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Pediatric Protein-Losing Enteropathy Clinical Presentation

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

History

Most commonly, protein-losing enteropathy presents with edema. When analyzing 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 (kwashiorkor).

Query about possible renal diseases (increased protein loss) or hepatic diseases (decreased protein synthesis) that could also result in hypoalbuminemia. Nephrotic syndrome or liver disease are often the primary cause of hypoalbuminemia. However, either can also increase the pressure in the intra-abdominal lymphatic system and thus also yield protein-losing enteropathy.

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

Alcohol consumption 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 in infancy.

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 firm, nodular liver, and/or tenderness in the right upper quadrant)
  • Chronic liver disease (eg, liver findings mentioned above along with jaundice, splenomegaly, abdominal wall venous prominence due to collateral circulation)

Perform a careful cardiac examination to evaluate for hepatosplenomegaly, ascites, (bilateral) 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, including dilated, tender loops of bowel
  • 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 are as follows:

  • Enteric lymphatic obstruction
    • Primary enteric lymphatic obstruction
      • Primary intestinal lymphangiectasia
      • Secondary intestinal lymphangiectasia (eg, mesointestinal fibrosis)[17]  
      • Whipple disease
      • Malrotation/volvulus                                 
      • Tuberculosis                                       
      • Sarcoidosis                                       
      • Amyloidosis[18]  
      • Radiation enteritis
      • Retroperitoneal fibrosis or tumor
      • Arsenic poisoning
      • Secondary to unusual causes of bowel infiltration - Leukemia,[19]  Gaucher disease,[20]  Langerhans cell histiocytosis,[21]  and infantile systemic hyalinosis syndrome[22]  
    • Cardiac causes of increased systemic venous pressure
      • Post–Fontan procedure
      • Constrictive pericarditis, including when seen with familial Mediterranean fever[23]
      • Congestive heart failure
      • Cardiomyopathy

Genetic causes include the following:

  • Congenital disorders of glycosylation (may involve enterocyte disruption without any ulceration)
  • Juvenile polyposis  [24]
  • Mutations in plasmalemma vesicle associated protein (PLVAP) may lead to deletion of the diaphragms of endothelial fenestrae, resulting in plasma protein extravasation and PLE [4]

Inflammation of the GI tract includes the following:

  • Infectious causes involving enterocyte disruption without ulceration                
    • Malaria
    • Measles
    • Rotavirus
    • Bacterial overgrowth
    • Clostridium difficile
    • Clostridium perfringens
    • Colonic malakoplakia
    • Cytomegalovirus          
    • Giardia lamblia
    • Helicobacter pylori
    • Strongyloides stercoralis
    • Histoplasmosis[25]
    • Mycobacterium avium complex[26]
    • Paracoccidiomycosis[27]         
  • Infectious causes with mucosal ulceration
    • Bacterial enterocolitis - Salmonellae, Shigella, Yersinia, Campylobacter, some forms of Escherichia coli
    • Toxin mediated enterocolitis - Clostridia difficile toxin, some forms of E coli and Shigella
    • Viral mediated enterocolitis - Cytomegalovirus (most commonly), herpes
    • Tuberculosis
  • Noninfectious causes with mucosal ulceration
    • May involve enterocyte disruption without any ulceration
    • Cow's milk/soy protein allergy
    • Eosinophilic gastroenteritis
    • Henoch-Schonlein purpura
    • Anastomotic ulceration/ischemia
    • Erosive gastritis
    • Graft versus host disease
    • Hirschsprung disease
    • Inflammatory bowel disease, including Crohn’s disease and ulcerative colitis[1]
    • Multiple polyposis
    • Necrotizing enterocolitis
    • Peptic esophagitis
    • Ulcerative jejunitis
    • Gastric carcinoma[28]
  • Noninfectious causes with breakdown of enterocyte barrier
    • Celiac disease (Gluten sensitive enteropathy)
    • Hypertrophic gastropathy (Menetrier disease)
    • Juvenile rheumatoid arthritis
    • Malnutrition
    • Systemic lupus erythematosus
    • Systemic phenobarbital hypersensitivity
    • Tropical sprue
    • Severe iron deficiency[29]
    • Budd Chiari syndrome/hepatic venous outlet obstruction/post-liver transplant[30]
    • Collagenous colitis/gastritis[31]
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Physical Examination

Edema is often noted, especially when the albumin becomes quite low.

Stigmata of heart disease, enteropathy, and infections, which can be limited to the GI tract or generalized, may also be suggested by physical findings.

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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 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.

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