Background

Liver involvement is an important cause of morbidity and mortality in patients with cystic fibrosis (CF). While liver biopsy is the gold standard for demonstrating involvement, its invasiveness prompts a search for noninvasive alternatives.

Objective

To evaluate liver involvement in pediatric patients with CF (versus healthy controls) using magnetic resonance (MR) elastography/spectroscopy and to correlate the imaging findings with clinical/laboratory characteristics.

Materials and methods

This was a single-center, prospective cross-sectional study conducted between April 2020 and March 2022 in patients with CF versus healthy controls. Patients with CF were divided into two subgroups: those with CF-related liver disease and those without. MR images were acquired on a 1.5-tesla machine. Kilopascal (kPa) values were derived from processing MR elastography images. MR spectroscopy was used to measure liver fat fraction, as an indication of hepatosteatosis. Groups were compared using either the Student’s t test or the Mann‒Whitney U test. The chi-square test or Fisher’s exact test were used to compare qualitative variables.

Results

Fifty-one patients with CF (12 ± 3.3 years, 32 boys) and 24 healthy volunteers (11.1 ± 2.4 years, 15 boys) were included in the study. Median liver stiffness (P=0.003) and fat fraction (P=0.03) were higher in the CF patients than in the controls. Median liver stiffness values were higher in CF patients with CF-related liver disease than in those without CF-related liver disease (P=0.002). Liver stiffness values of CF patients with high alanine aminotransferase (ALT), high gamma-glutamyl transferase, and thrombocytopenia were found to be higher than those without (P=0.004, P<0.001, P<0.001, respectively). Only the high ALT group showed a high fat fraction (P=0.002).

Conclusions

Patients with CF had higher liver stiffness than the control group, and patients with CF-related liver disease had higher liver stiffness than both the CF patients without CF-related liver disease and the control group. Patients with CF had a higher fat fraction than the control group. Noninvasive assessment of liver involvement using MR elastography/spectroscopy can support the diagnosis of CF-related liver disease and the follow-up of patients with CF.

Graphical abstract

中文翻译：

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囊性纤维化儿童患者与健康儿童的肝脏磁共振弹性成像和脂肪分数

背景

肝脏受累是囊性纤维化（CF）患者发病和死亡的重要原因。虽然肝活检是证明参与的金标准，但其侵入性促使人们寻找非侵入性替代方案。

客观的

使用磁共振 (MR) 弹性成像/光谱学评估 CF 儿科患者（与健康对照）的肝脏受累情况，并将影像学结果与临床/实验室特征相关联。

材料和方法

这是一项于 2020 年 4 月至 2022 年 3 月期间针对 CF 患者与健康对照进行的单中心前瞻性横断面研究。CF 患者分为两个亚组：患有 CF 相关肝病的患者和无 CF 相关肝病的患者。MR 图像是在 1.5 特斯拉机器上采集的。千帕 (kPa) 值是通过处理 MR 弹性成像图像得出的。磁共振波谱法用于测量肝脏脂肪分数，作为肝脂肪变性的指标。使用学生t检验或曼惠特尼U检验对各组进行比较。卡方检验或费舍尔精确检验用于比较定性变量。

结果

该研究包括 51 名 CF 患者（12 ± 3.3 岁，32 名男孩）和 24 名健康志愿者（11.1 ± 2.4 岁，15 名男孩）。CF 患者的中位肝脏硬度 ( P = 0.003) 和脂肪分数 ( P = 0.03) 高于对照组。患有 CF 相关肝病的 CF 患者的中位肝脏硬度值高于无 CF 相关肝病的患者 ( P =0.002)。发现患有高丙氨酸转氨酶（ALT）、高γ-谷氨酰转移酶和血小板减少症的 CF 患者的肝脏硬度值高于不患有这些疾病的患者（分别为P =0.004、P <0.001、P <0.001）。仅高 ALT 组表现出高脂肪分数（P =0.002）。

结论

CF患者的肝脏硬度高于对照组，CF相关肝病患者的肝脏硬度高于无CF相关肝病的CF患者和对照组。CF 患者的脂肪分数高于对照组。使用 MR 弹性成像/能谱对肝脏受累进行无创评估可以支持 CF 相关肝病的诊断和 CF 患者的随访。

图形概要