背侧胰腺发育不全临床特征及其并发症的文献分析

苏松 徐茂锦 钱阳阳 顾园婷 朱曙光 王天骄 郭晓榕 廖专 李兆申



·论著·

背侧胰腺发育不全临床特征及其并发症的文献分析

苏松 徐茂锦 钱阳阳 顾园婷 朱曙光 王天骄 郭晓榕 廖专 李兆申

目的 分析背侧胰腺发育不全的临床特征以及并发糖尿病、胰腺炎，同时合并其他器官先天畸形的情况。方法 以“短胰”、“胰腺发育不全”、“球形胰腺”、“背胰”作为检索词，对维普科技期刊数据库、中国生物医学文献服务系统、中国知网全文期刊数据库进行检索；以“dorsal pancreas agenesis”、“short pancreas”、“pancreas hypoplasia”作为检索词对PubMed数据库进行检索。分析背侧胰腺发育不全患者的临床表现，胰头特征，并发糖尿病、胰腺炎及合并其他器官先天畸形的情况。结果 国内数据库共检索到相关文献6篇，剔除重复病例2例，共计21例；国外数据库共纳入文献61篇，共计70例病例。总计91例。患者常以腹痛为主要表现，占比61.5%；其次是体检时发现，占比约15.3%；其他少见症状包括黄疸、乏力、上腹不适、糖尿病等。排除相关信息未描述的病例，胰头异常39例(61.9%)；并发糖耐量受损或糖尿病38例(56.7%)，其中胰岛素依赖18例，占血糖异常患者的47.4%；并发胰腺炎20例(26.7%)，其中15例(75.0%)为急性胰腺炎，1例(5.0%)为复发性胰腺炎，4例(20.0%)为慢性胰腺炎；合并其他器官先天畸形33例(36.3%)，其中21例次(63.6%)合并脾脏畸形，8例次(24.2%)合并心脏畸形，17例次(51.5%)合并多器官畸形(包括胃肠道畸形、奇静脉与下腔静脉融合、十二指肠及胆道闭锁、肾脏缺如等)。结论 背侧胰腺发育不全是以背侧胰管缺失作为主要诊断标准。糖尿病是最常见的并发症，其次为胰腺炎。

胰腺； 先天畸形； 糖尿病； 胰腺炎

背侧胰腺发育不全是一种罕见的胰腺先天畸形，属于常染色体遗传性疾病，以胰腺体尾部不发育或发育不全为主要特点。既往由于认识不足，也有文献描述其为“短胰腺”、“球形胰腺”等。患者常并发糖尿病、胰腺炎等诸多并发症，并可伴随多器官发育畸形[1-3]。随着影像诊断技术的发展，近年来背侧胰腺发育不全病例报道越来越多，但对该病的临床特征及并发症尚无系统报道。本研究通过检索国内外文献，系统分析背侧胰腺发育不全的临床特征、并发症及合并症的情况。

资料与方法

一、文献资料

2015年6月以“短胰”、“胰腺发育不全”、“球形胰腺”、“背胰”作为检索词，对维普科技期刊数据库、中国生物医学文献服务系统(sinomed)、中国知网全文期刊数据库进行检索，共检索到相关文献6篇，剔除重复病例2例，共计21例[2-7]。以“dorsal pancreas agenesis”、“short pancreas”、“pancreas hypoplasia”作为检索词对PubMed数据库进行检索，剔除重复及不相关文献，共纳入文献61篇，共计70例病例[8-68]。国内、外文献中的病例总计91例。

二、方法

分析背侧胰腺发育不全患者的临床表现，胰头特征，并发糖尿病、胰腺炎及合并其他器官先天畸形的情况。

结果

一、背侧胰腺发育不全患者的临床表现

患者常以腹痛为主要表现，占比61.5%；其次是无症状、于健康体检时发现背侧胰腺发育不全，占比15.3%；其他少见症状包括黄疸、乏力、上腹不适、糖尿病等。体格检查以腹部压痛为主要表现，占比62.3%，余无特征性表现。实验室检查常无异常，部分患者并发糖尿病、黄疸，胰腺炎可有相应生物化学指标改变。影像学检查以胰头异常表现为主。

二、背侧胰腺发育不全胰头特征

28例未描述胰头情况。余63例中39例(61.9%)胰头异常，其中胰头增大25例次(64.1%)，胰头缩小4例次(10.3%)，胰头钙化2例次(5.1%)，形状不规则、环形胰腺、合并肿瘤等14例次(35.9%)。胰头正常24例(38.1%)。

