Pulmonary arterial hypertension (PAH) describes a rare, progressive disease of the pulmonary vasculature, involving a group of clinical conditions that result in precapillary pulmonary hypertension (PH). PAH is characterized by proliferative vasculopathy, and subsequent right heart failure. For the past several decades research has focused on identification of underlying molecular causes of this disease. Recently, there have been a number of reported cases of patients with scurvy developing pulmonary arterial hypertension. Thus, it has been hypothesized that vitamin C deficiency results in non-hypoxic activation of hypoxia inducible transcription factors (HIF) and low nitric oxide (NO) level in the pulmonary vasculature, leading to subsequent pulmonary vasculopathy and an exaggerated pulmonary vasoconstrictive response. Immediate supplementation of vitamin C is considered the definitive treatment, preventing an otherwise fatal outcome. In this paper, we describe a patient with a fatal case of scurvy related PAH, who was admitted with ecchymosis, oral ulceration, and dyspnea.
May-Thurner syndrome (MTS) is a clinical condition where the left common iliac vein gets compressed by the overlying right common iliac artery anterior to the fifth lumbar vertebra and the sacral promontory. It results in vessel wall injury and predisposition to thrombosis. We present a case of a 21-year-old African-American man with no significant past medical history who came to the emergency department with left lower limb swelling associated with shortness of breath, and was eventually diagnosed to have extensive left lower extremity deep vein thrombosis (DVT) along with acute bilateral extensive pulmonary embolism (PE) as a consequence to MTS. MTS should be considered in the differential when young patients present with unprovoked or recurrent left-sided DVT. Diagnosis of this anatomical variant is critical as it may need long-term anticoagulation and consideration of pharmaco-mechanical intervention such as mechanical thrombectomy and venoplasty with or without stenting.
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