Membranoproliferative Glomerulonephritis and X-Linked Agammaglobulinemia : An Uncommon Association
Joint Authors
Sameiro-Faria, Maria
Mota, Conceição
Sampaio, Rita
Lavrador, Vasco
Correia, Filipa
Cândido, Cristina
Marques, Laura
Source
Issue
Vol. 2014, Issue 2014 (31 Dec. 2014), pp.1-3, 3 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2014-03-04
Country of Publication
Egypt
No. of Pages
3
Main Subjects
Abstract EN
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.
American Psychological Association (APA)
Lavrador, Vasco& Correia, Filipa& Sampaio, Rita& Cândido, Cristina& Sameiro-Faria, Maria& Marques, Laura…[et al.]. 2014. Membranoproliferative Glomerulonephritis and X-Linked Agammaglobulinemia : An Uncommon Association. Case Reports in Pediatrics،Vol. 2014, no. 2014, pp.1-3.
https://search.emarefa.net/detail/BIM-474952
Modern Language Association (MLA)
Lavrador, Vasco…[et al.]. Membranoproliferative Glomerulonephritis and X-Linked Agammaglobulinemia : An Uncommon Association. Case Reports in Pediatrics No. 2014 (2014), pp.1-3.
https://search.emarefa.net/detail/BIM-474952
American Medical Association (AMA)
Lavrador, Vasco& Correia, Filipa& Sampaio, Rita& Cândido, Cristina& Sameiro-Faria, Maria& Marques, Laura…[et al.]. Membranoproliferative Glomerulonephritis and X-Linked Agammaglobulinemia : An Uncommon Association. Case Reports in Pediatrics. 2014. Vol. 2014, no. 2014, pp.1-3.
https://search.emarefa.net/detail/BIM-474952
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-474952