Cohran Valeria C., Heubi James E.
Division of Pediatric Gastroenterology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229, USA.
Curr Treat Options Gastroenterol. 2003 Oct;6(5):403-415. doi: 10.1007/s11938-003-0043-4.
Cholestatic liver disease affects a small percentage of children, but therapy results in millions of healthcare dollars being spent each year. Close monitoring of nutritional status, pruritus, and complications from portal hypertension should improve the patient's quality of life and survival without liver transplantation. Other comorbid conditions, such as cardiac or renal disease, must also be integrated into the care plan and will affect the overall prognosis of the patient. Portal hypertension leads to ascites, variceal hemorrhage, and infection, which can result in significant mortality if not promptly recognized and treated. Surgical shunts are being used less because the expertise to perform endoscopic sclerotherapy and endoscopic band ligation is available at most medical centers. However, many cholestatic diseases, including biliary atresia, progressive familial intrahepatic cholestasis (PFIC) I, II, and III, and Alagille syndrome, may still require liver transplantation (Table 1). The cost of this procedure can exceed several hundred thousand dollars per patient, without including the annual costs of immunosuppressant therapy and medical monitoring. Meticulous medical management of nutrition and the sequelae of portal hypertension may prolong survival and reduce the potential morbidity and mortality of liver transplantation.
胆汁淤积性肝病影响一小部分儿童，但治疗每年花费数百万医疗费用。密切监测营养状况、瘙痒以及门静脉高压并发症，应能改善患者生活质量并提高其在不进行肝移植情况下的生存率。其他合并症，如心脏或肾脏疾病，也必须纳入护理计划，并会影响患者的总体预后。门静脉高压会导致腹水、静脉曲张出血和感染，如果不及时识别和治疗，可能会导致显著的死亡率。由于大多数医疗中心都具备进行内镜硬化治疗和内镜套扎术的专业知识，手术分流术的使用越来越少。然而，许多胆汁淤积性疾病，包括胆道闭锁、进行性家族性肝内胆汁淤积症（PFIC）I、II和III以及阿拉吉耶综合征，可能仍需要肝移植（表1）。该手术的费用可能超过每位患者数十万美元，这还不包括免疫抑制治疗和医疗监测的年度费用。精心的营养医疗管理以及门静脉高压的后遗症可能会延长生存期，并降低肝移植的潜在发病率和死亡率。
