Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Pediatric Protein-Losing Enteropathy Treatment & Management

  • Author: Simon S Rabinowitz, MD, PhD, FAAP; Chief Editor: Carmen Cuffari, MD  more...
 
Updated: Aug 11, 2016
 

Medical Care

Therapeutic approaches for protein-losing enteropathy depend on the underlying etiology.

In patients with primary intestinal lymphangiectasia, no direct method to address the protein-losing enteropathy is noted. Replacing fat in the diet with medium-chain triglycerides (MCTs) can improve fat malabsorption and the nutritional status of the patient. Supplementing fat-soluble vitamins (ie, A, D, E, K) is also important.

In protein-losing enteropathy associated with lymphatic obstruction, relieving the pressure in the lymphatic system decreases intestinal protein loss. Obstruction of lymphatics has been reported with structural heart disease, constrictive pericarditis, cardiomyopathy, and surgical repair of congenital heart disease. When obstruction of the intra-abdominal lymphatic system is the cause of protein-losing enteropathy, removal of long-chain triglycerides from the diet decreases the pressure in the lacteals and the lymphatic circulation. In addition, because of the increased pressure, there may be rupture of lacteals, which itself results in fat malabsorption. The use of MCT oil in these cases does not relieve any inflammation, but because MCT oil is not absorbed via the lymphatic system, it reduces the pressure of the lacteals.

Protein-losing enteropathy that results after heart surgery (with increased pressure in the right side) is a known postoperative complication of the Fontan procedure that has been a challenge to the surgical procedure's long-term success. Multiple treatments have been used, including corticosteroids, heparin, and additional surgical intervention (baffle fenestration or heart transplantation).[42]  Note the following:

  • As many as 13.4% of patients undergoing a Fontan procedure develop protein-losing enteropathy within 10 years of surgery, and the mortality rate associated with this complication has been reported to be as high as 56% in 5 years.
  • The use of steroids has produced temporary clinical and pathological resolution of protein-losing enteropathy.
  • Recently, a single-center retrospective review examined the use of budesonide, an oral steroid with extensive first pass metabolism, for 6 months or longer in Fontan related protein-losing enteropathy patients. This treatment was associated with significant symptomatic improvement and sustained increases in serum albumin but did not markedly change the ultimate outcome and was associated with significant side effects [43] .
  • Heparin has also been reported to improve protein-losing enteropathy in children after the Fontan procedure.
  • Heparin is thought to possibly have a stabilizing effect on the capillary endothelium, reducing protein leakage into the extravascular space and gut lumen, although the precise mechanism of action is unknown.
  • Although heparin has been successfully used to treat some patients with protein-losing enteropathy that develops after the Fontan procedure, it is by no means the treatment of choice for all the etiologies of protein-losing enteropathy.
  • A retrospective review of 42 patients with protein-losing enteropathy following the Fontan procedure found that treatments used more frequently in survivors included spironolactone (68%), octreotide (21%), sildenafil (19%), and surgical intervention (71%). [16]

Corticosteroids including budesonide, have been used in patients with protein-losing enteropathy associated with collagen vascular diseases, inflammatory bowel disease, heart surgery, and others. Sporadic case reports have documented the successful use of other agents such as cyclosporine for protein-losing enteropathy. Immunosuppressive drugs should not be used in cases of protein-losing enteropathy secondary to infections.

Case reports have described success in patients with protein-losing enteropathy secondary to primary intestinal lymphangiectasia (Waldmann disease) using everolimus[44]  and rapamycin in a child with tuberous sclerosis complex.[45]

Next

Surgical Care

Conner et al reported a case in which localized resection of the involved bowel successfully treated the condition.[46]

In patients who have undergone a Fontan procedure, fenestration of the baffle that separates the systemic venous pathway from the pulmonary venous atrium has been performed to treat protein-losing enteropathy, and in some cases the symptoms have resolved, presumably because of the decrease in systemic venous pressure.

