蘇松 徐茂錦 錢陽(yáng)陽(yáng) 顧園婷 朱曙光 王天驕 郭曉榕 廖專 李兆申

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·論著·

背側(cè)胰腺發(fā)育不全臨床特征及其并發(fā)癥的文獻(xiàn)分析

蘇松 徐茂錦 錢陽(yáng)陽(yáng) 顧園婷 朱曙光 王天驕 郭曉榕 廖專 李兆申

目的 分析背側(cè)胰腺發(fā)育不全的臨床特征以及并發(fā)糖尿病、胰腺炎，同時(shí)合并其他器官先天畸形的情況。方法 以“短胰”、“胰腺發(fā)育不全”、“球形胰腺”、“背胰”作為檢索詞，對(duì)維普科技期刊數(shù)據(jù)庫(kù)、中國(guó)生物醫(yī)學(xué)文獻(xiàn)服務(wù)系統(tǒng)、中國(guó)知網(wǎng)全文期刊數(shù)據(jù)庫(kù)進(jìn)行檢索；以“dorsal pancreas agenesis”、“short pancreas”、“pancreas hypoplasia”作為檢索詞對(duì)PubMed數(shù)據(jù)庫(kù)進(jìn)行檢索。分析背側(cè)胰腺發(fā)育不全患者的臨床表現(xiàn)，胰頭特征，并發(fā)糖尿病、胰腺炎及合并其他器官先天畸形的情況。結(jié)果 國(guó)內(nèi)數(shù)據(jù)庫(kù)共檢索到相關(guān)文獻(xiàn)6篇，剔除重復(fù)病例2例，共計(jì)21例；國(guó)外數(shù)據(jù)庫(kù)共納入文獻(xiàn)61篇，共計(jì)70例病例?？傆?jì)91例?；颊叱Ｒ愿雇礊橹饕憩F(xiàn)，占比61.5%；其次是體檢時(shí)發(fā)現(xiàn)，占比約15.3%；其他少見(jiàn)癥狀包括黃疸、乏力、上腹不適、糖尿病等。排除相關(guān)信息未描述的病例，胰頭異常39例(61.9%)；并發(fā)糖耐量受損或糖尿病38例(56.7%)，其中胰島素依賴18例，占血糖異?；颊叩?7.4%；并發(fā)胰腺炎20例(26.7%)，其中15例(75.0%)為急性胰腺炎，1例(5.0%)為復(fù)發(fā)性胰腺炎，4例(20.0%)為慢性胰腺炎；合并其他器官先天畸形33例(36.3%)，其中21例次(63.6%)合并脾臟畸形，8例次(24.2%)合并心臟畸形，17例次(51.5%)合并多器官畸形(包括胃腸道畸形、奇靜脈與下腔靜脈融合、十二指腸及膽道閉鎖、腎臟缺如等)。結(jié)論 背側(cè)胰腺發(fā)育不全是以背側(cè)胰管缺失作為主要診斷標(biāo)準(zhǔn)。糖尿病是最常見(jiàn)的并發(fā)癥，其次為胰腺炎。

胰腺； 先天畸形； 糖尿病； 胰腺炎

背側(cè)胰腺發(fā)育不全是一種罕見(jiàn)的胰腺先天畸形，屬于常染色體遺傳性疾病，以胰腺體尾部不發(fā)育或發(fā)育不全為主要特點(diǎn)。既往由于認(rèn)識(shí)不足，也有文獻(xiàn)描述其為“短胰腺”、“球形胰腺”等?；颊叱２l(fā)糖尿病、胰腺炎等諸多并發(fā)癥，并可伴隨多器官發(fā)育畸形[1-3]。隨著影像診斷技術(shù)的發(fā)展，近年來(lái)背側(cè)胰腺發(fā)育不全病例報(bào)道越來(lái)越多，但對(duì)該病的臨床特征及并發(fā)癥尚無(wú)系統(tǒng)報(bào)道。本研究通過(guò)檢索國(guó)內(nèi)外文獻(xiàn)，系統(tǒng)分析背側(cè)胰腺發(fā)育不全的臨床特征、并發(fā)癥及合并癥的情況。

資料與方法

一、文獻(xiàn)資料

2015年6月以“短胰”、“胰腺發(fā)育不全”、“球形胰腺”、“背胰”作為檢索詞，對(duì)維普科技期刊數(shù)據(jù)庫(kù)、中國(guó)生物醫(yī)學(xué)文獻(xiàn)服務(wù)系統(tǒng)(sinomed)、中國(guó)知網(wǎng)全文期刊數(shù)據(jù)庫(kù)進(jìn)行檢索，共檢索到相關(guān)文獻(xiàn)6篇，剔除重復(fù)病例2例，共計(jì)21例[2-7]。以“dorsal pancreas agenesis”、“short pancreas”、“pancreas hypoplasia”作為檢索詞對(duì)PubMed數(shù)據(jù)庫(kù)進(jìn)行檢索，剔除重復(fù)及不相關(guān)文獻(xiàn)，共納入文獻(xiàn)61篇，共計(jì)70例病例[8-68]。國(guó)內(nèi)、外文獻(xiàn)中的病例總計(jì)91例。

