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

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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.

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Acknowledgments

The authors would like to acknowledge the generous support from the University of Iowa Information Technology Service, Research Service. Mr. Timothy VanFosson developed the online research survey platform. This work was supported in part by NIH grant DK074409 to RJHS.

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Authors and Affiliations

  1. University of Iowa College of Nursing, 426 Nursing Building, Iowa City, IA 52242, USA

    Der-Fa Lu, Mikyung Moon, Lynne D. Lanning & Ann Marie McCarthy

  2. Kidneeds DDD Research Foundation, Greater Cedar Rapids Community Foundation, 324 3rd St SE, Cedar Rapids, IA 52401-1841, USA

    Lynne D. Lanning

  3. Departments of Internal Medicine, Molecular Physiology & Biophysics, Pediatrics and Otolaryngology, University of Iowa, 200 Hawkins Drive-21151 A PFP, Iowa City, IA 52242, USA

    Richard J. H. Smith

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  1. Der-Fa Lu
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Correspondence to Der-Fa Lu.

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Lu, DF., Moon, M., Lanning, L.D. et al. Clinical features and outcomes of 98 children and adults with dense deposit disease. Pediatr Nephrol 27, 773–781 (2012). https://doi.org/10.1007/s00467-011-2059-7

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  • DOI: https://doi.org/10.1007/s00467-011-2059-7

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