Study on establishment of an on－line registry of hereditary kidney diseases in children / 中华实用儿科临床杂志

ABSTRACT

Objective To explore the feasibility of establishing such an on_line registry of hereditary kidney diseases in Chinese children. Methods Selecting disease categories,designing input parameters,data quality and secu_rity are key factors of establishing an on_line registry of hereditary kidney diseases including general information,clini_cal data,relevant examinations,genetic testing,medication and follow_up. Results The first on_line,multi_cen_tered registry of children with hereditary kidney diseases in China was established using Java language and MySQL data_base. It contained 1 580 parameters and covered 6 major hereditary kidney diseases including Alport syndrome,protei_nuria related kidney disease,renal tubular disease,renal cystic disease,congenital anomalies of kidney and urinary tract and other hereditary kidney diseases. To date,a total of about 2 200 families from 32 tertiary hospitals have been regis_tered. About 648 families have well_documented follow_up records with a maximum follow_up of 13. 5 years. The registration system has data screening,export and simple statistical functions. The registry system had a clear interface, and was convenient and friendly to use. The input data could be real_time updated,and dedicated personnel was re_sponsible for data review and quality control to ensure security and reliability. Conclusions The on_line registry of children with hereditary kidney diseases not only facilitates standardized management of patients. Moreover,it provides a platform and a good foundation for the establishment and expansion of clinical research cohort.