Protein-Losing Enteropathy

Protein-Losing Enteropathy

No Results

No Results

processing….

Protein-losing enteropathy is characterized by the severe loss of serum proteins into the intestine. [1] Normal protein loss in the gastrointestinal tract mainly consists of sloughed enterocytes and pancreatic and biliary secretions. Albumin loss through the gastrointestinal tract normally accounts for 2-15% of the total body degradation of albumin, but, in patients with severe protein-losing gastrointestinal disorders, the enteric protein loss may reach up to 60% of the total albumin pool.

The serum protein level reflects the balance between protein synthesis, metabolism, and protein loss. Protein-losing enteropathy is characterized by more loss of proteins via the gastrointestinal tract than synthesis leading to hypoalbuminemia. It is not a single disease, but an atypical manifestation of other diseases. [2]

The pathophysiology of this disorder is directly related to the excessive leakage of plasma proteins into the lumen of the gastrointestinal tract. Mechanisms for gastrointestinal protein loss include lymphatic obstruction, mucosal disease with erosions, ulcerations, or increased mucosal permeability to proteins as a result of cell damage or death. Proteins entering the gastrointestinal tract are metabolized into constituent amino acids by gastric, pancreatic, and small intestinal enzymes and are reabsorbed. When the rate of gastrointestinal protein loss exceeds the body’s capacity to synthesize new proteins, hypoproteinemia develops. [3]

Primary gastrointestinal mucosal diseases (typically ulcerative/erosive) include the following:

Erosions or ulcerations of the esophagus, stomach, or duodenum

Regional enteritis

Graft versus host disease

Pseudomembranous colitis (Clostridium difficile) [4]

Mucosal-based neoplasia

Carcinoid syndrome

Idiopathic ulcerative jejunoileitis

Amyloidosis [5]

Kaposi sarcoma

Protein dyscrasia

Ulcerative colitis

Neurofibromatosis

Cytomegalovirus infection [6]

Increased interstitial pressure or lymphatic obstruction leading to protein loss can be caused by the following:

Tuberculosis

Sarcoidosis

Retroperitoneal fibrosis

Lymphoma

Intestinal endometriosis

Lymphoenteric fistula

Whipple disease

Cardiac disease (constrictive pericarditis or congestive heart failure)

Intestinal lymphangiectasia [7]

Nonerosive upper gastrointestinal diseases include the following:

Cutaneous burns [8]

Whipple disease

Connective tissue disorders

Acquired immunodeficiency syndrome (AIDS) [2]

Enteropathy, such as angioedema (idiopathic or hereditary) and Henoch-Schönlein purpura

Celiac sprue

Tropical sprue

Allergic gastroenteritis

Eosinophilic gastroenteritis

Giant hypertrophic gastritis (Ménétrier disease) [9, 10, 11]

Bacterial overgrowth

Intestinal parasites

Microscopic colitis

Dientamoeba fragilis [12]

Cases in which protein-losing enteropathy was the initial manifestation of systemic lupus erythematosus have been reported. [13, 14]

Protein-losing enteropathy can also occur as a complication of the Fontan procedure, an operation for several congenital heart abnormalities; the surgery allows systemic venous blood to reach the lungs without circulating through a ventricle. [15, 16, 17]  In children, protein-losing enteropathy may occur before or after the procedure, especially in the setting of hemodynamic disturbances. [18]

The prevalence rate is not known.

The prevalence rate is not known.

No racial, sexual, or age predilection exists.

A retrospective study by John et al indicated that the survival rate has increased for patients who develop protein-losing enteropathy as a complication of the Fontan procedure, an operation for the treatment of several types of congenital heart abnormalities. Although the 5-year mortality rate has been reported to be 50% for patients who develop protein-losing enteropathy following the surgery, the investigators found that in 42 such patients identified from Mayo Clinic clinical databases, the survival rate at 5 years after the diagnosis of enteropathy was 88%. [16]

The survival rate tended to be lower, however, in patients with a high Fontan pressure, reduced ventricular function (ejection fraction < 55%), and a New York Heart Association functional classification of greater than 2. Treatments associated with a higher survival rate included spironolactone, octreotide, and sildenafil, as well as the creation of fenestrae and Fontan obstruction relief. [16]

Morbidity and mortality of this condition directly relate to its cause, either primary gastrointestinal disease or a multisystem disorder.

