Preemptive liver transplant in two patients with primary hyperoxaluria type 1: Clinical significance of nephrolithiasis and nephrocalcinosis

Preemptive liver transplant in two patients with primary hyperoxaluria type 1: Clinical significance of nephrolithiasis and nephrocalcinosis
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2例原发性高草酸尿症1型患者的先发性肝移植:肾结石和肾钙质沉着症的临床意义

DOI:
10.1111/petr.14380
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发表时间:
2022
影响因子:
1.3
通讯作者:
Kasahara Mureo
Kasahara Mureo
中科院分区:
医学4区
文献类型:
--
作者:
Uchida Hajime;Sakamoto Seisuke;Kodama Tasuku;Nakao Toshimasa;Yanagi Yusuke;Shimizu Seiichi;Fukuda Akinari;Sato Mai;Kamei Koichi;Kasahara Mureo

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背景尽管肾结石(NL)和肾钙质沉着症(NC)是原发性高草酸尿症1型(PH 1)的常见特征,NL和NC的长期预后后抢先肝移植(PLT)尚未阐明。材料和方法我们描述的情况下,两个慢性肾脏疾病(CKD)结果第1例患者于7岁时接受PLT治疗,估计肾小球滤过率(eGFR)47.8 ml/min/1.73 m2。两年后,她因尿石症发生了几次梗阻性肾盂肾炎,其中一次发生了败血性休克。在这些事件发生的同时,既存NL和NC逐渐改善,在移植后8年X线片上消失。她的肾功能保持在eGFR 58.7 ml/min/1.73 m2。第2例患者在10岁时接受PLT,eGFR为58.9 ml/min/1.73 m2。她的肾功能得到维持,eGFR为65.9 ml/min/1.73 m2。她有反复的尿路结石,开始出现在LT后3年。放射学检查结果仍然显示双侧NL和NC,但肾盂结石显示轻度improvement. ConclusionsNo的回归NC在X射线上看到的,长期的维持与PH 1与CKD 3期患者的肾功能可以实现与PLT。在NL患者中,当LT后发生梗阻性肾盂肾炎时,由于移植后免疫抑制治疗,存在严重并发症的风险。
BackgroundAlthough nephrolithiasis (NL) and nephrocalcinosis (NC) are very common features of primary hyperoxaluria type 1 (PH1), the long‐term prognosis of NL and NC after preemptive liver transplantation (PLT) has not been elucidated.Material and MethodsWe describe the cases of two chronic kidney disease (CKD) stage three patients with different clinical courses after PLT for PH1.ResultsThe first patient underwent PLT at 7 years of age with an estimated glomerular filtration rate (eGFR) of 47.8 ml/min/1.73 m2. Two years later, she experienced several episodes of obstructive pyelonephritis due to urolithiasis, and developed septic shock in one of these episodes. At the same time as these episodes, preexisting NL and NC progressively improved, with disappearance on X‐ray disappeared at 8 years after transplantation. Her renal function has been maintained with an eGFR of 58.7 ml/min/1.73 m2. The second patient received PLT at 10 years of age with an eGFR of 58.9 ml/min/1.73 m2. Her renal function has been maintained with an eGFR of 65.9 ml/min/1.73 m2. She had repeated urolithiasis which started to appear at 3 years after LT. The radiological findings still show bilateral NL and NC, but the stones in the renal pelvis have shown mild improvement.ConclusionsRegardless of the regression in NC seen on X‐ray, long‐term maintenance of the renal function in patients with PH1 with CKD stage 3 can be achieved with PLT. In patients with NL, there is a risk of serious complications due to posttransplant immunosuppressive therapy when obstructive pyelonephritis occurs after LT.
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