Biomaterial Database

Scurvy-induced pulmonary arterial hypertension. 2023

Jehad Azar, and Mohammed Ayyad, and Yasmin Jaber, and Laith Azzam Ayasa

Pulmonary and Critical Care Department, Cleveland Clinic Foundation, Cleveland, Ohio, USA jehad.azar@gmail.com.

Pulmonary arterial hypertension (PAH) is a progressive disease of the pulmonary vasculature that results in precapillary pulmonary hypertension. PAH is caused by a group of clinical conditions involving multiple organ systems. Several cases have been reported in the literature demonstrating an association between vitamin C deficiency and PAH. Low endothelial nitric oxide levels in the pulmonary vasculature, combined with the inappropriate activation of hypoxia-inducible transcription factors, seen in patients with ascorbic acid deficiency, are believed to be the main contributors to the pathogenesis of pulmonary vasculopathy and the exaggerated pulmonary vasoconstrictive response seen in patients with scurvy-induced PAH. Vitamin C supplementation is considered the definitive treatment.

UI	MeSH Term	Description	Entries
D006976	Hypertension, Pulmonary	Increased VASCULAR RESISTANCE in the PULMONARY CIRCULATION, usually secondary to HEART DISEASES or LUNG DISEASES.	Pulmonary Hypertension
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000081029	Pulmonary Arterial Hypertension	A progressive rare pulmonary disease characterized by high blood pressure in the PULMONARY ARTERY.	Arterial Hypertension, Pulmonary,Hypertension, Pulmonary Arterial
D001206	Ascorbic Acid Deficiency	A condition due to a dietary deficiency of ascorbic acid (vitamin C), characterized by malaise, lethargy, and weakness. As the disease progresses, joints, muscles, and subcutaneous tissues may become the sites of hemorrhage. Ascorbic acid deficiency frequently develops into SCURVY in young children fed unsupplemented cow's milk exclusively during their first year. It develops also commonly in chronic alcoholism. (Cecil Textbook of Medicine, 19th ed, p1177)	Vitamin C Deficiency,Deficiency, Ascorbic Acid,Deficiency, Vitamin C,Ascorbic Acid Deficiencies,Deficiencies, Ascorbic Acid,Deficiencies, Vitamin C,Vitamin C Deficiencies
D012614	Scurvy	An acquired blood vessel disorder caused by severe deficiency of vitamin C (ASCORBIC ACID) in the diet leading to defective collagen formation in small blood vessels. Scurvy is characterized by bleeding in any tissue, weakness, ANEMIA, spongy gums, and a brawny induration of the muscles of the calves and legs.	Scorbutus,Hypoascorbemia,Hypoascorbemias,Scurvies
D065627	Familial Primary Pulmonary Hypertension	Familial or idiopathic hypertension in the PULMONARY CIRCULATION which is not secondary to other disease.	Heritable Pulmonary Arterial Hypertension,Idiopathic Pulmonary Arterial Hypertension,Idiopathic Pulmonary Hypertension,Pph1 With Hht,Primary Pulmonary Hypertension,Pulmonary Hypertension, Primary, 1,Pulmonary Hypertension, Primary, 1, With Hereditary Hemorrhagic Telangiectasia,Pulmonary Hypertension, Primary, Dexfenfluramine-Associated,Pulmonary Hypertension, Primary, Fenfluramine-Associated,Hypertension, Idiopathic Pulmonary,Hypertension, Primary Pulmonary,Pulmonary Hypertension, Idiopathic,Pulmonary Hypertension, Primary