{"title":"Cartilage-hair hypoplasia syndrome: immunological evaluation of two cases.","authors":"D Solé, P G Leser, D Soares, C K Naspitz","doi":"","DOIUrl":null,"url":null,"abstract":"<p><strong>Purpose: </strong>Immunological evaluation of patients with cartilage-hair hypoplasia. TYPE: Prospective and retrospective studies.</p><p><strong>Place: </strong>Division of Allergy, Clinical Immunology and Rheumatology-Dept. of Pediatrics-\"Escola Paulista de Medicina\".</p><p><strong>Patients: </strong>Two children with cartilage-hair hypoplasia syndrome.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>Cellular immunodeficiency and hypogammaglobulinemia were observed in one patient and normal values in the other.</p><p><strong>Conclusions: </strong>Immunological evaluation (cellular and humoral) should be performed in all patients with cartilage-hair hypoplasia.</p>","PeriodicalId":74720,"journal":{"name":"Revista paulista de medicina","volume":"111 1","pages":"314-9"},"PeriodicalIF":0.0000,"publicationDate":"1993-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Revista paulista de medicina","FirstCategoryId":"1085","ListUrlMain":"","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
Abstract
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: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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