Branched-Chain Amino Acid Database Integrated in MEDIPAD Software as a Tool for Nutritional Investigation of Mediterranean Populations.

Branched-chained amino acids (BCAA) are essential dietary components for humans and can act as potential biomarkers for diabetes development. To efficiently estimate dietary intake, we developed a BCAA database for 1331 food items found in the French Centre d'Information sur la Qualité des Aliments (CIQUAL) food table by compiling BCAA content from international tables, published measurements, or by food similarity as well as by calculating 267 items from Greek, Turkish, Romanian, and Moroccan mixed dishes. The database embedded in MEDIPAD software capable of registering 24 h of dietary recalls (24HDR) with clinical and genetic data was evaluated based on archived 24HDR of the Saint Pierre Institute (France) from 2957 subjects, which indicated a BCAA content up to 4.

Parent and health professional perspectives in the management of adolescents with diabetes: development of assessment instruments for international studies.

Objective: Assessment of quality of life (QOL) in adolescents with diabetes requires patient, parent and health professional input. Psychometrically robust instruments to assess parent and professional perspectives are required.

Research Design And Methods: Questionnaires concerning adolescent QOL were developed for completion by parents and health professionals.

Effects of a two-month rehabilitation program on substrate utilization during exercise in obese adolescents.

Background: We assessed the impact of an exercise and diet program for two weeks in a specialized institute and six weeks at home on body composition and exercise metabolism in obese adolescents.

Methods: Fourteen obese adolescents took part in the two-week training course and seven continued exercise and diet at home. The substrate crossover point and the maximal fat oxidation point (Lipox max) were determined before and after the program, using indirect calorimetry.

Insulin injection regimens and metabolic control in an international survey of adolescents with type 1 diabetes over 3 years: results from the Hvidore study group.

Unlabelled: The optimal insulin regimen for paediatric patients with type 1 diabetes remains controversial. Therefore this multicentre study was performed in adolescents over a 3-year period to assess metabolic control, severe hypoglycaemia, and weight gain in relation to insulin injection regimens. Out of 2873 children and adolescents in an international survey in 1995, 872 adolescents (433 boys, 439 girls, mean age in 1995 11.

Hepatitis C in a ward for cystic fibrosis and diabetic patients: possible transmission by spring-loaded finger-stick devices for self-monitoring of capillary blood glucose.

Objective: To identify the routes of transmission in a nosocomial outbreak of hepatitis C virus (HCV) infection.

Design: Epidemiological investigation, including screening for HCV of hospitalized patients, and a retrospective cohort study, review of hygiene and medical practices, and molecular comparison of HCV isolates.

Setting: A specialized care unit for cystic fibrosis (CF) and diabetic patients at an acute-care facility in the south of France.

Good metabolic control is associated with better quality of life in 2,101 adolescents with type 1 diabetes.

Objective: It is unclear whether the demands of good metabolic control or the consequences of poor control have a greater influence on quality of life (QOL) for adolescents with diabetes. This study aimed to assess these relations in a large international cohort of adolescents with diabetes and their families.

Research Design And Methods: The study involved 2,101 adolescents, aged 10-18 years, from 21 centers in 17 countries in Europe, Japan, and North America.

Persistent differences among centers over 3 years in glycemic control and hypoglycemia in a study of 3,805 children and adolescents with type 1 diabetes from the Hvidøre Study Group.

Objective: Twenty-one international pediatric diabetes centers from 17 countries investigated the effect of simple feedback about the grand mean HbA(1c) level of all centers and the average value of each center on changes in metabolic control, rate of severe hypoglycemia, and insulin therapy over a 3-year period.

Research Design And Methods: Clinical data collection and determination of HbA(1c) levels were conducted at a central location in 1995 (n = 2,780, age 0-18 years) and 1998 (n = 2,101, age 11-18 years).

Results: Striking differences in average HbA(1c) concentrations were found among centers; these differences remained after adjustment for the significant confounders of sex, age, and diabetes duration.

Insulin management and metabolic control of type 1 diabetes mellitus in childhood and adolescence in 18 countries. Hvidøre Study Group on Childhood Diabetes.

Insulin regimens and metabolic control in children and adolescents with Type 1 diabetes mellitus were evaluated in a cross-sectional, non-population-based investigation, involving 22 paediatric departments, from 18 countries in Europe, Japan, and North America. Blood samples and information were collected from 2873 children from March to August 1995. HbA1c was determined once and analysed centrally (normal range 4.

Factors associated with glycemic control. A cross-sectional nationwide study in 2,579 French children with type 1 diabetes. The French Pediatric Diabetes Group.

Objective: To determine on a large scale the multiple medical and nonmedical factors that influence glycemic control in the general population of children with diabetes, we performed a nationwide French cross-sectional study.

Research Design And Methods: We enrolled 2,579 patients aged 1-19 years with type 1 diabetes of > 1 year's duration. The study was center based: 270 centers were identified, 206 agreed to participate, and 147 included at least 90% of their patients.

Molecular study of the 5 alpha-reductase type 2 gene in three European families with 5 alpha-reductase deficiency.

