Kidney transplantation from donors with Marfan syndrome: report of 2 cases and literature review
10.3969/j.issn.1674-7445.2023239
- VernacularTitle:马方综合征来源供肾肾移植2例并文献复习
- Author:
Meng ZHANG
1
;
Yibin WANG
1
;
Yuchen WANG
1
;
Rumin LIU
1
;
Ziyan YAN
1
;
Renfei XIA
1
;
Wenli ZENG
1
;
Jialiang HUI
1
;
Minjie ZHOU
1
;
Jian XU
1
;
Yun MIAO
1
Author Information
1. Department of Organ Transplantation, Nanfang Hospital of Southern Medical University, Guangzhou 510515, China.
- Publication Type:OriginalArticle
- Keywords:
Kidney transplantation;
Marfan syndrome;
Hereditary disease;
Arterial dissection;
Delayed graft function;
Fibrillin-1;
Biopsy;
Remuzzi score
- From:
Organ Transplantation
2024;15(2):257-262
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate the feasibility and clinical experience of kidney transplantation from donors with Marfan syndrome (MFS). Methods Clinical data of 2 recipients undergoing kidney transplantation from the same MFS patient were retrospectively analyzed and literature review of 2 cases was conducted. Characteristics and clinical diagnosis and treatment of kidney transplantation from MFS patients were summarized. Results The Remuzzi scores of the left and right donor kidneys of the MFS patient during time-zero biopsy were 1 and 2. No significant difference was observed in the renal arteriole wall compared with other donors of brain death and cardiac death. Two recipients who received kidney transplantation from the MFS patient suffered from postoperative delayed graft function. After short-term hemodialysis, the graft function of the recipients received the left and right kidney began to gradually recover at postoperative 10 d and 20 d. After discharge, serum creatinine level of the recipient received the left kidney was ranged from 80 to 90 μmol/L, whereas that of the recipient received the right kidney kept declining, and the lowest serum creatinine level was 232 μmol/L before the submission date (at postoperative 43 d). Through literature review, two cases successfully undergoing kidney transplantation from the same MFS donor were reported. Both two recipients experienced delayed graft function, and then renal function was restored to normal. Until the publication date, 1 recipient has survived for 6 years, and the other recipient died of de novo cerebrovascular disease at postoperative 2 years. Conclusions MFS patients may serve as an acceptable source of kidney donors. However, the willingness and general conditions of the recipients should be carefully evaluated before kidney transplantation. Intraoperatively, potential risk of tear of renal arterial media should be properly treated. Extensive attention should be paid to the incidence of postoperative complications.
