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Circulation: Cardiovascular Interventions, ISSN 1941-7640, 12/2012, Volume 5, Issue 6, pp. 756 - 762
Journal Article
Journal Article
Clinics in Chest Medicine, ISSN 0272-5231, 09/2016, Volume 37, Issue 3, pp. 505 - 511
..., and highly penetrant pulmonary fibrosis in some subtypes, including HPS-1, HPS-2, and HPS-4. HPS pulmonary fibrosis shows many of the clinical, radiologic, and histologic features found in idiopathic pulmonary fibrosis, but occurs at a younger age... 
Biogenesis of lysosome-related organelle complex | Pulmonary fibrosis | Adaptor protein 3 | Albinism | Hermansky-Pudlak syndrome | Interstitial lung disease | MUTATION SCREEN REVEALS | APOPTOSIS | FORM | SUSCEPTIBILITY | COLITIS | RESPIRATORY SYSTEM | INFLAMMATION | MOUSE MODEL | LUNG-DISEASE | PULMONARY-FIBROSIS | IMMUNODEFICIENCY | Epistaxis - etiology | Hemorrhagic Disorders - physiopathology | Pulmonary Veins - abnormalities | Gastrointestinal Diseases - physiopathology | Humans | Gastrointestinal Diseases - etiology | Hypertension, Pulmonary - physiopathology | Telangiectasis - physiopathology | Epistaxis - physiopathology | Arteriovenous Malformations - physiopathology | Intracranial Arteriovenous Malformations - physiopathology | Albinism, Oculocutaneous - physiopathology | Hemorrhagic Disorders - complications | Liver Diseases - etiology | Intracranial Arteriovenous Malformations - etiology | Crohn Disease - etiology | Pulmonary Fibrosis - etiology | Albinism - physiopathology | Blood Coagulation Disorders - etiology | Arteriovenous Malformations - etiology | Pulmonary Artery - abnormalities | Albinism - complications | Albinism, Oculocutaneous - etiology | Telangiectasis - etiology | Hermanski-Pudlak Syndrome - complications | Pulmonary Fibrosis - physiopathology | Hermanski-Pudlak Syndrome - physiopathology | Crohn Disease - physiopathology | Liver Diseases - physiopathology | Hypertension, Pulmonary - etiology | Blood Coagulation Disorders - physiopathology
Journal Article
Circulation Research, ISSN 0009-7330, 05/2015, Volume 116, Issue 10, pp. 1680 - 1690
RATIONALE:Iron deficiency without anemia is prevalent in patients with idiopathic pulmonary arterial hypertension and associated with reduced exercise capacity and survival... 
hypertension, pulmonary | iron | vascular remodeling | glycolysis | STAT3 | APOPTOSIS | CARDIAC & CARDIOVASCULAR SYSTEMS | HEART-FAILURE | INVOLVEMENT | HEPCIDIN | PATHOLOGY | DICHLOROACETATE | HYPOXIA | SUPPLEMENTATION | METABOLISM | ARTERIAL-HYPERTENSION | PERIPHERAL VASCULAR DISEASE | HEMATOLOGY | Erythropoiesis | Antihypertensive Agents - pharmacology | Cell Proliferation | Homeostasis | Hypertension, Pulmonary - physiopathology | Male | Hypertrophy, Right Ventricular - etiology | Hypertension, Pulmonary - blood | Hypertension, Pulmonary - prevention & control | Hypertrophy, Right Ventricular - physiopathology | Pulmonary Artery - metabolism | Ferritins - blood | Time Factors | Deficiency Diseases - drug therapy | Arterial Pressure - drug effects | Hypertrophy, Right Ventricular - blood | Iron - deficiency | Benzamides - pharmacology | Vascular Remodeling - drug effects | Deficiency Diseases - blood | Disease Models, Animal | Iron - blood | Hematinics - pharmacology | Signal Transduction | Liver - metabolism | Ferric Compounds - pharmacology | Pulmonary Artery - physiopathology | Pulmonary Artery - drug effects | Pyrimidines - pharmacology | Imatinib Mesylate | Piperazines - pharmacology | Rats, Sprague-Dawley | Deficiency Diseases - complications | Animals | Maltose - analogs & derivatives | Maltose - pharmacology | Dichloroacetic Acid - pharmacology | Glycolysis | Transferrin - metabolism | Hypertension, Pulmonary - etiology | Hypertension, Pulmonary - pathology | Pulmonary Artery - pathology
Journal Article
JACC: Cardiovascular Interventions, ISSN 1936-8798, 2013, Volume 6, Issue 7, pp. 725 - 736
Journal Article
American Journal of Respiratory and Critical Care Medicine, ISSN 1073-449X, 02/2011, Volume 183, Issue 3, pp. 388 - 395
Journal Article
Journal of the American Heart Association, ISSN 2047-9980, 02/2013, Volume 2, Issue 1, pp. e005157 - n/a
Background Pulmonary arterial hypertension (PAH) is a vasculopathy characterized by enhanced pulmonary artery smooth muscle cell (PASMC... 
