TY - JOUR A2 - 德语,梅兰妮AU - Haftu,汉莎AU - Mustefa,穆罕默德AU - Gebrehiwot,Teklu PY - 2020 DA - 2020年1月14日TI - 锌作为单药治疗,由于Wilson病的替代治疗方案为失代偿性肝病?SP - 1275940 VL - 2020 AB -
背景。Wilson病是涉及铜代谢一个罕见的代谢障碍,以及患者可能具有可变程度的肝,神经的,和精神病的表现呈现。在肝介绍的情况下,治疗通常与潜在毒性的铜螯合剂(d青霉胺或特伦顿)发起。尽管锌是低毒性,低成本用于治疗威尔逊病的,它已被限定于辅助作为单一药物保养或无症状的患者。在患了严重的肝脏疾病患者使用锌单药治疗没有很好的研究。在我们的病例报告中,我们描述谁与肝功能衰竭患者使用单药治疗锌的严重肝功能表现呈现儿科患者。
病例报告。来自埃塞俄比亚的一名15岁的男性患者呈现出与他的眼睛黄染,容易疲劳性泛发全身肿胀(水肿和腹水)。他有高胆红素血症,凝血功能障碍,低蛋白血症,和疯狂的肝酶。他有一个凯泽 - 弗莱舍环用肉眼,这是由裂隙灯检查证实可见。He had very low serum ceruloplasmin (<8 mg/L) and high 24-hour urine copper (150 mcg/dl). In accordance with the scoring system proposed by the 8th International Meeting on Wilson Disease and Menkes Disease, a diagnosis of Wilson disease was made. Zinc monotherapy with low copper diet was initiated for decompensated liver disease due to Wilson disease because of the inaccessibility of chelators (D-penicillamine or Trientine). After months of treatment with zinc, the patient experienced normalization of hepatic synthetic function and resolution of hypoalbuminemia and coagulopathy. The patient had also clinically stabilized (ascites, lower extremity swelling, edema, and jaundice were improved. Currently, the patient is on follow-up almost for the last four years in the gastrointestinal clinic.
结论。我们的案例表明,锌对改善肝功能的治疗潜力。虽然锌有其自身的副作用,它是在那些资源有限的重要,也许另一种治疗选择(不能访问螯合剂)。这个例子希望能鼓励未来的锌单一疗法的调查和研究用于治疗失代偿期症状肝豆状核变性。SN - 2090-6587 UR - https://doi.org/10.1155/2020/1275940 DO - 10.1155 /一百二十七万五千九百四十分之二千〇二十〇JF - 病例报告肝病PB - Hindawi出版KW - ER -