Biomedical subjects

Jean Philippe Jais

Publications and source records attributed to Jean Philippe Jais.

4 recordsLinked to original sources

PubMed2003

Comparison between bisection/morcellation and myometrial coring for reducing large uteri during vaginal hysterectomy or laparoscopically assisted vaginal hysterectomy: results of a randomized prospective study.

BACKGROUND: Vaginal hysterectomy (VH) is being performed increasingly by gynecologic surgeons. Thus, enlarged uteri are more frequently removed vaginally, requiring reducing techniques to allow vaginal extraction. MATERIAL AND METHODS: We designed a randomized prospective study to compare bisection-morcellation and myometrial coring. Patients scheduled for VH or laparoscopically assisted vaginal hysterectomy (LAVH) were offered entry into the study. Endometrial cancer was an exclusion criterion. Uterine size was not a contraindication for vaginal surgery. We compared data from preoperative workup as well as from the operative and the postoperative course. Data were recorded prospectively. Results were analyzed with nonparametric tests and logistic regression models. RESULTS: Thirty patients were included in the study. Patients were similar in both groups. No severe peroperative complication occurred in this series. Operating time was comparable in both groups. Uteri weighed more than 280 g in more than 70% of patients in both groups. Myometrial coring failed more often than bisection-morcellation (25% vs. 0%, p = 0.06). Patients and uteri characteristics had no influence on the risk of failure, except for narrow uteri, which were associated with an increased risk of failure in the myometrial coring group only (68.3 vs. 83.9 mm, p = 0.01). Postoperative courses were similar for the two techniques, except for an increased rate of fever in the myometrial coring group (28%, p = 0.03). CONCLUSION: Both techniques appeared safe in this trial. Myometrial coring failed more frequently than bisection-morcellation, especially in the case of a narrow uterus. Postoperative fever was significantly more common after myometrial coring. Both techniques should be taught to resident surgeons.

Adult↗

PubMed2003

X-linked Alport syndrome: natural history and genotype-phenotype correlations in girls and women belonging to 195 families: a "European Community Alport Syndrome Concerted Action" study.

Alport syndrome (AS) is a type IV collagen hereditary disease characterized by progressive hematuric nephritis, hearing loss, and ocular changes. Mutations in the COL4A5 collagen gene are responsible for the more common X-linked dominant form of the disease characterized by much less severe disease in girls and women. A "European Community Alport Syndrome Concerted Action" (ECASCA) group was established to delineate the Alport syndrome phenotype in each gender and to determine genotype-phenotype correlations in a large number of families. Data concerning 329 families, 250 of them with an X-linked transmission, were collected. Characteristics of heterozygous girls and women belonging to the 195 families with proven COL4A5 mutation are compared with those of hemizygous boys and men. Hematuria was observed in 95% of carriers and consistently absent in the others. Proteinuria, hearing loss, and ocular defects developed in 75%, 28%, and 15%, respectively. The probability of developing end-stage renal disease or deafness before the age of 40 yr was 12% and 10%, respectively, in girls and women versus 90 and 80%, respectively, in boys and men. The risk of progression to end-stage renal disease appears to increase after the age of 60 yr in women. Because of the absence of genotype-phenotype correlation and the large intrafamilial phenotypic heterogeneity, early prognosis of the disease in X-linked Alport syndrome carriers remains moot. Risk factors for developing renal failure have been identified: the occurrence and progressive increase in proteinuria, and the development of a hearing defect.

Adolescent↗

PubMed2003

A dynamic Web application within an n-tier architecture: a Multi-Source Information System for end-stage renal disease.

A Multi-Source Information System (MSIS) has been designed for the Renal Epidemiology and Information Network (REIN) dedicated to End-Stage Renal Disease. Interoperability has been considered at 4 levels: semantics, network, formats and contents. An n-tier architecture has been chosen at the network level. It is made out of a universal client, a dynamic Web server connected to a production database and to a data warehouse. The MSIS is patient-oriented, based on a regional organization. Its implementation in the context of a regional experimentation is presented with insights on the design and underlying technologies. The n-tier architecture is a robust model and flexible enough to aggregate multiple information sources and integrate modular developments. The data warehouse is dedicated to support health care decision-making.

Data Display↗

PubMed2000

X-linked Alport syndrome: natural history in 195 families and genotype- phenotype correlations in males.

Alport syndrome (AS) is a type IV collagen hereditary disease characterized by the association of progressive hematuric nephritis, hearing loss, and, frequently, ocular changes. Mutations in the COL4A5 collagen gene are responsible for the more common X-linked dominant form of the disease. Considerable allelic heterogeneity has been observed. A "European Community Alport Syndrome Concerted Action" has been established to delineate accurately the AS phenotype and to determine genotype-phenotype correlations in a large number of families. Data concerning 329 families, 250 of them with an X-linked transmission, were collected. Characteristics of the 401 male patients belonging to the 195 families with COL4A5 mutation are presented. All male patients were hematuric, and the rate of progression to end-stage renal failure and deafness was mutation-dependent. Large deletions, non-sense mutations, or small mutations changing the reading frame conferred to affected male patients a 90% probability of developing end-stage renal failure before 30 yr of age, whereas the same risk was of 50 and 70%, respectively, in patients with missense or splice site mutation. The risk of developing hearing loss before 30 yr of age was approximately 60% in patients with missense mutations, contrary to 90% for the other types of mutations. The natural history of X-linked AS and correlations with COL4A5 mutations have been established in a large cohort of male patients. These data could be used for further evaluation of therapeutic approaches.

Adult↗