INTRODUCTION. Adult polycystic kidney disease (APKD) is the most common
hereditary kidney disease in humans. The course of the disease is accompanied
by numerous complications. OBJECTIVE The aim was to assess the prevalence,
clinical course and outcome of adult dominant polycystic kidney disease
(ADPKD) patients on renal replacement therapy. METHOD. Medical data on 700
haemodialyzed (HD) and 500 transplanted patients treated in 10 Serbian
centres from 1996 to 2000 were retrospectively analyzed. While ADPKD patients
accounted for 13% of HD patients in Serbia in 2000, in the period between
1996 and 2000, the percent of patients with ADPKD in the population of
patients starting HD in 8 examined centres changed from 13.5% to 6.9%.
RESULTS. The total number of 180 ADPKD patients on HD was analyzed (96 males;
aged 55 years at HD onset). Their HD lasted between 1 and 22 years: males
started HD 1.3 years earlier and spent on HD 1.1 years less than females. In
53% of HD patients one or more family members had ADPKD but the cause of
death was unknown for many family members. Hypertension was present in 75% of
ADPKD patients, anaemia in 37% and other organ involvement (usually liver)
was found in 53 patients. Fifty patients experienced AV fistula thrombosis
and a vascular prosthesis had to be used in 9 of them. A hundred and two HD
patients died (aged between 38 and 78 years, on HD for 5.3 years). The causes
of death were stroke (19.6%), cardiovascular diseases (29%), infections,
while 5% of patients died with the picture of acute abdomen. Among 500
transplanted patients, there were 20 patients with ADPKD (11 males, ages
between 35 and 56 years at the time of transplantation) and 14 of them
received graft from cadaver donor. Uni- or bilateral nephrectomy was done in
4 patients in the pretransplant preparation, and in another two early after
transplantation due to urinary infection. Three patients restarted HD in the
first 3 months after transplantation due to acute tubular necrosis and 10
patients died 56.5 months after the transplantation. The known causes of
death were cardiovascular disease (3 patients), severe gastrointestinal
bleeding (2 patients), infection (2 patients) and cancer (2 patients).
CONCLUSION. The obtained results showed that it was possible to provide a
favourable outcome of patients with APBB on renal replacement therapy in
spite of numerous complications.
Swyer-James syndrome: A cause of adult-onset dyspnea in a patient with adult polycystic kidney disease
