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Membranous nephropathy in childhood and its treatment
1Renal Unit, Clinical Science Laboratories, Guy's Hospital, London, UK.
Insights
Membranous nephropathy is rare in children but presents differently than in adults. Management involves searching for causes and considering corticosteroids or alkylating agents for progressive disease.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Internal Medicine
Background:
- Membranous nephropathy (MN) is typically a disease of middle-aged and elderly adults.
- It is an uncommon cause of proteinuria and nephrotic syndrome in children.
- Pediatric MN exhibits distinct clinical features compared to adult MN.
Purpose of the Study:
- To discuss the management of childhood membranous nephropathy.
- To highlight differences between pediatric and adult MN.
- To review treatment strategies for progressive pediatric MN.
Main Methods:
- Review of existing literature on pediatric and adult membranous nephropathy.
- Discussion of clinical presentation and management approaches.
- Consideration of treatment options based on disease progression.
Main Results:
- Childhood MN is associated with more secondary causes, frequent macroscopic hematuria, and relapsing courses.
- Renal venous thrombosis is absent in children, and progression to renal failure is exceptional.
- Treatment may involve corticosteroids, with alkylating agents considered for refractory cases.
Conclusions:
- An aggressive search for secondary causes is warranted in pediatric MN.
- Observation of proteinuria and renal function evolution is crucial.
- Optimal treatment regimens for pediatric and adolescent MN require further determination.
Abstract:
Membranous nephropathy is predominantly a disease of middle-aged and elderly individuals, and is thus rather an uncommon finding in proteinuric and nephrotic children. In children, it differs in several important respects from the disease as seen in adults: an apparent associated cause is more common, macroscopic haematuria is seen quite frequently, a relapsing course is more often noted, renal venous thrombosis is not found and evolution into renal failure is the exception. Nevertheless, a proportion of children with membranous nephropathy do evolve into renal failure, and their management is discussed with particular reference to recent papers on the treatment of membranous nephropathy in adults. An aggressive search for associated disease is worthwhile in children, and one should wait to see what the evolution or proteinuria and renal function may be. If a progressive course becomes evident, then a trial of treatment with corticosteroids is worthwhile, but if this is ineffective then a more aggressive approach involving the use of alkylating agents may be justified. It remains undetermined what the best regime in children and adolescents may be.