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Our objective was to compare the outcomes of dual kidney transplanataion (DKT) to single kidney transplantation (SKT) performed with grafts from expanded criteria donors (ECD) in recipients ≥65 years, focusing on surgical complications. All kidney transplantations (KT) performed between 2006 and 2014 in our institution were analysed. DKT was indicated according to the criteria of the French national Agence de la Biomedecine. Thirty-nine DKT and 155 SKT were included, with a median follow-up of 36 and 26.5 months, respectively. The rate of early surgical revisions was not significantly higher after DKT (23.1% vs 15.5% (P = 0.2593)) but more venous graft thromboses (12.8% vs 3.2% (P = 0.02)) were reported. The glomerular filtration rate (GFR) 24 months after KT was significantly higher after DKT (45.0 ± 16.3 vs 39.8 ± 13.8 ml/min/1.73m 2 ; P = 0.04) and allowed shorter waiting time without a significant increased risk of surgical revision, excepted for venous graft thrombosis, more frequent after DKT. Graft survivals were not significantly different and GFR was higher after DKT. DKT seems to remain an appropriate strategy to address the growing graft shortage in elderly patients., (© 2018 Steunstichting ESOT.)

The Xq28 duplication involving the MECP2 gene ( MECP2 duplication) has been mainly described in male patients with severe developmental delay (DD) associated with spasticity, stereotypic movements and recurrent infections. Nevertheless, only a few series have been published. We aimed to better describe the phenotype of this condition, with a focus on morphological and neurological features. Through a national collaborative study, we report a large French series of 59 affected males with interstitial MECP2 duplication. Most of the patients (93%) shared similar facial features, which evolved with age (midface hypoplasia, narrow and prominent nasal bridge, thick lower lip, large prominent ears), thick hair, livedo of the limbs, tapered fingers, small feet and vasomotor troubles. Early hypotonia and global DD were constant, with 21% of patients unable to walk. In patients able to stand, lower limbs weakness and spasticity led to a singular standing habitus: flexion of the knees, broad-based stance with pseudo-ataxic gait. Scoliosis was frequent (53%), such as divergent strabismus (76%) and hypermetropia (54%), stereotypic movements (89%), without obvious social withdrawal and decreased pain sensitivity (78%). Most of the patients did not develop expressive language, 35% saying few words. Epilepsy was frequent (59%), with a mean onset around 7.4 years of age, and often (62%) drug-resistant. Other medical issues were frequent: constipation (78%), and recurrent infections (89%), mainly lung. We delineate the clinical phenotype of MECP2 duplication syndrome in a large series of 59 males. Pulmonary hypertension appeared as a cause of early death in these patients, advocating its screening early in life., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Objective: To evaluate satisfaction rates and results in patients with penile prosthesis (5 PP)., Materials and Methods: From January 2000 till 2009, 50 patients with organic erectile dysfunction (ED) were implanted with hydraulics penile prosthesis by the same surgeon. Medical records were collected retrospectively. We analysed demographic characteristics, type of prosthesis used, per- and postoperative complications, prosthesis explantations and satisfaction rate measured by EDITS modified., Results: Mean age of patients was 60.2 ± 8.46 years (35-75). Thirty implanted patients (60%) were diabetics. We used a peno-scrotal incision in all patients. Three types of prosthesis were used; AMS Ambicor, AMS 700, Titan OTR. A bi-compartment and three-compartment prosthesis were implanted respectively in 24 and 26 patients. The prosthesis was retrieved in two patients secondary to a mechanical failure, in three patients for prosthesis erosion, in two patients for prosthesis infection. In four out of the seven explanted patients, a second prosthesis was implanted. The EDITS questionnary was realized by telephonic interview in 47 patients after a mean follow-up of 45 months (5-114). The response rate was of 83%. The satisfaction rate was of 81%., Conclusion: In our series, the satisfaction rate was better for the three-compartment prosthesis. After 45 months of follow-up, 73% (31/42) of patients had a functional prosthesis. The non-satisfaction most frequently found were patients who have complications as sepsis; mechanical failure, and prosthesis erosion, and subjective feeling of penile length reduction., (Copyright © 2013. Published by Elsevier Masson SAS.)

