eMedicine Specialties > Pediatrics: General Medicine > Gastroenterology

Protein-Losing Enteropathy: Follow-up

Author: Simon S Rabinowitz, MD, PhD, Professor of Clinical Pediatrics, New York Medical College; Chairman, Chief and Medical Administrator, Department of Pediatrics, Chief, Pediatric Gastroenterology and Nutrition, Richmond University Medical Center
Contributor Information and Disclosures

Updated: Aug 17, 2009

Follow-up

Further Outpatient Care

  • Patients who develop protein-losing enteropathy (PLE) after Fontan procedures represent a particularly vulnerable cohort with a high rate of significant morbidity and mortality.
  • Close follow-up coupled with an aggressive approach to reverse this problem is warranted.

Miscellaneous

Medicolegal Pitfalls

  • Failure to consider protein-losing enteropathy (PLE) in any patient who presents with edema
  • Failure to review all measurements during physical examination because weight alone may be misleading because of fluid retention
  • Failure to supplement diets of patients with protein-losing enteropathy with fat-soluble vitamins (ie, A, D, E, K)
  • Failure to recognize the poor prognosis of patients with protein-losing enteropathy after Fontan.
 
Acknowledgments

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



More on Protein-Losing Enteropathy

Overview: Protein-Losing Enteropathy
Differential Diagnoses & Workup: Protein-Losing Enteropathy
Treatment & Medication: Protein-Losing Enteropathy
Follow-up: Protein-Losing Enteropathy
References
Further Reading

References

  1. 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. Mar 2004;38(3):282-7. [Medline].

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

  3. 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. May 1998;115(5):1063-73. [Medline].

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

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

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

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

  8. Gordon RS. J Polym Sci. 1957;31:191.

  9. 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. Jan 2009;47(1):76-7. [Medline].

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

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

  12. 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. Aug 1991;86(8):986-91. [Medline].

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

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

  15. 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. Mar 24 2006;281(12):7809-15. [Medline].

  16. Bouhnik Y, Chaussade S, Robin P, Gobert JG, Couturier D, Guerre J. [Protein-losing enteropathy syndrome caused by a localization of Kaposi's sarcoma in AIDS]. Gastroenterol Clin Biol. Oct 1989;13(10):838-40. [Medline].

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

  18. 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.

  19. 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].

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

  21. Couper RT, Durie PR, Stafford SE, et al. Late gastrointestinal bleeding and protein loss after distal small- bowel resection in infancy. J Pediatr Gastroenterol Nutr. Nov 1989;9(4):454-60. [Medline].

  22. 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. Jun 1996;22(6):932-7. [Medline].

  23. Desmazures C, Giraudeaux V, Florent C, L'Hirondel C, Bernier JJ. [Intestinal clearance of alpha 1 anti-trypsin: a simple technique for diagnosis of protein losing enteropathy (author's transl)]. Nouv Presse Med. May 31 1980;9(24):1691-4. [Medline].

  24. 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. Mar 1997;130(3):474-8. [Medline].

  25. Ehringhaus C, Dominick HC, Schuller M. Protein-losing enteropathy associated with Clostridium perfringens infection [letter]. Lancet. Jul 29 1989;2(8657):268-9. [Medline].

  26. 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. Oct 1981;81(4):777-80. [Medline].

  27. 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. Sep-Dec 1990;53(5-6):488-98. [Medline].

  28. 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].

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

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

  31. Henley JD, Kratzer SS, Seo IS, Davis T. Endometriosis of the small intestine presenting as a protein-losing enteropathy. Am J Gastroenterol. Jan 1993;88(1):130-3. [Medline].

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

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

  34. 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. Nov 1992;15(4):452-4. [Medline].

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

  36. 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. Aug 1983;24(8):718-23. [Medline].

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

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

  39. 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. Aug 1998;73(8):777-9. [Medline].

  40. 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. May 1995;90(5):829-31. [Medline].

