Protein-losing enteropathy following the Fontan operation: Resolution with prednisone therapy

Rothman, Abraham; Snyder, John

doi:10.1016/0002-8703(91)90743-2

Cited by 69 publications

(47 citation statements)

References 3 publications

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“…First, steroid treatment has been shown to be effective in some patients, when used in high doses (1-2 mg/kg prednisone equivalent) for extended periods of time (weeks to months). [30][31][32] This phenomenon strongly suggests for the presence of an inflammatory component to the pathophysiology of this disease. Through anabolic effects, steroids may result in enteric cell membrane stabilization and perhaps also increase patient appetitie, reversing the negative nitrogen balance taking place in PLE.…”

Section: Diagnosis and Manifestations Of Ple After Fontanmentioning

confidence: 76%

Protein-Losing Enteropathy after Fontan Operation

Rychik¹

2007

Congenital Heart Disease

169

194

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A B S T R A C TProtein-losing enteropathy (PLE) is a poorly understood and enigmatic disease process affecting patients with single ventricle after Fontan operation. In those afflicted, PLE after Fontan operation results in significant morbidity and mortality. The pathophysiology of the disease is unknown; however, a proposed mechanism incorporates a combination of phenomena including: (1) altered hemodynamics, specifically low cardiac output; (2) increased mesenteric vascular resistance; (3) systemic inflammation; and (4) altered enterocyte basal membrane glycosaminoglycan make-up. A paradigm for the clinical management of PLE after Fontan operation is proposed.

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Section: Diagnosis and Manifestations Of Ple After Fontanmentioning

confidence: 76%

Protein-Losing Enteropathy after Fontan Operation

Rychik¹

2007

Congenital Heart Disease

169

194

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“…Pharmacological strategies directed at reducing intestinal inflammation and protein losses include heparin 161,162 and steroids. 158,163,164 Long-term steroid therapy requires close monitoring and treatment of side effects such as depression, osteoporosis, infection, adrenal insufficiency, and DM. 165 Periodic serum albumin infusions with diuretics may provide short-term relief of symptoms but rarely offer resolution of PLE.…”

Section: Managementmentioning

confidence: 99%

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

Lui¹,

Saïdi²,

Bhatt³

et al. 2017

Circulation

176

136

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ABSTRACT:Life expectancy and quality of life for those born with congenital heart disease (CHD) have greatly improved over the past 3 decades. While representing a great advance for these patients, who have been able to move from childhood to successful adult lives in increasing numbers, this development has resulted in an epidemiological shift and a generation of patients who are at risk of developing chronic multisystem disease in adulthood. Noncardiac complications significantly contribute to the morbidity and mortality of adults with CHD. Reduced survival has been documented in patients with CHD with renal dysfunction, restrictive lung disease, anemia, and cirrhosis. Furthermore, as this population ages, atherosclerotic cardiovascular disease and its risk factors are becoming increasingly prevalent. Disorders of psychosocial and cognitive development are key factors affecting the quality of life of these individuals. It is incumbent on physicians who care for patients with CHD to be mindful of the effects that disease of organs other than the heart may have on the well-being of adults with CHD. Further research is needed to understand how these noncardiac complications may affect the long-term outcome in these patients and what modifiable factors can be targeted for preventive intervention.

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“…Many possible therapies have been suggested and these are directed symptomatic relief with diuretics (110) and dietary modifications, (111,112) reduction in intestinal protein loss with the use of steroids (113)(114)(115)(116) and heparin (117)(118)(119)(120)(121)(122)(123) and improving the haemodynamics as described above.…”

Section: Protein-losing Enteropathy (Ple)mentioning

confidence: 99%

Management of the Fontan circulation in the adult patient

Clift

2017

SAHJ

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Protein-losing enteropathy following the Fontan operation: Resolution with prednisone therapy

Cited by 69 publications

References 3 publications

Protein-Losing Enteropathy after Fontan Operation

Protein-Losing Enteropathy after Fontan Operation

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

Management of the Fontan circulation in the adult patient

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