Objective: To determine the frequency and types of renal malformations, and to evaluate renal function in a cohort of patients with Kabuki syndrome (KS)., Study Design: Renal ultrasound scans and plasma creatinine measurements were collected from a French cohort of 94 patients with genotyped KS. Renal function was evaluated based on the estimated glomerular filtration rate. A genotype-phenotype study was conducted for renal and urinary tract malformations., Results: Renal malformations were present in 22% of cases, and urinary tract anomalies were present in 15%. Renal malformations were observed in 28% of the MLL2 mutation-positive group and in 0% of the MLL2 mutation-negative group (P = .015). No correlation was found between the presence or absence of renal or urinary tract malformations and the location or type of MLL2 mutation. Renal function was normal except for 1 patient with a MLL2 mutation diagnosed in the first days of life and severe renal disease due to unilateral renal agenesia and controlateral severe hypoplasia that progressed to the terminal stage at age 2 years., Conclusion: Our study emphasizes the need for ultrasound and renal function screening in children diagnosed with KS., (Copyright © 2013 Mosby, Inc. All rights reserved.)

Objective: To analyze the main clinical features, genetic mutations, and outcomes of patients of the French Congenital Central Hypoventilation Syndrome (CCHS) Registry., Design: A country-wide cohort established throughout a long-term multicenter effort., Patients: Seventy French patients with CCHS (29 male patients and 41 female patients)., Methods: The following items were analyzed: the most important moments of the disease course; the main clinical characteristics; associated pathologic conditions; management; clinical outcome; and genetic mutations., Results: An average of four new cases of CCHS per year was observed in the last 5 years. Thus, the incidence may be estimated to be 1 per 200,000 live births in France. The median age at diagnosis was 3.5 months (range, 0.5 to 15 months) before 1995 and < 2 weeks in the last 5 years (p = 0.01). CCHS occurred in isolation in 58 of 70 patients. In the remainder, it was associated with Hirschsprung disease (HSCR) [nine patients], Hirschsprung and neural crest tumor (two patients), and growth hormone deficiency (one patient). Among the 50 patients who lived beyond 1 year of age, all but one received nighttime ventilation, with 10 of them (20%) receiving it noninvasively. Three patients (6%) required daytime ventilatory support in addition to nighttime ventilation. The overall mortality rate was 38% (95% confidence interval [CI], 27 to 49%). The median age at death was 3 months (range, 0.4 months to 21 years). The 2-year mortality rate was greater in male patients than in female patients (p = 0.02; relative risk [RR], 2.71; 95% CI, 1.14 to 6.47) but was not affected by HSCR (p = 0.93; RR, 0.95; 95% CI, 0.28 to 3.2). The 43 patients who are currently alive (11 men; sex ratio, 0.4) have a mean age of 9 years (range, 2 months to 27 years). Among the 34 patients tested thus far, heterozygous mutations of the paired-like homeobox gene 2B (PHOX2B) gene were found in 31 patients (91%)., Conclusion: Our four major findings are the extreme rarity of CCHS, the improved recognition over time, the lack of effect of HSCR on the mortality rate, and the high frequency of PHOX2B mutations.

The present study is an attempt to outline the views held with the passage of time on the pathology of prostatic hypertrophy and upon the numerous palliative operations that have been used. As a natural limit, the author close to end the investigation at the beginning of the 20th century, when the principles of radical therapy were defined and accepted. It is demonstrated that a similar limit applies to the question of pathology, as the role of periurethral glands the ffue seat of prostatic hypertrophy was not definitely worked until the studies of Albarran (1900), Motz and Pérearnau (1905).

A randomized, double-blind, multicenter trial was performed comparing the association of orchiectomy plus the nonsteroid antiandrogen Anandron (300 mg daily) to orchiectomy plus placebo in the treatment of patients with stage C or D prostate cancer. The results for 98 evaluable stage D patients with a median follow-up of 23.4 +/- 8.9 months are given. Although there was no statistically significant difference between the two treatments with regard to any parameter (subjective response, best objective response according to NPCP criteria, and progression-free interval and survival), the results were invariably in favor of the combined treatment, as already reported in other trials on Anandron.