Gasser Rudolf W
Division of General Internal Medicine, Department of Internal Medicine, Medical University of Innsbruck, Innsbruck, Austria.
Wien Med Wochenschr. 2008;158(19-20):553-7. doi: 10.1007/s10354-008-0594-z.
Metabolic bone disease, mainly osteopenia/osteoporosis and occasionally osteomalacia, is a major extrahepatic manifestation of chronic cholestatic liver disease (synonym: hepatic osteodystrophy). Reduced bone mineral density is found in up to 60% and atraumatic fractures in about 20% of patients with chronic liver disease. Hepatic osteodystrophy is characterized by reduced formation and increased resorption of bone; major risk factors are chronic cholestasis and advanced cirrhosis. Pathogenetic mechanisms include genetic factors, abnormalities of calcium, vitamin D, vitamin K and bilirubin metabolism, IGF-1 deficiency, the RANKL/OPG-system, hypogonadism, drugs harmful to bone, lifestyle factors (smoking, alcoholism, immobility), malnutrition and low body mass index. Screening for osteopenia should be performed and reversible risk factors must be corrected. At present, bisphosphonates are the predominantly used specific drugs for the treatment of osteoporosis in chronic liver disease. After orthotopic liver transplantation bone mineral density improves in long-term follow-up. Studies are needed for fracture prevention in chronic liver disease.
代谢性骨病，主要为骨质减少/骨质疏松，偶尔也有骨软化症，是慢性胆汁淤积性肝病的一种主要肝外表现（同义词：肝性骨营养不良）。在高达60%的慢性肝病患者中可发现骨矿物质密度降低，约20%的患者会发生非创伤性骨折。肝性骨营养不良的特征是骨形成减少和骨吸收增加；主要危险因素是慢性胆汁淤积和晚期肝硬化。发病机制包括遗传因素、钙、维生素D、维生素K和胆红素代谢异常、胰岛素样生长因子-1缺乏、核因子κB受体活化因子配体/骨保护素系统、性腺功能减退、对骨骼有害的药物、生活方式因素（吸烟、酗酒、缺乏活动）、营养不良和低体重指数。应进行骨质减少筛查，必须纠正可逆性危险因素。目前，双膦酸盐是慢性肝病中治疗骨质疏松症最常用的特效药物。原位肝移植后，骨矿物质密度在长期随访中会有所改善。需要开展关于慢性肝病骨折预防的研究。
