Effects of cyclosporine A in hyperzincaemia and hypercalprotectinaemia
Effects of cyclosporine A in hyperzincaemia and hypercalprotectinaemia
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DOI：
10.1080/08035250500482255
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发表时间：
2006-07-01
期刊：
影响因子：
3.8
通讯作者：
Togari, Hajime
中科院分区：
文献类型：
--
作者：
Sugiura, Tokio;Goto, Kenji;Togari, Hajime
简介：高锌血症和高钙卫蛋白血症伴全身性炎症、复发性感染、肝脾肿大、关节炎、贫血、皮肤炎症和发育不良是一种极其罕见的疾病，目前尚无治疗报告。目的：观察环孢素A对高锌血症和高钙卫蛋白血症患者治疗前后血清细胞因子水平的影响。研究方法：一名10岁女孩因坏疽性脓疡、肝脾肿大、对补铁无反应的贫血、持续性炎症、关节炎和血清锌升高而入院。血清钙卫蛋白水平极高;因此，我们诊断为高锌血症和高钙卫蛋白血症，并开始环孢素A治疗。分别于治疗前及治疗后1年测定血清12种细胞因子水平。结果：环孢素A疗效显著。她的皮肤病变和关节疼痛得到缓解，生活质量明显改善。C反应蛋白下降，贫血改善。虽然锌水平下降，但钙卫蛋白仍处于极高水平。检查的细胞因子，白细胞介素-6血清水平下降，白细胞介素-8治疗后表现出显着的减少。结论：环孢素A是有效的高锌血症和高钙卫蛋白血症。血清白细胞介素-8可用于评估环孢素A治疗高锌血症和高钙卫蛋白血症的疗效。
Introduction: Hyperzincaemia and hypercalprotectinaemia with systemic inflammation, recurrent infections, hepatosplenomegaly, arthritis, anemia, cutaneous inflammation, and failure to thrive is an extremely rare disease and no therapy is reported. Aim: To evaluated the effects of cyclosporine A in hyperzincaemia and hypercalprotectinaemia in terms of serum cytokine level changes before and after treatment. Methods: A 10-year-old girl was admitted suffering from pyoderma gangrenosum, hepatosplenomegaly, anemia that was unresponsive to iron supplementation, persistent inflammation, arthritis, and increased serum zinc. The level of serum calprotectin was extremely high; therefore, we diagnosed hyperzincaemia and hypercalprotectinaemia and started cyclosporine A treatment. Twelve cytokines in serum were measured before and one year after treatment. Results: Cyclosporine A was very effective. Her skin lesion and joint pain were alleviated and quality of life was markedly improved. C-reactive protein had decreased and anemia had improved. While zinc levels had fallen, calprotectin remained at an extremely high level. Of the cytokines examined, interleukin -6 serum levels had fallen and interleukin -8 showed a marked reduction after treatment.Conclusion: Cyclosporine A is effective for hyperzincaemia and hypercalprotectinaemia. Serum interleukin -8 may be useful in assessing the therapeutic effects of cyclosporine A in hyperzincaemia and hypercalprotectinaemia.