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

  42. 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. Feb 1990;10(2):249-52. [Medline].

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

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

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

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

  47. 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. May 1998;115(5):1063-73. [Medline].

  48. 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].

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

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

  51. 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. Jun 1998;93(6):1005-6. [Medline].

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

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

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

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

  56. 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. Oct 1967;46(10):1643-56. [Medline].

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

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

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

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

  61. 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. Aug 1999;82(2):241-3. [Medline][Full Text].

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

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

  64. 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. Sep 16 1989;18(28):1393. [Medline].

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

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

  67. 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. Dec 2000;157(6):1917-25. [Medline][Full Text].

  68. 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. Jan-Feb 1996;24(1):155-63. [Medline].

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

  70. Yoshikawa I, Murata I, Tamura M, et al. A case of protein-losing gastropathy caused by acute Helicobacter pylori infection. Gastrointest Endosc. Feb 1999;49(2):245-8. [Medline].

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

Further Reading

  • Proujanksy R. Protein-Losing Enteropathy. In Walker, Durie, Hamilton, et al eds: Pediatric Gastrointestinal Disease. BC Decker Inc. 2002.
  • Levin MS. Miscellaneous diseases of the small intestine: Protein-losing gastroenteropathy. In Tadataka Y, ed: Textbook of Gastroenterology. KB Lippincott. 1996.

Keywords

protein-losing enteropathy, PLE, chronic diarrhea, hypoproteinemia, hypoalbuminemia, protein-losing gastroenteropathy, idiopathic hypoproteinemia, edema disease, nephrosis without nephrosis, nephrotic syndrome, liver disease, jaundice, splenomegaly, ascites, malrotation, tuberculosis, lymphoma, sarcoidosis, arsenic poisoning, Gaucher disease, Langerhans cell histiocytosis, constrictive pericarditis, congestive heart failure, cardiomyopathy, Clostridium difficile, Clostridium perfringens, Giardia lamblia, Helicobacter pylori, colonic malakoplakia, cytomegalovirus, malaria, measles, rotavirus, salmonella, schistosomiasis, graft versus host disease, Henoch-Schönlein purpura, Hirschsprung disease, inflammatory bowel disease, necrotizing enterocolitis, juvenile rheumatoid arthritis, malnutrition, systemic lupus erythematosus, tropical sprue, treatment, diagnosis

Contributor Information and Disclosures

Author

Simon S Rabinowitz, MD, PhD, Professor of Clinical Pediatrics, New York Medical College; Chairman, Chief and Medical Administrator, Department of Pediatrics, Chief, Pediatric Gastroenterology and Nutrition, Richmond University Medical Center
Simon S Rabinowitz, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American College of Gastroenterology, American Gastroenterological Association, American Medical Association, New York Academy of Sciences, North American Society for Pediatric Gastroenterology and Nutrition, Phi Beta Kappa, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Robert Baldassano, MD, Director, Center for Pediatric Inflammatory Bowel Disease, Division of Gastroenterology and Nutrition, Associate Professor, Department of Pediatrics, The Children's Hospital of Philadelphia, University of Pennsylvania
Robert Baldassano, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

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, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.

CME Editor

Steven M Schwarz, MD, FAAP, FACN, AGAF, Professor of Pediatrics, Children's Hospital at Downstate, SUNY-Downstate Medical Center
Steven M Schwarz, MD, FAAP, FACN, AGAF is a member of the following medical societies: American Academy of Pediatrics, American College of Nutrition, American College of Physician Executives, American Gastroenterological Association, American Pediatric Society, Gastroenterology Research Group, New York Academy of Medicine, North American Society for Pediatric Gastroenterology and Nutrition, and Society for Pediatric Research
Disclosure: TAP Pharmaceuticals Honoraria Speaking and teaching; Curemark, LLC Consulting fee Board membership; Centocor, Inc. Grant/research funds Independent contractor

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, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.