American Journal of Medical Case Reports
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American Journal of Medical Case Reports. 2019, 7(5), 79-86
DOI: 10.12691/ajmcr-7-5-2
Open AccessCase Report

A Very Uncommon Case of Pulmonary Arterial Hypertension

Jehad Azar1, Ali Varasteh1, Daniel Iltchev2, Mona Soliman1, Victor Baez1 and Basel Altaqi2,

1Internal Medicine Resident, St. Vincent Charity Medical Center, Cleveland, USA

2Pulmonary and Critical Care Medicine, St. Vincent Charity Medical Center, Cleveland, USA

Pub. Date: May 11, 2019

Cite this paper:
Jehad Azar, Ali Varasteh, Daniel Iltchev, Mona Soliman, Victor Baez and Basel Altaqi. A Very Uncommon Case of Pulmonary Arterial Hypertension. American Journal of Medical Case Reports. 2019; 7(5):79-86. doi: 10.12691/ajmcr-7-5-2


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.

pulmonary arterial hypertension scurvy right side heart failure hypoxia inducible transcription factors nitric oxide

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