The molecular basis of 5 alpha-reductase (5 alpha R) deficiency was investigated in four patients from three European families. In the French family, the first patient was raised as a female, and gonadectomy was performed before puberty. The second sibling, also raised as female, differed in that gonadal removal was performed after the onset of pubertal masculinization.

Molecular prenatal exclusion of familial partial androgen insensitivity (Reifenstein syndrome).

In a large family with Reifenstein syndrome, we previously performed molecular analysis of the androgen receptor gene. Direct sequencing showed a G-A point mutation at position 2818 of exon 7, which was responsible for an arginine-histidine substitution at position 840 of the androgen receptor. In this family, the proband's mother became pregnant and wished to know whether she was carrying an unaffected fetus.

[Klinefelter syndrome in 1993. Results of a multicenter study on 58 cases and review of the literature].

A retrospective multicenter study found 58 cases of Klinefelter syndrome of which 23 (39%) were diagnosed before puberty. Although as common as Down syndrome, Klinefelter syndrome is underdiagnosed and often recognized only in adulthood. Suggestive manifestations in infants, children, and teenagers include facial dysmorphism, micropenis, and delayed speech and should lead to examination of the karyotype.

Improvement of diabetic control and acceptability of a three-injection insulin regimen in diabetic adolescents. A multicenter controlled study.

Objective: To compare the effectiveness and acceptability of a three-injection insulin regimen with the conventional two-injection therapy in an unselected population of diabetic adolescents.

Research Design And Methods: Some 205 patients aged 10-18 yr with IDDM, who were previously treated with two daily insulin injections, were included without any selection into a randomized trial. They were either switched to three (regular prebreakfast, regular prelunch, and [regular+ultralente] predinner) or remained on two ([regular+intermediary] prebreakfast and predinner) subcutaneous injections.

Congenital adrenal hyperplasia associated with hyperphosphatemic rickets.

The authors describe a boy with precocious puberty due to adrenal hyperplasia associated with rickets, hypocalcemia, hyperphosphatemia, elevated PTH and alkaline phosphatase levels, and concentrations of 25-OH-D and 1,25-(OH)2D at the upper limit or above normal range. Treatment with hydrocortisone for 9 months did not normalize hypocalcemia and hyperphosphatemia. The addition of 1,25-(OH)2D3 (0.

1-year treatment with recombinant somatropin in prepubertal and pubertal growth hormone deficient patients: results from a French multicentre trial.

In this French study with recombinant somatropin, the stimulation of growth in 32 prepubertal (age 10.0 +/- 3.5 years; mean +/- SD) and 19 pubertal (age 14 +/- 1.

[Surgical problems posed by abnormalities of testicular migration].

Based on their experience and on a review of the literature, the authors study the surgical aspects of the undescended testis. A precise definition excluding the retractile testis is essential. Although the understanding of the pathogenesis remained incomplete, the present data give support to an early orchidopexy performed by a specialized surgeon following the failure of a human chorionic gonadotropin test.

[Treatment of precocious puberty with LH-RH agonists. Multicenter study using D-Trp-6-LH-RH in a programmed-release form].

LH-RH analogs, substituted in position 6 by D-tryptophane, D-serine(tBu), D-leucine or D-alanine induce a strong stimulation of the gonadotrophs, followed by a desensitization of the LH-RH receptors, which leads to a blockade of the gonadotropin secretion and to a hypogonadism. A delayed release preparation of D-Trp-6-LH-RH (Decapeptyl in microcapsules), designed to release the peptide for 28 days after intramuscular injection, was given to 69 girls and 18 boys with precocious puberty. In both, plasma levels of gonadotropins and gonadal steroids were suppressed within 3 weeks, whilst pituitary responses to LH-RH were almost abolished within 7 weeks.

Long term treatment of male and female precocious puberty by periodic administration of a long-acting preparation of D-Trp6-luteinizing hormone-releasing hormone microcapsules.

The efficacy and safety of a delayed release formulation of the LHRH agonist D-Trp6-LHRH (LHRH-A; im microcapsules) were tested in 16 girls, 0.9-8.8 yr old, and 10 boys, 2.

[Male pseudohermaphroditism caused by partial insensitivity to androgens. Clinical and biochemical heterogeneity].

Male pseudohermaphroditism due to partial androgen insensitivity (PAI) may be suspected clinically in case of incomplete masculinization of external genitalia in spite of age related plasma androgen levels. In 25 children or adolescents in whom PAI was suspected, the 5 alpha-reductase activity of external genitalia fibroblasts, the number of androgen receptor sites (Bmax) and the affinity of receptors for dihydrotestosterone (Kd) were studied. Clinical expression of PAI is highly polymorphic (Prader's type I to type IV), when most children (18/25) were considered as males.

[Persistant pubertal gynecomastia. Biological study (author's transl)].

17 cases of peristant pubertal gynecomastia (which differs from physiologic breast enlargement by their volume and duration) were studied. Plasma estrogens and urinary phenol steroids excretion were found higher than in normal boys. The main biological finding in these subjects was a decrease of the plasma testosterone/estradiol ratio, and a moderate but transient increase in plasma prolactin.