Ammann A J, Duquesnoy R J, Good R A
Clin Exp Immunol. 1970 Apr;6(4):587-95.
Endocrinological and immunological studies were performed in five patients with ataxia-telangiectasia, two patients with sex-linked non-lymphopenic hypogammaglobulinaemia, one patient with sex-linked lymphopenic hypogammaglobulinaemia, and two patients with hypopituitary dwarfism. One of five patients with ataxia-telangiectasia had deficient growth hormone responses to insulin-induced hypoglycaemia but normal responses to arginine infusion. One pubertal ataxia-telangiectasia patient had increased gonadotropin secretion. Patients with hypopituitary dwarfism failed to respond to insulin-induced hypoglycaemia and arginine infusion with increased growth hormone secretion. The patients with sex-linked lymphopenic and non-lymphopenic hypogammaglobulinaemia had normal endocrine function. Immunological studies indicated defects in humoral and cellular immunity in the patients with ataxia-telangiectasia and sex-linked lymphopenic and non-lymphopenic hypogammaglobulinaemia, whereas normal immunological function was demonstrated in the patients with hypopituitary dwarfism. No relationship between endocrinological and immunological function could be demonstrated in these patients.
对5例共济失调毛细血管扩张症患者、2例X连锁非淋巴细胞减少性低丙种球蛋白血症患者、1例X连锁淋巴细胞减少性低丙种球蛋白血症患者和2例垂体功能减退性侏儒症患者进行了内分泌学和免疫学研究。5例共济失调毛细血管扩张症患者中有1例对胰岛素诱导的低血糖生长激素反应不足，但对精氨酸输注反应正常。1例青春期共济失调毛细血管扩张症患者促性腺激素分泌增加。垂体功能减退性侏儒症患者对胰岛素诱导的低血糖和精氨酸输注无生长激素分泌增加的反应。X连锁淋巴细胞减少性和非淋巴细胞减少性低丙种球蛋白血症患者内分泌功能正常。免疫学研究表明，共济失调毛细血管扩张症患者以及X连锁淋巴细胞减少性和非淋巴细胞减少性低丙种球蛋白血症患者存在体液免疫和细胞免疫缺陷，而垂体功能减退性侏儒症患者免疫功能正常。在这些患者中未发现内分泌功能与免疫功能之间存在关联。
