Journal of the Formosan Medical Association
Volume 105, Issue 9 , Pages 770-774, 2006

Deficiency of the Seventh Component of Complement in a Taiwanese Boy

  • Yi-Chi Chiang

      Affiliations

    • Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan
    • Department of Pediatrics, Ho-Ping Branch, Taipei City Hospital, Taipei, Taiwan
    • ,
  • Shyh-Dar Shyur

      Affiliations

    • Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan
    • Corresponding Author InformationCorrespondence to: Dr Shyh-Dar Shyur, Department of Pediatrics, Mackay Memorial Hospital, 92 Chung-Shan North Road, Section 2, Taipei 104, Taiwan
    • ,
  • Li-Hsin Huang

      Affiliations

    • Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan
    • ,
  • Ta-Chzng Wen

      Affiliations

    • Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan
    • ,
  • Mao-Tsair Lin

      Affiliations

    • Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan
    • ,
  • Hwai-Chih Yang

      Affiliations

    • Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan
    • ,
  • Pei-Hsuan Liang

      Affiliations

    • Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan

Received 23 June 2005; received in revised form 12 August 2005; accepted 1 November 2005.

Article Outline

Inherited complement deficiencies are rare, particularly those associated with late components of the complement cascade. We report a 5-year-4-month-old Taiwanese boy with systemic meningococcal infection who had undetectable CH50 level of < 6 U/mL (normal, 32.6-39.8 U/mL). Levels of C3, C4, C5, C6 and C8 were normal, but C7 was undetectable (<5.8 mg/dL; reference, 55-85 mg/dL). The patient's sister was also C7-deficient (CH50 < 6 U/mL, C7 < 5.8 mg/dL). His father's CH50 was 25.9 U/mL and C7 was 27.8 mg/dL. His mother's CH50 was 31.2 U/mL and C7 was 22.7 mg/dL. His parents thus both had a partial complement deficiency, indicating an autosomal codominant inheritance pattern. Awareness of the possibility of late complement deficiency is important as they comprise a small percentage of patients who present with disseminated meningococcal disease or other serious infections caused by encapsulated organisms.

Key Words:  autosomal codominance , component deficiency , Neisseria meningitidis

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PII: S0929-6646(09)60207-2

doi:10.1016/S0929-6646(09)60207-2

Journal of the Formosan Medical Association
Volume 105, Issue 9 , Pages 770-774, 2006

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