软骨毛发发育不全综合征：两例病例的免疫学评估
Cartilage-hair hypoplasia syndrome: immunological evaluation of two cases.
作者信息
Solé D, Leser P G, Soares D, Naspitz C K
机构信息
Department of Pediatrics, Escola Paulista de Medicina, São Paulo, Brasil.
出版信息
Rev Paul Med. 1993 Jan-Feb;111(1):314-9.
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.
目的
软骨毛发发育不全患者的免疫学评估。
类型
前瞻性和回顾性研究。
地点
过敏、临床免疫学和风湿病科 - 儿科学系 - “圣保罗医学学校”。
患者
两名软骨毛发发育不全综合征患儿。
方法
临床和免疫学评估。研究了体液免疫（免疫球蛋白水平、脊髓灰质炎病毒抗体等）和T细胞免疫（用PHA、Con A和PWN刺激体外培养的淋巴细胞、总T细胞和亚群测定）。
结果
一名患者观察到细胞免疫缺陷和低丙种球蛋白血症，另一名患者各项指标正常。
结论
所有软骨毛发发育不全患者均应进行免疫学评估（细胞免疫和体液免疫）。
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