蛋白丢失性胃肠病低蛋白血症及水肿病理机制探讨与中西医治疗方法研究进展
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摘要
蛋白丢失性胃肠病(PLG)是多种病因所致大量血浆蛋白自消化道丢失,引发低蛋白血症与水肿的一组疾病,临床主要表现为局部水肿和低蛋白血症。明确该病的病理机制是治疗的基础,临床主张先对基础疾病进行治疗,在难以治愈的情况下,可采取对症治疗。但目前PLG确切的发病机制尚存争议,亦无特效治疗方法。为此,该文探讨了PLG型水肿与低蛋白血症的病理机制,并综述了近年来中西医临床治疗药物与方法,旨在为筛选针对PLG的特效治疗药物、适宜防治方法、准确诊断标准奠定文献基础。
Protein-losing gastrointestinal disease(PLG)is a group of diseases in which a large number of plasma proteins are lost from the digestive tract caused by various etiologies,causing hypoproteinemia and edema. The main clinical manifestations are local edema and hypoproteinemia. It is clear that the pathological mechanism of the disease is the basis of treatment. The clinical claim is to treat the underlying disease first,and in the case of difficulty in curing,symptomatic treatment can be taken. However,the exact pathogenesis of PLG is still controversial and there is no specific treatment. To this end,this article explores the pathological mechanism of PLG-type edema and hypoproteinemia,and reviews the clinical and therapeutic drugs and methods of traditional Chinese and Western medicine in recent years,aiming at screening for specific therapeutic drugs for PLG,suitable prevention and treatment methods,and accurate diagnostic criteria for establishing literature basis.
Protein-losing gastrointestinal disease(PLG)is a group of diseases in which a large number of plasma proteins are lost from the digestive tract caused by various etiologies,causing hypoproteinemia and edema. The main clinical manifestations are local edema and hypoproteinemia. It is clear that the pathological mechanism of the disease is the basis of treatment. The clinical claim is to treat the underlying disease first,and in the case of difficulty in curing,symptomatic treatment can be taken. However,the exact pathogenesis of PLG is still controversial and there is no specific treatment. To this end,this article explores the pathological mechanism of PLG-type edema and hypoproteinemia,and reviews the clinical and therapeutic drugs and methods of traditional Chinese and Western medicine in recent years,aiming at screening for specific therapeutic drugs for PLG,suitable prevention and treatment methods,and accurate diagnostic criteria for establishing literature basis.
引文
[1]朱晶晶,胡秀,徐秀英.蛋白丢失性肠病的临床特点分析[J].胃肠病学和肝病学杂志,2012,21(4):366-369.
[2]Amiot A. Protein-losing enteropathy[J]. Rev Med Interne,2015,36(7):467-473.
[3]Jr M R,Guyton A C. Effects of hypoproteinemia on fluid volumes and arterial pressure[J]. Am J Physiol,1983,245(2):284-293.
[4]李建国. Starling原理在液体治疗中意义[J].中国实用外科杂志,2015(2):155-159.
[5]Fadnes H O. Protein Concentration and Hydrostatic Pressure in Subcutaneous Tissue of Rats in Hypoproteinemia[J].Scandinavian Journal of Clinical&Laboratory Investigation,1975,35(5):441-446.
[6]Aukland K,Johnsen H M. Protein concentration and colloid osmotic pressure of rat skeletal muscle interstitial fluid[J]. Acta Physiologica,1974,91(3):354-364.
[7]Chang J M,Hwang S J,Chen H C,et al. Edema due to proteinlosing enteropathy--a disorder rarely considered by nephrologists[J]. Clinical Nephrology,2002,57(5):392.
[8]Deschênes G,Feraille E,Doucet A. Mechanisms of oedema in nephrotic syndrome:old theories and new ideas[J]. Nephrol Dial Transplant,2003,18(3):454-456.
