Risk Factor's Affecting long-term Outcome of Alport syndrome

Byun, Ji-Yoon; Baek, Seoung-Yon; Lee, Young-Mock; Kim, Ji-Hong; Lee, Jae Seung; Kim, Pyung-Kil; Hong, Soon-Won; Jeong, Hyeon-Joo; Kim, Soon-Il; Kim, Yu-Seun; Park, Ki-Il

J Korean Soc Pediatr Nephrol > Volume 5(2); 2001 > Article

J Korean Soc Pediatr Nephrol 2001;5(2): 164-175.

Alport 증후군의 예후와 관련된 위험요인 분석

변지윤, 백승연, 이영목, 김지홍, 이재승, 김병길, 홍순원, 정현주, 김순일, 김유선, 박기일

¹연세대학교 의과대학 소아과학교실, 신장질환연구소
²연세대학교 의과대학 소아과학교실, 신장질환연구소
³연세대학교 의과대학 소아과학교실, 신장질환연구소
⁴연세대학교 의과대학 소아과학교실, 신장질환연구소
⁵연세대학교 의과대학 소아과학교실, 신장질환연구소
⁶연세대학교 의과대학 소아과학교실, 신장질환연구소
⁷연세대학교 의과대학 병리학교실, 신장질환연구소
⁸연세대학교 의과대학 병리학교실, 신장질환연구소
⁹연세대학교 의과대학 외과학교실, 신장질환연구소
¹⁰연세대학교 의과대학 외과학교실, 신장질환연구소
¹¹연세대학교 의과대학 외과학교실, 신장질환연구소

Risk Factor's Affecting long-term Outcome of Alport syndrome

Ji-Yoon Byun, Seoung-Yon Baek, Young-Mock Lee, Ji-Hong Kim, Jae Seung Lee, Pyung-Kil Kim, Soon-Won Hong, Hyeon-Joo Jeong, Soon-Il Kim, Yu-Seun Kim, Ki-Il Park

¹Departments of Pediatrics, The Institute of Kidney Disease, Yonsei University College of Medicine
²Departments of Pediatrics, The Institute of Kidney Disease, Yonsei University College of Medicine
³Departments of Pediatrics, The Institute of Kidney Disease, Yonsei University College of Medicine
⁴Departments of Pediatrics, The Institute of Kidney Disease, Yonsei University College of Medicine
⁵Departments of Pediatrics, The Institute of Kidney Disease, Yonsei University College of Medicine
⁶Departments of Pediatrics, The Institute of Kidney Disease, Yonsei University College of Medicine
⁷Departments of Pathology, The Institute of Kidney Disease, Yonsei University College of Medicine
⁸Departments of Pathology, The Institute of Kidney Disease, Yonsei University College of Medicine
⁹Departments of Surgery, The Institute of Kidney Disease, Yonsei University College of Medicine
¹⁰Departments of Surgery, The Institute of Kidney Disease, Yonsei University College of Medicine
¹¹Departments of Surgery, The Institute of Kidney Disease, Yonsei University College of Medicine

ABSTRACT

Purpose : Alport syndrome is a hereditary nephrotic disease characterized by progressive nephrotic symptom, sensorineural hearing loss, ophthalmic abnormality, typical microscopic findings, and familial occurrence. In this study, we tried to find the risk factors related with its prognosis by taking a close observation on clinical symptoms of children with Alport syndrome reviewing retrospectively. Materials & methods : We chose children diagnosed as Alport syndrome in renal biopsy during 20 years(from 1980, Jan. until 1999, Dec.) who could receive follow up studies in tile department of pediatrics. They were divided into two groups by comparing renal function at the time of diagnosis and at current status. We compared several clinical aspects in them, and applied nonparametric test for statistical analysis.
Results : The sex ratio(male:female) of 24 children was 3:1. The most common clinical symptom presented at their first visit was gross hematuria. Among those 24 children, 11 cases($46%$) of progressing into chronic renal failure(Group II) were observed. Hypertension, proteinuria and edema were seen much frequently in group II. The level of serum protein, albumin, and creatinine clearance were decreased while BUN, creatinine were relatively increased. All the results were statistically significant. Conclusion Clinically significant risk factors related to prognosis in Alport syndrome were the presence of hypertension, edema, and proteinuria at the time of diagnosis. Also, the level of serum protein, albumin, BUN, creatinine, and glomerular filtration rate were proved to be important factors in predicting prognosis. We believe that studies on these possible risk factors would be of great help in treating and predicting prognosis of children suffering with Alport syndrome. (J Korean Soc Pediatr Nephrol 2001;5 : 164-75)