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      Chyloptysis causing plastic bronchitis

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          Abstract

          Chyloptysis is a rare clinical problem that is associated with conditions affecting lymphatic channels in the thorax. Diagnosis is usually made when the patients present with expectoration of milky-white sputum or of thick tenacious mucus in the shape of smaller bronchi (bronchial cast). Typically the symptoms resolve after coughing up of the bronchial casts. Pleural, mediastinal, pulmonary or lymphatic abnormalities result in chyloptysis. Lymphangiography and detection of lipids (cholesterol or triglycerides) in sputum help to establish the diagnosis. However, lymphangiography may not be positive in all patients. We report 2 patients with chyloptysis and bronchial casts with different etiologies. Abnormal lymphatics were demonstrated in one of our cases, but the second patients lymphangiogram was normal. In this patient we suspect that high venous filling pressures due to congestive heart failure had a causative effect in the setting of compromised lymphatic drainage in the thorax due to a prior history of radiation therapy to the chest for lymphoma.

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          Most cited references11

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          Chylothorax: aetiology, diagnosis and therapeutic options.

          Chylothorax is a rare condition that results from thoracic duct damage with chyle leakage from the lymphatic system into the pleural space, usually on the right side. It has multiple aetiologies and is usually discovered after it manifests itself as a pleural effusion. Diagnosis involves cholesterol and triglyceride measurement in the pleural fluid. Complications include malnutrition, immunosuppression and respiratory distress. Treatment may be either conservative or aggressive depending on the clinical scenario. In this review, we discuss the aetiology, diagnosis and treatment of chylothorax. English language publications in MEDLINE and references from relevant articles from January 1, 1980 to February 28, 2008 were reviewed. Keywords searched were chylothorax, aetiology, diagnosis and treatment. Copyright 2009 Elsevier Ltd. All rights reserved.
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            Dietary modification of chyle composition in chylothorax.

            Nutrition support has played a major role in the treatment of chylothorax, both to prevent malnutrition and to minimize chyle production and flow. This report evaluates chyle composition in a patient with chylothorax who was placed on a low-fat diet, medium-chain triglyceride diet, and total parenteral nutrition in sequence. Both triglyceride content and volume of chyle declined, but drainage persisted, ultimately requiring thoracic duct ligation. The chyle triglyceride while on total parenteral nutrition, which presumably originates from both the intestine and plasma, contained more long-chain unsaturated fatty acids than the circulating serum triglyceride. Of particular interest was the detection of an appreciable amount of medium-chain fatty acids in the chyle triglyceride, constituting 20% of the triglyceride fatty acids when an enteral formulation with medium-chain triglyceride as a sole fat source was administered. The finding of almost threefold more decanoic acid (C10:0) than octanoic acid (C8:0), despite the presence of considerably more octanoic acid in the original diet, suggests that trioctanoin may be a preferable medium-chain triglyceride substrate for the nonsurgical treatment of chylothorax.
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              Lymphangioleiomyomatosis: current and future.

              Lymphangioleiomyomatosis is a rare slowly progressive lung disease that affects almost exclusively young women of reproductive age. It occurs sporadically or in association with Tuberous Sclerosis Complex. LAM is characterized by cystic remodeling of the lung parenchyma, due to proliferation of abnormal smooth muscle-like LAM cells and presence of extra pulmonary manifestations such as lymphadenopathy, angiomyolipomas and abdominal lymphangioleiomyomas. The most common clinical manifestations are progressive dyspnea on exertion, pneumothorax and chylous effusions. Currently there is no curative treatment for the disease, but the ongoing study of the genetic and molecular pathways implicated in the pathogenesis of the disease could lead to targeted therapy.
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                Author and article information

                Contributors
                Journal
                Respir Med Case Rep
                Respir Med Case Rep
                Respiratory Medicine Case Reports
                Elsevier
                2213-0071
                08 June 2014
                2014
                08 June 2014
                : 13
                : 4-6
                Affiliations
                [a ]University of Vermont College of Medicine, United States
                [b ]Pulmonary, Allergy, Critical Care and Sleep Medicine, University of Minnesota, MMC 276, 420 Delaware St SE, Minneapolis, MN 55455, United States
                Author notes
                []Corresponding author. Tel.: +1 612 626 9338. bharg005@ 123456umn.edu bhargavam@ 123456gmail.com
                Article
                S2213-0071(14)00003-3
                10.1016/j.rmcr.2013.12.001
                4246250
                26029546
                ec859095-5d87-4da8-91d8-6421a5aa7091
                © 2014 The Authors

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/3.0/).

                History
                Categories
                Case Report

                chyloptysis,bronchial cast,heart failure,thoracic duct duplication

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