We analysed the 49 probands of autosomal dominant polycystic kidney disease (ADPKD) in Korea to elucidate clinical and genetic characteristics. 1) Family history of renal disease or ADPKD was taken from 44% of probands; hypertension 88%, cerebrovascular attack 64%, end stage renal disease 16%. 2) From the family screening with renal ultrasonography, we have confirmed dominant trait in 24 families. We found 2 families which have suspicious new mutations. 3) We performed linkage analysis of 15 families. The PKD-1 to non PKD-1 ratio was 13 : 2. 4) The male to femal ratio was 17 : 32 and age at diagnosis was 41 (24-65)years (mean (range)) in male, 45 (26-68) years in female. 5) The factors leading to the diagnosis of ADPKD were flank pain (23%), incidental finding (17%), palpable abdominal mass (11%), headache (9%) and gross hematuria (9%) 3) Hypertension (80%), azotemia (43%), flank pain (42%), renal calcification (42%), gross hematuria (33%) and cyst hemorrhage (25%) were renal complications. There were 2 renal cell carcinoma cases. 4)Liver cyst (82%) was the most common extrarenal manifestations. There were colonic diverticulosis (13%), cerebral artery aneurysm (7%), adrenal cyst (4%) and pancreatic cyst (2%). Results of our study revealed the clinical and genetic characteristics of ADPKD in Korea. We found only 44% of family history of renal disease, variable initial manifestations, variable renal and extrarenal complications. And we also found the similar percentage of PKD-2 (13%) to that (5-15%) of western countries, but it is necessary to study with more patients and families.
Aneurysm ; Azotemia ; Carcinoma, Renal Cell ; Cerebral Arteries ; Diagnosis ; Diverticulosis, Colonic ; Female ; Flank Pain ; Headache ; Hematuria ; Hemorrhage ; Humans ; Hypertension ; Incidental Findings ; Kidney Failure, Chronic ; Korea* ; Male ; Mass Screening ; Pancreatic Cyst ; Polycystic Kidney, Autosomal Dominant* ; Ultrasonography

Objectives: To evaluate undergraduate medical education in Korea, we aimed at the development of the students' satisfaction questionnaire. Also analysis of the factors affecting student satisfa ction was intended. Subjects: Total students of 33 medical schools in whole country were included as a target population. However, finally 5,452 students from 25 schools participated in the survey. Methods: We developed the questionnaire, asking student satisfaction for six categories of medical education in terms of educational input and process; educator, facility, educational environments. Each school administered questionnaire to their students, and responses with the angle of the distribution of the satisfaction and the factors affecting the satisfaction were analysed by the authors. Results: Overall satisfaction of students was not so high. The older the school, and the larger the students number, the higher the satisfaction level of the students. Students who live in the dormitory were more satisfied with medical education than those live alone. There was no significant correlation between satisfaction level and individual factors of the students such as sex, academic performance level, etc. Conclusion:It is necessary to develop continuous program that evaluate and improve the quality of medical education. Among them measurement in student satisfaction may be one of the useful.
Education, Medical* ; Education, Medical, Undergraduate ; Health Services Needs and Demand ; Humans ; Korea ; Schools, Medical ; Surveys and Questionnaires