Cardiac transplantation has also been performed for the management of intractable protein-losing enteropathy related to previous Fontan surgery, with complete resolution of symptoms in most cases and with survival comparable to non-Fontan procedures undergoing transplantation.[47]

Previous
Next

Diet

In patients whose protein-losing enteropathy is related to lymphatic pathology, decreasing the lymphatic circulation provides some benefit. This requires dietary limitation of long-chain triglycerides because their absorption from the gut stimulates lymphatic flow. In order to provide adequate energy, medium-chain triglycerides must be added as an alternative source of lipid calories.

As described below, fat soluble vitamins must also be supplemented because their absorption is compromised in these patients.

Previous
 
 
Contributor Information and Disclosures
Author

Simon S Rabinowitz, MD, PhD, FAAP Professor of Clinical Pediatrics, Vice Chairman, Clinical Practice Development, Pediatric Gastroenterology, Hepatology, and Nutrition, State University of New York Downstate College of Medicine, The Children's Hospital at Downstate

Simon S Rabinowitz, MD, PhD, FAAP is a member of the following medical societies: American Gastroenterological Association, American Academy of Pediatrics, Phi Beta Kappa, American Association for the Advancement of Science, American College of Gastroenterology, American Medical Association, New York Academy of Sciences, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Jessica A Epstein State University of New York Downstate College of Medicine

Jessica A Epstein is a member of the following medical societies: American Academy of Pediatrics, American Medical Association, American Psychiatric Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

David A Piccoli, MD Chief of Pediatric Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia; Professor, University of Pennsylvania School of Medicine

David A Piccoli, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Nothing to disclose.

Chief Editor

Carmen Cuffari, MD Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, Royal College of Physicians and Surgeons of Canada

Disclosure: Received honoraria from Prometheus Laboratories for speaking and teaching; Received honoraria from Abbott Nutritionals for speaking and teaching.

Additional Contributors

Robert Baldassano, MD Director, Center for Pediatric Inflammatory Bowel Disease, Children's Hospital of Philadelphia; Professor, Department of Pediatrics, Division of Gastroenterology and Nutrition, University of Pennsylvania School of Medicine

Robert Baldassano, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Received consulting fee from Abbott, Inc for consulting.

Brianna Devito University at Buffalo, The State University of New York

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author Barry K Wershil, MD, to the original writing and development of this article.

References
  1. Cakir M, Ersoz S, Akbulut UE. Disseminated cytomegalovirus infection and protein losing enteropathy as presenting feature of pediatric patient with Crohn's disease. Pediatr Gastroenterol Hepatol Nutr. 2015 Mar. 18 (1):60-5. [Medline].

  2. Magdo HS, Stillwell TL, Greenhawt MJ, Stringer KA, Yu S, Fifer CG, et al. Immune Abnormalities in Fontan Protein-Losing Enteropathy: A Case-Control Study. J Pediatr. 2015 Aug. 167 (2):331-7. [Medline].

  3. Schumacher KR, Stringer KA, Donohue JE, Yu S, Shaver A, Caruthers RL, et al. Fontan-associated protein-losing enteropathy and plastic bronchitis. J Pediatr. 2015 Apr. 166 (4):970-7. [Medline].

  4. Elkadri A, Thoeni C, Deharvengt SJ, Murchie R, Guo C, Stavropoulos JD, et al. Mutations in Plasmalemma Vesicle Associated Protein Result in Sieving Protein-Losing Enteropathy Characterized by Hypoproteinemia, Hypoalbuminemia, and Hypertriglyceridemia. Cell Mol Gastroenterol Hepatol. 2015 Jul. 1 (4):381-394.e7. [Medline].

  5. Damen G, de Klerk H, Huijmans J, den Hollander J, Sinaasappel M. Gastrointestinal and other clinical manifestations in 17 children with congenital disorders of glycosylation type Ia, Ib, and Ic. J Pediatr Gastroenterol Nutr. 2004 Mar. 38(3):282-7. [Medline].

  6. Bode L, Freeze HH. Applied glycoproteomics--approaches to study genetic-environmental collisions causing protein-losing enteropathy. Biochim Biophys Acta. 2006 Apr. 1760(4):547-59. [Medline].

  7. Meadows J, Jenkins K. Protein-losing enteropathy: integrating a new disease paradigm into recommendations for prevention and treatment. Cardiol Young. 2011 Aug. 21(4):363-77. [Medline].

