IMMUNOREGULATORY ABNORMALITIES IN MUCOCUTANEOUS LYMPH-NODE SYNDROME
IMMUNOREGULATORY ABNORMALITIES IN MUCOCUTANEOUS LYMPH-NODE SYNDROME
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DOI：
10.1016/0090-1229(82)90075-7
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发表时间：
1982-01-01
期刊：
影响因子：
--
通讯作者：
GEHA, RS
中科院分区：
文献类型：
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作者：
LEUNG, DYM;SIEGEL, RL;GEHA, RS
对21例粘膜皮肤淋巴结综合征（MCLS）急性期儿童的免疫状态进行了评估，并与对照组（包括年龄匹配的正常儿童和患有急性发热性非细菌性疾病的儿童）进行了比较。与对照组相比，在MCLS急性期研究的18例患者中，有13例循环[单克隆抗体]T8阳性（T8+）抑制细胞/细胞毒性T细胞显著减少（P < 0.001），但[单克隆抗体]T3阳性（T3+）总T细胞和[单克隆抗体]T4阳性（T4+）辅助T细胞的百分比正常（P < 0.05）。3例患者中有3例携带ia的T细胞数量显著增加，提示存在循环活化的辅助细胞。18例急性MCLS患者中有17例的循环细胞自发分泌IgG （P < 0.01）和IgM (P < 0.001)，这是通过反向溶血斑块试验测定的。20例急性MCLS患者中有18例的单核细胞对51cr标记的正常人皮肤成纤维细胞的细胞毒性增加（P < 0.01）。在MCLS恢复期的随访研究表明，大多数患者的免疫异常逐渐消退。免疫调节异常可能导致该综合征的发病机制。
The immune status of 21 children in the acute phase of mucocutaneous lymph node syndrome (MCLS) was assessed and compared to that of control populations, consisting of age-matched normal children and of children suffering from acute febrile nonbacterial illnesses. In contrast to the controls, 13 of 18 patients studied during the acute phase of MCLS had a significant reduction in circulating [monoclonal antibody] T8-positive (T8+) suppressor/cytotoxic T cells (P < 0.001), but normal percentages of [monoclonal antibody] T3-positive (T3+) total T cells and of [monoclonal antibody] T4-positive (T4+) helper T cells (P > 0.05). Three of 3 patients had a significantly increased number of Ia-bearing T cells, suggesting the presence of circulating activated helper cells. Seventeen of 18 patients with acute MCLS had a significantly elevated number of circulating cells spontaneously secreting IgG (P < 0.01) and IgM (P < 0.001) as determined by a reverse hemolytic plaque assay. Mononuclear cells from 18 of 20 patients with acute MCLS had increased cytotoxicity against 51Cr-labeled normal human skin fibroblasts (P < 0.01). Follow-up studies during the convalescence phase of MCLS indicated that most of the patients had gradual resolution of their immunologic abnormalities. Immunoregulatory abnormalities may contribute to the pathogenesis of this syndrome.