Lee YT, Sung JJ. Protein-losing enteropathy. Gastrointest Endosc. 2004 Nov. 60(5):801-2. [Medline].

Laine L, Garcia F, McGilligan K, et al. Protein-losing enteropathy and hypoalbuminemia in AIDS. AIDS. 1993 Jun. 7(6):837-40. [Medline].

Waldmann TA, Wochner RD, Strober W. The role of the gastrointestinal tract in plasma protein metabolism. Studies with 51Cr-albumin. Am J Med. 1969 Feb. 46(2):275-85. [Medline].

Rybolt AH, Bennett RG, Laughon BE, et al. Protein-losing enteropathy associated with Clostridium difficile infection. Lancet. 1989 Jun 17. 1(8651):1353-5. [Medline].

Akgun A, Tani Acar E, Taner MS, et al. Scintigraphic diagnosis of protein-losing enteropathy secondary to amyloidosis. Turk J Gastroenterol. 2005 Mar. 16(1):41-3. [Medline].

Cheong JL, Cowan FM, Modi N. Gastrointestinal manifestations of postnatal cytomegalovirus infection in infants admitted to a neonatal intensive care unit over a five year period. Arch Dis Child Fetal Neonatal Ed. 2004 Jul. 89(4):F367-9. [Medline].

Pratz KW, Dingli D, Smyrk TC, et al. Intestinal lymphangiectasia with protein-losing enteropathy in Waldenstrom macroglobulinemia. Medicine (Baltimore). 2007 Jul. 86(4):210-4. [Medline].

Venkatesh B, Gough J, Ralston DR, et al. Protein losing enteropathy in critically ill adult patients with burns: a preliminary report. Intensive Care Med. 2004 Jan. 30(1):162-6. [Medline].

Citrin Y, Sterling K, Halsted JA. The mechanism of hypoproteinemia associated with giant hypertrophy of the gastric mucosa. N Engl J Med. 1957 Nov 7. 257(19):906-12. [Medline].

Overholt BF, Jeffries GH. Hypertrophic, hypersecretory protein-losing gastropathy. Gastroenterology. 1970 Jan. 58(1):80-7. [Medline].

Fretzayas A, Moustaki M, Alexopoulou E, Nicolaidou P. Menetrier’s disease associated with Helicobacter pylori: three cases with sonographic findings and a literature review. Ann Trop Paediatr. 2011. 31(2):141-7. [Medline].

Ito R, Sakagami J, Kataoka K, et al. Chronic diarrhea and protein-losing gastroenteropathy caused by Dientamoeba fragilis. J Gastroenterol. 2004 Nov. 39(11):1117-9. [Medline].

Murali A, Narasimhan D, Krishnaveni J, Rajendiran G. Protein losing enteropathy in systemic lupus erythematosus. J Assoc Physicians India. 2013 Oct. 61(10):747-9. [Medline].

Lim DH, Kim YG, Bae SH, et al. Factors related to outcomes in lupus-related protein-losing enteropathy. Korean J Intern Med. 2015 Nov. 30(6):906-12. [Medline].

Vyas H, Driscoll DJ, Cetta F, et al. Gastrointestinal bleeding and protein-losing enteropathy after the Fontan operation. Am J Cardiol. 2006 Sep 1. 98(5):666-7. [Medline].

John AS, Johnson JA, Khan M, et al. Clinical outcomes and improved survival in patients with protein-losing enteropathy after the Fontan operation. J Am Coll Cardiol. 2014 Jul 8. 64(1):54-62. [Medline].

Dori Y, Keller MS, Fogel MA, et al. MRI of lymphatic abnormalities after functional single-ventricle palliation surgery. AJR Am J Roentgenol. 2014 Aug. 203(2):426-31. [Medline].

Patel JK, Loomes KM, Goldberg DJ, Mercer-Rosa L, Dodds K, Rychik J. Early impact of Fontan operation on enteric protein loss. Ann Thorac Surg. 2016 Mar. 101(3):1025-30. [Medline].

Waldmann TA. Protein-losing enteropathy. Gastroenterology. 1966 Mar. 50(3):422-43. [Medline].

Chau TN, Mok MY, Chan EY, et al. Evaluation of performance of measurement of faecal a(1)-antitrypsin clearance and technetium-99m human serum albumin scintigraphy in protein-losing enteropathy. Digestion. 2011. 84(3):199-206. [Medline].

