Deficiency of the Seventh Component of Complement in a Taiwanese Boy
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,...
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| Format: | Article |
| Language: | English |
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Elsevier
2006-01-01
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| Series: | Journal of the Formosan Medical Association |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S0929664609602072 |
| _version_ | 1819182797937442816 |
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| author | Yi-Chi Chiang Shyh-Dar Shyur Li-Hsin Huang Ta-Chzng Wen Mao-Tsair Lin Hwai-Chih Yang Pei-Hsuan Liang |
| author_facet | Yi-Chi Chiang Shyh-Dar Shyur Li-Hsin Huang Ta-Chzng Wen Mao-Tsair Lin Hwai-Chih Yang Pei-Hsuan Liang |
| author_sort | Yi-Chi Chiang |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-22T22:51:51Z |
| format | Article |
| id | doaj.art-adaa98cfda6144bea708903e21805928 |
| institution | Directory Open Access Journal |
| issn | 0929-6646 |
| language | English |
| last_indexed | 2024-12-22T22:51:51Z |
| publishDate | 2006-01-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Journal of the Formosan Medical Association |
| spelling | doaj.art-adaa98cfda6144bea708903e218059282022-12-21T18:09:55ZengElsevierJournal of the Formosan Medical Association0929-66462006-01-01105977077410.1016/S0929-6646(09)60207-2Deficiency of the Seventh Component of Complement in a Taiwanese BoyYi-Chi Chiang0Shyh-Dar Shyur1Li-Hsin Huang2Ta-Chzng Wen3Mao-Tsair Lin4Hwai-Chih Yang5Pei-Hsuan Liang6Department of Pediatrics, Mackay Memorial Hospital, Taipei, TaiwanDepartment of Pediatrics, Mackay Memorial Hospital, Taipei, TaiwanDepartment of Pediatrics, Mackay Memorial Hospital, Taipei, TaiwanDepartment of Pediatrics, Mackay Memorial Hospital, Taipei, TaiwanDepartment of Pediatrics, Mackay Memorial Hospital, Taipei, TaiwanDepartment of Pediatrics, Mackay Memorial Hospital, Taipei, TaiwanDepartment of Pediatrics, Mackay Memorial Hospital, Taipei, TaiwanInherited 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.http://www.sciencedirect.com/science/article/pii/S0929664609602072autosomal codominancecomponent deficiencyNeisseria meningitidis |
| spellingShingle | Yi-Chi Chiang Shyh-Dar Shyur Li-Hsin Huang Ta-Chzng Wen Mao-Tsair Lin Hwai-Chih Yang Pei-Hsuan Liang Deficiency of the Seventh Component of Complement in a Taiwanese Boy Journal of the Formosan Medical Association autosomal codominance component deficiency Neisseria meningitidis |
| title | Deficiency of the Seventh Component of Complement in a Taiwanese Boy |
| title_full | Deficiency of the Seventh Component of Complement in a Taiwanese Boy |
| title_fullStr | Deficiency of the Seventh Component of Complement in a Taiwanese Boy |
| title_full_unstemmed | Deficiency of the Seventh Component of Complement in a Taiwanese Boy |
| title_short | Deficiency of the Seventh Component of Complement in a Taiwanese Boy |
| title_sort | deficiency of the seventh component of complement in a taiwanese boy |
| topic | autosomal codominance component deficiency Neisseria meningitidis |
| url | http://www.sciencedirect.com/science/article/pii/S0929664609602072 |
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