[9]Lima M S,Dos S B V,Zeinad A,et al. Association of proteinlosing enteropathy caused by eosinophilic gastroenteritis with essential thrombocytosis:case report[J]. Clinics,2006,61(61):271-274.
[10]Umar S B,Dibaise J K. Protein-losing enteropathy:case illustrations and clinical review[J]. American Journal of Gastroenterology,2010,105(1):43-49.
[11]Horowitz I N,Tai K. Hypoalbuminemia in critically ill children[J]. Arch Pediatr Adolesc Med,2007,161(11):1048-1052.
[12]刘倩,王立中,陈梦娜.低蛋白血症对慢性心力衰竭预后的影响分析[J].中国当代医药,2018,25(19):24-27.
[13]苏艺胜,张北平,赵喜颖,等.溃疡性结肠炎合并蛋白丢失性肠病、多发血栓形成1例[J].广东医学,2018,39(4):15-16.
[14]徐美华,贺帼英,张桂英,等.以低蛋白血症为主要表现的蛋白丢失性胃肠病病因和误诊分析[J].中国现代医学杂志,2010,20(3):422-425.
[15]Kobayashi Y,Shimojima Y,Kondo Y,et al. Protein-losing Gastroenteropathy Related to Mixed Connective Tissue Disease:A Case Report of a Successful Outcome and Literature Review[J].Internal Medicine,2017,56(15):2057-2062.
[16]陈真,苏丽,李梦涛,等.系统性红斑狼疮并发蛋白丢失性肠病的危险因素[J].中华临床免疫和变态反应杂志,2017,11(3):264-268.
[17]Iwasa T,Matsubayashi N. Protein-loosing enteropathy associated with rotavirus infection in an infant[J]. World Journal of Gastroenterology,2008,14(10):1630-1632.
[18]Kurolap A,Eshach-Adiv O,Gonzaga-Jauregui C,et al.Establishing the role of PLVAP in protein-losing enteropathy:a homozygous missense variant leads to an attenuated phenotype[J].Journal of Medical Genetics,2018,1(5):299.
[19]闰斌,刘庆森,黄启阳,等.原发性小肠淋巴管扩张症1例并文献复习[J].胃肠病学和肝病学杂志,2013,22(5):473-474.
[20]Vignes S,Bellanger J. Primary intestinal lymphangiectasia(Waldmann's disease)[J]. Orphanet Journal of Rare Diseases,2008,3(1):1-8.
[21]Gras P,Gottrand F,Godart F. Gastrointestinal haemorrhage due to lymphangiectasia caused by protein-losing enteropathy in the Fontan circulation[J]. Cardiology in the Young,2017,27(8):1.
[22]Bendixen G,Jarnum S,Soltoft J,et al. IgG and albumin turnover in Crohn's disease[J]. Scandinavian Journal of Gastroenterology,2010,3(5):481-489.
[23]LevittDG,LevittMD.Proteinlosingenteropathy:comprehensive review of the mechanistic association with clinical and subclinical disease states[J]. Clinical&Experimental Gastroenterology,2017,10:147.
[24]王新华.中医治疗蛋白丢失性胃肠病的体会[J].中医杂志,2001,42(12):715-716.
[25]庄睿丹,唐鲁静,方优红,等.儿童肠内营养47例临床分析[J].中华儿科杂志,2016,54(7):500-503.
[26]Kienast W,Haeseler K,Herterich R. Protein-losing enteropathy following the Fontan procedure in a child with intestinal lactase deficiency treated with lactose-free diet[J]. Cardiology in the Young,2014,24(1):175-177.
[27]Rivet C,Lapalus M G,Dumortier J,et al. Use of capsule endoscopy in children with primary intestinal lymphangiectasia[J]. Gastrointestinal Endoscopy,2006,64(4):649-650.
[28]Mizuochi T,Suda K,Seki Y,et al. Successful diuretics treatment of protein-losing enteropathy in Noonan syndrome[J].Pediatrics International,2015,57(2):39-41.
