[Discordant evolution in monozygotic twins with familial nephropathy]. / Evolution discordante chez deux soeurs jumelles monozygotes atteintes de néphropathie familiale.

Two monozygotic twin sisters presented a familial nephropathy, discovered at 5 years of age. The clinical course was quite different for each patient: end-stage renal failure occurred at 5.5 years of age for one and at 14 years for the other. This difference raises the hypothesis of the role of environmental factors on the clinical expression of an inherited disease.

[Polycystic kidney and Bourneville's tuberous sclerosis. Apropos of 2 cases]. / Polykystose rénale et sclérose tubéreuse de Bourneville. A propos de 2 observations.

Abdominal tumor is discovered in a 4 months old boy and a 2 years old another. The paraclinical exploration leads to the diagnosis of polycystic disease. But the clinical course permits to precise that the renal lesions are those of tuberous sclerosis. Indeed the children exhibit adenoma sebaceum at ages of 4 and 7 years.

[The role of dialysis in the treatment of terminal renal insufficiency in children]. / Place de la dialyse dans le traitement de l'insuffisance rénale terminale de l'enfant.

In order to analyze the place of dialysis in a hemodialysis/transplantation program, the duration of each treatment modality, mortality rate and quality of inclusion in the social network were studied. Complications which arose during hemodialysis were evaluated by comparing the 1970's and the 1980's. Sixty children with terminal renal failure, aged 3 to 15 years, were entered in a hemodialysis/transplantation program between May 1971 and December 1988. Patients were followed up until December 1989. Among the 47 (78%) survivors at the end of the follow-up period, 25 had a functioning renal transplant and 22 were undergoing dialysis. Among the 13 deaths, 7 occurred during renal transplantation or immediately after loss of the transplant and 6 occurred under dialysis. Mean duration of treatment, including both dialysis and transplantation, was 7 years 11 months. Mean time spent under dialysis was 4 years 9 months. Time spent with a functioning transplant was 3 years 10 months for the 46 transplant recipients. Mean time spent on the transplant waiting list fell from 3 years 6 months before 1980 to 2 years after 1980. Virtually no cases of renal osteodystrophy, acute arterial hypertension or hepatitis B were seen after 1980 as a result of the use of higher-potency vitamin D derivatives, recent antihypertensive drugs including ACE inhibitors, and the Hevac B vaccine. Similarly, safety and patient comfort during dialysis improved substantially, as well as the quality of rehabilitation. Growth remained a significant problem although improvements can be expected to occur in the near future. Hemodialysis is an indispensable complement to transplantation.(ABSTRACT TRUNCATED AT 250 WORDS)