蛋白質流失腸病變及其處置用藥介紹.indd

Transcription

1 Pharmaceutical Sciences 藥物科學蛋白質流失腸病變及其處置用藥介紹 摘要 巫櫻桃 1 蕭如君 1 許凱倫 2,3 1,3 李建瑩 1 中山醫學大學附設醫院藥劑科藥師 2 中山醫學大學附設醫院醫師 3 中山醫學大學醫學系 (protein-losing enteropathy, PLE) (hypoproteinemia) (hypoalbuminemia) (ascites) (pleural effusion) (pericardial effusion) PLE (primary intestinal lymphangiectasia) PLE PLE PLE protein-losing enteropathy (PLE) hypoalbuminemia edema 壹 前言 PLE (primary intestinal lymphangiectasia) ( ) PLE PLE 1 PLE (hypoproteinemia) (hypoalbuminemia) (ascites) (pleural effusion) (pericardial effusion) (albumin) (IgA, IgG, IgM) fibrinogen transferrin ceruloplasmin (proteinuria) 32 THE JOURNAL OF TAIWAN PHARMACY Vol.31 No.3 Sep

2 2 PLE / 1 2 貳 致病因 albumin 10% PLE PLE 一 發炎性滲出 (Crohn's disease) (ulcerative colitis) nonsteroidal anti-inflammatory drug (NSAID) 二 無侵蝕或潰瘍表現之黏膜性疾病 (menetrier's disease) (systemic lupus erythematosus) (rheumatoid arthritis) 三 淋巴管阻塞 (primary intestinal lymphangiectasia) fontan operation ( ) (mesenteric venous thrombosis) 藥物科學 fontan operation PLE fontan operation PLE 5-15% PLE 5 50% 2 參 診斷流程與臨床評估 PLE ( ) PLE albumin IgA, IgG, IgM fibrinogen transferrin ceruloplasmin PLE (primary intestinal lymphangiectasia) (lymphocytopenia) (steatorrhea) / (lymphatic obstruction) (congenital lymphangiectasia) (lymphocytopenia) 肆 PLE 治療一 飲食治療 PLE ( 2 g/kg/ (medium-chain triglyceride) 藥學雜誌 Sep

3 藥Pharmaceutical Sciences 物科學2 圖一蛋白質流失腸病變 ( protein-losing enteropathy, PLE) 的描述與評估 1 二 潛在疾病治療 (constrictive pericarditis) 2 三 輔助性藥物治療 ( 一 )Albumin Albumin albumin albumin PLE 3 ( 二 )Heparin Heparin (glycosaminoglycan) III (antithrombin III) Heparin PLE PLE heparin fontan operation PLE 4,5 heparin antithrombin III heparin 3 fontan operation PLE heparin heparin ( 3 ) albumin 1-antitrypsin heparin (osteopenia) heparin 5,000 units/m 2 34 THE JOURNAL OF TAIWAN PHARMACY Vol.31 No.3 Sep

4 4 albumin (furosemide) heparin 5,000 units/day albumin 1- antitrypsin PLE 5 ( 三 )Corticosteroid PLE (corticosteroid) prednisone 1-2 mg/kg/ PLE budesonide budesonide 6-9 mg/kg/ budesonide albumin PLE 6 budesonide budesonide ( 四 )Octreotide Octreotide somastatin analogue octreotide (200 g, ) 3 fontan operation PLE octreotide 藥物科學 heparin PLE 3 1 budesonide octreotide (Sandostatin LAR DEPOT) 20 mg/ budesonide (Cushing's syndrome) (adrenal insufficiency) octreotide 2 fontan operation PLE heparin 3 octreotide (Sandostatin LAR DEPOT) 10 mg/ 3 octreotide (Sandostatin 50 g)/ 3 octreotide albumin g/dl g/dl 3 7 octreotide fontan operation PLE 7 Octreotide PLE 8 octreotide Octreotide PLE octreotide Octreotide (22-33 %) 3 ( 五 )Sildenafil PLE (central venous pressure, CVP) CVP 12 mmhg PLE sildenafil ( 0.5- 藥學雜誌 Sep

