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Farhoudi A, Bazargan N, Pourpak Z, et al. Two related cases of primary complement deficiencies. Iran J Allergy Asthma Immunol. 2003;2(2):69-74doi: 02.02/ijaai.6974.
Farhoudi, A., Bazargan, N., Pourpak, Z., & Mahmoudi, M. (2003). Two related cases of primary complement deficiencies. Iranian journal of allergy, asthma, and immunology, 2(2), 69-74. https://doi.org/02.02/ijaai.6974
Farhoudi, A, et al. "Two related cases of primary complement deficiencies." Iranian journal of allergy, asthma, and immunology vol. 2,2 (2003): 69-74. doi: https://doi.org/02.02/ijaai.6974
Farhoudi A, Bazargan N, Pourpak Z, Mahmoudi M. Two related cases of primary complement deficiencies. Iran J Allergy Asthma Immunol. 2003 Jun;2(2):69-74. doi: 02.02/ijaai.6974. PMID: 17301359.
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Iran J Allergy Asthma Immunol. 2003 Jun;2(2):69-74. doi: 02.02/ijaai.6974.

Two related cases of primary complement deficiencies.

Iranian journal of allergy, asthma, and immunology

A Farhoudi, Nasrin Bazargan, Zabra Pourpak, Maryam Mahmoudi

Affiliations

  1. Immunology, Asthma and Allergy Research Institute; Children Hospital Medical Center; Tehran University of Medical Sciences, Tehran, Iran.

PMID: 17301359 DOI: 02.02/ijaai.6974

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 C1INH levels were 4.3 to 7 mgldL which were very low compared with normal healthy subjects (C1INH was 40-50 mg/dL in ten normal controls) and C4 was lower than normal but other immunological tests were normal. Other causes of angioederna 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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