  8. Ohuchi H, Yasuda K, Miyazaki A, Kitano M, Sakaguchi H, Yazaki S, et al. Haemodynamic characteristics before and after the onset of protein losing enteropathy in patients after the Fontan operation. Eur J Cardiothorac Surg. 2013 Mar. 43(3):e49-57. [Medline].

  9. Yu JJ, Yun TJ, Yun SC, Im YM, Lee SC, Shin HJ, et al. Low pulmonary vascular compliance predisposes post-Fontan patients to protein-losing enteropathy. Int J Cardiol. 2011 Sep 22. [Medline].

  10. Kim GB, Kwon BS, Bae EJ, Noh CI, Choi JY. Significance of circulating hepatocyte growth factor in protein-losing enteropathy after Fontan operation. Pediatr Cardiol. 2011 Oct. 32(7):917-23. [Medline].

  11. Mertens L, Hagler DJ, Sauer U, Somerville J, Gewillig M. Protein-losing enteropathy after the Fontan operation: an international multicenter study. PLE study group. J Thorac Cardiovasc Surg. 1998 May. 115(5):1063-73. [Medline].

  12. Ono M, Kasnar-Samprec J, Hager A, Cleuziou J, Burri M, Langenbach C, et al. Clinical outcome following total cavopulmonary connection: a 20-year single-centre experience. Eur J Cardiothorac Surg. 2016 Mar 23. [Medline].

  13. 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].

  14. Magdo HS, Stillwell TL, Greenhawt MJ, Stringer KA, Yu S, Fifer CG, et al. Immune Abnormalities in Fontan Protein-Losing Enteropathy: A Case-Control Study. J Pediatr. 2015 Aug. 167 (2):331-7. [Medline].

  15. Schumacher KR, Gossett J, Guleserian K, Naftel DC, Pruitt E, Dodd D, et al. Fontan-associated protein-losing enteropathy and heart transplant: A Pediatric Heart Transplant Study analysis. J Heart Lung Transplant. 2015 Sep. 34 (9):1169-76. [Medline].

  16. John AS, Johnson JA, Khan M, Driscoll DJ, Warnes CA, Cetta F. 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].

  17. Mahjoub F, Sani MN, Tabari AK, Monajemzadeh M, Zandieh S. An unusual cause of protein losing enteropathy in a 2.5-year-old girl: meso-intestinal fibrosis. European J Pediatr Surg Rep. 2014 Jun. 2 (1):29-31. [Medline]. [Full Text].

  18. Weng MT, Wong JM, Shin SR, Yao M, Wei SC. Papillary thyroid cancer associated with light-chain amyloidosis initially presenting with small-bowel bleeding and protein-losing enteropathy. J Clin Oncol. 2010 Oct 10. 28(29):e557-9. [Medline].

  19. Hwang YY, Leung AY, Ng IO, Chan GS, Chan KW, Tse E, et al. Protein-losing enteropathy due to T-cell large granular lymphocyte leukemia. J Clin Oncol. 2009 Apr 20. 27(12):2097-8. [Medline].

  20. Burrow TA, Cohen MB, Bokulic R, et al. Gaucher disease: progressive mesenteric and mediastinal lymphadenopathy despite enzyme therapy. J Pediatr. 2007 Feb. 150(2):202-6. [Medline].

  21. Damry N, Hottat N, Azzi N, Ferster A, Avni F. Unusual findings in two cases of Langerhans' cell histiocytosis. Pediatr Radiol. 2000 Mar. 30(3):196-9. [Medline].

  22. Alreheili K, AlMehaidib A, Alsaleem K, Banemi M, Aldekhail W, Al-Mayouf SM. Intestinal lymphangiectasia in a patient with infantile systemic hyalinosis syndrome: a rare cause of protein-losing enteropathy. Ann Saudi Med. 2012 Mar-Apr. 32(2):206-8. [Medline].

  23. Gökçe I, Gökçe S, Kiliç A, Bozlar U, Kocaoglu M, Ongürü O. Familial Mediteranean fever with protein-losing enteropathy due to constrictive pericarditis. World J Pediatr. 2011 Nov. 7(4):365-7. [Medline].

