Patients usually become symptomatic before the second year of life, with recurrent pyogenic and opportunistic infections such as P. carinii and Cryptosporidium.In this article, we reported three cases that have recurrent infections and reviewed the clinical features of this rare disease.
📌 3 Key Takeaways
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Objective & Hypothesis:The hyper IgM syndrome is a rare, inherited immune deficiency disorder and somatic.
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Key Findings & Data:The most common form of hyper IgM syndrome is of X-linked inheritance and caused by CD40 ligand gene mutations.
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Conclusion & Impact:Patients usually become symptomatic before the second year of life, with recurrent pyogenic and opportunistic infections such as P. carinii and Cryptosporidium.In this article, we reported three cases that have recurrent infections and reviewed the clinical features of this rare disease.
🔬Design:Review & Systematic Survey
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters
Abstract
The hyper IgM syndrome is a rare, inherited immune deficiency disorder and somatic. The most common form of hyper IgM syndrome is of X-linked inheritance and caused by CD40 ligand gene mutations. Patients usually become symptomatic before the second year of life, with recurrent pyogenic and opportunistic infections such as P. carinii and Cryptosporidium.In this article, we reported three cases that have recurrent infections and reviewed the clinical features of this rare disease.
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Cite This Article
USTA, M., AKDAĞ, A., ERGÜVEN, M., ÖZKAN, H., ÜÇEL, R. (2022). Hyper IgM syndrome. Göztepe Tıp Dergisi, Cilt: 20 - Sayı: 3, 183–184. https://ajindex.com/articles/526395
Ann O'Neill Shigeoka: X-linked immunodeficiency with hyper IgM. Medicine Journal 3(3):1-21, 2002
Delves PJ, Roitt IM: Advances in immunology: The immune sys- tem (second of two parts). N Engl Med 134; 343(2): 108-117, 2000
Levy J, Espanol-Boren T, Thomas C: Clinical spectrum of X-lin- ked Hyper IgM syndrome. J Pediatrics 131:47-54, 1997
Revy P, Muto T, Levy Y: Activation-induced cytidine deaminase (AID) deficiency causes the autosomal recessive form of the hyper- IgM syndrome(HIGM2). Cell 102(5):565-75, 2000 (Medline)
Ochs HD,WinkeIstein J: Disorders of the B-cellsystem.Immuno- logic disorders in infants and children 311-314, 1996
Gulino A, Virginia MD, Notarangelo: Hyper IgM syndromesr Current opinion in rheumatology 3:422-429, 2003
Ostenstad B, Giliani S, Mellbye OJ: A boy with HIM and NK cell deficiency. Clin Exp Immunol 107(2):230-4, 1997.