三、背侧胰腺发育不全并发糖尿病

24例未描述血糖水平。余67例中38例(56.7%)并发糖耐量受损或糖尿病，其中18例为胰岛素依赖，占血糖异常患者的47.4%。血糖正常29例(43.3%)。

四、背侧胰腺发育不全并发胰腺炎

16例未描述是否并发胰腺炎。余75例中20例(26.7%)并发胰腺炎，其中15例(75.0%)为急性胰腺炎，1例(5.0%)为复发性胰腺炎，4例(20.0%)为慢性胰腺炎。未并发胰腺炎55例(73.3%)。

五、背侧胰腺发育不全合并其他器官先天畸形

91例中合并其他器官先天畸形33例(36.3%)，其中21例次(63.6%)合并脾脏畸形，8例次(24.2%)合并心脏畸形，17例次(51.5%)合并多器官畸形(包括胃肠道畸形、奇静脉与下腔静脉融合、十二指肠及胆道闭锁、肾脏缺如等)。未合并其他器官畸形58例(63.7%)。

讨论

胚胎发育过程中内胚层分化形成腹侧胰腺和背侧胰腺并在妊娠第7周时彼此融合，最终，腹侧胰腺形成胰腺钩突、胰头的绝大部分及主胰管胰头段，而背侧胰腺则形成胰腺体尾部、小部分胰头以及副胰管和体尾部主胰管[1]，这一过程中任何基因突变或表达异常均有可能导致胰腺发育不全。最新研究发现，同源异型基因GATA6和GATA4以及人类HNF1B基因参与胰腺发育调控，其中GATA6基因突变是最常见的导致胰腺发育不全的原因，该基因突变还常常导致先天性心脏病。而GATA4基因突变除了导致胰腺发育不全，还会导致新生儿或儿童糖尿病[69-71]。人类HNF1B基因则对胰腺的形态发育以及胰腺内分泌细胞分化均具有调控作用[72]。

背侧胰腺发育不全的诊断主要依靠影像学检查，典型征象包括胰腺仅有头部残留，胰头可增大、正常或缩小，胰腺体尾部部分缺失。考虑到慢性胰腺炎胰腺萎缩、胰腺脂肪浸润或者胰腺远端切除的患者影像学检查也可表现为上述征象，因此有部分学者提出以完全或部分背侧胰管缺失作为背侧胰腺发育不全的诊断标准[1,49,61,73]。影像学检查手段主要包括US、CT、MRCP、ERCP以及EUS，其中US检查容易受胃肠道气体的干扰，诊断的敏感性及特异性较低，仅可作为初筛。若不能除外胰腺发育不全，则推荐MRCP作为首选检查，因为CT的胰管显示效果不及MRI，且具有放射性，而ERCP和EUS属于有创性检查，并有诱发急性胰腺炎的风险[35,41]。本研究结果显示，91例背侧胰腺发育不全患者影像学检查的胰头异常发生率61.9%，其中除胰头增大外，合并胰头萎缩、环形胰腺及肿瘤、胰头钙化等情况占所有胰头异常病例的51.3%，表明背侧胰腺发育不全患者易并发其他胰腺疾患，而胰头单纯性增大则常被认为是其代偿所致[73]。

背侧胰腺发育不全患者常无明显症状而偶然发现，或表现为反复发作的急性胰腺炎及糖尿病[65,74]。本组91例中胰腺炎总体发生率为26.7%，其中急性胰腺炎占绝大部分。关于背侧胰腺发育不全并发急性胰腺炎的机制目前还不清楚，大部分学者认为可能是因为oddi括约肌功能失调、残存的腹侧胰腺胰液分泌代偿性增多以及胰管内压升高所致[67,75]。也有学者认为患者基因易感性与并发胰腺炎具有重要关系。由于背侧胰腺发育不全的基因学基础复杂，常合并有多基因异常，这些异常的基因可同时增加患者对急、慢性胰腺炎的易感性[35]。

