Radiology, Cliniques Universitaires St Luc, Avenue Hippocrate 10, Brussels 1200, Belgium.
Pediatr Radiol. 2010 Jul;40(7):1231-5. doi: 10.1007/s00247-009-1531-z. Epub 2010 Feb 5.
Hepatic involvement is frequent in patients with cystic fibrosis (CF), with focal biliary cirrhosis being the pathognomonic hepatic manifestation. In around one-quarter of CF patients, it results in CF-associated liver disease (CFLD). This occurs as a relatively early complication with the majority of patients presenting in childhood or their early teens. However, a normal US does not preclude significant liver fibrosis and liver biopsy is an invasive procedure that is hampered by potential sampling errors. Transient elastography (TE) (Fibroscan) is a non-invasive, user-friendly and quick technique that provides an objective and reproducible measure of liver stiffness. This is accomplished with a device using an US probe mounted in the axis of a vibrator. Vibrations are transmitted by the transducer, inducing an electronic shear wave that propagates through the underlying tissue.
We aimed to prospectively compare TE and transabdominal US scanning in children and adults attending a CF clinic.
A total of 134 consecutive patients with documented CF were prospectively studied. In each case, transient elastography measurement was performed immediately after the routine annual US evaluation of the liver. Sonographic appearance of the liver was classified from 1 to 5. Ten validated TE measurements were performed in each patient with the result expressed in kilopascals (kPa). The median value was considered representative of the elastic modulus of the liver.
Measurements were performed in 59 CF adults, 75 CF children and 31 control children. There was no relationship between age and liver stiffness in either the control group or CF patients. Elasticity values of controls, CF pancreatic sufficient (PS) patients and pancreatic insufficient (PI) CF patients with a US score <3 were comparable and significantly lower than in CF patients with a US score > or = 3 (all PI) (P < 0.002). Median elasticity in CF patients was significantly higher in males (4.7 kPa) than in females (3.9 kPa) (P = 0.0013).
Considering the limitations of US and the low risk-benefit rate of liver biopsy in most CF patients, this preliminary study suggests that TE is an attractive non-invasive way to assess and follow-up liver disease in these patients.
囊性纤维化（CF）患者常出现肝脏受累，局灶性胆管性肝硬化是其特有的肝脏表现。约四分之一的 CF 患者会出现 CF 相关性肝病（CFLD）。该病通常为早期并发症，大多数患者在儿童或青少年时期发病。然而，肝脏超声正常并不能排除显著的肝纤维化，且肝活检是一种具有潜在采样误差的侵袭性操作。瞬时弹性成像（TE）（Fibroscan）是一种非侵入性、用户友好且快速的技术，可提供肝硬度的客观、可重复的测量。该技术通过一个安装在振动器轴上的超声探头的设备来实现。振动由换能器传递，从而产生传播穿过下方组织的电子剪切波。
我们旨在前瞻性比较 TE 和经腹超声扫描在 CF 门诊就诊的儿童和成人中的应用。
共对 134 例确诊 CF 的连续患者进行前瞻性研究。在每种情况下，TE 测量均在肝脏的常规年度超声评估后立即进行。肝脏的超声表现从 1 分到 5 分进行分类。对每位患者进行 10 次经过验证的 TE 测量，结果以千帕斯卡（kPa）表示。中位数被认为代表肝脏的弹性模量。
在 59 例 CF 成人、75 例 CF 儿童和 31 例对照儿童中进行了测量。在对照组或 CF 患者中，年龄与肝硬度之间均无相关性。对照组、CF 胰腺功能正常（PS）患者和 US 评分<3 的 CF 胰腺功能不全（PI）患者的弹性值相当，且明显低于 US 评分≥3 的 CF 患者（均为 PI）（均 P<0.002）。CF 患者中，男性（4.7kPa）的中位弹性显著高于女性（3.9kPa）（P=0.0013）。
鉴于超声的局限性以及大多数 CF 患者肝活检的低风险效益比，这项初步研究表明，TE 是评估和随访这些患者肝脏疾病的一种有吸引力的非侵入性方法。
