Ocular manifestations in liver transplant recipients with familial amyloid polyneuropathy
Ocular manifestations in liver transplant recipients with familial amyloid polyneuropathy
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DOI：
10.1111/j.1600-0420.2007.01098.x
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发表时间：
2008-08-01
影响因子：
3.4
通讯作者：
Suhr, Ole B.
中科院分区：
文献类型：
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作者：
Sandgren, Ola;Kjellgren, Daniel;Suhr, Ole B.
目的：评估瑞典肝移植（LT）受体家族性淀粉样多发性神经病（FAP）术后眼部受累情况：48例LT受体进行常规眼科检查，特别注意眼前段和玻璃体中的淀粉样蛋白沉积。医疗记录进行了仔细检查的时候，LT的神经功能缺损的信息。诊断是安全的，在所有情况下，通过检查活检标本中的淀粉样蛋白沉积和淀粉样蛋白生成转甲状腺素蛋白（ATTR）Val 30 Met mutation.Results阳性基因检测：6例患者（12.5%）开发玻璃体混浊后LT观察期内。第一次出现混浊是在移植后40个月，也就是全身性疾病发生后8年。4名患者（8%）发生继发性青光眼，第一例发生在手术后18个月和发病后6.5年。16名患者（33%）在透镜前表面出现沉积物。在10例患者（21%）中发现了扇形瞳孔边缘。结论：眼部并发症的患病率随着LT后时间的推移而增加，定期随访是必要的，特别是要揭示青光眼的发展-一种具有隐匿症状的并发症，患者通常不知道。
Purpose: To evaluate postoperative ocular involvement in Swedish liver transplant (LT) recipients with familial amyloid polyneuropathy (FAP).Methods: Routine ophthalmological examinations were performed in 48 LT recipients, with particular attention given to amyloid deposition in the anterior segment and the vitreous body. Medical records were scrutinized for information regarding neurological impairment at the time of the LT. The diagnosis was secured in all cases by examining for amyloid deposits in biopsy specimens and positive genetic testing for amyloidogenic transthyretin (ATTR) Val30Met mutation.Results: Six patients (12.5%) developed vitreous opacities within the post-LT observation period. The first opacities were seen 40 months after transplantation, 8 years after the onset of systemic disease. Four patients (8%) developed secondary glaucoma, the first of which was observed 18 months after the procedure and 6.5 years after the onset of disease. Sixteen patients (33%) developed deposits on the anterior surface of the lens. Scalloped pupillary margins were noted in 10 patients (21%).Conclusion: The prevalence of eye complications increases with time after LT and regular follow-up is necessary, especially to disclose the development of glaucoma - a complication with insidious symptoms of which patients are normally unaware.