title: [Prevention and clinical course of polycystic kidney disease in adults. Examination and case report of 134 patients].
abstract: Adult polycystic kidney disease is inherited in an autosomal dominant fashion and it ranks third as the cause of end-stage renal disease, with 9% of patients in dialysis treatment. It is possible to achieve an effective primary or secondary preventive action because of its clinical, evolutive and hereditary features. We studied 134 patients with different degrees of renal function, considering the beginning and the evolution of the disease. The collected data were compared with other reports. Furthermore the finding of cysts in organs other than the kidney, the coexistence of intracranial aneurysms, high blood pressure and pregnancy were examined. We report a study carried out on 17 families of patients in hemodialysis because of MPRA, which included 118 subjects. We were only able to examine 40 adults (34%) completely and among these five patients were discovered to have MPRA. For 29 of the subjects (41%) didn't complete the examinations or refused further investigations. The contemporary presence of suggestive family history and data obtained by urography, nephrotomography, nephrosonography and the study of renal function permit a reliable diagnosis. The incidence of the disease could only be reduced by a constant prevention, but there are considerable difficulties caused by the little attention given to the disease by the medical class and people in general.
mesh_terms: D000293:Adolescent; D000328:Adult; D005260:Female; D006801:Humans; D006973:Hypertension / Q000150:complications; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000628:therapy; D007677:Kidney Function Tests; D008297:Male; D008875:Middle Aged; D007690:Polycystic Kidney Diseases / Q000209:etiology / Q000235:genetics / Q000517:prevention & control*; D011247:Pregnancy; D011248:Pregnancy Complications; D006435:Renal Dialysis
vernacular_title: Prevenzione e clinica della malatia policistica del rene dell'aduto (MPRA). Esame di una casistica di 134 pazienti.