Tolerance to proinsulin-2 is due to radioresistant thymic cells.

Proinsulin is a key Ag in type 1 diabetes, but the mechanisms regulating proinsulin immune tolerance are unknown. We have shown that preproinsulin-2 gene-deficient mice (proins-2(-/-)) are intolerant to proinsulin-2. In this study, we analyzed the mechanisms underlying T cell-mediated tolerance to proinsulin-2 in 129/Sv nonautoimmune mice.

[Artificial supporting methods for the treatment of acute organ failure].

Improvement of organ supporting devices clearly represents a major progress amongst the other numerous medical advances that permitted the development of intensive care. The areas in which these advances are the most consistent are multiple. The most well-established progresses concern respiratory support (including non invasive ventilation, prone positioning, nitric oxide administration, and possibly partial liquid ventilation), mechanical circulatory support and continuous renal replacement therapies.

Impaired sexual activity in male adults with partial androgen insensitivity.

Context: Choosing the sex of rearing of an XY neonate with a major sexual ambiguity and a mutated androgen receptor remains one of the more difficult questions of neonatal endocrinology. A direct consequence of this choice is the accomplishment of sexual function in adulthood. There is very limited knowledge of the sexual performance of patients with partial androgen insensitivity syndrome.

[Gamete and embryo donations].

Medical taking part in procreation in gamete and embryo donation is recent in France and has been included in the field of medically assisted procreation as defined by a law since 1994. Theses activities are strictly regulated. If procreation using donor sperm has been broadly carried out for 30 years in the CECOS leading to the birth of approximately 40,000 children, oocyte and embryo donation had a more recent and limited development.

[Antinuclear antibodies].

Antinuclear antibodies react with various nuclear autoantigens. Testing for them is critical for diagnosing connective tissue diseases, but they may be also found in other inflammatory diseases and in the elderly. They constitute a group of heterogeneous autoantibodies, and their specificity helps to discriminate between various connective tissue diseases.

Puberty in subjects with complete androgen insensitivity syndrome.

Background: Androgen receptor defects affect the regulation of the gonadotropic axis. However, little is known about the timing of pubertal maturation in complete androgen insensitivity syndrome (CAIS).

Aims: To evaluate growth, skeletal maturation and gonadotropin and sex steroid secretion in patients with CAIS and intact gonads at puberty.

Prepubertal gynecomastia in Peutz-Jeghers syndrome: incomplete penetrance in a familial case and management with an aromatase inhibitor.

Background: Peutz-Jeghers syndrome (PJS) is a rare autosomal-dominant disorder characterized by multiple gastrointestinal hamartomatous polyps, mucocutaneous pigmentation and increased predisposition to various neoplasms. Endocrine manifestations in PJS include gynecomastia due to calcified Sertoli cell testicular tumors usually referred to as large-cell calcifying Sertoli cell tumors (LSCT).

Objective: To evaluate the value of endocrine markers and aromatase inhibitor treatment in children with PJS and LSCT.

[Spongioplasty in the treatment of hypospadias].

Objective: Hypospadias is a frequent anomaly, for which many repair techniques have been proposed. In 2000, we described a method of one-stage anatomical correction, called spongioplasty. The present study evaluated the functional and cosmetic results of this technique.

Three-month sustained-release triptorelin (11.25 mg) in the treatment of central precocious puberty.

Objective: Depot GnRH agonists are commonly used in the treatment of central precocious puberty (CPP). The triptorelin 11.25 mg 3-month depot, currently used in adult indications, had not previously been evaluated in CPP.

Fetal lung growth in congenital diaphragmatic hernia.

Background: In spite of significant therapeutic progress, the prognosis of congenital diaphragmatic hernia (CDH) remains pejorative in those forms in which the liver is herniated into the chest. The severity of this malformation relies on the pulmonary hypoplasia due to lung compression by the herniated viscera in the thoracic cavity, particularly the liver. This impaired growth concerns the whole pulmonary tissue, i.

[Delayed testing for the diagnosis of fungi in the urines. Evaluation of the BD Vacutainer C&S tubes for the storage of urine samples at room temperature].

The study included 37 urine samples which have been artificially infected with low levels (10(3) CFU/mL) of various fungi strains. We compared the effects of sample storage, up to 48 hours, at room temperature, in a urine evacuated tube containing specific additives with storage at + 4 degrees C, for the same length of time, in a urine evacuated tube without any additives. There have been no differences of results (speed of growth and colony size) between the 2 modes of storage.

