Protein-Losing Enteropathy

Updated: Oct 24, 2016
  • Author: Naeem Aslam, MD; Chief Editor: Burt Cagir, MD, FACS  more...
  • Print
Overview

Background

Protein-losing enteropathy is characterized by the severe loss of serum proteins into the intestine. [1] Normal protein loss in the gastrointestinal tract mainly consists of sloughed enterocytes and pancreatic and biliary secretions. Albumin loss through the gastrointestinal tract normally accounts for 2-15% of the total body degradation of albumin, but, in patients with severe protein-losing gastrointestinal disorders, the enteric protein loss may reach up to 60% of the total albumin pool.

The serum protein level reflects the balance between protein synthesis, metabolism, and protein loss. Protein-losing enteropathy is characterized by more loss of proteins via the gastrointestinal tract than synthesis leading to hypoalbuminemia. It is not a single disease, but an atypical manifestation of other diseases. [2]

Next:

Pathophysiology

The pathophysiology of this disorder is directly related to the excessive leakage of plasma proteins into the lumen of the gastrointestinal tract. Mechanisms for gastrointestinal protein loss include lymphatic obstruction, mucosal disease with erosions, ulcerations, or increased mucosal permeability to proteins as a result of cell damage or death. Proteins entering the gastrointestinal tract are metabolized into constituent amino acids by gastric, pancreatic, and small intestinal enzymes and are reabsorbed. When the rate of gastrointestinal protein loss exceeds the body's capacity to synthesize new proteins, hypoproteinemia develops. [3]

Previous
Next:

Etiology

Primary gastrointestinal mucosal diseases (typically ulcerative/erosive) include the following:

  • Erosions or ulcerations of the esophagus, stomach, or duodenum
  • Regional enteritis
  • Graft versus host disease
  • Pseudomembranous colitis ( Clostridium difficile) [4]
  • Mucosal-based neoplasia
  • Carcinoid syndrome
  • Idiopathic ulcerative jejunoileitis
  • Amyloidosis [5]
  • Protein dyscrasia
  • Neurofibromatosis
  • Cytomegalovirus infection [6]

Increased interstitial pressure or lymphatic obstruction leading to protein loss can be caused by the following:

  • Retroperitoneal fibrosis
  • Lymphoma
  • Intestinal endometriosis
  • Lymphoenteric fistula
  • Cardiac disease (constrictive pericarditis or congestive heart failure)
  • Intestinal lymphangiectasia [7]

Nonerosive upper gastrointestinal diseases include the following:

  • Cutaneous burns [8]
  • Whipple disease
  • Connective tissue disorders
  • Acquired immunodeficiency syndrome (AIDS) [2]
  • Enteropathy, such as angioedema (idiopathic or hereditary) and Henoch-Schönlein purpura
  • Tropical sprue
  • Allergic gastroenteritis
  • Eosinophilic gastroenteritis
  • Giant hypertrophic gastritis (Ménétrier disease) [9, 10, 11]
  • Bacterial overgrowth
  • Intestinal parasites
  • Microscopic colitis
  • Dientamoeba fragilis [12]

Cases in which protein-losing enteropathy was the initial manifestation of systemic lupus erythematosus have been reported. [13, 14]

Protein-losing enteropathy can also occur as a complication of the Fontan procedure, an operation for several congenital heart abnormalities; the surgery allows systemic venous blood to reach the lungs without circulating through a ventricle. [15, 16, 17]  In children, protein-losing enteropathy may occur before or after the procedure, especially in the setting of hemodynamic disturbances. [18]

Previous
Next:

Epidemiology

Frequency

United States

The prevalence rate is not known.

International

The prevalence rate is not known.

Mortality/Morbidity

Morbidity and mortality of this condition directly relate to its cause, either primary gastrointestinal disease or a multisystem disorder.

Race

No racial predilection exists.

Sex

No sex predilection exists.

Age

No age predilection exists.

Previous