二、方法

分析背側(cè)胰腺發(fā)育不全患者的臨床表現(xiàn)，胰頭特征，并發(fā)糖尿病、胰腺炎及合并其他器官先天畸形的情況。

結(jié)果

一、背側(cè)胰腺發(fā)育不全患者的臨床表現(xiàn)

患者常以腹痛為主要表現(xiàn)，占比61.5%；其次是無(wú)癥狀、于健康體檢時(shí)發(fā)現(xiàn)背側(cè)胰腺發(fā)育不全，占比15.3%；其他少見(jiàn)癥狀包括黃疸、乏力、上腹不適、糖尿病等。體格檢查以腹部壓痛為主要表現(xiàn)，占比62.3%，余無(wú)特征性表現(xiàn)。實(shí)驗(yàn)室檢查常無(wú)異常，部分患者并發(fā)糖尿病、黃疸，胰腺炎可有相應(yīng)生物化學(xué)指標(biāo)改變。影像學(xué)檢查以胰頭異常表現(xiàn)為主。

二、背側(cè)胰腺發(fā)育不全胰頭特征

28例未描述胰頭情況。余63例中39例(61.9%)胰頭異常，其中胰頭增大25例次(64.1%)，胰頭縮小4例次(10.3%)，胰頭鈣化2例次(5.1%)，形狀不規(guī)則、環(huán)形胰腺、合并腫瘤等14例次(35.9%)。胰頭正常24例(38.1%)。

三、背側(cè)胰腺發(fā)育不全并發(fā)糖尿病

24例未描述血糖水平。余67例中38例(56.7%)并發(fā)糖耐量受損或糖尿病，其中18例為胰島素依賴，占血糖異常患者的47.4%。血糖正常29例(43.3%)。

四、背側(cè)胰腺發(fā)育不全并發(fā)胰腺炎

16例未描述是否并發(fā)胰腺炎。余75例中20例(26.7%)并發(fā)胰腺炎，其中15例(75.0%)為急性胰腺炎，1例(5.0%)為復(fù)發(fā)性胰腺炎，4例(20.0%)為慢性胰腺炎。未并發(fā)胰腺炎55例(73.3%)。

五、背側(cè)胰腺發(fā)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%)合并脾臟畸形，8例次(24.2%)合并心臟畸形，17例次(51.5%)合并多器官畸形(包括胃腸道畸形、奇靜脈與下腔靜脈融合、十二指腸及膽道閉鎖、腎臟缺如等)。未合并其他器官畸形58例(63.7%)。

討論

胚胎發(fā)育過(guò)程中內(nèi)胚層分化形成腹側(cè)胰腺和背側(cè)胰腺并在妊娠第7周時(shí)彼此融合，最終，腹側(cè)胰腺形成胰腺鉤突、胰頭的絕大部分及主胰管胰頭段，而背側(cè)胰腺則形成胰腺體尾部、小部分胰頭以及副胰管和體尾部主胰管[1]，這一過(guò)程中任何基因突變或表達(dá)異常均有可能導(dǎo)致胰腺發(fā)育不全。最新研究發(fā)現(xiàn)，同源異型基因GATA6和GATA4以及人類HNF1B基因參與胰腺發(fā)育調(diào)控，其中GATA6基因突變是最常見(jiàn)的導(dǎo)致胰腺發(fā)育不全的原因，該基因突變還常常導(dǎo)致先天性心臟病。而GATA4基因突變除了導(dǎo)致胰腺發(fā)育不全，還會(huì)導(dǎo)致新生兒或兒童糖尿病[69-71]。人類HNF1B基因則對(duì)胰腺的形態(tài)發(fā)育以及胰腺內(nèi)分泌細(xì)胞分化均具有調(diào)控作用[72]。

背側(cè)胰腺發(fā)育不全的診斷主要依靠影像學(xué)檢查，典型征象包括胰腺僅有頭部殘留，胰頭可增大、正常或縮小，胰腺體尾部部分缺失。考慮到慢性胰腺炎胰腺萎縮、胰腺脂肪浸潤(rùn)或者胰腺遠(yuǎn)端切除的患者影像學(xué)檢查也可表現(xiàn)為上述征象，因此有部分學(xué)者提出以完全或部分背側(cè)胰管缺失作為背側(cè)胰腺發(fā)育不全的診斷標(biāo)準(zhǔn)[1,49,61,73]。影像學(xué)檢查手段主要包括US、CT、MRCP、ERCP以及EUS，其中US檢查容易受胃腸道氣體的干擾，診斷的敏感性及特異性較低，僅可作為初篩。若不能除外胰腺發(fā)育不全，則推薦MRCP作為首選檢查，因?yàn)镃T的胰管顯示效果不及MRI，且具有放射性，而ERCP和EUS屬于有創(chuàng)性檢查，并有誘發(fā)急性胰腺炎的風(fēng)險(xiǎn)[35,41]。本研究結(jié)果顯示，91例背側(cè)胰腺發(fā)育不全患者影像學(xué)檢查的胰頭異常發(fā)生率61.9%，其中除胰頭增大外，合并胰頭萎縮、環(huán)形胰腺及腫瘤、胰頭鈣化等情況占所有胰頭異常病例的51.3%，表明背側(cè)胰腺發(fā)育不全患者易并發(fā)其他胰腺疾患，而胰頭單純性增大則常被認(rèn)為是其代償所致[73]。

