eMedicine Specialties > Pediatrics: General Medicine > Gastroenterology

Protein-Losing Enteropathy: Treatment & Medication

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

Treatment

Medical Care

Therapeutic approaches for protein-losing enteropathy (PLE) 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, malabsorption of the fat-soluble vitamins can occur secondary to the dilatation and rupture of the lacteals. 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 sometimes reversible after the use of corticosteroids or heparin or after surgical intervention (baffle fenestration of heart transplant).9
    • 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.
    • 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.
  • Corticosteroids 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.

Surgical Care

  • 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 heart surgery, with complete resolution of symptoms.
  • Conner et al reported a case in which localized resection of the involved bowel successfully treated the condition.10

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.

Medication

Vitamins

In protein-losing enteropathy (PLE), providing supplementation with fat-soluble vitamins (eg, A, D, E, K) is important. These agents are necessary for growth and health. For healthy individuals, they are needed in small amounts only and are available in the foods of a daily diet. However, soluble vitamin supplementation is essential in patients with protein-losing enteropathy because the small amounts available in a regular diet are insufficient in the face of the malabsorption that occurs.


ADEK vitamins (AquADEKs Pediatric Liquid, ADEKs Chewable)

PO multinutrient specially formulated for use under medical supervision to provide nutritional supplementation in individuals with malabsorptive conditions. Each 1 mL dose contains water-miscible forms of fat-soluble vitamins A (5,751 U), D (400 U), E (65 U), and K (400 mcg) plus other nutrients, including vitamin C (15 mcg), B-complex vitamins, biotin, selenium (10 mcg), and zinc gluconate (5 mg). Available as chewable tab or pediatric drops.

Adult

Pediatric

<12 months: 1 mL PO qd
1-3 years: 2 mL PO qd
4-10 years: 1 tab PO qd
>10 years: 2 tabs PO qd

Best administered with supplementary pancreatic enzymes for individuals who require enzyme therapy for control of steatorrhea or improved fat absorption; vitamin K interferes with actions of anticoagulant drugs

Pregnancy

A - Fetal risk not revealed in controlled studies in humans

Precautions

Do not exceed recommended doses; contraindicated in pregnancy if vitamin A exceeds RDA; exclude pernicious anemia before using because folic acid in doses >0.1 mg/d may mask symptoms; for chewable tab, chew or crush tab thoroughly before swallowing


Vitamin A (Aquasol A)

Needed for night vision and growth of skin, bones, male reproductive organs, and female reproductive organs.

Adult

Pediatric

Doses given PO qd
<1 year: 375 mcg
1-3 years: 400 mcg
4-6 years: 500 mcg
7-10 years: 700 mcg
>10 years: 800-1000 mcg
Adolescent males: 1000 mcg
Adolescent females: 800 mcg
Retinol equivalents (RE): 0.3 mcg RE = 1 U vitamin A

Cholestyramine and colestipol decrease effects; mineral oil and neomycin may decrease absorption of vitamin A

Pregnancy

A - Safe in pregnancy

Precautions

Pregnancy category X if dose exceeds RDA


Ergocalciferol (Calciferol, Drisdol)

Form of vitamin D used in vitamin supplements, necessary for strong bones and teeth.

Adult

Pediatric

Premature infants: 10-20 mcg/d PO (400-800 U), not to exceed 750 mcg/d (300,000 U)
Infants and healthy children: 10 mcg/d PO (400 U)
1 mcg = 40 USP U

Colestipol, mineral oil, and cholestyramine may decrease absorption from small intestine; thiazide diuretics may increase effects of vitamin D

Documented hypersensitivity; hypercalcemia, malabsorption syndrome

Pregnancy

C - Safety for use during pregnancy has not been established.

Precautions

Pregnancy category C per manufacturer; expert analysis category A, category D if dosage exceeds RDA; adequate dietary calcium needed for clinical response; maintain adequate fluid intake; caution in impaired renal function, renal stones, heart disease, or arteriosclerosis


Vitamin E (Vita-Plus E Softgels, Vitec)

Protects polyunsaturated fatty acids in membranes from attack by free radicals and protects red blood cells against hemolysis.

Adult

Pediatric

1 U/kg/d PO of water-miscible vitamin E

Mineral oil decreases absorption; delays absorption of iron and increases effects of anticoagulants

Pregnancy

B - Usually safe but benefits must outweigh the risks.

Precautions

Pregnancy factor is C with large doses of vitamin E; may induce vitamin K deficiency; necrotizing enterocolitis may occur when large doses of vitamin E given


Vitamin K (AquaMEPHYTON)

Fat-soluble vitamin absorbed by the gut and stored in the liver; necessary for the function of clotting factors in the coagulation cascade; used to replace essential vitamins not obtained in sufficient quantities in the diet or to further supplement levels.

Adult

Pediatric

2.5-5 mg/d PO
1-2 mg/dose as a single dose IV/IM

Effects of warfarin, sodium, and dicumarol are antagonized by phytonadione

Pregnancy

C - Safety for use during pregnancy has not been established.

Precautions

Ineffective in hereditary hypoprothrombinemia; rapid infusion may result in flushing and a feeling of constriction in chest; relatively nontoxic, even in massive doses

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
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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.

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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

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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.

 
 
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