Protein losing enteropathy: Difference between revisions
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==Overview== | ==Overview== | ||
Protein losing enteropathy is the loss of plasma proteins from the gastrointestinal tract caused by an array of abnormalities | |||
==Common Causes== | ==Common Causes== | ||
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#[[Lymphatic obstruction]] | #[[Lymphatic obstruction]] | ||
===Primary Gastrointestinal Diseases === | ===Primary Gastrointestinal Diseases=== | ||
=====Mucosal Erosions/Ulcerations===== | =====Mucosal Erosions/Ulcerations===== | ||
Primary gastrointestinal diseases causing erosion or [[ulceration]] of the [[mucosa]] of the [[gut]] leading to fecal loss of proteins such as: | Primary [[gastrointestinal]] diseases causing erosion or [[ulceration]] of the [[mucosa]] of the [[gut]] leading to fecal loss of proteins such as: | ||
*[[Inflammatory bowel diseases]] (Crohn disease, Ulcerative colitis) | *[[Inflammatory bowel diseases]] ([[Crohn disease]], [[Ulcerative colitis]]) | ||
*[[Malignancies]] involving the gut mucosa | *[[Malignancies]] involving the gut [[mucosa]] | ||
*Graft vs. host disease | *Graft vs. host disease | ||
*Esophageal and gastric erosions or ulcerations | *[[Esophageal]] and [[gastric]] erosions or [[Ulceration|ulcerations]] | ||
*[[Carcinoid syndrome]] | *[[Carcinoid syndrome]] | ||
*Bacterial infection with [[Clostridium difficile]] causing [[pseudomembranous colitis]] | *Bacterial infection with [[Clostridium difficile]] causing [[pseudomembranous colitis]] | ||
*Parasitic infection with [[Giardia]] | *[[Parasitic]] infection with [[Giardia]] | ||
=====Non-Erosive/Ulcerative Mucosal involvement===== | =====Non-Erosive/Ulcerative Mucosal involvement===== | ||
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*[[Hypertrophic gastritis]] | *[[Hypertrophic gastritis]] | ||
*[[Connective tissue disorders]]: [[Systemic lupus erythematosus]] | *[[Connective tissue disorders]]: [[Systemic lupus erythematosus]] | ||
*Cutaneous [[burns]] | *[[Cutaneous]] [[burns]] | ||
===Lymphatic Obstruction=== | ===Lymphatic Obstruction=== | ||
Conditions responsible for causing lymphatic obstruction leading to the leakage of lymph into the lumen of gut such as: | Conditions responsible for causing [[lymphatic]] obstruction leading to the leakage of [[lymph]] into the lumen of gut such as: | ||
*[[Lymphoma]] | *[[Lymphoma]] | ||
*Congenital or acquired lymphatic diseases | *[[Congenital]] or acquired lymphatic diseases | ||
*Lymphatic [[filariasis]] | *Lymphatic [[filariasis]] | ||
*[[Sarcoidosis]] | *[[Sarcoidosis]] | ||
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*[[Fortan surgical procedure]] | *[[Fortan surgical procedure]] | ||
*[[Cirrhosis]] with [[portal hypertension]] | *[[Cirrhosis]] with [[portal hypertension]] | ||
*[[Retroperitoneal fibrosis]] | *[[Retroperitoneal fibrosis]] | ||
==Complete Differential Diagnosis Of Underlying Causes== | ==Complete Differential Diagnosis Of Underlying Causes== | ||
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==Diagnosis== | ==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. | 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'''=== | ==='''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 <sup>51</sup>Cr-albumin. | 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 <sup>51</sup>Cr-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. | *[[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) | 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. | [[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. | *51Cr-labeled albumin can also be measured followed by stool collection to determine the amount of protein loss into the gastrointestinal tract. | ||
==='''Imaging Studies'''=== | ==='''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. | 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. | *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. | ||
Revision as of 14:41, 10 February 2021
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
Common Causes
Most cases of protein losing enteropathy are caused as a result of:
- Primary gastrointestinal disorders
- 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:
- Inflammatory bowel diseases (Crohn disease, Ulcerative colitis)
- Malignancies involving the gut mucosa
- Graft vs. host disease
- Esophageal and gastric erosions or ulcerations
- Carcinoid syndrome
- Bacterial infection with Clostridium difficile causing pseudomembranous colitis
- Parasitic infection with Giardia
Non-Erosive/Ulcerative Mucosal involvement
- Celiac disease
- Eosinophilic gastritis
- Hypertrophic gastritis
- Connective tissue disorders: Systemic lupus erythematosus
- Cutaneous burns
Lymphatic Obstruction
Conditions responsible for causing lymphatic obstruction leading to the leakage of lymph into the lumen of gut such as:
- Lymphoma
- Congenital or acquired lymphatic diseases
- Lymphatic filariasis
- Sarcoidosis
- Cardiovascular diseases: Congestive heart failure, Restrictive pericarditis
- Intestinal Tuberculosis
- Fortan surgical procedure
- Cirrhosis with portal hypertension
- Retroperitoneal fibrosis
Complete Differential Diagnosis Of Underlying Causes
- Acute gastroenteritis
- AIDS
- Allergic Gastroenteritis
- Amyloidosis
- Angioedema
- Bacterial overgrowth
- Carcinoid Syndrome
- Celiac Sprue
- Clostridium Difficile
- Congestive Heart Failure
- Connective tissue disorders
- Constrictive pericarditis
- Crohn's Disease
- Duodenal erosions or ulcerations
- Esophageal erosions or ulcerations
- Graft vs. Host Disease
- Henoch-Schonlein Purpura
- Idiopathic ulcerative jejunoileitis
- Intestinal endometriosis
- Intestinal parasites
- Kaposi Sarcoma
- Lymphoenteric fistula
- Lymphoma
- Menetrier's Disease
- Microscopic colitis
- Mucosal-based neoplasm
- Neurofibromatosis
- Protein dyscrasia
- Pseudomembranous colitis
- Retroperitoneal fibrosis
- Sarcoidosis
- Stomach (erosions, ulcerations)
- Tropical sprue
- Tuberculosis
- Ulcerative Colitis
- Whipple's Disease
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.
- Radiographic contrast studies and endoscopy can be performed to evaluate the ulcerative or erosive gastrointestinal causes of the protein loss.
- For detecting cardiac diseases causing loss of protein, echocardiography or radionuclide scanning of the heart can be performed.
Other tests:
- All patients should undergo basic laboratory tests such as complete blood test, liver and renal function tests.
- Work up for autoimmune diseases such as systemic lupus erythematosus should be ordered if there is a suspicion of connective tissue disorder causing protein loss in the gastrointestinal tract.
- 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.