Terlizzi V, Tosco A, Tomaiuolo R, Sepe A, Amato N, Casale A, Mercogliano C, De Gregorio F, Improta F, Elce A, Castaldo G, Raia V
Department of Translational Medical Sciences, University of Naples Federico II, Italy.
CEINGE-Advanced Biotechnology, Naples, Italy; Department of Biochemistry and Biotechnology, University of Naples Federico II, Italy.
J Cyst Fibros. 2014 Sep;13(5):579-84. doi: 10.1016/j.jcf.2014.01.007. Epub 2014 Feb 11.
Currently no tools to predict risk of acute (AP) and recurrent pancreatitis (ARP) in children with cystic fibrosis (CF) are available. We assessed the prevalence of AP/ARP and tested the potential role of Pancreatic Insufficiency Prevalence (PIP) score in a cohort of children with CF.
We identified two groups of children, on the basis of presence/absence of AP/ARP, who were compared for age at diagnosis, clinical features, genotypes and sweat chloride level. PIP score was calculated for each patient.
10/167 (5.9%) experienced at least one episode of AP during follow up; 10/10 were pancreatic sufficient (PS). Patients with AP/ARP showed a PIP score ≤0.25 more frequently (6/10) than patients without AP/ARP. The odds ratio (95% CI) of developing pancreatitis was 4.54 (1.22-16.92) for patients with PIP <0.25 when compared with those who have a PIP score >0.25 (p 0.0151). PIP score was correlated with sweat chloride test (p < 0.01).
PIP score, PS status and normal/borderline sweat chloride levels could be applied to predict pancreatitis development in children with CF. ARP could lead to pancreatic insufficiency.
目前尚无用于预测囊性纤维化（CF）患儿发生急性胰腺炎（AP）和复发性胰腺炎（ARP）风险的工具。我们评估了AP/ARP的患病率，并在一组CF患儿中测试了胰腺功能不全患病率（PIP）评分的潜在作用。
我们根据是否存在AP/ARP将两组儿童进行了识别，比较了他们的诊断年龄、临床特征、基因型和汗液氯化物水平。为每位患者计算PIP评分。
10/167（5.9%）在随访期间经历了至少一次AP发作；其中10/10为胰腺功能正常（PS）。与无AP/ARP的患者相比，发生AP/ARP的患者PIP评分≤0.25更为常见（6/10）。与PIP评分>0.25的患者相比，PIP<0.25的患者发生胰腺炎的比值比（95%CI）为4.54（1.22-16.92）（p=0.0151）。PIP评分与汗液氯化物试验相关（p<0.01）。
PIP评分、PS状态以及正常/临界汗液氯化物水平可用于预测CF患儿胰腺炎的发生。ARP可能导致胰腺功能不全。
