Chronic Intestinal Failure Center, Digestive Disease Department, St. Orsola-Malpighi Hospital, Department of Medical and Surgical Sciences, University of Bologna, Bologna 40138, Italy.
Intestinal Failure Unit, Salford Royal Hospital & University of Manchester, Salford M68HD, UK.
Clin Nutr. 2019 Jun;38(3):1198-1205. doi: 10.1016/j.clnu.2018.04.019. Epub 2018 May 8.
BACKGROUND & AIMS: Intestinal failure associated liver disease (IFALD) has been defined using numerous criteria; however the clinical relevance of these criteria has never been compared. We therefore aimed to evaluate the prevalence, incidence, evolution of IFALD diagnosed by different criteria and to assess any clinical features that may be associated with its occurrence.
A cross sectional (CS) and retrospective study were carried out on adults on home parenteral nutrition (HPN) for chronic intestinal failure (CIF) managed at a single center. Inclusion criteria at CS: age ≥18 years, benign disease. Collected data included: patient demographics, CIF and HPN characteristics, episodes of central venous catheter related bloodstream infection (CRBSI). IFALD was diagnosed by 9 criteria based on liver function tests and liver ultrasound (US) imaging. IFALD diagnoses were categorized as steatosis (2 criteria), cholestasis (3 criteria) or fibrosis (2 criteria) and unclassified (2 criteria). Prevalence was assessed at CS and at starting HPN (baseline, BS). Evolution was assessed as change of IFALD between BS and CS. Incidence was calculated as patients who developed IFALD from BS to CS.
A total of 113 patients were included. At CS, IFALD prevalence range in each diagnostic categories was: cholestasis 5-15%; steatosis 17-43%; fibrosis 10-20%; unclassified 7-38%. A 28.5% of patients did not have IFALD according to any criteria. Two cholestasis criteria and one fibrosis criterion were significantly (P < 0.05) associated with a short bowel syndrome as the pathophysiological mechanism of CIF, HPN requirement and the number of CRBSI episodes. At BS, IFALD prevalence range was: cholestasis 13-40%; steatosis 27-90%; fibrosis 2-5%; unclassified 8-75%. The incidence range of IFALD was: cholestasis 0-7%; steatosis 0-39%; fibrosis 7-18%; unclassified 4-9%. IFALD steatosis diagnosed by US was the most frequent diagnosis at both CS prevalence and incidence assessments. Notably, IFALD criteria normalized in various percentages (2-70%), depending on the diagnostic categories, between BS and CS.
This is the first study to systematically demonstrate that the frequency of IFALD varies greatly depending on diagnostic criteria used, confirming the need for a consensus definition to be used between different national and international IF units. IFALD can be present at HPN initiation but may resolve thereafter; further work is required to evaluate the factors associated with improvement.
肠衰竭相关肝病（IFALD）已经有许多标准来定义；然而，这些标准的临床相关性从未被比较过。因此，我们旨在评估不同标准诊断的 IFALD 的患病率、发病率、演变，并评估任何可能与 IFALD 发生相关的临床特征。
对在单中心接受家庭肠外营养（HPN）治疗慢性肠衰竭（CIF）的成年人进行了一项横断面（CS）和回顾性研究。CS 的纳入标准为：年龄≥18 岁，良性疾病。收集的数据包括：患者人口统计学、CIF 和 HPN 特征、中心静脉导管相关血流感染（CRBSI）发作。IFALD 是根据肝功能检查和肝脏超声（US）成像的 9 项标准诊断的。IFALD 诊断分为脂肪变性（2 项标准）、胆汁淤积（3 项标准）或纤维化（2 项标准）和未分类（2 项标准）。在 CS 和开始 HPN（基线，BS）时评估患病率。通过比较 BS 和 CS 之间的 IFALD 变化来评估演变。从 BS 到 CS 期间发生 IFALD 的患者的发病率来计算。
共纳入 113 名患者。在 CS 时，每种诊断类别的 IFALD 患病率范围如下：胆汁淤积 5-15%；脂肪变性 17-43%；纤维化 10-20%；未分类 7-38%。根据任何标准，有 28.5%的患者没有 IFALD。两个胆汁淤积标准和一个纤维化标准与短肠综合征作为 CIF 的病理生理机制、HPN 需求和 CRBSI 发作次数显著相关（P<0.05）。在 BS 时，IFALD 的患病率范围为：胆汁淤积 13-40%；脂肪变性 27-90%；纤维化 2-5%；未分类 8-75%。IFALD 的发病率范围为：胆汁淤积 0-7%；脂肪变性 0-39%；纤维化 7-18%；未分类 4-9%。在 CS 的患病率和发病率评估中，US 诊断的 IFALD 脂肪变性是最常见的诊断。值得注意的是，BS 和 CS 之间，根据诊断类别，IFALD 标准的正常化比例在 2-70%之间变化。
这是第一项系统地证明 IFALD 的频率因使用的诊断标准而异的研究，证实了需要在不同的国家和国际 IF 单位之间使用一致的定义。IFALD 可能在开始 HPN 时就存在，但此后可能会消退；需要进一步工作来评估与改善相关的因素。