  24. Yamashita K, Saito M, Itoh M, et al. Juvenile polyposis complicated with protein losing gastropathy. Intern Med. 2009. 48(5):335-8. [Medline].

  25. Kok J, Chen SC, Anderson L, et al. Protein-losing enteropathy and hypogammaglobulinaemia as first manifestations of disseminated histoplasmosis coincident with Nocardia infection. J Med Microbiol. 2010 May. 59:610-3. [Medline].

  26. Ogawa E, Murata M, Unno M, et al. Protein-losing enteropathy during highly active antiretroviral therapy in a patient with AIDS-related disseminated Mycobacterial avium complex infection. J Infect Chemother. 2009 Aug. 15(4):252-6. [Medline].

  27. Berni AD, Elias Júnior E, Arão Filho A, Paniago AM. Protein-losing enteropathy in paracoccidioidomycosis identified by scintigraphy: report of three cases. Braz J Infect Dis. 2010 Sep-Oct. 14(5):540-3. [Medline].

  28. Yamamoto M, Nishibuchi I, Matsuyama A, et al. Gastric carcinoma with protein-losing gastroenteropathy: report of a case. Surg Today. 2011 Jan. 41(1):125-9. [Medline].

  29. Salstrom JL, Kent M, Liang X, Wang M. Toddlers with anasarca and severe anemia: a lesson in preventive medicine. Curr Opin Pediatr. 2012 Feb. 24(1):129-33. [Medline].

  30. Hourigan SK, Anders RA, Mitchell SE, Schwarz KB, Lau H, Karnsakul W. Chronic diarrhea, ascites, and protein-losing enteropathy in an infant with hepatic venous outflow obstruction after liver transplantation. Pediatr Transplant. 2012 Nov. 16(7):E328-31. [Medline].

  31. Camarero Salces C, Enes Romero P, Redondo C, Rizo Pascual JM, Roy Ariño G. Collagenous colitis and collagenous gastritis in a 9 year old girl: a case report and review of the literature. Acta Gastroenterol Belg. 2011 Sep. 74(3):468-74. [Medline].

  32. Morava E, Tiemes V, Thiel C, et al. ALG6-CDG: a recognizable phenotype with epilepsy, proximal muscle weakness, ataxia and behavioral and limb anomalies. J Inherit Metab Dis. 2016 Jun 10. [Medline].

  33. Yang C, Dehner LP. Protein-losing enteropathy with intestinal lymphangiectasia in skeletal dysplasia with Lys650Met mutation. Am J Med Genet A. 2016 May 23. [Medline].

  34. Stephen J, Vilboux T, Haberman Y, Pri-Chen H, Pode-Shakked B, Mazaheri S, et al. Congenital protein losing enteropathy: an inborn error of lipid metabolism due to DGAT1 mutations. Eur J Hum Genet. 2016 Feb 17. [Medline].

  35. Jackson CC, Best L, Lorenzo L, Casanova JL, Wacker J, Bertz S, et al. A Multiplex Kindred with Hennekam Syndrome due to Homozygosity for a CCBE1 Mutation that does not Prevent Protein Expression. J Clin Immunol. 2016 Jan. 36 (1):19-27. [Medline].

  36. Frosk P, Chodirker B, Simard L, El-Matary W, Hanlon-Dearman A, Schwartzentruber J, et al. A novel CCBE1 mutation leading to a mild form of hennekam syndrome: case report and review of the literature. BMC Med Genet. 2015 Apr 30. 16:28. [Medline].

  37. Higuchi R, Booka M, Suzuki H, Tsuno H. Protein-losing enteropathy and erythema caused by egg allergy in a breast-fed infant. Pediatr Int. 2016 May. 58 (5):422-424. [Medline].

  38. Liu NF, Lu Q, Wang CG, Zhou JG. Magnetic resonance imaging as a new method to diagnose protein losing enteropathy. Lymphology. 2008 Sep. 41(3):111-5. [Medline].