remodeling | RAGE | pulmonary hypertension | Sugen | PPARγ | GAMMA | ACTIVATION | CARDIAC & CARDIOVASCULAR SYSTEMS | PROTEIN-RECEPTOR | ATHEROSCLEROSIS | PPAR gamma | S100 PROTEINS | PATHWAY | DISEASE | EXPRESSION | AXIS | Up-Regulation | Cell Proliferation | Humans | Middle Aged | Monocrotaline | Hypertension, Pulmonary - physiopathology | Male | Hypertrophy, Right Ventricular - etiology | PPAR gamma - metabolism | Pulmonary Artery - metabolism | RNA Interference | Receptors, Immunologic - agonists | Hypertension, Pulmonary - drug therapy | Indoles | S100 Proteins - pharmacology | Myocytes, Smooth Muscle - drug effects | Hypertrophy, Right Ventricular - metabolism | Receptor for Advanced Glycation End Products | Disease Models, Animal | Muscle, Smooth, Vascular - drug effects | Signal Transduction | Rats | Familial Primary Pulmonary Hypertension | Hypoxia - complications | Rats, Sprague-Dawley | Bone Morphogenetic Protein Receptors, Type II - metabolism | Hypertrophy, Right Ventricular - prevention & control | Receptors, Immunologic - genetics | Hypertension, Pulmonary - etiology | Muscle, Smooth, Vascular - metabolism | Myocytes, Smooth Muscle - pathology | Pyrroles | Hypertension, Pulmonary - chemically induced | Muscle, Smooth, Vascular - physiopathology | Case-Control Studies | Arterial Pressure | Transfection | Adult | Female | Myocytes, Smooth Muscle - metabolism | STAT3 Transcription Factor - metabolism | Cells, Cultured | Hypertension, Pulmonary - genetics | Hypertension, Pulmonary - metabolism | Muscle, Smooth, Vascular - pathology | Animals | Glycation End Products, Advanced - metabolism | Aged | Hypertension, Pulmonary - pathology | Receptors, Immunologic - metabolism | Apoptosis
Journal Article
Chest, ISSN 0012-3692, 2016, Volume 149, Issue 5, pp. 1313 - 1324
.... Chronic pulmonary complications in SCD... 
Pulmonary/Respiratory | airway hyperresponsiveness | sleep-disordered breathing | pulmonary embolism | pulmonary hypertension | sickle cell disease | Pulmonary embolism | Sickle cell disease | Pulmonary hypertension | Sleep-disordered breathing | Airway hyperresponsiveness | YOUNG-ADULTS | OBSTRUCTIVE SLEEP-APNEA | RISK-FACTORS | AMERICAN-COLLEGE | LUNG-FUNCTION | CHILDREN | FUNCTION ABNORMALITIES | ACUTE CHEST SYNDROME | RESPIRATORY SYSTEM | ARTERIAL-HYPERTENSION | ASTHMA | CRITICAL CARE MEDICINE | Sleep Apnea Syndromes - physiopathology | Anemia, Sickle Cell - physiopathology | Asthma - physiopathology | Sleep Apnea Syndromes - etiology | Anemia, Sickle Cell - complications | Humans | Hypertension, Pulmonary - physiopathology | Lung - physiopathology | Respiratory Sounds - etiology | Respiratory Sounds - physiopathology | Pulmonary Embolism - etiology | Asthma - etiology | Respiratory Function Tests | Hypertension, Pulmonary - etiology | Pulmonary Embolism - physiopathology | Complications and side effects | Lung diseases, Obstructive | Sickle cell anemia | Research | Risk factors | RV, right ventricular | PE, pulmonary embolism | PVR, pulmonary vascular resistance | ACS, acute chest syndrome | HbS, hemoglobin S | Hb, hemoglobin | SCD, sickle cell disease | AHI, apnea-hypopnea index | PH, pulmonary hypertension | AHR, airway hyperresponsiveness | PAH, pulmonary arterial hypertension | RCT, randomized controlled trial | SDB, sleep-disordered breathing | NT-pro-BNP, N-terminal pro-B-type natriuretic peptide | Dlco, diffusion capacity of the lung for carbon monoxide | HbC, hemoglobin C | Recent Advances in Chest Medicine | TRV, tricuspid regurgitant jet velocity | PFT, pulmonary function testing | VOE, vaso-occlusive event | SCD-PH, sickle cell disease-associated pulmonary hypertension
Journal Article