계명대학교 의학도서관 Repository

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

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Author(s)
Der-Fa LuMikyung MoonLynne D. LanningAnn Marie McCarthyRichard J. H. Smith
Keimyung Author(s)
Moon, Mi Kyung
Department
Dept. of Nursing (간호학)
Journal Title
Pediatric Nephrology
Issued Date
2012
Volume
27
Issue
5
Keyword
Dense deposit diseaseMPGN type IIPatient Information Survey
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
Keimyung Author(s)(Kor)
문미경
Publisher
College of Nursing
Citation
Der-Fa Lu et al. (2012). Clinical features and outcomes of 98 children and adults with dense deposit disease. Pediatric Nephrology, 27(5), 773–781. doi: 10.1007%2Fs00467-011-2059-7
Type
Article
ISSN
0931-041X
DOI
10.1007%2Fs00467-011-2059-7
URI
https://kumel.medlib.dsmc.or.kr/handle/2015.oak/33655
Appears in Collections:
2. College of Nursing (간호대학) > Dept. of Nursing (간호학)
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