L-Carnitine Deficiency Causing Hypoxic Respiratory Failure Due to Recurrent Flash Pulmonary Edema

Abstract

SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease I SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: We present a unique case of recurrent flash pumonary edema associated with L-carnitine deficiency. The purpose of our case is to establish clinical significance of L-carnitine replacement, since its deficiency causes pulmonary edema, especially in chronic hepatitis patients. CASE PRESENTATION: 45-y/o African American female with history of cirrhosis from chronic Hepatitis B on Lamivudine was admitted with one week of productive cough with pleuritic pain, nausea and lethargy. On physical exam there were decreased breath sounds bilaterally. ProBNP was elevated at 5297 ng/L. Chest X-ray (pic 1) and CT chest (pic 2) showed bilateral pulmonary vascular congestion without pleural effusions. 2D Transthoracic ECHO showed LVEF-60% with diastolic dysfunction. Upon admission, patient was intubated for acute hypoxic respiratory failure secondary to pulmonary edema. HIV-PCR, PCP, Urine for Legionella Antigen, and AFB cultures were negative. Patient was started on antibiotics for pneumonia based on BAL cultures that grew ESBL E.coli. Patient's severe sepsis significantly improved, but failed multiple weaning trials. She developed recurrent flash pulmonary edema, as noticed on CXR, at the time of weaning from mechanical ventilation that prompted us to check serum carnitine level. Her carnitine levels were extremely low. Despite multiple aggressive attempts to diurese the patient, her clinical condition continued to deteriorate, due to recurrent flash pulmonary edema, requiring hemodialysis until carnitine was replaced. Following carnitine replacement patient’s clinical condition significantly improved, recurrent pulmonary edema completely resolved and hemodialysis was discontinued. DISCUSSION: Serum carnitine is readily measurable and can easily be substituted, but there are no guidelines yet for adults (1). Carnitine levels in patients with chronic hepatitis are lower than in healthy individuals. There are case reports that suggest secondary carnitine deficiency due to some medications like Valproate, Adefovir and Zidovudine. In our patient, carnitine deficiency could be due to chronic hepatitis or even Lamivudine (2,3). CONCLUSIONS: We can clearly establish from our case that carnitine deficiency must be considered as a reversible cause for recurrent flash pulmonary edema in chronic hepatitis patients. Prompt recognition and replacement of carnitine reverses the clinical symptoms. Reference #1: E. Gilbert. Carnitine deficiency. Pathology, 1985;17(2):161-71. Reference #2: M. Vilaseca, R. Artuch, C. Sierra, et al. Low serum carnitine in HIV-infected children on antiretroviral treatment. European Journal of Clinical Nutrition, 2003;57:1317-1322. Reference #3: L. Jansen, A. Niet, F. Stelma, et al. HBsAg loss in patients treated with peginterferon alfa-2a and adefovir is associated with SLC16A9 gene variation and lower plasma carnitine levels. Journal of Hepatology, 2014;61(4):730-737. DISCLOSURE: The following authors have nothing to disclose: Alexandra Sokolova, Shruti Patel, Jagadish Akella No Product/Research Disclosure Information