Surgery versus medical follow-up in patients with asymptomatic primary hyperparathyroidism: a decision analysis.

Objectives: To examine the cost-effectiveness of strategies for management of primary asymptomatic hyperparathyroidism: surgical strategies and medical follow-up versus surgery.

Design: We used a Markov state-transition decision-analytic model for an hypothetical cohort of 55-year-old women to compare with a lifetime horizon costs and effectiveness of bilateral neck exploration (BNE), unilateral neck exploration (UNE), video-assisted parathyroidectomy (VAP) and lifelong medical follow-up shifting for either BNE or UNE in case of disease progression.

Methods: Data on localization tests, complications and treatment efficacies were derived from a systematic review of the literature.

[Treatment of precocious puberty by GnRH agonists].

The objective of GnRH agonist treatment in precocious puberty is to block pubertal development, and to reduce the action of sex steroids on bone maturation in order to restore normal long-term growth of the skeleton. Currently available studies show a positive benefit-risk ratio of GnRH analogs in the treatment of precocious puberty. Adult heights obtained are in average greater than those predicted in the absence of treatment and close to the normal target height.

Quantification of tipranavir in human plasma by high-performance liquid chromatography with UV detection.

A simple method for the quantification of tipranavir, a new non-peptidic protease-inhibitor, was developed. An internal standard, prazepam, was added to 100 microl of plasma before a liquid-liquid extraction by 3 ml of tert-butyl methyl ether. The extracts were evaporated to dryness and reconstituted with 100 microl of mobile phase before being injected in the chromatographic system.

[Intravenous immunoglobulins: therapeutic indications].

Intravenous immunoglobulins (IVIg) are preparations of normal human IgG obtained from large pools of healthy blood donors. IVIg can be used at low doses to treat patients with primary or secondary immune deficiencies and at high doses as an immunomodulatory agent in many autoimmune and systemic inflammatory diseases, especially hematologic and neurologic diseases. Its mechanisms of action are multiple, complex, and not yet well elucidated.

Intrachromosomal insertion mimicking a pericentric inversion: molecular cytogenetic characterization of a three break rearrangement of chromosome 20.

Intrachromosomal insertions are uncommon rearrangements, in which a chromosomal segment is intercalated into another part of the same chromosome. The insertion may occur in the same arm (paracentric) or in the other arm (pericentric). The cytogenetic recognition of these structurally rearranged chromosomes can be difficult, and intrachromosomal insertions can be easily mistaken for inversions.

Simple method to determine beta-lactam resistance phenotypes in Pseudomonas aeruginosa using the disc agar diffusion test.

Background: Pseudomonas aeruginosa is a major opportunistic bacterial pathogen in nosocomial infections because of the increasing prevalence of resistance to many of the commonly used antibiotics. To ensure optimal efficiency of antibiotic treatment against this species, antibiotic susceptibility tests must be interpreted with caution. Most microbiologists now consider it essential to characterize the antibiotic resistance expressed by isolates.

[Proteasome inhibitors].

Introduction: Proteasome is involved in cell cycle and apoptosis regulation. Its deregulation could participate to oncogenesis.

Exegesis: Bortezomib is the first proteasome inhibitor to reach clinical development.

[Post-exposure vaccination against hepatitis A].

The dramatic decrease in the incidence of hepatitis A in France renders routine vaccination unecessary. However, the main problem concerns prophylaxis for persons exposed to an index case, because nonspecific immunoglobulins are recommended but unavailable in France for this indication. The vaccination of persons who come into contact with an index case is possible in the days following exposure.

[Post-licensing surveillance of vaccines].

Pharmacoepidemiological studies are essential in the post-licensing surveillance of vaccines in order to evaluate the potential benefits and risks of vaccines used in common practice. Surveillance is required to detect rare or unanticipated vaccine adverse events and to ensure confidence in vaccination. Epidemiological studies provide data on the long-term protection conferred by vaccination, and the incidence and associated mortality of, and population susceptibility to, diseases preventable by vaccine.

[Medium and long-term evaluation of spongioplasty in hypospadias repair].

Introduction: Spongioplasty for the treatment of hypospadias consists of tubulization urethroplasty combined with dissection and midline approximation of the two divergent hemicorpora spongiosa. The objective of this study was to evaluate the medium-term and long-term cosmetic and functional results, and satisfaction of patients operated by this technique.

Material And Methods: Fifty spongiopasties were performed by the same surgeon between 1990 and 1996.