[29]Kijmassuwan T,Tanpowpong P,Molagool S,et al. A rare cause of multiple small bowel ulcers and strictures in a 10-yearold child[J]. Turkish Journal of Gastroenterology the Official Journal of Turkish Society of Gastroenterology,2018,29(1):110.
[30]Ozeki M,Hori T,Kanda K,et al. Everolimus for Primary Intestinal Lymphangiectasia With Protein-Losing Enteropathy[J].Pediatrics,2016,137(3):1-5.
[31]Kobayashi Y,Shimojima Y,Kondo Y,et al. Protein-losing Gastroenteropathy Related to Mixed Connective Tissue Disease:A Case Report of a Successful Outcome and Literature Review[J].Internal Medicine,2017,56(15):2057-2062.
[32]SansinaneaP,CarricaSA,MarcosJ,etal.Proteinlosingenteropathyassociatedwithrefractorysystemic lupus erythematosus with a good response to rituximab[J].Reumatologia Clinica,2016,12(1):47-49.
[33]Cherqaoui B,Chausset A,Stephan J L,et al. Intravenous immunoglobulins for severe gastrointestinal involvement in pediatric Henoch-Sch?nlein purpura:A French retrospective study[J]. Archives De Pediatrie,2016,23(6):584-590.
[34]Shah S N,Todoric K,Tarrant T K. Improved outcomes on subcutaneous IgG in patients with humoral immunodeficiency and co-morbid bowel disease[J]. Clinical Case Reports&Reviews,2015,1(7):151.
[35]IwasakiK,MorimotoM,OtaG,etal.Partialsmall intestinal resection for successful surgical management of refractory protein-losing gastroenteropathy in systemic lupus erythematosus:A case report and literature review[J].Medicine,2018,97(30):11357.
[36]Fiske W H,Tanksley J,Nam K T,et al. Efficacy of cetuximab in the treatment of Menetrier's disease[J]. Science Translational Medicine,2009,1(8):8-18.
[37]TamuraK,MatsudaK,YokoyamaS,etal.Successful laparoscopic resection for cap polyposis:case report,literature review[J]. Surgical Case Reports,2018,4(1):69.
[38]Seul Gi Cha,Gi Beom Kim,Chung Il Noh,et al. A new surgical strategy for medically uncontrolled protein-losing enteropathy after Fontan surgery[J].Interactive CardioVascular and Thoracic Surgery,2018,10(10):629-631.
[39]Okada S,Muneuchi J,Nagatomo Y,et al. Successful Treatment of Protein-Losing Enteropathy and Plastic Bronchitis by Biphasic Cuirass Ventilation in a Patient with Failing Fontan Circulation[J]. International Heart Journal,2018,59(4):873-876.
[40]Dho S H,Lim J C,Kim L K. Beyond the Role of CD55 as a Complement Component[J]. Immune Netw,2018,18(1):11.
[41]Ozen A,Comrie W A,Ardy R C,et al. CD55 Deficiency,EarlyOnset Protein-Losing Enteropathy,and Thrombosis[J]. N Engl J Med,2017,377(1):52-61.
[42]Gray G M,Cooper H L. Protein digestion and absorption[J].Gastroenterology,1971,61(4):535-544.
[2]Amiot A. Protein-losing enteropathy[J]. Rev Med Interne,2015,36(7):467-473.
[3]Jr M R,Guyton A C. Effects of hypoproteinemia on fluid volumes and arterial pressure[J]. Am J Physiol,1983,245(2):284-293.
[4]李建国. Starling原理在液体治疗中意义[J].中国实用外科杂志,2015(2):155-159.
[5]Fadnes H O. Protein Concentration and Hydrostatic Pressure in Subcutaneous Tissue of Rats in Hypoproteinemia[J].Scandinavian Journal of Clinical&Laboratory Investigation,1975,35(5):441-446.
