Natural history of liver disease in cystic fibrosis
Natural history of liver disease in cystic fibrosis
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DOI：
10.1002/hep.510300527
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发表时间：
1999-11-01
期刊：
影响因子：
13.5
通讯作者：
Strandvik, B
中科院分区：
文献类型：
--
作者：
Lindblad, A;Glaumann, H;Strandvik, B
全球囊性纤维化 (CF) 人群的中位年龄有所增加，这表明肝病的患病率将会增加。本研究的目的是评估控制良好的 CF 患者人群 15 年期间 CF 相关肝病的自然史。 1976 年至 1993 年间，对 124 名患者进行了年度肝功能检查 (LFT) 随访。在整个研究期间，对 15 名患者进行了肝活检随访。超过 50% 的患者在婴儿期有病理性 LFT，后来恢复正常。在研究期间，大约 25% 的 4 岁或以上儿童具有肝病生化标志物。约10%的患者在活检时确诊为肝硬化或晚期纤维化，4%的患者患有肝硬化并伴有临床肝病。严重肝病主要发生在青春期前和青春期。在对 15 名患者进行肝活检前瞻性随访时，只有 3 名患者出现进行性纤维化。未发现具体的危险因素，但在明显脂肪变性的患者中更常见缺乏必需脂肪酸（P < .05）。没有患者在成年后出现临床肝病，肝活检的组织学变化通常不是进行性的。尽管患者群体的中位年龄有所增加，但在研究期结束时，肝病的发生率并未增加。现代治疗可能会对肝病产生积极影响，因为与以前报道的相比，这种疾病似乎不太常见、进展较慢且严重程度也较低。
The median age of the population with cystic fibrosis (CF) has increased worldwide, which has led to the suggestion that the prevalence of liver disease would increase. The aim of this study was to evaluate the natural history of CF-associated liver disease over a 15-year period in a well-controlled population of patients with CF. During the years 1976 through 1993, 124 patients were followed up by yearly liver function tests (LFTs). Fifteen patients were followed up with liver biopsies throughout the whole study period. More than 50% of the patients had pathological LFTs in infancy, later being normalized. Approximately 25% of children 4 years of age or older had biochemical markers of liver disease during the study period. In about 10% of the patients, cirrhosis or advanced fibrosis was confirmed at biopsy and 4% of patients had cirrhosis with clinical liver disease. Severe liver disease developed mainly during prepuberty and puberty. Of the 15 patients prospectively followed up with liver biopsies, only 3 had progressive fibrosis. No specific risk factor was identified, but deficiency of essential fatty acids was found more often in patients with marked steatosis (P < .05). No patient developed clinical liver disease in adulthood and the histological changes in the liver biopsies were usually not progressive. Liver disease was no more frequent at the end of the study period although the median age of the patient population had increased. Modern treatment might positively influence liver disease because it seemed less common, less progressive, and less serious than previously reported.