/winged-helix domain of the scurfin protein, indicating that the mutations may disrupt critical DNA interactions.