多篇研究报道糖尿病是背侧胰腺发育不全最常见的并发症，但所纳入的病例常常较少，数据差异较大[1,16,35]。本组糖尿病或糖耐量受损的发生率为56.7%，其中近一半患者需要胰岛素治疗，与Schnedl等[1]的数据基本一致。既往这部分患者大多被诊断为2型糖尿病，但根据美国糖尿病协会最新分型标准，继发于胰腺外分泌疾患的糖尿病应被划分为3c型，且已有研究报道在一个背侧胰腺发育不全家系中家系成员都存在肝细胞胰岛素抵抗，这也与3c型糖尿病的病理生理特点相吻合[76-77]。此外，因为背侧胰腺发育不全在某种程度上类似胰腺体尾部切除术，而胰腺手术正是3c型糖尿病主要病因之一。根据2012年国际胰腺病研讨会的指南建议，3c型糖尿病由于其特殊的病理生理特点，治疗应采取个体化策略[78]。

背侧胰腺发育不全患者还常合并多种其他器官先天畸形。本组合并其他器官先天畸形的发生率高达36.2%，以多脾综合征最为常见，其次是心脏畸形，且合并畸形的患者中超过半数是多器官畸形，包括内脏反位、胃肠道畸形、奇静脉与下腔静脉融合、十二指肠及胆道闭锁等[1,58]。提示背侧胰腺发育不全患者如出现肿瘤、占位等需手术治疗的情况时，术前应仔细评估术区及毗邻解剖结构。

总之，背侧胰腺发育不全是一种罕见的常染色体遗传性疾病，以胰腺体尾部缺失为主要特点，诊断以背侧胰管缺失为主要标准。患者常并发糖尿病、胰腺炎以及伴有多种其他器官先天畸形，其中糖尿病是最常见的并发症，近一半的糖尿病患者需要胰岛素治疗。胰腺炎的发生可能与患者基因易感性相关。对于需要手术的患者，术前应仔细评估术区及毗邻器官的异常。

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(本文编辑：吕芳萍)

Literature review on clinical features and associated complications of dorsal pancreas agenesis

SuSong,XuMaojin,QianYangyang,GuYuanting,ZhuShuguang,WangTianjiao,GuoXiaorong,LiaoZhuan,LiZhaoshen.

DepartmentofEndocrinology,ChanghaiHospital,SecondMilitaryMedicalUniversity,Shanghai200433,China

XuMaojin,Email:xumaojinch@126.com

Objective To analysis the clinical features of dorsal pancreas agenesis (DPA) and the associated diabetes, pancreatitis and other congenital organ malformations. Methods Chinese databases of Sinomed, CQVIP and CNKI using the term of short pancreas, pancreas agenesis, bulbar pancreas and dorsal pancreas, and English databases of PubMed using the term of dorsal pancreas agenesis, short pancreas and pancreas hypoplasia were searched. The clinical manifestation, pancreatic head characteristics and associations with diabetes, pancreatitis and other congenital organ malformations were analyzed. Results Six related publications from Chinese databases were searched and 21 patients were included with 2 cases excluded. Sixty-one publications from English database were searched and 71 patients were included. Thus, a total of 91 patients with DPA were analyzed. Abdominal pain was the most common manifestation, which was reported by 61.5% of the patients. 15.3% patients were identified during regular physical examination. Other manifestations including jaundice, fatigue, abdominal discomfort and diabetes were rare. After removing cases

Pancreas； Congenital abnormalities; Diabetes mellitus; Pancreatitis

10.3760/cma.j.issn.1674-1935.2016.05.009

200433 上海，第二军医大学长海医院内分泌科(苏松、徐茂锦)，消化内科(钱阳阳、顾园婷、朱曙光、王天骄、郭晓榕、廖专、李兆申)

徐茂锦，Email: xumaojinch@126.com

with insufficient information, 39 patients (61.9%) carried abnormal pancreatic head. Prevalence of diabetes or impaired glucose tolerance was 56.7% and the percentage of insulin-dependent diabetes in patients with abnormal glycaemia was 47.3% (n=18). 20 patients (26.7%) were associated with pancreatitis, including 15 patients (75.0%) with acute pancreatitis, 1 patient (5.0%) with recurrent pancreatitis, and 4 patients (20.0%) with chronic pancreatitis. Thirty-three patients (36.2%) suffered other congenital organ malformations, including 21 patients (63.6%) with splenic malformation, 8 patients (24.2%) with heart malformation, and 17 patients (51.5%) with multi-organs malformations like gastrointestinal malformation, azygos vein and inferior cava vena fusion, duodenal and biliary atresia and renal absence. Conclusions The main diagnostic criteria of DPA was the absence of dorsal pancreatic duct. Diabetes was the most common complication followed by pancreatitis.

2016-03-01)