Results 141 to 150 of about 1,353,059 (301)

Role of the soluble epoxide hydrolase and cytochrome P450-derived epoxyeicosatrienoic acids in hypoxic pulmonary vasoconstriction and hypoxia-induced pulmonary vascular remodelling [PDF]

, 2009
Hypoxic pulmonary vasoconstriction (HPV) redistributes pulmonary blood flow from areas of low oxygen partial pressure to areas of normal or relativity high oxygen availability, thus optimising the matching of perfusion to ventilation and preventing ...
Keserü, Benjamin
core  

Descriptive Epidemiology From the Myhre Syndrome Foundation Registry: The Value of Self‐Reported Data

American Journal of Medical Genetics Part C: Seminars in Medical Genetics, EarlyView.
ABSTRACT Myhre syndrome is an ultrarare genetic disease characterized by short stature, distinct craniofacial features, cardiovascular and respiratory fibrosis and stenosis, neurodevelopmental delays, autism, intellectual disability, and hearing loss. The natural history of Myhre syndrome is still not fully understood due to a small patient population ...
Mary K. Young, Armelle Pindon, Maggie R. Brand, Kate Wears, Katherine H. Young, Alyssa Mendel, Michael J. Lyons   +6 more
wiley   +1 more source

Dasatinib‐Induced Pulmonary Arterial Hypertension in Chronic Myeloid Leukaemia: A Case Report and Literature Review

Respirology Case Reports
Dasatinib, a second‐generation tyrosine kinase inhibitor used for treating chronic myeloid leukaemia (CML), is associated with rare but significant adverse effects, including pulmonary arterial hypertension.
Sathish Krishnan, Sashi Adigopula
doaj   +1 more source

Therapy for Myhre Syndrome: Goals, Misconceptions, and Current Agents

American Journal of Medical Genetics Part C: Seminars in Medical Genetics, EarlyView.
ABSTRACT Myhre Syndrome (MYHRS, MIM #139210) is a rare, multisystem connective tissue disorder caused by recurrent heterozygous gain‐of‐function pathogenic variants in the SMAD4 gene, a key player in TGF‐β signaling and a regulator of extracellular matrix homeostasis.
Alessandro De Falco, Alfonso Manuel D'Alessio, Nicola Brunetti‐Pierri   +2 more
wiley   +1 more source

Update on the PHA Pulmonary Hypertension Care Center Network: Early Experience With the National Accreditation Program

, 2018
World Health Organization (WHO) Group 1 pulmonary hypertension (PH) (PAH, pulmonary arterial hypertension) and WHO Group 4 PH (CTEPH, chronic thromboembolic pulmonary hypertension) are rare diseases of the pulmonary vascular system characterized by ...
Michael Patrick Gray, Olivia Onyeador, Joel A. Wirth   +2 more
core   +1 more source

Review of the Molecular and Developmental Basis of Myhre Syndrome, Bench Research

American Journal of Medical Genetics Part C: Seminars in Medical Genetics, EarlyView.
ABSTRACT Myhre syndrome (MS) is a connective‐tissue disorder within the acromelic dysplasia spectrum. It is characterized by congenital craniofacial, skeletal, cutaneous anomalies, respiratory, cardiovascular along with intellectual disability, deafness, and progressive fibrosis.
Camille Viaut, Valerie Cormier‐Daire
wiley   +1 more source

Posthumously Diagnosed Myhre Syndrome Presenting With Pleural Remodeling and Endometrial Cancer

American Journal of Medical Genetics Part C: Seminars in Medical Genetics, EarlyView.
ABSTRACT Myhre syndrome (OMIM 139210) is a genetic condition defined by neurodevelopmental disability, characteristic facial features, and multisystem proliferative fibrosis. While various types of lung disease have been reported, pleural remodeling leading to restrictive lung disease has not yet been described.
Jeanette Saffir, Karee Morgan, Karigynn Chaimson, Blake E. Funke, Kim L. Sandler, Melissa Hogan, Thomas Cassini   +6 more
wiley   +1 more source

Tezepelumab in Chinese Patients With Severe Chronic Rhinosinusitis With Nasal Polyps: A Prespecified Subgroup Analysis of the Phase 3 WAYPOINT Trial

International Forum of Allergy &Rhinology, EarlyView.
ABSTRACT Background Chronic rhinosinusitis with nasal polyps (CRSwNP) in Chinese patients often exhibits a mixed Type 1/2/3 inflammatory phenotype (63%), potentially impacting the efficacy of biologics targeting Type 2 inflammation. This prespecified subgroup analysis of WAYPOINT (NCT04851964) evaluated the efficacy and safety of tezepelumab in Chinese
Li Hu, Houyong Li, Xicai Sun, Quan Liu, Yu Xu, Jiangang Fan, Andrew Foster, Claudia Chen, Yun Chon, Dehui Wang   +9 more
wiley   +1 more source

Protein biomarkers in pulmonary arterial hypertension: advances, clinical relevance, and translational challenges

Journal of Translational Medicine
Pulmonary arterial hypertension (PAH) is a progressive and life-threatening disease characterized by pulmonary vasoconstriction and right ventricular dysfunction.
Yanqin Niu, Jinglin Tian, Steeve Provencher, Sebastien Bonnet, Olivier Boucherat, François Potus, Deming Gou   +6 more
doaj   +1 more source

A Comparison of Frailty Indices in Predicting Postoperative Outcomes After Endoscopic Sinus Surgery

International Forum of Allergy &Rhinology, EarlyView.
ABSTRACT Objective To compare the discriminatory performance of the Risk Analysis Index (RAI) and modified Frailty Index‐5 (mFI‐5) in predicting postoperative outcomes following endoscopic sinus surgery (ESS). Study Design Retrospective cohort study. Methods Adult ESS patients were identified from the ACS‐NSQIP database (2005–2020).
Akshay Warrier, David Remyes, Tenesha Boyd, Ailish Nagpal, Aman M Patel, David W. Wassef, Jean Anderson Eloy   +6 more
wiley   +1 more source