[6]Aukland K,Johnsen H M. Protein concentration and colloid osmotic pressure of rat skeletal muscle interstitial fluid[J]. Acta Physiologica,1974,91(3):354-364.
[7]Chang J M,Hwang S J,Chen H C,et al. Edema due to proteinlosing enteropathy--a disorder rarely considered by nephrologists[J]. Clinical Nephrology,2002,57(5):392.
[8]Deschênes G,Feraille E,Doucet A. Mechanisms of oedema in nephrotic syndrome:old theories and new ideas[J]. Nephrol Dial Transplant,2003,18(3):454-456.
[9]Lima M S,Dos S B V,Zeinad A,et al. Association of proteinlosing enteropathy caused by eosinophilic gastroenteritis with essential thrombocytosis:case report[J]. Clinics,2006,61(61):271-274.
[10]Umar S B,Dibaise J K. Protein-losing enteropathy:case illustrations and clinical review[J]. American Journal of Gastroenterology,2010,105(1):43-49.
[11]Horowitz I N,Tai K. Hypoalbuminemia in critically ill children[J]. Arch Pediatr Adolesc Med,2007,161(11):1048-1052.
[12]刘倩,王立中,陈梦娜.低蛋白血症对慢性心力衰竭预后的影响分析[J].中国当代医药,2018,25(19):24-27.
[13]苏艺胜,张北平,赵喜颖,等.溃疡性结肠炎合并蛋白丢失性肠病、多发血栓形成1例[J].广东医学,2018,39(4):15-16.
[14]徐美华,贺帼英,张桂英,等.以低蛋白血症为主要表现的蛋白丢失性胃肠病病因和误诊分析[J].中国现代医学杂志,2010,20(3):422-425.
[15]Kobayashi Y,Shimojima Y,Kondo Y,et al. Protein-losing Gastroenteropathy Related to Mixed Connective Tissue Disease:A Case Report of a Successful Outcome and Literature Review[J].Internal Medicine,2017,56(15):2057-2062.
[16]陈真,苏丽,李梦涛,等.系统性红斑狼疮并发蛋白丢失性肠病的危险因素[J].中华临床免疫和变态反应杂志,2017,11(3):264-268.
[17]Iwasa T,Matsubayashi N. Protein-loosing enteropathy associated with rotavirus infection in an infant[J]. World Journal of Gastroenterology,2008,14(10):1630-1632.
[18]Kurolap A,Eshach-Adiv O,Gonzaga-Jauregui C,et al.Establishing the role of PLVAP in protein-losing enteropathy:a homozygous missense variant leads to an attenuated phenotype[J].Journal of Medical Genetics,2018,1(5):299.
[19]闰斌,刘庆森,黄启阳,等.原发性小肠淋巴管扩张症1例并文献复习[J].胃肠病学和肝病学杂志,2013,22(5):473-474.
[20]Vignes S,Bellanger J. Primary intestinal lymphangiectasia(Waldmann's disease)[J]. Orphanet Journal of Rare Diseases,2008,3(1):1-8.
[21]Gras P,Gottrand F,Godart F. Gastrointestinal haemorrhage due to lymphangiectasia caused by protein-losing enteropathy in the Fontan circulation[J]. Cardiology in the Young,2017,27(8):1.
[22]Bendixen G,Jarnum S,Soltoft J,et al. IgG and albumin turnover in Crohn's disease[J]. Scandinavian Journal of Gastroenterology,2010,3(5):481-489.
[23]LevittDG,LevittMD.Proteinlosingenteropathy:comprehensive review of the mechanistic association with clinical and subclinical disease states[J]. Clinical&Experimental Gastroenterology,2017,10:147.
[24]王新华.中医治疗蛋白丢失性胃肠病的体会[J].中医杂志,2001,42(12):715-716.
[25]庄睿丹,唐鲁静,方优红,等.儿童肠内营养47例临床分析[J].中华儿科杂志,2016,54(7):500-503.
