| Autor: |
Morse JH; Department of Medicine, Columbia University College of Physicians and Surgeons, New York, NY 10032., Barst RJ, Whitman HH 3rd, Fotino M, Jacobs JC |
| Jazyk: |
angličtina |
| Zdroj: |
The Journal of rheumatology [J Rheumatol] 1989 Dec; Vol. 16 (12), pp. 1536-41. |
| Abstrakt: |
We report a small kindred in which the father and daughter with positive antinuclear antibodies (ANA) (proband) had diffuse cutaneous and visceral scleroderma (systemic sclerosis, SSc), antitopoisomerase autoantibodies and shared the HLA-A23 C- B- DR5 DRw52 DQw3 haplotype. The ANA- granddaughter (daughter of the proband) was noted to have severe isolated pulmonary hypertension within the first 6 months of life, and had the other maternal HLA-A1 Cw8 B14 DR1 DQw1 haplotype, which included a B1, B2 duplication of the C4B allele. All 3 members shared DRw52. The possibility that neonatal pulmonary hypertension represents an isolated autoimmune disease or a hitherto undescribed neonatal syndrome is proposed and the immunogenetic autoantibody implications are discussed. |
| Databáze: |
MEDLINE |
| Externí odkaz: |
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