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小儿活体供肝肝移植术后的中央桥脑髓鞘溶解症:一例报告并文献复习

Central pontine myelinolysis following pediatric living donor liver transplantation: a case report and review of literature.

作者信息

Uchida Hajime, Sakamoto Seisuke, Sasaki Kengo, Hamano Ikumi, Shigeta Takanobu, Kanazawa Hiroyuki, Fukuda Akinari, Nosaka Shunsuke, Kubota Masaya, Kasahara Mureo

机构信息

Transplantation Center, National Center for Child Health and Development, Tokyo, Japan.

出版信息

Pediatr Transplant. 2014 Jun;18(4):E120-3. doi: 10.1111/petr.12255. Epub 2014 Apr 12.

Abstract

CPM is one of the most serious neurological complications that can occur after OLT and is characterized by symmetrical demyelinization in the basis pontis. The etiology of CPM remains unclear, although the rapid correction of the serum sodium and CNI concentrations may be associated with the development of CPM. With recent advances in MRI technology, early diagnosis of CPM has become possible. Here, we present the case of a five-yr-old female who developed CNI-associated CPM after undergoing LDLT. A decreased level of consciousness and dysphasia was noted one wk after LDLT, and MRI revealed findings compatible with a diagnosis of CPM. The patient fully recovered from the neurological deficits related to CPM following the switch from the CNI to sirolimus. We propose MRI to be promptly considered for patients with abnormal neurological findings, together with the substitution of CNI with an mTOR inhibitor as a management regimen for CNI-related CPM.

摘要

渗透性脱髓鞘综合征(CPM)是肝移植术后可能发生的最严重的神经并发症之一,其特征是脑桥基底部出现对称性脱髓鞘。尽管血清钠和钙调神经磷酸酶抑制剂(CNI)浓度的快速纠正可能与CPM的发生有关,但其病因仍不清楚。随着磁共振成像(MRI)技术的最新进展,CPM的早期诊断已成为可能。在此,我们报告一例5岁女性在接受活体肝移植(LDLT)后发生CNI相关CPM的病例。LDLT术后1周发现意识水平下降和言语困难,MRI显示的结果符合CPM的诊断。在从CNI转换为西罗莫司后,患者从与CPM相关的神经功能缺损中完全康复。我们建议,对于有异常神经学表现的患者应及时进行MRI检查,并将CNI替换为雷帕霉素靶蛋白(mTOR)抑制剂,作为CNI相关CPM的一种治疗方案。

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