Liver transplantation for progressive familial intrahepatic cholestasis: Clinical and histopathological findings, outcome and impact on growth
Liver transplantation for progressive familial intrahepatic cholestasis: Clinical and histopathological findings, outcome and impact on growth
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DOI：
10.1111/j.1399-3046.2007.00722.x
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发表时间：
2007-09-01
影响因子：
1.3
通讯作者：
Kilic, Murat
中科院分区：
文献类型：
--
作者：
Aydogdu, Sema;Cakir, Murat;Kilic, Murat
在这项研究中，我们分析了进行性家族性肝内胆汁淤积症患者接受肝移植的人口统计学特征，临床和组织病理学结果。我们还分析了肝移植对生长和骨矿物质含量的影响。大多数患者主要在前六个月内出现黄疸。在初次入院时，8名患者身材矮小（身高SD评分< 2），4名患者体重SD评分< 2。肝移植在43.2 +/- 27个月（范围9至96个月）时进行，在首次入院后6.5 +/- 3.5个月后进行。感染、手术并发症和渗透性腹泻合并严重代谢性酸中毒的发生率分别为41.4%、16.6%和33.3%。1例患者发生移植后淋巴组织增生性疾病。总体而言，1年移植物和患者生存率分别为69.2%和75%。在第1年结束时，仅2例患者的身高SD评分< 2。身高增加对移植时和肝移植后1年测量的全身BMD增加的线性回归系数r = 0.588（p = 0.074）。身高增长与移植时的年龄和PELD评分之间没有相关性，性别和供体类型之间也没有差异。肝移植是一种有效的治疗方法，具有良好的结果和低发病率，并增加与肝硬化相关的PFIC患者的生长加速。
In this study, we analyze the demographic features, clinical and histopathologlical findings in patients who underwent liver transplantation for progressive familial intrahepatic cholestasis. We also analyze outcome and impact of liver transplantation on growth and bone mineral content. Most of the patients were presented with jaundice mainly beginning within the first six months. At the time of initial admission; eight patients had short stature {height SD score < 2), and four patients had weight SD score < 2. Liver transplantation were performed at the age of 43.2 +/- 27 months (range 9 to 96 months), 6.5 +/- 3.5 months later after the first admission. Infection, surgical complications and osmotic diarrhea associated with severe metabolic acidosis were noted in 41.4%, 16.6% and 33.3%, respectively. One patient developed posttransplant lymphoproliferative disorder. Overall; 1 year graft and patient survival was 69.2% and 75%, respectively. At the end of the 1st year only 2 patients had height SD score < 2. Linear regression of height gain against increase in total body BMD measured at the time of transplantation and 1 year after liver transplantation gave a coefficient r = 0.588 (p = 0.074). No correlation was found between the height gain and age and PELD score at time of transplantation, and no difference was noted between the sexes and donor type. Liver transplantation is effective treatment modality with good outcome and little morbidity, and increases the growth acceleration in patients with PFIC associated with cirrhosis.