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Childhood-onset dense deposit disease: a rare cause of proteinuria
K Taranta-Janusz1, A Wasilewska, B Szynaka
1Department of Pediatrics and Nephrology, Medical University of Białystok, Waszyngtona 17, 15-274, Białystok, Poland.
Insights
Dense deposit disease (DDD) in children can have a benign course, presenting with proteinuria but stable kidney function. Early detection through urine screening is crucial for timely management of this rare complement-related kidney disease.
Area of Science:
- Nephrology
- Complement System Biology
- Pediatric Renal Diseases
Background:
- Dense deposit disease (DDD) is a rare kidney disorder stemming from complement alternative pathway dysregulation.
- It involves C3 accumulation, often linked to C3 nephritic factor autoantibodies or Factor H mutations/autoantibodies.
- DDD is histologically defined by dense C3 deposits with minimal immunoglobulin.
Observation:
- A child with DDD presented with moderate proteinuria and no clinical symptoms, requiring no immunosuppressive therapy.
- Laboratory tests showed decreased serum C3 levels, normal renal function, and stable proteinuria throughout follow-up.
- Genetic analysis identified the patient carried a Factor H H402 risk allele.
Findings:
- The child experienced a benign clinical course of DDD, maintaining stable renal function.
- Proteinuria persisted but did not progress to nephrotic syndrome.
- The presence of the Factor H H402 risk allele may be associated with a milder DDD phenotype.
Implications:
- Routine urine screening in children can detect asymptomatic kidney diseases like DDD.
- Early diagnosis and close monitoring are vital for managing DDD.
- Understanding genetic factors like Factor H alleles can inform prognosis and treatment strategies for DDD.
Introduction:
Dense deposit disease (DDD) is a rare renal disease related to the dysregulation of the alternative pathway of the complement cascade, caused by several factors including the presence of an autoantibody to C3 nephritic factor, mutations in factor H and autoantibodies to this protein. DDD is characterized by C3 accumulation with absent or scanty immunoglobulin deposition.
Case Presentation:
Herein we report the case of a child with benign course of DDD, who presented with moderate proteinuria and lack of clinical symptoms without immunosuppressive treatment. Laboratory testing revealed moderate proteinuria, normal serum creatinine, total protein, and albumin levels, but significantly decreased serum C3 level. The results of renal biopsy were consistent with DDD. Genetic analysis revealed that the patient carried one copy of the H402 risk allele of factor H. The level of proteinuria did not change during the follow-up period and no nephrotic syndrome signs occurred. Renal function was stable.
Conclusion:
In conclusion, a program of urine screening for asymptomatic proteinuria and hematuria to detect children with kidney disease before they experience loss of kidney functions should be considered. Children diagnosed with DDD should have the opportunity to get treatment early on and to be followed very closely.
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