背側(cè)胰腺發(fā)育不全患者常無(wú)明顯癥狀而偶然發(fā)現(xiàn)，或表現(xiàn)為反復(fù)發(fā)作的急性胰腺炎及糖尿病[65,74]。本組91例中胰腺炎總體發(fā)生率為26.7%，其中急性胰腺炎占絕大部分。關(guān)于背側(cè)胰腺發(fā)育不全并發(fā)急性胰腺炎的機(jī)制目前還不清楚，大部分學(xué)者認(rèn)為可能是因?yàn)閛ddi括約肌功能失調(diào)、殘存的腹側(cè)胰腺胰液分泌代償性增多以及胰管內(nèi)壓升高所致[67,75]。也有學(xué)者認(rèn)為患者基因易感性與并發(fā)胰腺炎具有重要關(guān)系。由于背側(cè)胰腺發(fā)育不全的基因?qū)W基礎(chǔ)復(fù)雜，常合并有多基因異常，這些異常的基因可同時(shí)增加患者對(duì)急、慢性胰腺炎的易感性[35]。

多篇研究報(bào)道糖尿病是背側(cè)胰腺發(fā)育不全最常見(jiàn)的并發(fā)癥，但所納入的病例常常較少，數(shù)據(jù)差異較大[1,16,35]。本組糖尿病或糖耐量受損的發(fā)生率為56.7%，其中近一半患者需要胰島素治療，與Schnedl等[1]的數(shù)據(jù)基本一致。既往這部分患者大多被診斷為2型糖尿病，但根據(jù)美國(guó)糖尿病協(xié)會(huì)最新分型標(biāo)準(zhǔn)，繼發(fā)于胰腺外分泌疾患的糖尿病應(yīng)被劃分為3c型，且已有研究報(bào)道在一個(gè)背側(cè)胰腺發(fā)育不全家系中家系成員都存在肝細(xì)胞胰島素抵抗，這也與3c型糖尿病的病理生理特點(diǎn)相吻合[76-77]。此外，因?yàn)楸硞?cè)胰腺發(fā)育不全在某種程度上類似胰腺體尾部切除術(shù)，而胰腺手術(shù)正是3c型糖尿病主要病因之一。根據(jù)2012年國(guó)際胰腺病研討會(huì)的指南建議，3c型糖尿病由于其特殊的病理生理特點(diǎn)，治療應(yīng)采取個(gè)體化策略[78]。

背側(cè)胰腺發(fā)育不全患者還常合并多種其他器官先天畸形。本組合并其他器官先天畸形的發(fā)生率高達(dá)36.2%，以多脾綜合征最為常見(jiàn)，其次是心臟畸形，且合并畸形的患者中超過(guò)半數(shù)是多器官畸形，包括內(nèi)臟反位、胃腸道畸形、奇靜脈與下腔靜脈融合、十二指腸及膽道閉鎖等[1,58]。提示背側(cè)胰腺發(fā)育不全患者如出現(xiàn)腫瘤、占位等需手術(shù)治療的情況時(shí)，術(shù)前應(yīng)仔細(xì)評(píng)估術(shù)區(qū)及毗鄰解剖結(jié)構(gòu)。

總之，背側(cè)胰腺發(fā)育不全是一種罕見(jiàn)的常染色體遺傳性疾病，以胰腺體尾部缺失為主要特點(diǎn)，診斷以背側(cè)胰管缺失為主要標(biāo)準(zhǔn)?；颊叱２l(fā)糖尿病、胰腺炎以及伴有多種其他器官先天畸形，其中糖尿病是最常見(jiàn)的并發(fā)癥，近一半的糖尿病患者需要胰島素治療。胰腺炎的發(fā)生可能與患者基因易感性相關(guān)。對(duì)于需要手術(shù)的患者，術(shù)前應(yīng)仔細(xì)評(píng)估術(shù)區(qū)及毗鄰器官的異常。

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(本文編輯：呂芳萍)

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，第二軍醫(yī)大學(xué)長(zhǎng)海醫(yī)院內(nèi)分泌科(蘇松、徐茂錦)，消化內(nèi)科(錢陽(yáng)陽(yáng)、顧園婷、朱曙光、王天驕、郭曉榕、廖專、李兆申)

徐茂錦，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)