Clinical features and outcomes of 98 children and adults with dense deposit disease

Authors
Der-Fa LuMikyung MoonLynne D. LanningAnn Marie McCarthyRichard J. H. Smith
Department
Dept. of Nursing (간호학)
Issue Date
2012
Citation
Pediatric Nephrology, Vol.27(5) : 773-781, 2012
ISSN
0931-041X
Abstract
Background Dense deposit disease (DDD) is an ultra-rare renal disease. Methods In the study reported here, 98 patients and their families participated in a descriptive patient-centered survey using an online research format. Reports were completed by patients (38%) or their parents (62%). Age at diagnosis ranged from 1.9 to 38.9 years (mean 14 years). Results The majority of patients presented with proteinuria and hematuria; 50% had hypertension and edema. Steroids were commonly prescribed, although their use was not evidence-based. One-half of the patients with DDD for 10 years progressed to end-stage renal disease (ESRD), with young females having the greatest risk for renal failure. Of first allografts, 45% failed within 5 years, most frequently due to recurrent disease (70%). Type 1 diabetes (T1D) was present in over 16% of families, which represents a 116-fold increase in incidence compared with the general population (p<0.001). Conclusions Based on these findings, we suggest that initiatives are needed to explore the high incidence of T1D in family members of DDD patients and the greater risk for progression to ESRD in young females with DDD. These efforts must be supported by sufficient numbers of patients to establish evidence-based practice guidelines for disease management. An international collaborative research survey should be implemented to encourage broad access and participation. Keywords: Dense deposit disease, MPGN type II, Patient Information Survey
Keywords
Dense deposit diseaseMPGN type IIPatient Information Survey
URI
http://kumel.medlib.dsmc.or.kr/handle/2015.oak/33655
Appears in Collections:
1. Journal Papers (연구논문) > 2. College of Nursing (간호대학) > Dept. of Nursing(간호학)
Keimyung Author(s)
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