  39. Kinsell L, Margen S, Tarver H. J Clin Invest. 1950. 29:238.

  40. Gordon RS. J Polym Sci. 1957. 31:191.

  41. Martinez-Sanchis B, Cortés-Vizcaíno V, Frontado-Morales L, Sopena-Novales P. Intestinal uptake of (99m)Tc-MDP: a case report of protein-losing enteropathy correlated with pathology findings from autopsy. Ann Nucl Med. 2011 Feb. 25(2):139-41. [Medline].

  42. Liu M, Liu L, Zhou HM. [Reversal of protein-losing enteropathy with heparin therapy in a patient after Fontan operation]. Zhonghua Er Ke Za Zhi. 2009 Jan. 47(1):76-7. [Medline].

  43. Schumacher KR, Cools M, Goldstein BH, Ioffe-Dahan V, King K, Gaffney D, et al. Oral budesonide treatment for protein-losing enteropathy in Fontan-palliated patients. Pediatr Cardiol. 2011 Oct. 32(7):966-71. [Medline].

  44. Ozeki M, Hori T, Kanda K, Kawamoto N, Ibuka T, Miyazaki T, et al. Everolimus for Primary Intestinal Lymphangiectasia With Protein-Losing Enteropathy. Pediatrics. 2016 Mar. 137 (3):e20152562. [Medline].

  45. Pollack SF, Geffrey AL, Thiele EA, Shah U. Primary intestinal lymphangiectasia treated with rapamycin in a child with tuberous sclerosis complex (TSC). Am J Med Genet A. 2015 Sep. 167A (9):2209-12. [Medline].

  46. Bac DJ, Van Hagen PM, Postema PT, et al. Octreotide for protein-losing enteropathy with intestinal lymphangiectasia. Lancet. 1995 Jun 24. 345(8965):1639. [Medline].

  47. Bai JC, Sambuelli A, Niveloni S, et al. Alpha 1-antitrypsin clearance as an aid in the management of patients with celiac disease. Am J Gastroenterol. 1991 Aug. 86(8):986-91. [Medline].

  48. Belamarich PF, Ortiz A, Mones RL. Severe hypoproteinemia and edema in association with varicella infection. Clin Pediatr (Phila). 1991 Jan. 30(1):56-8. [Medline].

  49. Boccon-Gibod LA, Krichen HA, Carlier-Mercier LM, et al. Digestive tract involvement with exudative enteropathy in Langerhans cell histiocytosis. Pediatr Pathol. 1992 Jul-Aug. 12(4):515-24. [Medline].

  50. Bode L, Murch S, Freeze HH. Heparan sulfate plays a central role in a dynamic in vitro model of protein-losing enteropathy. J Biol Chem. 2006 Mar 24. 281(12):7809-15. [Medline].

  51. Bryan RL, Newman J, Alexander-Williams J. Giant inflammatory polyposis in ulcerative colitis presenting with protein losing enteropathy. J Clin Pathol. 1990 Apr. 43(4):346-7. [Medline]. [Full Text].

  52. Chiu NT, Lee BF, Hwang SL, et al. Protein-losing enteropathy: diagnosis with 99mTc-labeled human serum albumin scintigraphy. Radiol. Apr 2001. 219(1):86-90.

  53. Cho CS, Min JK, Park SH, et al. Protein losing enteropathy associated with Henoch-Schonlein purpura in a patient with rheumatoid arthritis. Scand J Rheumatol. 1996. 25(5):334-6. [Medline].

  54. Cohen HA, Shapiro RP, Frydman M, Varsano I. Childhood protein-losing enteropathy associated with Helicobacter pylori infection. J Pediatr Gastroenterol Nutr. 1991 Aug. 13(2):201-3. [Medline].

  55. Connor FL, Angelides S, Gibson M, et al. Successful resection of localized intestinal lymphangiectasia post-Fontan: role of (99m)technetium-dextran scintigraphy. Pediatrics. 2003 Sep. 112(3 Pt 1):e242-7. [Medline]. [Full Text].

