Protein losing enteropathy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ; Associate Editor(s)-in-Chief: Please Take Over This Page and Apply to be Editor-In-Chief for this topic: There can be one or more than one Editor-In-Chief. You may also apply to be an Associate Editor-In-Chief of one of the subtopics below. Please mail us [2] to indicate your interest in serving either as an Editor-In-Chief of the entire topic or as an Associate Editor-In-Chief for a subtopic. Please be sure to attach your CV and or biographical sketch.

Synonyms and keywords: Protein loss, protein deficiency, GI protein loss, gastrointestinal protein loss, protein-losing gastroenteropathy, protein-losing gastroenteropathy, gastroenteropathy, gastric protein loss, helicobacter pylori, H pylori, giant hypertrophic gastropathy, menetrier disease, ménétrier, disease, loss of plasma proteins from the gastrointestinal tract, excessive leakage of plasma proteins into the lumen of the gastrointestinal tract, lymphatic obstruction, mucosal disease with erosions, ulcerations, swelling of the legs, peripheral edema, decreased plasma oncotic pressure

Overview

Protein losing enteropathy is the loss of plasma proteins from the gastrointestinal tract caused by an array of abnormalities

Historical Perspective

  • [Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
  • In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
  • In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].

Classification

  • [Disease name] may be classified according to [classification method] into [number] subtypes/groups:
  • [group1]
  • [group2]
  • [group3]
  • Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3]

Pathophysiology

  • The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
  • The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
  • On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
  • On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Causes

Common Causes

Most cases of protein losing enteropathy are caused as a result of:

  1. Primary gastrointestinal disorders
  2. Lymphatic obstruction

Primary Gastrointestinal Diseases

Mucosal Erosions/Ulcerations

Primary gastrointestinal diseases causing erosion or ulceration of the mucosa of the gut leading to fecal loss of proteins such as:[1][2]

Non-Erosive/Ulcerative Mucosal involvement

Lymphatic Obstruction

Conditions responsible for causing lymphatic obstruction leading to the leakage of lymph into the lumen of gut such as:

Complete Differential Diagnosis Of Underlying Causes

Diagnosis

As hypoproteinemia is the key factor in evaluating a patient for protein losing enteropathy, other common causes of hypoproteinemia such as nephrotic syndrome, impaired protein synthesis due to chronic liver disease and malnutrition must be excluded first.

Laboratory Studies

As the most prominent laboratory finding is a decrease in serum concentration of albumin and globulin, the diagnostic work up protein losing enteropathy consist of quantitative measurements of Alpha-1 antitrypsin or 51Cr-albumin.

  • Alpha-1 antitrypsin (A1AT) used as an endogenous marker is a sensitive and inexpensive laboratory test performed to diagnose protein losing enteropathy and has become the current standard for quantitating protein losing enteropathy. Measurement of fecal volume and fecal loss of alpha-1 antitrypsin depicts the plasma concentration of alpha-1 antitrypsin as;

Alpha 1-AT plasma concentration = ((stool volume) x (stool alpha 1-AT)) / (serum alpha-1 AT)

Gastrointestinal loss of alpha-1 antitrypsin is measured in feces and a clearance greater than 27mL/day is considered diagnostic for protein losing enteropathy.

  • 51Cr-labeled albumin can also be measured followed by stool collection to determine the amount of protein loss into the gastrointestinal tract.

Imaging Studies

Following the detection of abnormal amounts of alpha-1 antitrypsin in the stool, the following tests can be performed to detect the specific etiology for the protein loss into the gastrointestinal lumen.

  • Administration of technetium-99 labeled macromolecules such as albumin. Imaging is required to localize the primary site of protein leakage with no requirement for fecal collection. Scintigraphy is becoming popular in the diagnosis and localization of the site of protein leakage.  
  • For the diagnosis of lymphatic obstruction, computed tomography, lymphangiography or magnetic resonance imaging can be used.

Other tests:

  • Biopsy samples of the mucosa and stool cultures for ova and parasites should be performed if there is a suspicion of ulcerative or erosive gastrointestinal disease and parasitic infection, respectively.

Treatment

Treatment depends upon the underlying condition.

References

  1. Akkelle BS, Tutar E, Sengul OK, Celikel CA, Ertem D (2018). "A Rare Complication of Giardiasis in Children: Protein-losing Enteropathy". Pediatr Infect Dis J. 37 (12): e345–e347. doi:10.1097/INF.0000000000002025. PMID 30408010.
  2. Zubiaga Toro L, Ruiz-Tovar J, Castro MJ, Ortiz de Solórzano FJ, Luque de León E, Jiménez JM; et al. (2019). "Whipple disease after bariatric surgery: from malabsorption to malnutrition status". Nutr Hosp. 36 (1): 238–241. doi:10.20960/nh.02258. PMID 30834767.