Cerebral thrombotic microangiopathy and antiphospholipid antibodies in Aicardi-Goutieres syndrome--report of two sisters
| Autor: | S. J. Bakke, Frode L. Jahnsen, P. Lebon, M. Rasmussen, K. Skullerud |
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| Rok vydání: | 2005 |
| Předmět: |
Systemic disease
Thrombotic microangiopathy Autopsy Aicardi syndrome Basal Ganglia Diseases immune system diseases Medicine Humans Lupus Erythematosus Systemic skin and connective tissue diseases Autoimmune disease Lupus erythematosus business.industry Brain Infant General Medicine medicine.disease Antiphospholipid Syndrome Connective tissue disease Radiography Child Preschool Pediatrics Perinatology and Child Health Immunology Antibodies Antiphospholipid Aicardi–Goutières syndrome Female Neurology (clinical) Atrophy Intracranial Thrombosis business |
| Zdroj: | Neuropediatrics. 36(1) |
| ISSN: | 0174-304X |
| Popis: | Cerebral thrombotic microangiopathy was found at autopsy in one of two sisters with Aicardi-Goutieres syndrome, whereas the other revealed increased serum levels of anticardiolipin IgG antibodies (measured only in the living sister); both typical features of systemic lupus erythematosus. These findings add support to the suggestion that Aicardi-Goutieres syndrome and systemic lupus erythematosus are closely related disorders in which dysregulated production of interferon-alpha might play a crucial role. |
| Databáze: | OpenAIRE |
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