  56. Dansinger ML, Johnson S, Jansen PC, et al. Protein-losing enteropathy is associated with Clostridium difficile diarrhea but not with asymptomatic colonization: a prospective, case- control study. Clin Infect Dis. 1996 Jun. 22(6):932-7. [Medline].

  57. Donnelly JP, Rosenthal A, Castle VP, Holmes RD. Reversal of protein-losing enteropathy with heparin therapy in three patients with univentricular hearts and Fontan palliation. J Pediatr. 1997 Mar. 130(3):474-8. [Medline].

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

  59. Ghilain JM, Martiat P, Fiasse R, et al. Exudative enteropathy caused by an acute graft-vs-host reaction. Apropos of a case report [in French]. Acta Gastroenterol Belg. 1990 Sep-Dec. 53(5-6):488-98. [Medline].

  60. Grill BB, Hillemeier AC, Gryboski JD. Fecal alpha 1-antitrypsin clearance in patients with inflammatory bowel disease. J Pediatr Gastroenterol Nutr. 1984. 3(1):56-61. [Medline].

  61. Groisman GM, George J, Berman D, Harpaz N. Resolution of protein-losing hypertrophic lymphocytic gastritis with therapeutic eradication of Helicobacter pylori. Am J Gastroenterol. 1994 Sep. 89(9):1548-51. [Medline].

  62. Hardikar W, Smith AL, Chow CW. Neonatal protein-losing enteropathy caused by intestinal lymphatic hypoplasia in siblings. J Pediatr Gastroenterol Nutr. 1997 Aug. 25(2):217-21. [Medline].

  63. Inoue Y, Ohtake T, Koga H, et al. Tc-99m albumin scintigraphy in protein-losing gastroenteropathy caused by gastric polyposis. Clin Nucl Med. 1998 May. 23(5):322-3. [Medline].

  64. Jacobs ML, Rychik J, Byrum CJ, Norwood WI Jr. Protein-losing enteropathy after Fontan operation: resolution after baffle fenestration. Ann Thorac Surg. 1996 Jan. 61(1):206-8. [Medline].

  65. Jain A, Reif S, O'Neil K, Gandhi A, Rossi T. Small intestinal bacterial overgrowth and protein-losing enteropathy in an infant with AIDS. J Pediatr Gastroenterol Nutr. 1992 Nov. 15(4):452-4. [Medline].

  66. Jarnum S, Westergaard H, Yssing M, Jensen H. Quantitation of gastrointestinal protein loss by means of FE59-labeled iron dextran. Gastroenterology. 1968 Aug. 55(2):229-41. [Medline].

  67. John AS, Driscoll DJ, Warnes CA, Phillips SD, Cetta F. The use of oral budesonide in adolescents and adults with protein-losing enteropathy after the Fontan operation. Ann Thorac Surg. 2011 Oct. 92(4):1451-6. [Medline].

  68. Kanter KR, Mahle WT, Vincent RN, Berg AM, Kogon BE, Kirshbom PM. Heart transplantation in children with a Fontan procedure. Ann Thorac Surg. 2011 Mar. 91(3):823-9; discussion 829-30. [Medline].

  69. Karbach U, Ewe K, Bodenstein H. Alpha 1-antitrypsin, a reliable endogenous marker for intestinal protein loss and its application in patients with Crohn's disease. Gut. 1983 Aug. 24(8):718-23. [Medline].

  70. Karbach U, Ewe K, Dehos H. Antiinflammatory treatment and intestinal alpha 1-antitrypsin clearance in active Crohn's disease. Dig Dis Sci. 1985 Mar. 30(3):229-35. [Medline].

  71. Keaney NP, Kelleher J. Faecal excretion of alpha 1-antitrypsin in protein-losing enteropathy. Lancet. 1980 Mar 29. 1(8170):711. [Medline].

  72. Kelly AM, Feldt RH, Driscoll DJ, Danielson GK. Use of heparin in the treatment of protein-losing enteropathy after Fontan operation for complex congenital heart disease. Mayo Clin Proc. 1998 Aug. 73(8):777-9. [Medline].

  73. Koga H, Iida M, Aoyagi K, et al. Generalized giant inflammatory polyposis in a patient with ulcerative colitis presenting with protein-losing enteropathy. Am J Gastroenterol. 1995 May. 90(5):829-31. [Medline].

