protein-losing enteropathy

Protein-losing enteropathy (PLE) is a rare condition characterized by an excessive loss of plasma proteins into the gastrointestinal tract, leading to abnormally low levels of protein in the blood [5][6]. This occurs when albumin and other protein-rich materials leak into the intestine, causing symptoms such as hypoalbuminemia, diarrhea, and malabsorption [1][4].

PLE is often a complication of an underlying gastrointestinal problem or previous heart surgery [11], and can also be caused by disruptions in the lymphatic system, which plays a crucial role in immune function and nutrient delivery to the body [14]. The condition can occur in many clinical conditions, including nearly all gastrointestinal diseases and a large number of non-gut conditions [13].

Symptoms of PLE may include weight loss, fatigue, and swelling due to fluid retention [1][6]. In children, PLE is rare but can cause significant nutritional problems when it does occur [3]. The condition requires a team approach for management, involving medical treatments and dietary changes to help manage symptoms and prevent complications [10].

PLE is characterized by an excessive loss of proteins via the gastrointestinal tract, leading to hypoalbuminemia [5][8]. It is essential to rule out other causes of hypoproteinemia and diarrhea or malabsorption when diagnosing PLE, as well as performing imaging studies and assessing systemic manifestations of the condition [15].

References: [1] - Context result 1 [3] - Context result 3 [4] - Context result 4 [5] - Context result 5 [6] - Context result 6 [13] - Context result 13 [14] - Context result 14 [15] - Context result 15

Protein-losing enteropathy (PLE) can manifest in various ways, depending on the underlying cause and individual factors. Here are some common signs and symptoms associated with PLE:

• Edema: Swelling of the legs, arms, or other areas due to peripheral edema secondary to decreased plasma albumin levels [9][11]
• Diarrhea: Frequent bowel movements or loose stools can be a symptom of PLE, especially if the condition is caused by an underlying gastrointestinal issue [3]
• Feeding intolerance: Difficulty eating or digesting food, which can lead to weight loss and malnutrition [3]
• Weight loss: Unintentional weight loss due to malabsorption of nutrients and proteins [4][10]
• Ascites: Retention of fluid in the stomach, which can cause discomfort and shortness of breath [12]
• Hypoalbuminemia: Low levels of albumin in the blood, which can lead to edema and other complications [8]

It's essential to note that the symptoms of PLE can vary widely depending on the underlying cause and individual factors. Some people may experience mild symptoms, while others may have more severe manifestations.

References: [3] - Context 3 [4] - Context 4 [8] - Context 8 [9] - Context 9 [10] - Context 10 [11] - Context 11 [12] - Context 12

Protein-losing enteropathy (PLE) can be diagnosed using various tests, which are aimed at detecting the loss of serum proteins from the digestive tract.

Laboratory Tests

• Serum protein electrophoresis: This test measures the levels of different types of proteins in the blood, including albumin and globulin. Low levels of these proteins may indicate PLE.
• Lymphocyte count: An elevated lymphocyte count can suggest lymphatic obstruction or other underlying conditions contributing to PLE.
• Serum cholesterol: Elevated serum cholesterol levels can be a sign of lymphatic obstruction.

Imaging Tests

• Colonoscopy: This procedure allows for the visualization of the colon and rectum, which may reveal inflammation, ulcers, or other abnormalities that could be causing PLE.
• Esophagogastroduodenoscopy (EGD): EGD is used to examine the upper part of the digestive tract, including the esophagus, stomach, and duodenum. This test can help identify conditions such as gastritis or ulcers that may contribute to PLE.
• Small intestine biopsy: A biopsy of the small intestine can be performed to rule out other conditions that may cause protein loss.

Specialized Tests

• Alpha-1-antitrypsin (A1AT) clearance test: This is a specific test used to diagnose PLE, which measures the levels of A1AT in the blood and stool.
• Small bowel capsule endoscopy: This procedure involves swallowing a small camera that takes pictures of the inside of the small intestine, allowing for the visualization of any abnormalities.

Other Tests

• Stool cultures: These tests can help identify bacterial or parasitic infections that may be contributing to PLE.
• Serologic evaluation: This test measures the levels of certain antibodies in the blood, which can indicate autoimmune disorders or other underlying conditions.
• Radiographic imaging: Imaging tests such as X-rays or CT scans may be used to rule out other conditions that could be causing protein loss.

It's worth noting that a combination of these tests is often necessary to accurately diagnose PLE and determine its underlying cause.

Protein-losing enteropathy (PLE) can be effectively managed through various drug treatments, which aim to correct the underlying cause of the condition.

• Corticosteroids: Corticosteroids, such as prednisone, may be prescribed to reduce inflammation and swelling in the intestines, thereby alleviating protein loss [6]. However, their effectiveness can vary depending on the underlying cause of PLE.
• Complement inhibitors: Complement inhibitors, like eculizumab, have been shown to be effective in treating PLE caused by CD55 deficiency [7].
• **Heparin

Protein-losing enteropathy (PLE) is a condition characterized by the excessive loss of plasma proteins through the gastrointestinal tract, leading to hypoalbuminemia and other complications. Establishing an accurate diagnosis of PLE requires a thorough differential diagnosis to rule out other causes of hypoproteinemia.

Possible Causes:

• Increased lymphatic pressure [7]
• Collagenous and lymphocytic colitis [8]
• Hypoalbuminaemia [8]
• Hypogammaglobulinaemia [8]
• Inflammatory bowel disease [8]
• Congenital heart disease, cancers, immune disorders, inflammation of the gastrointestinal tract, and other conditions [5]

Other Conditions to Consider:

• Common Variable Immunodeficiency (CVID) [4]
• Systemic Lupus Erythematosus (SLE) [3]
• Ileal Crohn's disease [3]
• Primary biliary cholangitis [14]
• Cardiac and liver diseases, lupus, sarcoidosis, and other non-gut conditions [15]

Diagnostic Approach:

Establishing a diagnosis of PLE may require a multidisciplinary approach and the use of various diagnostic tests, including:

• Measurement of serum proteins and albumin levels
• Gastrointestinal clearance of alpha1-antitrypsin [12]
• Endoscopy and biopsy to rule out other conditions
• Imaging studies to evaluate lymphatic pressure and other potential causes

References:

[3] Alessio M. Differential diagnosis of hypoalbuminemia in childhood: protein losing enteropathy associated to ... (search result 1) [4] PLE can occur during CVID and requires higher IgG replacement therapy dosage. PLE can also mimic CVID and is associated with milder immunological abnormalities. (search result 4) [5] PLE has been linked to more than 60 diseases and disorders, including congenital heart disease, cancers, immune disorders, inflammation of the gastrointestinal ... (search result 5) [7] by MJAM Braamskamp · 2010 · Cited by 205 — PLE is a rare complication of various diseases, mostly gastrointestinal or cardiac conditions that result into loss of proteins in the gastrointestinal tract. (search result 7) [8] Differential diagnosis of protein-losing enteropathy. Collagenous and lymphocytic colitis. Hypoalbuminaemia. Hypogammaglobulinaemia. Inflammatory bowel disease. (search results 8, 9, 10, 11, 12, 13)