TWO RELATED CASES OF PRIMARY COMPLEMENT DEFICIENCY

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FARHOUDI A.; BAZARGAN N.; MAHMOUDI M.; POURPAK Z.

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Paper Information

Journal: IRANIAN JOURNAL OF ALLERGY, ASTHMA AND IMMUNOLOGY Year:2003 | Volume:2 | Issue:2 Page(s): 69-74

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Title

TWO RELATED CASES OF PRIMARY COMPLEMENT DEFICIENCY

Author(s)

FARHOUDI A. | BAZARGAN N. | MAHMOUDI M. | POURPAK Z. | Issue Writer Certificate

Keywords

PRIMARY C3 DEFICIENCY

HEREDITARY ANGIOEDEMA

CL ESTERASE INHIBITOR

STANAZOL.

Abstract

Primary complement deficiencies are rare and two related patients are reported
here. The first patient is a 41- year- old man with eighteen episodes of pneumococcal
meningitis and other purulent infections. The serum C3 level was checked
at three separate times, showing that this was a primary C3 deficient case; other
immunological tests were however normal. This patient now takes prophylactic
antibiotics and the meningitis has not recurred, but he does have glomerulonephritis.
The second case is a 40 - year-old woman with repeated episodes of
orofacial and laryngeal edema and dyspnea. The serum C INH levels were 4.3 to
7 mg/dL which were very low compared with normal healthy subjects (C,INH
was 40-50 mg/dL in ten normal controls) and C4 was lower than normal but other
immunological tests were normal. Other causes of angioedema such as
lymphoproliferative disorders were excluded. She had hereditary angioedema without
a family background. The condition may be due to genetic mutation. The
angioedema was controlled with Danazol and Stanasol. As our patients are related,
this may suggest a genetic relationship between these two disorders.

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APA: Copy

FARHOUDI, A., BAZARGAN, N., MAHMOUDI, M., & POURPAK, Z.. (2003). TWO RELATED CASES OF PRIMARY COMPLEMENT DEFICIENCY. IRANIAN JOURNAL OF ALLERGY, ASTHMA AND IMMUNOLOGY (IJAAI), 2(2), 69-74. SID. https://sid.ir/paper/291071/en

Vancouver: Copy

FARHOUDI A., BAZARGAN N., MAHMOUDI M., POURPAK Z.. TWO RELATED CASES OF PRIMARY COMPLEMENT DEFICIENCY. IRANIAN JOURNAL OF ALLERGY, ASTHMA AND IMMUNOLOGY (IJAAI)[Internet]. 2003;2(2):69-74. Available from: https://sid.ir/paper/291071/en

IEEE: Copy

A. FARHOUDI, N. BAZARGAN, M. MAHMOUDI, and Z. POURPAK, “TWO RELATED CASES OF PRIMARY COMPLEMENT DEFICIENCY,” IRANIAN JOURNAL OF ALLERGY, ASTHMA AND IMMUNOLOGY (IJAAI), vol. 2, no. 2, pp. 69–74, 2003, [Online]. Available: https://sid.ir/paper/291071/en

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