Pregnancy complicated by Caroli's disease with polycystic kidney disease: A case report and following observations
Pregnancy complicated by Caroli's disease with polycystic kidney disease: A case report and following observations
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DOI：
10.1111/j.1447-0756.2008.00891.x
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发表时间：
2008-08-01
影响因子：
1.6
通讯作者：
Kaneki, Shizuo
中科院分区：
文献类型：
--
作者：
Tsunoda, Mika;Ohba, Takashi;Kaneki, Shizuo
Caroli‘s病和Caroli’s综合征是罕见的先天性疾病，其特征是肝内胆管呈非梗阻性囊性扩张。这些疾病通常与常染色体隐性遗传性多囊肾病有关。一名怀孕11周的年轻女性被转诊至本院，以便在妊娠期妥善处理Caroli病。核磁共振成像和实验室测试显示，Caroli病合并多囊肾病引起的慢性肾功能衰竭。她接受了饮食控制、促红细胞生成素和预防性口服抗生素。她的怀孕过程平淡无奇，在怀孕37周时生下了孩子。此后，她的肾功能逐渐恶化。产后5年开始血液透析。虽然合并Caroli‘s病或Caroli’s综合征的妊娠过程多种多样，可能包括危及生命的情况，但如果仔细处理预防胆道和肾脏感染，结果是可以预期的。
Caroli's disease and Caroli's syndrome are rare congenital disorders characterized by non-obstructive cystic dilatation of the intrahepatic bile ducts. These disorders are often associated with autosomal recessive polycystic kidney disease. A young woman at 11 weeks of gestation was referred to our hospital for proper management of Caroli's disease during pregnancy. Magnetic resonance imaging and laboratory tests revealed Caroli's disease with chronic renal failure caused by polycystic kidney disease. She received diet control, erythropoietin and prophylactic oral antibiotics. Her pregnancy course was uneventful, and she gave birth at 37 weeks of gestation. Thereafter, her renal function gradually worsened. Hemodialysis was begun 5 years after parturition. Though the courses of pregnancies complicated by Caroli's disease or Caroli's syndrome are variable and can include life-threatening conditions, uneventful outcomes can be expected if careful management prevents biliary and renal infection.