Somatic deletion of the imprinted 11p15 region in sporadic persistent hyperinsulinemic hypoglycemia of infancy is specific of focal adenomatous hyperplasia and endorses partial pancreatectomy.

Sporadic persistent hyperinsulinemic hypoglycemia of infancy (PHHI) or nesidioblastosis is a heterogeneous disorder characterized by profound hypoglycemia due to inappropriate hypersecretion of insulin. An important diagnostic goal is to distinguish patients with a focal hyperplasia of islet cells of the pancreas (FoPHHI) from those with a diffuse abnormality of islets (DiPHHI) because management strategies differ significantly. 16 infants with sporadic PHHI resistant to diazoxide and who underwent pancreatectomy were investigated. Selective pancreatic venous sampling coupled with peroperative surgical examination and analysis of extemporaneous frozen sections allowed us to identify 10 cases with FoPHHI and 6 cases with DiPHHI. We show here that in cases of FoPHHI, but not those of DiPHHI, there was specific loss of maternal alleles of the imprinted chromosome region 11p15 in cells of the hyperplastic area of the pancreas but not in normal pancreatic cells. This somatic event is consistent with a proliferative monoclonal lesion. It involves disruption of the balance between monoallelic expression of several maternally and paternally expressed genes. Thus, we provide the first molecular explanation of the heterogeneity of sporadic forms of PHHI such that it is possible to perform only partial pancreatectomy, limited to the focal somatic lesion, so as to avoid iatrogenic diabetes in patients with focal adenomatous hyperplasia.

Simple pediatric nutritional risk score to identify children at risk of malnutrition.

BACKGROUND: Although hospitalized children are at risk of malnutrition, routine screening of nutritional status has been hindered by lack of a validated nutritional assessment tool. OBJECTIVE: Our aim was to develop a simple pediatric nutritional risk score that could be used at hospital admission to identify patients at risk of acute malnutrition during hospitalization. DESIGN: Nutritional risk was assessed prospectively in 296 children. Anthropometric measurements, food intake, ability to eat and retain food, medical condition, and symptoms interfering with feeding (pain, dyspnea, and depression) were evaluated within 48 h of admission. Pathology was classified as mild (grade 1), moderate (grade 2), or severe (grade 3). The risk of weight loss was investigated with stepwise logistic regression. RESULTS: Weight loss during hospitalization occurred in 65% of the children and was >2% of admission weight in 45% of patients. Multivariate analysis indicated that food intake <50%, pain, and grade 2 and 3 pathologic conditions (P = 0.0001 for all) were associated with weight losses of >2%. The nutritional risk score ranged from 0 to 5 and was calculated by adding the values for the significant risk factors as follows: 1 for food intake <50%, 1 for pain, 1 for grade 2 pathologic condition, and 3 for grade 3 pathologic condition. A score of 1 or 2 indicated moderate risk and a score >/=3 indicated high risk of malnutrition. CONCLUSIONS: This simple score is suitable for routine use to identify patients at risk of malnutrition during hospitalization. Implementation may prevent hospital-acquired malnutrition.

Outbreak of nosocomial urinary tract infections due to Pseudomonas aeruginosa in a paediatric surgical unit associated with tap-water contamination.

An outbreak of 14 cases of urinary tract infections by Pseudomonas aeruginosa, including six symptomatic infections, occurred from September to November 1994 in a paediatric surgical unit. During the outbreak, urine samples from patients and multiple samples from the environment of patients were tested for the presence of P. aeruginosa. Bacterial isolates were studied by pulsed-field gel electrophoresis. Genotypic analysis showed that most of the isolates from children were different. Multiple P. aeruginosa isolates were also found in the tap water, as the only putative source of contamination. Two of these isolates were identified in two infected patients, indicating possible direct contamination of patients via tap water and this was related to the distal colonization of faucets. Bacteria were eradicated from tap water by replacement of taps. The cluster of cases of P. aeruginosa urinary infection was, therefore, related to multiple contaminations through tap water. These results illustrate an unexpected risk of nosocomial infection and emphasizes the importance of checking tap water to prevent bacterial contamination through handwashing in contaminated water.

Autologous blood donation for elective surgery in children weighing 8-25 kg.

To determine whether autologous blood donation can be used safely and efficiently in children weighing 8-25 kg, we studied children whose perioperative blood losses were expected to exceed 25% of total blood volume. Blood donations were performed in pediatric units, under the direction of an anesthesiologist and a blood bank physician experienced in paediatric care. Twenty-four children, median age 6 years (1-13), were included. They underwent surgery mainly for digestive or urological disorders, and for orthopedic defects. Forty blood collections were performed of the 46 prescribed. Phlebotomies could not be performed in 1 child because of the mother's apprehension, and in 5 cases because of venous access problems. All phlebotomies were hemodynamically well tolerated. Hemodilution was also performed in 17 children, and cell saver used in 2. Allogeneic blood transfusion was avoided in 21/24 children.