Frequent respiratory tract infections in the canine model of X-linked ectodermal dysplasia are not caused by an immune deficiency
Frequent respiratory tract infections in the canine model of X-linked ectodermal dysplasia are not caused by an immune deficiency
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DOI：
10.1016/j.vetimm.2005.04.005
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发表时间：
2005-08-15
影响因子：
1.8
通讯作者：
Felsburg, PJ
中科院分区：
文献类型：
--
作者：
Casal, ML;Mauldin, EA;Felsburg, PJ
与许多患有X连锁少汗性外胚层发育不良（XHED）的人类患者一样，XHED犬患肺部疾病的风险增加。由于频繁的感染和由于机会性呼吸道感染导致的意外死亡，先前在受影响的犬中怀疑局部免疫系统缺陷。设计实验以检查全身和局部体液和细胞反应、T细胞的发育和功能以及胸腺形态。这些实验中使用的所有犬在检查时均为临床健康，并将其免疫应答与正常同窝仔进行比较。正常犬和XHED犬的血清免疫球蛋白浓度略有不同，但均在正常范围内。XHED犬对破伤风类毒素疫苗接种反应适当，表明全身B和浆细胞功能正常。比较正常和受累犬的胸腺形态，并评估T细胞的功能。受影响犬血液和胸腺中T和B细胞的数量和表型均在正常范围内，表明T细胞发育正常。巨噬细胞和中性粒细胞的细胞毒性和吞噬能力也正常。与此相反，分泌型伊加浓度在受影响的狗显着高于正常狗，而泪液分泌物显着减少。这些结果表明，分泌型伊加的代偿机制，使总量等于正常犬。本研究中呈现的结果表明，XHED犬具有相对完整的免疫系统，并表明对于具有同源形式的XHED的人类也是如此。(C)2005 Elsevier B.V.保留所有权利。
As in many human patients with X-linked hypohidrotic ectodermal dysplasia (XHED), XHED dogs are at an increased risk for pulmonary disorders. Localized immune system defects had been suspected previously in affected dogs because of frequent infections and unexpected deaths due to opportunistic respiratory tract infections. Experiments were designed to examine systemic and localized humoral and cellular responses, development and function of T cells, and thymic morphology. All dogs used in these experiments were clinically healthy at the time of examination and their immune responses were compared to normal littermates. Serum immunoglobulin concentrations differed somewhat between normal dogs and dogs affected with XHED but they were all within normal ranges. The XHED dogs responded appropriately to vaccination with tetanus toxoid suggesting normal systemic B and plasma cell function. Thymic morphology was compared between normal and affected dogs and T cells were assessed for functionality. Numbers and phenotypes of T and B cells in blood and thymus of affected dogs were within normal limits suggesting normal development of T cells. Cytotoxic and phagocytic ability of macrophages and neutrophils was also normal in affected dogs. In contrast, the secretory IgA concentrations found in affected dogs were significantly higher than in normal dogs, while lacrimal secretions were significantly decreased. These results suggest a compensatory mechanism for secretory IgA, so that the total amount equals that in normal dogs. The results presented in this study indicate that the XHED dogs have a relatively intact immune system and suggest that the same is true for humans with the homologous form of XHED. (C) 2005 Elsevier B.V. All rights reserved.