Furfaro F, Bezzio C, Maconi G. Protein-losing enteropathy in inflammatory bowel diseases. Minerva Gastroenterol Dietol. 2015 Dec. 61(4):261-5. [Medline].

Bindl L, Buderus S, Bindl C, Lentze MJ. Protein-losing enteropathy: report of four cases and review of etiology, diagnostic work-up and treatment. Klin Padiatr. 2005. 217:201-10.

Florent C, L’Hirondel C, Desmazures C, et al. Intestinal clearance of alpha 1-antitrypsin. A sensitive method for the detection of protein-losing enteropathy. Gastroenterology. 1981 Oct. 81(4):777-80. [Medline].

Magdo HS, Stillwell TL, Greenhawt MJ, et al. Immune abnormalities in Fontan protein-losing enteropathy: a case-control study. J Pediatr. 2015 Aug. 167(2):331-7. [Medline].

Albright F, Bartter FC, Forbes AP. The fate of human serum albumin administered intravenously to a patient with idiopathic hypoalbuminemia and hypoproteinemia. Trans Assoc Am Physicians. 1949. 204:62.

Ferrante M, De Hertogh G, Penninckx F, et al. Protein-losing enteropathy in Crohn’s disease. Clin Gastroenterol Hepatol. 2005 Jun. 3(6):A25. [Medline].

Stark ME, Batts KP, Alexander GL. Protein-losing enteropathy with collagenous colitis. Am J Gastroenterol. 1992 Jun. 87(6):780-3. [Medline].

Ozeki T, Ogasawara N, Izawa S, et al. Protein-losing enteropathy associated with collagenous colitis cured by withdrawal of a proton pump inhibitor. Intern Med. 2013. 52(11):1183-7. [Medline].

Nakaya Y, Kaku Hosokawa S, Kataoka Y, et al. Acute onset collagenous colitis associated with protein-losing enteropathy. J Gen Fam Med. 2017 Jun. 18(3):135-8. [Medline]. [Full Text].

Jeffries GH, Chapman A, Sleisenger MH. Low-fat diet in intestinal lymphangiectasia. Its effect on albumin metabolism. N Engl J Med. 1964 Apr 9. 270:761-6. [Medline].

Lee HJ, Rha MY, Cho YY, Kim ER, Chang DK. A case of protein supplement effect in protein-losing enteropathy. Clin Nutr Res. 2012 Jul. 1(1):94-8. [Medline]. [Full Text].

Yeaton P, Frierson HF Jr. Octreotide reduces enteral protein losses in Ménétrier’s disease. Am J Gastroenterol. 1993 Jan. 88(1):95-8. [Medline].

Levitt DG, Levitt MD. Protein losing enteropathy: comprehensive review of the mechanistic association with clinical and subclinical disease states. Clin Exp Gastroenterol. 2017. 10:147-68. [Medline]. [Full Text].

Naeem Aslam, MD Fellow, Department of Medicine, Division of Gastroenterology/Hepatology, University of Louisville School of Medicine

Naeem Aslam, MD is a member of the following medical societies: American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Richard Wright, MD Professor and Chief, Department of Medicine, Division of Gastroenterology/Hepatology, University of Louisville School of Medicine

Richard Wright, MD is a member of the following medical societies: American Association for Physician Leadership, American College of Physicians, American Gastroenterological Association, American Medical Association, American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Noel Williams, MD, FRCPC FACP, MACG, Professor Emeritus, Department of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada; Professor, Department of Internal Medicine, Division of Gastroenterology, University of Alberta, Edmonton, Alberta, Canada

Noel Williams, MD, FRCPC is a member of the following medical societies: Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Burt Cagir, MD, FACS Clinical Professor of Surgery, The Commonwealth Medical College; Director, General Surgery Residency Program, Robert Packer Hospital; Attending Surgeon, Robert Packer Hospital and Corning Hospital

Burt Cagir, MD, FACS is a member of the following medical societies: American College of Surgeons, American Medical Association, Society for Surgery of the Alimentary Tract

Disclosure: Nothing to disclose.

Terence David Lewis, MBBS, MBBS 

Terence David Lewis, MBBS, MBBS is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Medical Association, California Medical Association, Royal College of Physicians and Surgeons of Canada, Sigma Xi

Disclosure: Nothing to disclose.

Protein-Losing Enteropathy

Research & References of Protein-Losing Enteropathy|A&C Accounting And Tax Services
Source