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Disorders of the Pleura |

Unusual Presentation of Alpha-1 Antitrypsin Deficiency

Mahmoud Amarna, MD; Souheil Abdel Nour, MD; Haytham Adada, MD; Wael El Minaoui, MD; Ryland Byrd, MD; Thomas Roy, MD
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East Tennessee State University, Johnson City, TN


Chest. 2013;144(4_MeetingAbstracts):496A. doi:10.1378/chest.1693642
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Abstract

SESSION TITLE: Pleural Cases

SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Monday, October 28, 2013 at 04:15 PM - 05:15 PM

INTRODUCTION: Alpha-1 antitrypsin deficiency (AAD) is a rare genetic disorder. We present a patient who was diagnosed with AAD after presenting with a transudative chylothorax (TC). Chylothorax is typically exudative in nature, and TC is unusual but reported in association with liver disease. AAD was considered and documented after computerized tomography (CT) suggested emphysematous changes in the lungs and cirrhoitic changes in the liver. To our knowledge, this is the first case report of chylothorax associated with AAD induced hepatic disease.

CASE PRESENTATION: A 65 year-old white nonsmoker female patient was seen with new onset dyspnea and nonproductive cough for 6 weeks. She had no previous surgeries. She did not use ethanol. Her chest roentgenogram (CXR) showed a large right pleural effusion(Figure1). CT of the chest with IV contrast also demonstrated a small left pleural effusion, emphysema and broncheictasis but ruled out lymphadenopathy. CT of the abdomen suggested cirrhosis and splenomegaly. Echocardiogram was normal . 2000 cc of cloudy pleural fluid was obtained by thoracentesis. The fluid was transudative by Light’s criteria for protein and LDH values(Table 1). Chylothorax was diagnosed by the elevated triglyceride level. The chylothorax was managed conservatively with sodium restriction and diuresis. It resolved in one month (Figure 2). Pulmonary function tests revealed a moderate obstructive disease with a moderate decrease in diffusion capacity. Alpha-1 antritrypsin level was 28.20 mg/dL and homozygous (PiZZ) on genetic study.

DISCUSSION: Chylothorax is usually caused by a pathology in the thoracic duct related to malignancies and surgical trauma. These effuions are typically exudates and rich in triglycerides. However, TC has also been reported in patients with amyloidosis, hepatic cirrhosis, nephrotic syndrome, superior vena cava obstruction, heart failure, and chylous ascites . TC associated with hepatic cirrhosis can be caused by transdiaphragmatic movement of chylous ascites or from portal hypertension in the absence of ascites. Chylothorax is best treated by therapy directed at the underlying disease. Patients who are symptomatic due to benign chylothorax can be managed by drainage and dietary control measures to limit the flow of chyle.

CONCLUSIONS: We would like to call attention to the unusual association of a transudative chylothorax with cirrhosis of the liver caused by AAD. After a review of the literature, we could not find another report of AAD-induced cirrhosis presenting with respiratory symptoms due to chylothorax.

Reference #1: Diaz-Guzman E, Culver DA, Stoller JK. Transudative chylothorax: report of two cases and review of the literature. Lung 2005; 183:169

Reference #2: Agrawal V, Doelken P, Sahn SA. Pleural fluid analysis in chylous pleural effusion. Chest 2008; 133:1436

Reference #3: Maldonado F, Cartin-Ceba R, Hawkins FJ, Ryu JH. Medical and surgical management of chylothorax and associated outcomes. Am J Med Sci 2010; 339:314.

DISCLOSURE: The following authors have nothing to disclose: Mahmoud Amarna, Souheil Abdel Nour, Haytham Adada, Wael El Minaoui, Ryland Byrd, Thomas Roy

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