5 藥Pharmaceutical Sciences 物科學2 mg/kg/dose/6h) PLE 9 ( 六 ) 其它 PLE PLE - albumin PLE 1. fontan operation PLE (total protein) albumin Cyclosporine (autoimmune disease) (scleroderma) PLE cyclosporine spironolactone fontan operation PLE Furosemide 1 mg/kg 伍 結論 PLE fontan operation PLE PLE The Introduction and Medication Management of Protein-losing Enteropathy Ying-Tao Wu 1, Ju-Chun Shiao 1, Kai-Lun Sheu 2,3, Chien-Ying Lee 1,3 Department of Pharmacy, Chung Shan Medical University Hospital 1 Department of Family and Community Medicine, Chung Shan Medical University Hospital 2 School of Medicine, Chung Shan Medical University 3 Abstract The main clinical feature of protein-losing enteropathy (PLE) is primarily characterized by excessive leakage of serum proteins into the gastrointestinal tract, resulting in common clinical manifestations such as hypoproteinemia, hypoalbuminemia, edema, malabsorption, 36 THE JOURNAL OF TAIWAN PHARMACY Vol.31 No.3 Sep

6 pleural effusion or pericardial effusion may even appear. There are many factors those might cause PLE, but most patients do not show serious clinical signs. However, for persons having primary intestinal lymphangiectasia, autoimmune disease or post-operation congenital heart disease leading to PLE, high mortality rate is frequently observed. Although the treatment of PLE has advanced greatly in recent years, this disease is still difficult to treat. Several successful clinical cases from the past can help us provide better care in managing patients with PLE. 參考資料 : 1. Johnson JN, Driscoll DJ, O'Leary PW: Protein-Losing Enteropathy and the Fontan Operation. Nutr Clin Pract 2012;27: Milovic V, Grand RJ: Protein-losing gastroenteropathy. Retrieved from UpToDate clinical knowledge base, institutional edition. This literature review current through 2015 Jan. 3. John AS, Johnson JA, Khan M, et al: Clinical Outcomes and Improved Survival in Patients With Protein- Losing Enteropathy After the Fontan Operation. JACC 2014;64: Donnelly JP, Rosenthal A, Castle VP, et al: Reversal of protein-losing enteropathy with heparin therapy in three patients with univentricular hearts and Fontan palliation. J Pediatr 1997;130: Kelly AM, Feldt RH, Driscoll DJ, et al: Use of heparin in the treatment of protein-losing enteropathy after fontan operation for complex congenital heart disease. Mayo Clin Proc 1998;73: Rychik J, Piccoli DA, Barber G: Usefulness of corticosteroid therapy for protein-losing enteropathy after the Fontan procedure. Am J Cardiol 1991; 68: John AS, Phillips SD, Driscoll DJ, et al: The Use of Octreotide to Successfully Treat Protein-losing Enteropathy Following the Fontan Operation. Congenit Heart Dis 2011;6: Thacker D, Patel A, Dodds K, et al: Use of oral budesonide in the management of protein-losing enteropathy after the Fontan operation. Ann Thorac Surg 2010;89: Ohuchi H, Yasuda K, Miyazaki A, et al: Haemodynamic characteristics before and after the onset of protein losing enteropathy in patients after the Fontan operation. European Journal of Cardio-Thoracic Surgery 2013; 43: Kim SJ, Park IS, Song JY, et al: Reversal of protein-losing enteropathy with calcium replacement in a patient after Fontan operation. Ann Thorac Surg 2004;77: Thacker D, Patel A, Dodds K, et al: Use of oral budesonide in the management of protein-losing enteropathy after the Fontan operation. Ann Thorac Surg 2010; 89: Ringel RE, Peddy SB: Effect of high-dose spironolactone on protein-losing enteropathy in patients with Fontan palliation of complex congenital heart disease. Am J Cardiol 2003;91: Elinoff JM, Rame JE, Forfia PR, et al: A pilot study of the effect of spironolactone therapy on exercise capacity and endothelial dysfunction in pulmonary arterial hypertension: study protocol for a randomized controlled trial. Trials 2013;14: Sep 藥學雜誌 藥物科學body weight loss and electrolyte imbalance. In some rare cases, symptoms such as ascites,