Reversal of protein losing enteropathy with prednisone in adults with modified fontan operations: long term palliation or bridge to cardiac transplantation?
نویسندگان
چکیده
Protein losing enteropathy (PLE), defined as severe loss of serum protein into the intestine, occurs in 4-13% of patients after the Fontan procedure and carries a dismal prognosis with a five year survival between 46% and 59%. Chronically raised systemic venous pressure is thought to be responsible for the development of PLE in these patients, with perhaps superimposed immunological or inflammatory factors. The success rate of contemporary medical, transcatheter, and surgical treatments attempting to reduce systemic venous pressure ranges from 19% to 40%. Prednisone treatment for PLE has been tried, with variable success rates reported in children. The effect of prednisone in adult patients with PLE after the Fontan procedure is largely unknown. Two cases of PLE in adults (a 39 year old woman and a 25 year old man) after modified Fontan procedure who responded dramatically to oral prednisone treatment are reported, suggesting that a trial of this "non-invasive" treatment should be considered as long term palliation or bridge to cardiac transplantation.
منابع مشابه
CASE STUDY Reversal of protein losing enteropathy with prednisone in adults with modified Fontan operations: long term palliation or bridge to cardiac transplantation?
Protein losing enteropathy (PLE), defined as severe loss of serum protein into the intestine, occurs in 4–13% of patients after the Fontan procedure and carries a dismal prognosis with a five year survival between 46% and 59%. Chronically raised systemic venous pressure is thought to be responsible for the development of PLE in these patients, with perhaps superimposed immunological or inflamma...
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1 Hill D, Feldt R, Porter C, Puga F, Danielson G, Perrault J. Protein-losing enteropathy after Fontan operation: a preliminary report. Circulation 1989;80(suppl II):490. 2 Rothman A, Snyder J. Protein-losing enteropathy following the Fontan operation: resolution with prednisone therapy. Am HeartJ 1991;121:618-619. 3 Rychick J, Piccoli DA, Barber G. Usefulness of corticosteroid therapy for prote...
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BACKGROUND Protein-losing enteropathy (PLE) is a disorder characterized by abnormal and often profound enteric protein loss. It's relatively uncommon complication of Fontan and other complex congenital heart disease (CCHD) procedures. Because of the complexity and rarity of this disease process, the pathogenesis and pathophysiology of protein-losing enteropathy remain poorly understood, and att...
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ورودعنوان ژورنال:
- Heart
دوره 82 2 شماره
صفحات -
تاریخ انتشار 1999