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Adult polycystic kidney disease in Hong Kong Chinese: an autopsy study
1Department of Pathology, University of Hong Kong.
Insights
Adult polycystic kidney disease (APKD) is less common in Hong Kong than in Western countries. This study found APKD in 0.295% of autopsies, with common complications including polycystic liver and end-stage renal disease (ESRD).
Area of Science:
- Nephrology
- Autopsy studies
- Epidemiology
Background:
- Adult polycystic kidney disease (APKD) is a genetic disorder.
- Understanding its prevalence and characteristics in diverse populations is crucial.
Purpose of the Study:
- To determine the frequency, clinical features, and prognosis of APKD in Hong Kong Chinese patients.
- To compare these findings with data from Western countries.
Main Methods:
- Retrospective autopsy study of 41 Chinese patients diagnosed with APKD.
- Analysis of autopsy records from 1964 to 1991.
- Life table analysis for survival estimation.
Main Results:
- APKD occurred in 0.295% of autopsies in Hong Kong, lower than Western frequencies.
- 46% of patients reached end-stage renal disease (ESRD).
- Polycystic liver (63%) and left ventricular hypertrophy (59%) were common; ruptured berry aneurysms caused death in some cases.
Conclusions:
- APKD in Hong Kong Chinese appears less frequent than in Western populations.
- Key complications include ESRD, polycystic liver, and cardiovascular issues.
- Survival probabilities decrease significantly with age, with no observed gender-based differences.
Abstract:
To appraise the frequency, clinical features and prognosis of adult polycystic kidney disease (APKD) in Hong Kong, 41 Chinese patients who had an autopsy diagnosis of APKD were studied retrospectively. The APKD patients constituted 0.295% of 13,890 (or one in 339) autopsies performed during the study period (1964-1991). The frequency of APKD in autopsied populations in Western countries varies from one in 261 to one in 1,019 with an average of one in 503. Nineteen (46%) patients reached end-stage renal disease (ESRD) at the time of death. Ruptured berry aneurysm caused death in 3 patients while 3 others had spontaneous intracerebral hemorrhage. Left ventricular hypertrophy was found in 24 (59%) patients. Polycystic liver was present in 26 (63%) patients. This frequency was higher than the 4% to 60% (average 47%) reported in similar studies conducted in Western countries. The probability of patients being alive and without ESRD, estimated using a life table analysis, was 83%, 54%, 32% and 12% by the age of 40, 50, 60 and 70 respectively. No difference in frequency, clinical features and survival between male and female APKD patients could be demonstrated.