Membranous nephropathy in three paediatric patients

Detalhes bibliográficos
Autor(a) principal: Nobre,Susana
Data de Publicação: 2012
Outros Autores: Santos,Raquel, Gomes,Clara, Cunha,Fernanda X., Correia,António J.
Tipo de documento: Relatório
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692012000100007
Resumo: Membranous nephropathy in childhood is usually secondary to an underlying cause. Idiopathic membranous nephropathy is a rare cause of paediatric asymptomatic proteinuria/nephrotic syndrome. We reviewed the only three cases of membranous nephropathy followed in a paediatric hospital over the last ten years. All patients were female with mean age at diagnosis of 11.3 years (10-13 years). Patients 1 and 2 presented with steroid-resistant nephrotic syndrome. Patient 3 had a history of episodes of gross haematuria, and subnephrotic-range proteinuria refractory to enalapril and losartan. All patients had normal renal function. Patient 2 had hypertension and microscopic haematuria. Patients 1 and 2 underwent renal biopsy at an average of 51.5 days after initial symptoms; in patient 3, renal biopsy was performed seven years after initial presentation with episodes of gross haematuria, and five years after discovery of proteinuria. Histopathological features indicated membranous nephropathy; in patients 2 and 3, some findings suggested the coexistence of a systemic clinical condition. Secondary causes were sought in all patients. Six months after diagnosis, patient 1 developed facial skin lesions suggestive of discoid lupus erythematosus and later had positive autoantibodies (ANA, ENA, anti-SSA). Corticosteroids and angiotensin II receptor antagonists or angiotensin converting enzyme inhibitors were given to all patients. Those with nephritic syndrome required the addition of ciclosporin to achieve remission. At the latest evaluation (mean follow-up 31.3 months), patients 1 and 3 were in remission, and patient 2 had sub-nephrotic range proteinuria as the result of poor adherence to medication. All had normal renal function and blood pressure. All were receiving treatment with prednisolone and enalapril, and patients 1 and 2 were also receiving ciclosporin. Diagnosing idiopathic membranous nephropathy in children can be challenging. At least one of our patients had atypical features suggestive of an underlying cause so may have had secondary membranous nephropathy. Follow-up must continue, even after remission, with continued monitoring for underlying systemic disease. Treatment options are angiotensin converting enzyme inhibitors or angiotensin II receptor antagonists, corticosteroids and immunosuppressive drugs such as ciclosporin.
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spelling Membranous nephropathy in three paediatric patientsCiclosporinidiopathic membranous nephropathynephrotic syndromesteroidsMembranous nephropathy in childhood is usually secondary to an underlying cause. Idiopathic membranous nephropathy is a rare cause of paediatric asymptomatic proteinuria/nephrotic syndrome. We reviewed the only three cases of membranous nephropathy followed in a paediatric hospital over the last ten years. All patients were female with mean age at diagnosis of 11.3 years (10-13 years). Patients 1 and 2 presented with steroid-resistant nephrotic syndrome. Patient 3 had a history of episodes of gross haematuria, and subnephrotic-range proteinuria refractory to enalapril and losartan. All patients had normal renal function. Patient 2 had hypertension and microscopic haematuria. Patients 1 and 2 underwent renal biopsy at an average of 51.5 days after initial symptoms; in patient 3, renal biopsy was performed seven years after initial presentation with episodes of gross haematuria, and five years after discovery of proteinuria. Histopathological features indicated membranous nephropathy; in patients 2 and 3, some findings suggested the coexistence of a systemic clinical condition. Secondary causes were sought in all patients. Six months after diagnosis, patient 1 developed facial skin lesions suggestive of discoid lupus erythematosus and later had positive autoantibodies (ANA, ENA, anti-SSA). Corticosteroids and angiotensin II receptor antagonists or angiotensin converting enzyme inhibitors were given to all patients. Those with nephritic syndrome required the addition of ciclosporin to achieve remission. At the latest evaluation (mean follow-up 31.3 months), patients 1 and 3 were in remission, and patient 2 had sub-nephrotic range proteinuria as the result of poor adherence to medication. All had normal renal function and blood pressure. All were receiving treatment with prednisolone and enalapril, and patients 1 and 2 were also receiving ciclosporin. Diagnosing idiopathic membranous nephropathy in children can be challenging. At least one of our patients had atypical features suggestive of an underlying cause so may have had secondary membranous nephropathy. Follow-up must continue, even