[26]Kienast W,Haeseler K,Herterich R. Protein-losing enteropathy following the Fontan procedure in a child with intestinal lactase deficiency treated with lactose-free diet[J]. Cardiology in the Young,2014,24(1):175-177.
[27]Rivet C,Lapalus M G,Dumortier J,et al. Use of capsule endoscopy in children with primary intestinal lymphangiectasia[J]. Gastrointestinal Endoscopy,2006,64(4):649-650.
[28]Mizuochi T,Suda K,Seki Y,et al. Successful diuretics treatment of protein-losing enteropathy in Noonan syndrome[J].Pediatrics International,2015,57(2):39-41.
[29]Kijmassuwan T,Tanpowpong P,Molagool S,et al. A rare cause of multiple small bowel ulcers and strictures in a 10-yearold child[J]. Turkish Journal of Gastroenterology the Official Journal of Turkish Society of Gastroenterology,2018,29(1):110.
[30]Ozeki M,Hori T,Kanda K,et al. Everolimus for Primary Intestinal Lymphangiectasia With Protein-Losing Enteropathy[J].Pediatrics,2016,137(3):1-5.
[31]Kobayashi Y,Shimojima Y,Kondo Y,et al. Protein-losing Gastroenteropathy Related to Mixed Connective Tissue Disease:A Case Report of a Successful Outcome and Literature Review[J].Internal Medicine,2017,56(15):2057-2062.
[32]SansinaneaP,CarricaSA,MarcosJ,etal.Proteinlosingenteropathyassociatedwithrefractorysystemic lupus erythematosus with a good response to rituximab[J].Reumatologia Clinica,2016,12(1):47-49.
[33]Cherqaoui B,Chausset A,Stephan J L,et al. Intravenous immunoglobulins for severe gastrointestinal involvement in pediatric Henoch-Sch?nlein purpura:A French retrospective study[J]. Archives De Pediatrie,2016,23(6):584-590.
[34]Shah S N,Todoric K,Tarrant T K. Improved outcomes on subcutaneous IgG in patients with humoral immunodeficiency and co-morbid bowel disease[J]. Clinical Case Reports&Reviews,2015,1(7):151.
[35]IwasakiK,MorimotoM,OtaG,etal.Partialsmall intestinal resection for successful surgical management of refractory protein-losing gastroenteropathy in systemic lupus erythematosus:A case report and literature review[J].Medicine,2018,97(30):11357.
[36]Fiske W H,Tanksley J,Nam K T,et al. Efficacy of cetuximab in the treatment of Menetrier's disease[J]. Science Translational Medicine,2009,1(8):8-18.
[37]TamuraK,MatsudaK,YokoyamaS,etal.Successful laparoscopic resection for cap polyposis:case report,literature review[J]. Surgical Case Reports,2018,4(1):69.
[38]Seul Gi Cha,Gi Beom Kim,Chung Il Noh,et al. A new surgical strategy for medically uncontrolled protein-losing enteropathy after Fontan surgery[J].Interactive CardioVascular and Thoracic Surgery,2018,10(10):629-631.
[39]Okada S,Muneuchi J,Nagatomo Y,et al. Successful Treatment of Protein-Losing Enteropathy and Plastic Bronchitis by Biphasic Cuirass Ventilation in a Patient with Failing Fontan Circulation[J]. International Heart Journal,2018,59(4):873-876.
[40]Dho S H,Lim J C,Kim L K. Beyond the Role of CD55 as a Complement Component[J]. Immune Netw,2018,18(1):11.
[41]Ozen A,Comrie W A,Ardy R C,et al. CD55 Deficiency,EarlyOnset Protein-Losing Enteropathy,and Thrombosis[J]. N Engl J Med,2017,377(1):52-61.
[42]Gray G M,Cooper H L. Protein digestion and absorption[J].Gastroenterology,1971,61(4):535-544.