Acquired combined immunodeficiency associated with protein losing enteropathy complicating Fontan operation

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Acquired combined immunodeficiency associated with protein losing enteropathy complicating Fontan operation.

It is vitally important that the immunological aspect of protein losing enteropathy following Fontan procedures is highlighted, in order to decrease significant morbidity and mortality

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Protein-losing enteropathy after the Fontan operation.

Patients were observed after the Fontan operation to determine the frequency and severity of protein-losing enteropathy. A total of 427 patients who survived for 30 days after the Fontan operation, performed between 1973 and January 1987, were analyzed and, thus far, protein-losing enteropathy has developed in 47 of 427. The cumulative risk for the development of protein-losing enteropathy by 1...

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Clinical outcomes and improved survival in patients with protein-losing enteropathy after the Fontan operation.

BACKGROUND Patients with protein-losing enteropathy (PLE) following the Fontan operation have a reported 50% mortality at 5 years after diagnosis. OBJECTIVES The aim of this study was to review outcomes in patients with PLE following the Fontan operation. METHODS From 1992 to 2010, 42 patients (55% male) with PLE following the Fontan operation were identified from clinical databases at the ...

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Protein losing enteropathy associated with collagen diseases.

Four patients with collagen diseases are described, who developed protein losing enteropathy in the course of their disease. Their protein losing enteropathies subsided after treatment with 60 mg/day oral prednisolone. Immunohistological studies of the small intestine showed deposits of C3 in the capillary walls of villi in two patients. Increased capillary permeability due to autoimmune phenom...

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Protein Losing Enteropathy after Fontan Surgery – Clinical and Diagnostical Aspects

Protein losing enteropathy (PLE) is the massive enteric loss of serum protein. PLE may appear in several diseases associated with intestinal mucous membrane damages with or without infection. PLE is mostly associated with total cavopulmonary connection (TCPC) or Fontan-type circulation in patients with a functionally univentricular heart. TCPC is performed at an age of about 2 years or older an...

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ژورنال

عنوان ژورنال: Heart

سال: 2003

ISSN: 0007-0769

DOI: 10.1136/heart.89.10.1130