PLE Description
PLE or Protein Losing Enteropathy is a syndrome in which children lose protein modules from the blood serum into the intestinal track. The loss of proteins also results in an impaired immune system which can not fight off infection. This impact on the immune system is a common cause of the high mortality rate.
PLE is life threatening and is associated with an alarming mortality rate of 54%.
Children with congenital heart defects must undergo surgical repair early in life in hopes of a long term survival. Patients with complex lesions such as tricuspid atresia, single ventricle syndrome, and hypoplastic left heart syndrome frequently undergo a staged surgical approach known as the Fontan operation.
This procedure has saved the lives of many children. But, in addition to immediate complications from the procedure, these children are also at risk for PLE.
The precise mechanism of this condition and why it afflicts some children and not others is unknown. Many theories have been postulated, however none of these has sufficiently explained a direct cause and effect relationship. Various medical and surgical treatments have been tried with limited success. Further research is necessary. Once the cause is better understood, more effective treatment options will be available and this devastating complication will be avoided.
Between 2,000 and 5,000 children have been diagnosed with PLE. Of the children that have major cardiac surgery, about 7-10% become afflicted with PLE. Of these, there is only a 50% survival rate.
Technical Definition:
Abstract cited in
Protein Losing Enteropathy after Fontan Surgery- Clinical and Diagnostical Aspects
A. Tarnok; J. Bocsi; D. Lenz; J. Janousek
Department of Pediatric Cardiology, Cardiac Center Leipzig GmbH,
University of Leipzig, Germany
Transfus Med Hemother 2007; 34: 164-167
"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 cardiopulmonary 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 and has a high survival rate of >90%. Time of PLE onset after TCPC is variable, the exact etiology is still unclear. Increased central venous pressure due to the absence of a subpulmonary ventricle may be a main reason for PLE< affecting 2-15% of the patients with a survival rate of 40% at 5 years and 20% at 10 years. Also immunological reasons for PLE are suspected. Major clinical signs are edemas, ascites, pleural effusion, diarrhea, malnutrition, fatigue, weight loss, and reduced physical development. The most impaired laboratory signs are elevated fecal 1-antitrypsin and 1-antitrypsin clearance, hypoproteinemia (hypoalbuminemia, hypo-Y- globulinemia), lymphadenitis (selective T helper cell loss) and secondary lymphangiectasia. Therapy considerations should have the aim to decrease central venous pressure to improve hemodynamics. Medical treatment consists of substitution of e.g. albumin, Y-globulin, glucocorticoid, heparin or calcium, but still >60% of the patients remain symptomatic.
Copyright 2007 S.Karger GmbH, Freiburg
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