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一名患有肝脏和骨骼异常的患儿肝移植后出现短暂性高磷酸酶血症。

Transient hyperphosphatasemia following pediatric liver transplantation in a patient with hepatic and skeletal abnormalities.

作者信息

Murthy Vishakantha, Altawallbeh Ghaith, Larson-Nath Catherine, Karger Amy B, Thomas Stefani N

机构信息

Department of Laboratory Medicine and Pathology, School of Medicine, University of Minnesota, Minneapolis, MN, USA.

Department of Pediatrics, Division of Pediatric Gastroenterology, School of Medicine, University of Minnesota, Minneapolis, MN, USA.

出版信息

Clin Chim Acta. 2021 Aug;519:48-50. doi: 10.1016/j.cca.2021.03.030. Epub 2021 Apr 5.

Abstract

BACKGROUND

We report a unique case of transient hyperphosphatasemia in a pediatric patient with a history of hepatic and skeletal abnormalities.

PATIENT AND METHODS

A 2-month old male was diagnosed with progressive familial intrahepatic cholestasis type-2 and osteoporosis after marked increases in liver function tests were noted at 1 month of age. He underwent a second liver transplantation at 1 y. The increased liver function test trend resolved a few weeks post-transplantation. Four months after successful liver transplantation, unexplained significant increases in alkaline phosphatase (ALP) were observed, and they persisted for almost 9 months. Among the etiologies under consideration for the isolated increased ALP activity were viral infections and macro-ALP.

RESULTS

A persistent trend in abnormally increased ALP for 9 months was investigated leading to a confirmed diagnosis of transient hyperphosphatasemia (TH).

CONCLUSION

Pediatric post-liver transplant patients with skeletal and hepatic abnormalities including isolated markedly increased ALP activities represent a previously undescribed TH patient population. The 4.3% prevalence of TH in pediatric liver transplant recipients within our healthcare system is considerably higher than the previously reported prevalence of 2.1% for patients within the United States.

摘要

背景

我们报告了一例患有肝脏和骨骼异常病史的儿科患者出现短暂性高磷酸酶血症的独特病例。

患者与方法

一名2个月大的男性在1月龄时肝功能检查显著升高后被诊断为2型进行性家族性肝内胆汁淤积症和骨质疏松症。他在1岁时接受了第二次肝移植。移植后几周,肝功能检查升高的趋势得到缓解。肝移植成功4个月后,观察到碱性磷酸酶(ALP)出现不明原因的显著升高,并持续了近9个月。在考虑孤立性ALP活性升高的病因中包括病毒感染和巨ALP。

结果

对持续9个月的ALP异常升高趋势进行了调查,最终确诊为短暂性高磷酸酶血症(TH)。

结论

患有骨骼和肝脏异常(包括孤立性ALP活性显著升高)的儿科肝移植后患者代表了一个此前未被描述的TH患者群体。我们医疗系统中儿科肝移植受者中TH的患病率为4.3%,远高于美国此前报告的2.1%的患病率。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3b54/8532144/e5d920bff025/nihms-1748261-f0001.jpg

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