  74. Konar A, Brown CB, Hancock BW, Moss S. Protein losing enteropathy as a sole manifestation of non-Hodgkin''s lymphoma. Postgrad Med J. 1986 May. 62(727):399-400. [Medline]. [Full Text].

  75. Korman SH, Bar-Oz B, Mandelberg A, Matoth I. Giardiasis with protein-losing enteropathy: diagnosis by fecal alpha 1- antitrypsin determination. J Pediatr Gastroenterol Nutr. 1990 Feb. 10(2):249-52. [Medline].

  76. Laine L, Politoske EJ, Gill P. Protein-losing enteropathy in acquired immunodeficiency syndrome due to intestinal Kaposi's sarcoma. Arch Intern Med. 1987 Jun. 147(6):1174-5. [Medline].

  77. Laster L, Waldmann TA, Fenster LF, Singleton JW. Albumin metabolism in patients with Whipple''s disease. J Clin Invest. 1966 May. 45(5):637-44. [Medline]. [Full Text].

  78. Madina EH, Soliman AT, Morsi MR. Protein losing enteropathy in the different forms of protein-energy malnutrition. J Trop Pediatr. 1987 Oct. 33(5):254-6. [Medline].

  79. Matoth I, Granot E, Gorenstein A, et al. Gastrointestinal protein loss in children recovering from burns. J Pediatr Surg. 1991 Oct. 26(10):1175-8. [Medline].

  80. Mertens L, Hagler DJ, Sauer U, et al. Protein-losing enteropathy after the Fontan operation: an international multicenter study. PLE study group. J Thorac Cardiovasc Surg. 1998 May. 115(5):1063-73. [Medline].

  81. Meuwissen SG, Ridwan BU, Hasper HJ, Innemee G. Hypertrophic protein-losing gastropathy. A retrospective analysis of 40 cases in The Netherlands. The Dutch Menetrier Study Group. Scand J Gastroenterol Suppl. 1992. 194:1-7. [Medline].

  82. Molina JF, Brown RF, Gedalia A, Espinoza LR. Protein losing enteropathy as the initial manifestation of childhood systemic lupus erythematosus. J Rheumatol. 1996 Jul. 23(7):1269-71. [Medline].

  83. Nakano S, Kuramochi S, Hosoda Y, Miura S. Protein-losing enteropathy due to intestinal lymphangiectasia accompanied by mesenteric lymph node fibrosis. Pathol Int. 1996 Nov. 46(11):883-6. [Medline].

  84. Nakase H, Itani T, Mimura J, et al. Transient protein-losing enteropathy associated with cytomegalovirus infection in a noncompromised host: a case report. Am J Gastroenterol. 1998 Jun. 93(6):1005-6. [Medline].

  85. Petersen VP, Hastrup J. Protein losing enteropathy in constrictive pericarditis. Acta Med Scand. 1963. 173:401.

  86. 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].

  87. Rychik J, Piccoli DA, Barber G. Usefulness of corticosteroid therapy for protein-losing enteropathy after the Fontan procedure. Am J Cardiol. 1991 Sep 15. 68(8):819-21. [Medline].

  88. Sierra C, Calleja F, Picazo B, Martinez-Valverde A. Protein-losing enteropathy secondary to Fontan procedure resolved after cardiac transplantation. J Pediatr Gastroenterol Nutr. 1997 Feb. 24(2):229-30. [Medline].

  89. Strober W, Wochner RD, Carbone PP, Waldmann TA. Intestinal lymphangiectasia: a protein-losing enteropathy with hypogammaglobulinemia, lymphocytopenia and impaired homograft rejection. The Journal of Clinical Investigation. 1967 Oct. 46(10):1643-56. [Medline].

  90. Su J, Smith MB, Rerknimitr R, Morrow D. Small intestine bacterial overgrowth presenting as protein-losing enteropathy. Dig Dis Sci. 1998 Mar. 43(3):679-81. [Medline].

