Partial C4 deficiency in two children with systemic lupus erythematosus
Systemic lupus erythematosus (SLE) is a rare disease in childhood. Here two cases with SLE are presented, both with C4 null alleles yielding a functional C4 deficiency. The first case, a 14-year-old girl with a C4A null allele only, had a mild disease course, whereas the second child, a seven...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Hacettepe University Institute of Child Health
1995-04-01
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| Series: | The Turkish Journal of Pediatrics |
| Online Access: | https://turkjpediatr.org/article/view/3551 |
| _version_ | 1826543601901895680 |
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| author | A Bakkaloğlu M Pascual J A Schifferli S Ozen N Beşbaş U Saatçi |
| author_facet | A Bakkaloğlu M Pascual J A Schifferli S Ozen N Beşbaş U Saatçi |
| author_sort | A Bakkaloğlu |
| collection | DOAJ |
| description |
Systemic lupus erythematosus (SLE) is a rare disease in childhood. Here two cases with SLE are presented, both with C4 null alleles yielding a functional C4 deficiency. The first case, a 14-year-old girl with a C4A null allele only, had a mild disease course, whereas the second child, a seven-year-old boy with both C4A0 and C4B0, had a more relentless course leading to death in five years. We conclude that complement activation by the classical pathway might be an essential mechanism that protects against the emergence of autoimmune or immune-complex diseases, and that the deficient state in our patients predisposed them to the early development of SLE.
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| first_indexed | 2025-03-14T04:48:40Z |
| format | Article |
| id | doaj.art-42f75c440dc64aa8ae7e4ba230d08b03 |
| institution | Directory Open Access Journal |
| issn | 0041-4301 2791-6421 |
| language | English |
| last_indexed | 2025-03-14T04:48:40Z |
| publishDate | 1995-04-01 |
| publisher | Hacettepe University Institute of Child Health |
| record_format | Article |
| series | The Turkish Journal of Pediatrics |
| spelling | doaj.art-42f75c440dc64aa8ae7e4ba230d08b032025-03-07T19:45:09ZengHacettepe University Institute of Child HealthThe Turkish Journal of Pediatrics0041-43012791-64211995-04-01372Partial C4 deficiency in two children with systemic lupus erythematosusA Bakkaloğlu0M PascualJ A SchifferliS OzenN BeşbaşU SaatçiDepartment of Pediatrics, Hacettepe University Faculty of Medicine, Ankara. Systemic lupus erythematosus (SLE) is a rare disease in childhood. Here two cases with SLE are presented, both with C4 null alleles yielding a functional C4 deficiency. The first case, a 14-year-old girl with a C4A null allele only, had a mild disease course, whereas the second child, a seven-year-old boy with both C4A0 and C4B0, had a more relentless course leading to death in five years. We conclude that complement activation by the classical pathway might be an essential mechanism that protects against the emergence of autoimmune or immune-complex diseases, and that the deficient state in our patients predisposed them to the early development of SLE. https://turkjpediatr.org/article/view/3551 |
| spellingShingle | A Bakkaloğlu M Pascual J A Schifferli S Ozen N Beşbaş U Saatçi Partial C4 deficiency in two children with systemic lupus erythematosus The Turkish Journal of Pediatrics |
| title | Partial C4 deficiency in two children with systemic lupus erythematosus |
| title_full | Partial C4 deficiency in two children with systemic lupus erythematosus |
| title_fullStr | Partial C4 deficiency in two children with systemic lupus erythematosus |
| title_full_unstemmed | Partial C4 deficiency in two children with systemic lupus erythematosus |
| title_short | Partial C4 deficiency in two children with systemic lupus erythematosus |
| title_sort | partial c4 deficiency in two children with systemic lupus erythematosus |
| url | https://turkjpediatr.org/article/view/3551 |
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