Lenaerts Catherine, Lapierre Chantale, Patriquin Heidi, Bureau Nathalie, Lepage Guy, Harel François, Marcotte Jacques, Roy Claude C
Department of Pediatrics, Hôpital Sainte-Justine, University of Montreal, Montreal, Quebec, Canada.
J Pediatr. 2003 Sep;143(3):343-50. doi: 10.1067/S0022-3476(03)00329-9.
To investigate routine ultrasonography (US) as an early marker and to identify risk factors for the development of cirrhosis and portal hypertension (PHT) in cystic fibrosis (CF).
A cohort of 106 children with CF aged 5.9+/-2.3 years were followed for 10.4+/-0.2 years in a CF clinic.
At enrollment, the US was normal, but biochemical and/or clinical disease was present in 10%. By the end of the study, 19 had developed US changes, eight with evidence of PHT. At the time of the initial US change, only 36.4% of those had, at the end of the study, either a heterogeneous or a nodular parenchyma, and only 50% of those with PHT had biochemical and/or clinical disease. Of the 30 patients treated with ursodeoxycholic acid for biochemical and/or clinical disease with (n=15) and without (n=15) associated US changes, PHT developed in six of the former and two of the latter. Univariate analysis and logistic regression showed that children with more severe disease in terms of forced expiratory volume in one second were at somewhat greater risk (P<.06) of PHT developing.
US was an early marker of liver disease and more severe CF disease, a predictor of progressive liver disease. A controlled trial should be done to assess isolated US-detected disease as an indication for UDCA.
研究常规超声检查（US）作为囊性纤维化（CF）患者肝硬化和门静脉高压（PHT）发生的早期标志物，并确定其危险因素。
在一家CF诊所对106名年龄为5.9±2.3岁的CF儿童进行了为期10.4±0.2年的队列研究。
入组时，超声检查正常，但10%的患者存在生化和/或临床疾病。到研究结束时，19名患者出现了超声检查变化，其中8名有PHT证据。在最初出现超声检查变化时，到研究结束时，只有36.4%的患者出现实质不均匀或结节状改变，只有50%的PHT患者存在生化和/或临床疾病。在30名因生化和/或临床疾病接受熊去氧胆酸治疗的患者中，15名有相关超声检查变化，15名无相关超声检查变化，前者中有6名发生了PHT，后者中有2名发生了PHT。单因素分析和逻辑回归显示，一秒用力呼气量方面疾病较严重的儿童发生PHT的风险略高（P<0.06）。
超声检查是肝病和更严重CF疾病的早期标志物，是进行性肝病的预测指标。应进行一项对照试验，以评估超声检查单独发现的疾病作为熊去氧胆酸治疗指征的情况。
