Abstract：
Porto-sinusoidal vascular disease (PSVD) has been described as the prominent pathology in liver explants of patients with cystic fibrosis (CF), but data outside the transplant setting are lacking. We aimed to investigate the prevalence of portal hypertension (PH) in CF-associated liver disease (CFLD) and develop an algorithm to classify liver involvement in CF patients.
This is a cross-sectional study of consecutive paediatric and adult patients in a tertiary centre between 2018 and 2019, who underwent ultrasound, liver (LSM) and spleen stiffness (SSM) measurement. CFLD was defined according to physical examination, liver tests and ultrasound findings. PSVD was likely if there were PH signs in the absence of advanced chronic liver disease (CF-ACLD, LSM <10 kPa). A historical cohort was used to validate the prognostic significance of the new definitions.
Fifty (27.5%) patients met CFLD criteria. At least one sign of PH was found in 47 (26%) patients, but most (81%) had LSM <10 kPa and were likely to have PSVD; only 9 (5%) had CF-ACLD. PSVD and CFLD (LSM <10 kPa) co-existed in most (23/36) cases. In the historical cohort (n = 599 patients), likely PSVD and CFLD+PH were independently associated with a 2-fold and 3.5-fold increase in mortality compared to patients without PH, respectively. In 34 patients with SSM, values <21 and >50 kPa accurately diagnosed specific signs of PH.
PSVD is the prevailing cause of PH in CF patients. We developed a new diagnostic algorithm based on clinical and elastosonography criteria to classify liver involvement in patients with CF.
This is a cross-sectional study of consecutive paediatric and adult patients in a tertiary centre between 2018 and 2019, who underwent ultrasound, liver (LSM) and spleen stiffness (SSM) measurement. CFLD was defined according to physical examination, liver tests and ultrasound findings. PSVD was likely if there were PH signs in the absence of advanced chronic liver disease (CF-ACLD, LSM <10 kPa). A historical cohort was used to validate the prognostic significance of the new definitions.
Fifty (27.5%) patients met CFLD criteria. At least one sign of PH was found in 47 (26%) patients, but most (81%) had LSM <10 kPa and were likely to have PSVD; only 9 (5%) had CF-ACLD. PSVD and CFLD (LSM <10 kPa) co-existed in most (23/36) cases. In the historical cohort (n = 599 patients), likely PSVD and CFLD+PH were independently associated with a 2-fold and 3.5-fold increase in mortality compared to patients without PH, respectively. In 34 patients with SSM, values <21 and >50 kPa accurately diagnosed specific signs of PH.
PSVD is the prevailing cause of PH in CF patients. We developed a new diagnostic algorithm based on clinical and elastosonography criteria to classify liver involvement in patients with CF.
摘要：
目的：窦口血管疾病（PSVD）已被描述为囊性纤维化（CF）患者肝外植体的突出病理，但是缺乏移植设置之外的数据。我们旨在调查CF相关肝病（CFLD）中门静脉高压（PH）的患病率，并开发一种对CF患者肝脏受累进行分类的算法。
方法：这是一项针对2018年至2019年在三级中心连续接受超声检查的儿科和成年患者的横断面研究，肝脏（LSM）和脾脏硬度（SSM）测量。CFLD是根据体检定义的，肝脏检查和超声检查结果。如果在没有晚期慢性肝病的情况下有PH体征，则PSVD很可能（CF-ACLD，LSM<10kPa)。使用历史队列来验证新定义的预后意义。
结果：50例（27.5%）患者符合CFLD标准。在47例(26%)患者中发现至少一种PH征象,但大多数(81%)的LSM<10kPa,可能有PSVD;只有9(5%)的患者有CF-ACLD.PSVD和CFLD（LSM<10kPa）在大多数（23/36）病例中共存。在历史队列中（n=599名患者），与无PH患者相比，PSVD和CFLD+PH可能与死亡率增加2倍和3.5倍独立相关。分别。在34例SSM患者中，值<21和>50kPa可准确诊断PH的特定体征。
结论：PSVD是CF患者PH的主要原因。我们开发了一种基于临床和弹性成像标准的新诊断算法，以对CF患者的肝脏受累进行分类。
方法：这是一项针对2018年至2019年在三级中心连续接受超声检查的儿科和成年患者的横断面研究，肝脏（LSM）和脾脏硬度（SSM）测量。CFLD是根据体检定义的，肝脏检查和超声检查结果。如果在没有晚期慢性肝病的情况下有PH体征，则PSVD很可能（CF-ACLD，LSM<10kPa)。使用历史队列来验证新定义的预后意义。
结果：50例（27.5%）患者符合CFLD标准。在47例(26%)患者中发现至少一种PH征象,但大多数(81%)的LSM<10kPa,可能有PSVD;只有9(5%)的患者有CF-ACLD.PSVD和CFLD（LSM<10kPa）在大多数（23/36）病例中共存。在历史队列中（n=599名患者），与无PH患者相比，PSVD和CFLD+PH可能与死亡率增加2倍和3.5倍独立相关。分别。在34例SSM患者中，值<21和>50kPa可准确诊断PH的特定体征。
结论：PSVD是CF患者PH的主要原因。我们开发了一种基于临床和弹性成像标准的新诊断算法，以对CF患者的肝脏受累进行分类。
