Protein-Losing Enteropathy (PLE)

What is protein-losing enteropathy (PLE)?

Protein-losing enteropathy is a broad term that refers to the loss of serum proteins from the digestive tract. This causes an abnormally low level of albumin (a protein made by the liver) and other proteins in the blood stream and can lead to edema (retention of fluid in tissue) and ascites (retention of fluid in the stomach). In some cases the loss of protein is due to abnormalities in lymphatic flow. 

Lymph vessels carry lymph fluid to veins, where it returns to the bloodstream, playing a crucial role in immune function and fat and protein transport. When something disrupts or damages normal lymphatic flow, it can cause protein rich lymphatic fluid to leak into the intestine. 

Causes

Protein-losing enteropathy has several potential causes, including:

  • Heart failure
  • Previous surgical repair of a single ventricle heart condition
  • Cancer
  • Infections
  • Intestinal lymphangiectasia
  • Inflammatory bowel disease

Signs and symptoms

Some of the symptoms of protein-losing enteropathy include:

  • Diarrhea
  • Feeding intolerance (when an infant or child is unable to eat or digest food without getting sick)
  • Edema (fluid retention)
  • Ascites (fluid retention in the abdomen)

Diagnosis

Doctors use a combination of imaging and laboratory tests to diagnose protein-losing enteropathy. Normally the diagnosis is confirmed by testing the stool for loss of protein. Other tests may include X-ray, computerized tomography (CT), ultrasound or MRI

Additionally, lymphatic imaging modalities, such as intranodal lymphangiography, liver lymphangiogram, and dynamic contrast magnetic resonance lymphangiography, might be used to determine the exact source of the lymphatic leak.

Treatment

Treatment of PLE can be challenging, but we are now working on several new treatment options for patients with this disorder. At The Children’s Hospital of Philadelphia and the Hospital of the University of Pennsylvania, patients are treated by a team of experts who specialize in lymphatic imaging and interventions through the Center for Lymphatic Imaging and Interventions.

The team will determine the best treatment approach for each patient’s condition. When lymphatic imaging is able to identify the source of the leak that’s causing protein-losing enteropathy, it can potentially be sealed during an embolization procedure.

Long-term outlook

Experts at the Center for Lymphatic Imaging and Interventions have been developing new imaging and treatment options for these patients. It is too early to tell what the long-term outlook will be for these new treatment options.

Follow-up care

Most of the follow-up care will be done by your or your child’s primary physician in coordination with our team for special needs related to the procedure or the lymphatic disorder. This can include recommendations for new medications, medication weans or diet recommendations.

Research

Experts from the Hospital of the University of Pennsylvania and CHOP are collaborating on research to better understand the lymphatic system, develop new lymphatic interventional procedures, and to improve lymphatic imaging modalities.

Why choose us

Our team has the most experience in the world treating both children and adults with protein-losing enteropathy. We have developed a variety of innovative imaging and interventional techniques that have advanced the minimally invasive treatment options available to patients.