Protein‐Losing Enteropathy and the Fontan Operation

Johnson, Jonathan N.; Driscoll, David J.; O’Leary, Patrick

doi:10.1177/0884533612444532

Cited by 68 publications

(63 citation statements)

References 49 publications

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“…This procedure markedly increases the central venous pressure and in 3%–18% of subjects, PLE appears from 2 to 10 years after the surgery 48. This rare form of PLE (prevalence of <2,000 in USA49) has been intensively investigated because it is a leading cause of morbidity and mortality in patients undergoing the Fontan procedure 50,51.…”

Section: Increased Lymphatic Pressurementioning

confidence: 99%

Protein losing enteropathy: comprehensive review of the mechanistic association with clinical and subclinical disease states

Levitt¹,

Levitt²

2017

CEG

116

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Protein losing enteropathy (PLE) has been associated with more than 60 different conditions, including nearly all gastrointestinal diseases (Crohn’s disease, celiac, Whipple’s, intestinal infections, and so on) and a large number of non-gut conditions (cardiac and liver disease, lupus, sarcoidosis, and so on). This review presents the first attempt to quantitatively understand the magnitude of the PLE in relation to the associated pathology for three different disease categories: 1) increased lymphatic pressure (e.g., lymphangiectasis); 2) diseases with mucosal erosions (e.g., Crohn’s disease); and 3) diseases without mucosal erosions (e.g., celiac disease). The PLE with lymphangiectasis results from rupture of the mucosal lymphatics, with retrograde drainage of systemic lymph into the intestinal lumen with the resultant loss of CD4 T cells, which is diagnostic. Mucosal erosion PLE results from macroscopic breakdown of the mucosal barrier, with the epithelial capillaries becoming the rate-limiting factor in albumin loss. The equation derived to describe the relationship between the reduction in serum albumin (CP) and PLE indicates that gastrointestinal albumin clearance must increase by at least 17 times normal to reduce the CP by half. The strengths and limitations of the two quantitative measures of PLE (51Cr-albumin or α1-antitrypsin [αAT] clearance) are reviewed. αAT provides a simple quantitative diagnostic test that is probably underused clinically. The strong, unexplained correlation between minor decreases in CP and subsequent mortality in seemingly healthy individuals raises the question of whether subclinical PLE could account for the decreased CP and, if so, could the mechanism responsible for PLE play a role in the increased mortality? A large-scale study correlating αAT clearance with serum albumin concentrations will be required in order to determine the role of PLE in the regulation of the serum albumin concentration of seemingly healthy subjects.

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Section: Increased Lymphatic Pressurementioning

confidence: 99%

Protein losing enteropathy: comprehensive review of the mechanistic association with clinical and subclinical disease states

Levitt¹,

Levitt²

2017

CEG

116

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“…150,160 The treatment of PLE typically requires multiple strategies, but the efficacy of each treatment strategy remains unknown. Intestinal therapies comprise a low-fat/highprotein diet and medium-chain triglycerides, which act to reduce intestinal lymphatic flow and fluid losses.…”

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

180

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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“…Increased pressure in the lymphatic system may cause trauma to the duct and lead to the development of chylothorax. 35 Medical management for resolution of a chylous effusion can be multifactorial. Chylothorax is diagnosed by the biochemical analysis of the pleural fluid, with identification of elevated triglyceride 300 TOPICS IN CLINICAL NUTRITION/JULY-SEPTEMBER 2012 concentration (>150 mg/dL), lymphocyte concentration greater than 80%, and high total protein content.…”

Section: Chylothoraxmentioning

confidence: 99%

Nutrition Through the Life Cycle in Patients With Congenital Heart Disease

Bailer

Savoca

2012

Topics in Clinical Nutrition

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Single ventricle physiology, including hypoplastic left heart syndrome, encompasses the most severe cardiac lesions. Despite improvements in palliation and care, failure to thrive remains a major concern specifically among the neonatal and infant population. This population may develop other nutritional challenges throughout the life span, which adversely affect growth, morbidity, and mortality. This review of the literature uniquely describes the staged surgical palliation and discusses clinical practice related to nutritional complications associated with the disease process, such as chylothorax and protein-losing enteropathy. A registered dietitian is an essential part of the multidisciplinary team to provide expert medical nutrition therapy for this challenging population.

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Protein‐Losing Enteropathy and the Fontan Operation

Cited by 68 publications

References 49 publications

Protein losing enteropathy: comprehensive review of the mechanistic association with clinical and subclinical disease states

Protein losing enteropathy: comprehensive review of the mechanistic association with clinical and subclinical disease states

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

Nutrition Through the Life Cycle in Patients With Congenital Heart Disease

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