软骨毛发发育不全综合征2例免疫学评价。

Revista paulista de medicina Pub Date : 1993-01-01

D Solé, P G Leser, D Soares, C K Naspitz

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引用次数: 0

摘要

目的:对软骨毛发育不全患者进行免疫学评价。类型:前瞻性和回顾性研究。地点:变态反应、临床免疫及风湿病科儿科的"Escola Paulista de Medicina"患者:2例患有软骨毛发发育不全综合征的儿童。方法:临床及免疫学评价。研究体液免疫(免疫球蛋白水平、脊髓灰质炎病毒抗体等)和T细胞免疫(PHA、Con A和PWN刺激的体外培养淋巴细胞，总T细胞和亚群测定)。结果:1例出现细胞免疫缺陷和低丙种球蛋白血症，1例正常。结论:所有软骨毛发育不全患者均应进行免疫学评估(细胞和体液)。

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Cartilage-hair hypoplasia syndrome: immunological evaluation of two cases.

Purpose: Immunological evaluation of patients with cartilage-hair hypoplasia. TYPE: Prospective and retrospective studies.

Place: Division of Allergy, Clinical Immunology and Rheumatology-Dept. of Pediatrics-"Escola Paulista de Medicina".

Patients: Two children with cartilage-hair hypoplasia syndrome.

Methods: Clinical and immunological evaluation. Humoral immunity (immunoglobulin levels, poliovirus antibodies, etc.) and T cell immunity (in vitro cultured lymphocytes stimulated with PHA, Con A and PWN, total T cell and subset determination) were studied.

Results: Cellular immunodeficiency and hypogammaglobulinemia were observed in one patient and normal values in the other.

Conclusions: Immunological evaluation (cellular and humoral) should be performed in all patients with cartilage-hair hypoplasia.

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