RESUMEN
A case of a 22 years old woman with autosomal-recessive form of kidney polycystosis is presented. The diagnosis was made in early childhood. A combination of renal anomaly and hepatic fibrosis with manifestations of portal hypertension was present. No deviations from the other internal organs were found. At the age of 12 she entered into the stage of chronic renal failure. The last five years she is on dialysis treatment. She had survived several acute bleedings from esophageal varices. The authors are of the opinion that the case is of interest since patients with autosomal-recessive renal polycystosis very rarely reach majority.
Asunto(s)
Aberraciones Cromosómicas/diagnóstico , Genes Recesivos , Enfermedades Renales Poliquísticas/diagnóstico , Adulto , Aberraciones Cromosómicas/patología , Trastornos de los Cromosomas , Várices Esofágicas y Gástricas/diagnóstico , Várices Esofágicas y Gástricas/patología , Femenino , Fibrosis/patología , Hemorragia Gastrointestinal/diagnóstico , Hemorragia Gastrointestinal/patología , Humanos , Hipertensión Portal/diagnóstico , Hipertensión Portal/patología , Fallo Renal Crónico/diagnóstico , Fallo Renal Crónico/patología , Fallo Renal Crónico/terapia , Hígado/patología , Enfermedades Renales Poliquísticas/patología , Diálisis RenalRESUMEN
In patients with chronic renal failure the presence and frequency of acquired cystic changes in the kidneys (acquired cystic renal disease) were studied. 46 patients, 21 to 62 years of age and duration of hemodialysis treatment from 2 up to 126 months, were examined. The patients with polycystic kidneys were excluded. Cysts were found in 67.4% of the patients. They were classified into five groups. Between the duration of the hemodialysis treatment (the chronic renal failure respectively) and the development of the cystic renal disease correlation was found. The correlation between the length of the kidneys and the cystic changes is statistically significant. There is no correlation with the sex, age, basic disease, hematologic indices, diuresis and arterial pressure of the patients. In 50% of the patients examined splenomegaly was found the cause of which is not known.