Pediatric Protein-Losing Enteropathy Clinical Presentation

  • Author: Simon S Rabinowitz, MD, PhD, FAAP; Chief Editor: Carmen Cuffari, MD   more...
 
Updated: Dec 5, 2011
 

History

Most commonly, protein-losing enteropathy presents with edema. When analyzinig the cause of edema, certain aspects of the history and physical examination should be emphasized.

A complete dietary history should be obtained to evaluate for possible protein malnutrition, which results in diminished albumin synthesis (kwashiokor).

Query about possible renal diseases (increased protein loss) or hepatic diseases (decreased protein synthesis) that results in hypoalbuminemia. Usually nephrotic syndrome or liver disease alone would be the only cause for the hypoalbuminemia, but either can also increase the pressure in the intra-abdominal lymphatic system, producing protein-losing enteropathy.

Urinary tract issues (urinary frequency, urine color, pain with urination) or a history of high blood pressure should lead to an evaluation for renal disease.

Alcohol intake or a previous history of hepatitis, fatigue, or jaundice should lead to an evaluation for liver disease

Obtain a complete GI history, looking for any suggestions of diarrhea, hematochezia, and abdominal issues (ie, gut sources of excessive protein loss).

Primary lymphangiectasia may be long-standing; therefore, questions about symptoms may date back to the neonatal period. Query the patient or parents about other lymphatic abnormalities, especially asymmetric edema that might have been present.

Obtain a cardiac history, including congenital heart disease, prior episodes of pericarditis, serious streptococcal infection, and prior heart surgery.

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Physical

Begin the physical examination by taking appropriate anthropometric measurements, including the following:

  • Head circumference
  • Height
  • Weight
  • Triceps skinfold thickness as an assessment of the nutritional status (if available)

Emphasize that weight alone may be misleading because fluid retention can occur in the setting of hypoalbuminemia.

Examine the patient for evidence of the following:

  • Acute liver disease (eg, enlarged liver, tenderness in the right upper quadrant)
  • Signs of chronic liver disease (eg, jaundice, splenomegaly, abdominal wall venous prominence due to collateral circulation)

Perform a careful cardiac examination to evaluate for hepatomegaly, ascites, lower lobe rales, and jugular vein distention are suggestive of increased right-sided pressures in the heart as the cause for protein-losing enteropathy.

The finding of high blood pressure may suggest renal or cardiac disease.

GI findings compatible with protein-losing enteropathy include the following:

  • Abdominal tenderness or distension
  • Macroscopic or microscopic blood and mucus determined on rectal examination

Localized edema is suggestive of primary intestinal lymphangiectasia.

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Causes

As indicated above, many disease processes can lead to protein-losing enteropathy. The following is an approach to categorizing the underlying etiology.

Lymphatic losses

Genetic causes

  • Congenital disorders of glycosylation (may involve enterocyte disruption without any ulceration)
  • Juvenile polyposis[8]

Inflammation of the GI tract

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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 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: Abbott nutrition Honoraria Speaking and teaching

Coauthor(s)

Esther N Prince, MD  Pediatric Gastroenterology Fellow, State University of New York Downstate Medical Center

Disclosure: Nothing to disclose.

Specialty Editor Board

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, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Abbott, Inc Consulting fee Consulting

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, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

Steven M Schwarz, MD, FAAP, FACN, AGAF  Professor of Pediatrics, Children's Hospital at Downstate, State University of New York 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: Curemark, LLC Consulting fee Board membership; Centocor, Inc. Grant/research funds Independent contractor; Johnson & Johnson, 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.

Additional Contributors

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.

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