  91. Sunagawa T, Kinjo F, Gakiya I, et al. Successful long-term treatment with cyclosporin A in protein losing gastroenteropathy. Intern Med. 2004 May. 43(5):397-9. [Medline].

  92. Sunheimer RL, Finck C, Mortazavi S, et al. Primary lupus-associated protein-losing enteropathy. Ann Clin Lab Sci. 1994 May-Jun. 24(3):239-42. [Medline].

  93. Sutton DL, Kamath KR. Giardiasis with protein-losing enteropathy. J Pediatr Gastroenterol Nutr. 1985 Feb. 4(1):56-9. [Medline].

  94. Therrien J, Webb GD, Gatzoulis MA. Reversal of protein losing enteropathy with prednisone in adults with modified Fontan operations: long term palliation or bridge to cardiac transplantation?. Heart. 1999 Aug. 82(2):241-3. [Medline]. [Full Text].

  95. Tift WL, Lloyd JK. Intestinal lymphangiectasia. Long-term results with MCT diet. Arch Dis Child. 1975 Apr. 50(4):269-76. [Medline].

  96. Tsutsumi A, Sugiyama T, Matsumura R, et al. Protein losing enteropathy associated with collagen diseases. Ann Rheum Dis. 1991 Mar. 50(3):178-81. [Medline].

  97. Tusseau F, Beaugerie L, Cardon B, Rozenbaum W, Gendre JP, Le Quintrec Y. [Anasarca caused by exudative enteropathy due to digestive Kaposi's sarcoma]. Presse Med. 1989 Sep 16. 18(28):1393. [Medline].

  98. Waldmann TA. Gastrointestinal protein loss demonstrated by 51Cr-labelled albumin. Lancet. 1961. July:121-3.

  99. Wargny D, Dichy J, de Broucker F, et al. Protein-losing enteropathy caused by intestinal metastases of melanosarcoma [in French]. J Radiol. 1989 Oct. 70(10):577-9. [Medline].

  100. Westphal V, Murch S, Kim S, et al. Reduced heparan sulfate accumulation in enterocytes contributes to protein-losing enteropathy in a congenital disorder of glycosylation. Am J Pathol. 2000 Dec. 157(6):1917-25. [Medline]. [Full Text].

  101. Wing-Harkins DL, Dellinger GW, Lynch C, Mihas AA. Eosinophilic gastro-enteritis associated with protein-losing enteropathy and protein C deficiency. J Int Med Res. 1996 Jan-Feb. 24(1):155-63. [Medline].

  102. Yamada M, Sumazaki R, Adachi H, et al. Resolution of protein-losing hypertrophic gastropathy by eradication of Helicobacter pylori. Eur J Pediatr. 1997 Mar. 156(3):182-5. [Medline].

  103. Zellers TM, Brown K. Protein-losing enteropathy after the modified Fontan operation: oral prednisone treatment with biopsy and laboratory proved improvement. Pediatr Cardiol. 1996 Mar-Apr. 17(2):115-7. [Medline].

  104. Gursu HA, Varan B, Erdogan I. Use of oral budesonide in the management of protein-losing enteropathy due to restrictive cardiomyopathy. Cardiol Young. 2014 Aug. 24 (4):764-6. [Medline].

  105. Mizuochi T, Suda K, Seki Y, Yanagi T, Yoshimoto H, Kudo Y, et al. Successful diuretics treatment of protein-losing enteropathy in Noonan syndrome. Pediatr Int. 2015 Apr. 57 (2):e39-41. [Medline].

  106. Schumacher KR, Gossett J, Guleserian K, Naftel DC, Pruitt E, Dodd D, et al. Fontan-associated protein-losing enteropathy and heart transplant: A Pediatric Heart Transplant Study analysis. J Heart Lung Transplant. 2015 Sep. 34 (9):1169-76. [Medline].

  107. Khalesi M, Nakhaei AA, Seyed AJ, Treglia G, Zakavi SR, Sadeghi R, et al. Diagnostic accuracy of nuclear medicine imaging in protein losing enteropathy : systematic review and meta-analysis of the literature. Acta Gastroenterol Belg. 2013 Dec. 76 (4):413-22. [Medline].

 
Previous
Next
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.