中华肝脏病杂志
中華肝髒病雜誌
중화간장병잡지
CHINESE JOURNAL OF HEPATOLOGY
2009年
11期
861-866
,共6页
肝硬化%肝硬化%胆汁性%原发性%抗线粒体抗体%免疫球蛋白M%熊去氧胆酸%肝移植
肝硬化%肝硬化%膽汁性%原髮性%抗線粒體抗體%免疫毬蛋白M%熊去氧膽痠%肝移植
간경화%간경화%담즙성%원발성%항선립체항체%면역구단백M%웅거양담산%간이식
Liver cirrhosis: Primagy biliary cirrhosis%Antimitochondrial antibodies%Immunoglobulin M%Ursodexycholic acid%Liver transplantation
目的 研究中国人原发性胆汁性肝硬化(PBC)的临床特点.方法 检索所有有关中国人PBC的文献报道并进行系统分析. 结果自1955-2007年共有103篇PBC相关文献,2740例病例报道(剔除重复报道).16篇文献中985例患者纳入研究,其中女:男为6.82:1,平均年龄42.0～56.2岁,发病至确诊的平均时间为12.0～98.4个月.最常见的症状为乏力(72.40%)、黄疸(67.41%)、纳差(68.58%)、瘙痒(45.60%),无症状患者占20%.最常见的体征为脾肿大(57.53%)、肝肿大(43.56%)和腹水(18.45%).肝功能检查见碱性磷酸酶、γ谷氨酰转肽酶显著升高,免疫球蛋白以1gM升高为主.抗线粒体抗体及其M2型阳性率分别为88.98%与82.65%.30.96%患者进行了肝活组织检查.伴发疾病以十燥综合征最常见(9.14%),其次为类风湿性关节炎(3.95%)和2型糖尿病(2.54%).使用熊去氧胆酸治疗者68.05%(345/507)有效.最主要死亡原因为上消化道出血(41.67%)和肝衰竭(41.67%).肝移植仍是终末期肝病惟一有效的治疗方法.结论 中国人PBC的特点同国外报道基本相似.进一步的研究应侧重于流行病学调查、无症状患者的早期诊断及长期随访、免疫学机制、高质量的临床试验以及肝移植作用等方面.
目的 研究中國人原髮性膽汁性肝硬化(PBC)的臨床特點.方法 檢索所有有關中國人PBC的文獻報道併進行繫統分析. 結果自1955-2007年共有103篇PBC相關文獻,2740例病例報道(剔除重複報道).16篇文獻中985例患者納入研究,其中女:男為6.82:1,平均年齡42.0～56.2歲,髮病至確診的平均時間為12.0～98.4箇月.最常見的癥狀為乏力(72.40%)、黃疸(67.41%)、納差(68.58%)、瘙癢(45.60%),無癥狀患者佔20%.最常見的體徵為脾腫大(57.53%)、肝腫大(43.56%)和腹水(18.45%).肝功能檢查見堿性燐痠酶、γ穀氨酰轉肽酶顯著升高,免疫毬蛋白以1gM升高為主.抗線粒體抗體及其M2型暘性率分彆為88.98%與82.65%.30.96%患者進行瞭肝活組織檢查.伴髮疾病以十燥綜閤徵最常見(9.14%),其次為類風濕性關節炎(3.95%)和2型糖尿病(2.54%).使用熊去氧膽痠治療者68.05%(345/507)有效.最主要死亡原因為上消化道齣血(41.67%)和肝衰竭(41.67%).肝移植仍是終末期肝病惟一有效的治療方法.結論 中國人PBC的特點同國外報道基本相似.進一步的研究應側重于流行病學調查、無癥狀患者的早期診斷及長期隨訪、免疫學機製、高質量的臨床試驗以及肝移植作用等方麵.
목적 연구중국인원발성담즙성간경화(PBC)적림상특점.방법 검색소유유관중국인PBC적문헌보도병진행계통분석. 결과자1955-2007년공유103편PBC상관문헌,2740례병례보도(척제중복보도).16편문헌중985례환자납입연구,기중녀:남위6.82:1,평균년령42.0～56.2세,발병지학진적평균시간위12.0～98.4개월.최상견적증상위핍력(72.40%)、황달(67.41%)、납차(68.58%)、소양(45.60%),무증상환자점20%.최상견적체정위비종대(57.53%)、간종대(43.56%)화복수(18.45%).간공능검사견감성린산매、γ곡안선전태매현저승고,면역구단백이1gM승고위주.항선립체항체급기M2형양성솔분별위88.98%여82.65%.30.96%환자진행료간활조직검사.반발질병이십조종합정최상견(9.14%),기차위류풍습성관절염(3.95%)화2형당뇨병(2.54%).사용웅거양담산치료자68.05%(345/507)유효.최주요사망원인위상소화도출혈(41.67%)화간쇠갈(41.67%).간이식잉시종말기간병유일유효적치료방법.결론 중국인PBC적특점동국외보도기본상사.진일보적연구응측중우류행병학조사、무증상환자적조기진단급장기수방、면역학궤제、고질량적림상시험이급간이식작용등방면.
Objective To summarize the clinical features, diagnosis and treatment of patients with primary biliary cirrhosis (PBC) in China. Methods Systematic analysis of clinical characteristics by search-ing the Chinese literatures. Results From 1955 to 2007, 2740 PBC patients were reported in 103 papers (duplicated reports were deleted). The detailed information of 985 patients from 16 papers were colleted.Female : male was 6.82:1. The age range was 42 to 56.2-year-old. The time from onset to diagnosis was 12 to 98.4 months. The most common symptoms were fatigue (72.40%), jaundice (67.41%), anorexia (68.58%)and pruritus (45.60%). 20% patients were asymptomatic at onset. The most frequent physical signs were splenomegaly (57.53%), hepatomegaly (43.56%) and ascites (18.45%). Serum alkaline phosphatase (ALP)and y glutamyl transpeptidase (GGT) levels were malkedly elevated in most of these patients. The immuno-logical marks of AMA and M2 were positive in 88.98% and 82.65% patients, respectively. The most common comobidity were Sjogren syndrome (9.14%), rheumatoid arthritis (3.95%) and diabetes type Ⅱ (2.54%). Of the 507 patients treated with ursodeoxycholic acid (UDCA), 345 patients got complete or partial clinical biochemical response. The common complications were gastrointestinal bleeding (41.67%) and liver failure (41.67%). Liver transplantation was the only effective way for the treatment of the end-stage liver disease.Conclusion The clinical feature of primary biliary cirrhosis in China was similar to the oversea literatures.Further-research should focus on epidemic investigation, early diagnosis, long term follow up of asymptom-atic patients immunological mechanism and the efficacy of liver transplantation.
