What Is Protein Losing Enteropathy (PLE)?

Protein-losing enteropathy (PLE) is a disease condition that occurs when albumin and other protein-rich materials leak into the intestinal cavity. The most abundant protein in the blood, albumin’s many functions in the body include hormone transport and retaining water in the bloodstream to maintain fluid balance and osmotic pressures. In the hospital setting, low albumin (hypoalbuminemia) is associated with poor surgical outcomes and increased mortality.

Symptoms of PLE

Patients with PLE may experience the following symptoms:

  • Hypoalbuminemia (low levels of albumin in the blood)
  • Diarrhea
  • Tissue swelling (edema)
  • Ascites (excess fluid trapped in the abdomen)
  • Pleural and pericardial effusions (excess fluid around the heart)
  • Hypoproteinemia (lower than normal protein levels in the body)
  • Severe malnutrition

Causes of PLE

PLE is very complex and has many proposed causes, including damage to the inner layers of the intestine and bowel wall structure. As important as the causes of PLE, however, are its effects, which include decreased protein levels in the blood plasma, fluid imbalance as a result of albumin depletion and lymphatic system dysfunction.

PLE has been linked to more than 60 diseases and disorders, including congenital heart disease, cancers, immune disorders, inflammation in any part of the gastrointestinal tract and all of the intestinal and digestive diseases.

One of the ways that PLE works in patients with heart disease has only recently come to light. In these patients, PLE is known to cause elevated pressure inside the blood vessels. This led researchers to consider whether this increased pressure could cause congestion in the liver that in turn would result in increased production of lymph in the surrounding lymph system. The test to evaluate the lymph system associated with the liver is called liver lymphangiography. When the researchers performed this test in patients with heart disease, it showed that lymph was leaking from the liver into the intestines.

Normal liver lymphatic system Liver lymphatic system with PLE

A recent report from Penn Medicine and Children’s Hospital of Philadelphia (CHOP) found that leakage of albumin-rich lymph into the intestine, predominantly through rupture of abnormal lymphatic vessels linking the liver and duodenum (the first part of the small intestine), is a principal mechanism of albumin loss in patients with congestive heart disease (CHD) exhibiting PLE.

Diagnosis of Protein Losing Enteropathy (PLE)

PLE is a diagnosis of exclusion. Once suspected from its symptoms, PLE can be confirmed by measuring albumin and protein levels in the blood, plasma, and urine, and of alpha-1-antitrypsin, a sensitive protein marker, in the feces.

In selected patients, dynamic contrast-enhanced MR lymphangiography is performed in conjunction with upper endoscopy in order to confirm the location of the leakage point in the duodenum, by injecting of the blue dye in the liver lymphatic system.

Blue dye in liver lymphatic system used to diagnose Protein-losing enteropathy (PLE)

Blue dye injected in liver lymphatic system to diagnose PLE

Treatment of PLE at Penn

Clinician researchers at Penn Medicine and CHOP confirmed that protein-rich lymph was leaking from the lymphatic vessels linking the liver and small intestine in CHD patients with PLE. They then realized that embolization, a technique that uses medical glue to block lymphatic vessels, could be an effective treatment for PLE. The researchers then conducted a study to assess the efficacy of therapeutic imaging and embolization of the affected vessels.

The study determined that liver lymphatic embolization results in sustained improvement of serum albumin levels and the symptoms of PLE in patients with radiographic and endoscopic confirmation of lymphatic leakage into the duodenum.

Procedure

Embolization (closure) of the liver and retroperitoneal lymphatic vessels, that leak into the intestinal with surgical glue diluted with a contrast agent can result in resolution of the patient’s symptoms.

The embolization is performed by injecting of the glue through very small needle inserted in the liver lymphatic vessels under fluoroscopic guidance.

Liver lymphatic embolization procedure

The glue propagates and spreads through the retroperitoneal lymphatic ducts until it reaches the holes in duodenum and closes them.

Penn Programs & Services for Protein Losing Enteropathy (PLE)

Lymphatic Disorders

Penn Interventional Radiology is a world leader in treating lymphatic flow disorders and conditions of the lymphatic system.

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