Membranoproliferative Glomerulonephritis and X-Linked Agammaglobulinemia: An Uncommon Association
Introduction. X-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by agammaglobulinemia requiring replacement treatment with immunoglobulin. The association of XLA and membranoproliferative glomerulonephritis (MPGN) is unexpected and, to our knowledge, only one case was pre...
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doaj-4559b2392e934058825cd8c6c36d36ed2020-11-25T00:10:17ZengHindawi LimitedCase Reports in Pediatrics2090-68032090-68112014-01-01201410.1155/2014/480947480947Membranoproliferative Glomerulonephritis and X-Linked Agammaglobulinemia: An Uncommon AssociationVasco Lavrador0Filipa Correia1Rita Sampaio2Cristina Cândido3Maria Sameiro-Faria4Laura Marques5Conceição Mota6Pediatric Infectious Diseases and Immunodeficiencies Unit, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099-001 Porto, PortugalPediatric Infectious Diseases and Immunodeficiencies Unit, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099-001 Porto, PortugalDepartment of Pathology, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099-001 Porto, PortugalDepartment of Pediatrics, Centro Hospitalar de Trás-os-Montes e Alto Douro, Avenida Noruega, 5000-508 Vila Real, PortugalPediatric Nephrology Department, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099-001 Porto, PortugalPediatric Infectious Diseases and Immunodeficiencies Unit, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099-001 Porto, PortugalPediatric Nephrology Department, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099-001 Porto, PortugalIntroduction. X-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by agammaglobulinemia requiring replacement treatment with immunoglobulin. The association of XLA and membranoproliferative glomerulonephritis (MPGN) is unexpected and, to our knowledge, only one case was previously published. Case Report. The authors report the case of a 10-year-old boy with family history and prenatal diagnosis of XLA, treated from birth with intravenous immunoglobulin replacement therapy. He presented with pneumonia, macroscopic hematuria, nephrotic proteinuria, hypoalbuminemia, and hypercholesterolemia with normal renal function and serum complement levels. Renal histology showed immune complex mediated MPGN. He was started on high dose prednisolone and ramipril and switched to weekly subcutaneous immunoglobulin. After a 4-month treatment, hematuria and proteinuria significantly improved and prednisolone was gradually tapered without relapse. Conclusion. The pathogenic process underlying MPGN development in this patient is unknown but residual humoral immunity might play an important role. Thus, this case highlights the risk of autoimmune disorders among patients with XLA.http://dx.doi.org/10.1155/2014/480947 |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Vasco Lavrador Filipa Correia Rita Sampaio Cristina Cândido Maria Sameiro-Faria Laura Marques Conceição Mota |
spellingShingle |
Vasco Lavrador Filipa Correia Rita Sampaio Cristina Cândido Maria Sameiro-Faria Laura Marques Conceição Mota Membranoproliferative Glomerulonephritis and X-Linked Agammaglobulinemia: An Uncommon Association Case Reports in Pediatrics |
author_facet |
Vasco Lavrador Filipa Correia Rita Sampaio Cristina Cândido Maria Sameiro-Faria Laura Marques Conceição Mota |
author_sort |
Vasco Lavrador |
title |
Membranoproliferative Glomerulonephritis and X-Linked Agammaglobulinemia: An Uncommon Association |
title_short |
Membranoproliferative Glomerulonephritis and X-Linked Agammaglobulinemia: An Uncommon Association |
title_full |
Membranoproliferative Glomerulonephritis and X-Linked Agammaglobulinemia: An Uncommon Association |
title_fullStr |
Membranoproliferative Glomerulonephritis and X-Linked Agammaglobulinemia: An Uncommon Association |
title_full_unstemmed |
Membranoproliferative Glomerulonephritis and X-Linked Agammaglobulinemia: An Uncommon Association |
title_sort |
membranoproliferative glomerulonephritis and x-linked agammaglobulinemia: an uncommon association |
publisher |
Hindawi Limited |
series |
Case Reports in Pediatrics |
issn |
2090-6803 2090-6811 |
publishDate |
2014-01-01 |
description |
Introduction. X-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by agammaglobulinemia requiring replacement treatment with immunoglobulin. The association of XLA and membranoproliferative glomerulonephritis (MPGN) is unexpected and, to our knowledge, only one case was previously published. Case Report. The authors report the case of a 10-year-old boy with family history and prenatal diagnosis of XLA, treated from birth with intravenous immunoglobulin replacement therapy. He presented with pneumonia, macroscopic hematuria, nephrotic proteinuria, hypoalbuminemia, and hypercholesterolemia with normal renal function and serum complement levels. Renal histology showed immune complex mediated MPGN. He was started on high dose prednisolone and ramipril and switched to weekly subcutaneous immunoglobulin. After a 4-month treatment, hematuria and proteinuria significantly improved and prednisolone was gradually tapered without relapse. Conclusion. The pathogenic process underlying MPGN development in this patient is unknown but residual humoral immunity might play an important role. Thus, this case highlights the risk of autoimmune disorders among patients with XLA. |
url |
http://dx.doi.org/10.1155/2014/480947 |
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