after remission, with continued monitoring for underlying systemic disease. Treatment options are angiotensin converting enzyme inhibitors or angiotensin II receptor antagonists, corticosteroids and immunosuppressive drugs such as ciclosporin.Sociedade Portuguesa de Nefrologia2012-01-01info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/reporttext/htmlhttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692012000100007Portuguese Journal of Nephrology & Hypertension v.26 n.1 2012reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPenghttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692012000100007Nobre,SusanaSantos,RaquelGomes,ClaraCunha,Fernanda X.Correia,António J.info:eu-repo/semantics/openAccess2024-02-06T17:04:38Zoai:scielo:S0872-01692012000100007Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-20T02:18:48.651604Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse
dc.title.none.fl_str_mv Membranous nephropathy in three paediatric patients
title Membranous nephropathy in three paediatric patients
spellingShingle Membranous nephropathy in three paediatric patients
Nobre,Susana
Ciclosporin
idiopathic membranous nephropathy
nephrotic syndrome
steroids
title_short Membranous nephropathy in three paediatric patients
title_full Membranous nephropathy in three paediatric patients
title_fullStr Membranous nephropathy in three paediatric patients
title_full_unstemmed Membranous nephropathy in three paediatric patients
title_sort Membranous nephropathy in three paediatric patients
author Nobre,Susana
author_facet Nobre,Susana
Santos,Raquel
Gomes,Clara
Cunha,Fernanda X.
Correia,António J.
author_role author
author2 Santos,Raquel
Gomes,Clara
Cunha,Fernanda X.
Correia,António J.
author2_role author
author
author
author
dc.contributor.author.fl_str_mv Nobre,Susana
Santos,Raquel
Gomes,Clara
Cunha,Fernanda X.
Correia,António J.
dc.subject.por.fl_str_mv Ciclosporin
idiopathic membranous nephropathy
nephrotic syndrome
steroids
topic Ciclosporin
idiopathic membranous nephropathy
nephrotic syndrome
steroids
description Membranous nephropathy in childhood is usually secondary to an underlying cause. Idiopathic membranous nephropathy is a rare cause of paediatric asymptomatic proteinuria/nephrotic syndrome. We reviewed the only three cases of membranous nephropathy followed in a paediatric hospital over the last ten years. All patients were female with mean age at diagnosis of 11.3 years (10-13 years). Patients 1 and 2 presented with steroid-resistant nephrotic syndrome. Patient 3 had a history of episodes of gross haematuria, and subnephrotic-range proteinuria refractory to enalapril and losartan. All patients had normal renal function. Patient 2 had hypertension and microscopic haematuria. Patients 1 and 2 underwent renal biopsy at an average of 51.5 days after initial symptoms; in patient 3, renal biopsy was performed seven years after initial presentation with episodes of gross haematuria, and five years after discovery of proteinuria. Histopathological features indicated membranous nephropathy; in patients 2 and 3, some findings suggested the coexistence of a systemic clinical condition. Secondary causes were sought in all patients. Six months after diagnosis, patient 1 developed facial skin lesions suggestive of discoid lupus erythematosus and later had positive autoantibodies (ANA, ENA, anti-SSA). Corticosteroids and angiotensin II receptor antagonists or angiotensin converting enzyme inhibitors were given to all patients. Those with nephritic syndrome required the addition of ciclosporin to achieve remission. At the latest evaluation (mean follow-up 31.3 months), patients 1 and 3 were in remission, and patient 2 had sub-nephrotic range proteinuria as the result of poor adherence to medication. All had normal renal function and blood pressure. All were receiving treatment with prednisolone and enalapril, and patients 1 and 2 were also receiving ciclosporin. Diagnosing idiopathic membranous nephropathy in children can be challenging. At least one of our patients had atypical features suggestive of an underlying cause so may have had secondary membranous nephropathy. Follow-up must continue, even after remission, with continued monitoring for underlying systemic disease. Treatment options are angiotensin converting enzyme inhibitors or angiotensin II receptor antagonists, corticosteroids and immunosuppressive drugs such as ciclosporin.
publishDate 2012
dc.date.none.fl_str_mv 2012-01-01
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dc.publisher.none.fl_str_mv Sociedade Portuguesa de Nefrologia
publisher.none.fl_str_mv Sociedade Portuguesa de Nefrologia
dc.source.none.fl_str_mv Portuguese Journal of Nephrology & Hypertension v.26 n.1 2012
reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
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repository.name.fl_str_mv Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
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