Poddar Ujjal, Yachha Surender K, Borkar Vibhor, Srivastava Anshu
Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
Dig Liver Dis. 2017 Jul;49(7):796-801. doi: 10.1016/j.dld.2017.02.019. Epub 2017 Mar 6.
BACKGROUND/AIMS: In view of paucity of literature we analyzed our experience of acute recurrent pancreatitis (ARP) to study clinical profile and long-term outcome.
Over 13 years, 93 consecutive children (≤18 years) diagnosed to have ARP were included in this study. Magnetic resonance cholangiopancreatography was done at baseline and on follow-up. Common mutations for serine-protease-inhibitor (SPINK1 N34S), protease inhibitor (PRSS1 R122S) and cystic fibrosis transmembrane conductance regulator (CFTR deltaF508, 5T) were studied in 22 idiopathic cases.
The median age of the children with ARP was 13 (10-14.5) years, 53 were males. Etiology included biliary in 14 (15%), pancreas divisum in 6 (7%), others in 3 (3.5%) and idiopathic in the remaining 70 (75%). SPINK1 mutation was found in 10/22 (45%) cases. Over a median follow-up of 25.5 (8.25-48) months, 37 (42%) of 88 (5 lost to follow-up) developed chronic pancreatitis (CP). On multivariate analysis idiopathic etiology (p<0.03), presence of SPINK1 mutation (p=0.01), longer follow-up (p<0.001) were associated with progression to CP.
Biliopancreatic structural/obstructive causes should always be looked for. It seems ARP is a precursor of CP and progression is associated with idiopathic etiology and presence of genetic mutations. Hence, patients with ARP should be kept on regular follow-up to detect CP.
背景/目的:鉴于相关文献较少,我们分析了我们在急性复发性胰腺炎(ARP)方面的经验,以研究其临床特征和长期预后。
在13年的时间里,本研究纳入了93例连续诊断为ARP的18岁及以下儿童。在基线和随访时均进行了磁共振胰胆管造影。对22例特发性病例研究了丝氨酸蛋白酶抑制剂(SPINK1 N34S)、蛋白酶抑制剂(PRSS1 R122S)和囊性纤维化跨膜传导调节因子(CFTR deltaF508、5T)的常见突变。
ARP患儿的中位年龄为13(10 - 14.5)岁,男性53例。病因包括胆汁性14例(15%)、胰腺分裂症6例(7%)、其他3例(3.5%),其余70例(75%)为特发性。在22例病例中有10例(45%)发现SPINK1突变。在中位随访25.5(8.25 - 48)个月期间,88例(5例失访)中有37例(42%)发展为慢性胰腺炎(CP)。多因素分析显示,特发性病因(p<0.03)、存在SPINK1突变(p = 0.01)、随访时间较长(p<0.001)与进展为CP相关。
应始终寻找胆胰结构性/梗阻性病因。ARP似乎是CP的前驱疾病,其进展与特发性病因和基因突变的存在有关。因此,ARP患者应定期随访以检测CP。