Two related cases of primary complement deficiency.

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 normal. This patient now takes prophylactic antibiotics and the meningitis had 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 which is very low compared with normal healthy subjects (C1INH was 40-50 mg/dl) 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.

• Authors: A Farhoudi, N Bazargan, Z Pourpak, M Mahmoudi
• Journal: Immunological investigations (Immunol Invest) Vol. 32 Issue 4 Pg. 313-21 (Nov 2003) ISSN: 0882-0139 [Print] England
• PMID: 14603997 (Publication Type: Case Reports, Journal Article)

Chemical References

• Anti-Bacterial Agents
• Complement C3
• Stanozolol
• Danazol

Topics

• Adult
• Anti-Bacterial Agents (therapeutic use)
• Complement C3 (deficiency)
• Danazol (therapeutic use)
• Dyspnea (complications)
• Edema (complications, drug therapy)
• Female
• Genetic Diseases, Inborn (blood, complications, genetics)
• Glomerulonephritis (complications)
• Humans
• Male
• Meningitis, Pneumococcal (complications, drug therapy)
• Pedigree
• Stanozolol (therapeutic use)