A5066198655- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Medical Imaging and Pathology Studies
- Sarcoidosis and Beryllium Toxicity Research
- Pulmonary Hypertension Research and Treatments
- Tracheal and airway disorders
- Vascular Anomalies and Treatments
- Chronic Obstructive Pulmonary Disease (COPD) Research
- Systemic Sclerosis and Related Diseases
- Sharing Economy and Platforms
- Vascular Malformations and Hemangiomas
- Cardiovascular Issues in Pregnancy
- Pleural and Pulmonary Diseases
- Transplantation: Methods and Outcomes
- Inflammatory Myopathies and Dermatomyositis
- Head and Neck Surgical Oncology
- Respiratory Support and Mechanisms
- Myasthenia Gravis and Thymoma
- Autoimmune and Inflammatory Disorders
- Trauma Management and Diagnosis
- Heart Failure Treatment and Management
- Research on Leishmaniasis Studies
- Pneumonia and Respiratory Infections
- Lymphoma Diagnosis and Treatment
- Otitis Media and Relapsing Polychondritis
- Cancer Diagnosis and Treatment
Mayo Clinic in Arizona
2005-2024
Cleveland Clinic
2014-2024
RTI International
2024
Johns Hopkins University
2024
Massachusetts General Hospital
2024
Cleveland Clinic Lerner College of Medicine
2010
Case Western Reserve University
2010
Mayo Clinic
2004-2009
Pulmonary and Allergy Associates
2008
Pulmonary and Critical Care Associates
2006
Idiopathic pulmonary fibrosis is a progressive, fatal disease. This prospective, randomised, double-blind, multicentre, parallel-group, placebo-controlled phase II trial (NCT00903331) investigated the efficacy and safety of endothelin receptor antagonist macitentan in idiopathic fibrosis. Eligible subjects were adults with <3 years duration histological pattern usual interstitial pneumonia on surgical lung biopsy. The primary objective was to demonstrate that (10 mg once daily) positively...
Background Pulmonary hypertension (PH) accompanying COPD (PH-COPD) is associated with worse outcomes than alone. There are currently no approved therapies to treat PH-COPD. The PERFECT study ( ClinicalTrials.gov : NCT03496623 ) evaluated the safety and efficacy of inhaled treprostinil (iTRE) in this patient population. Methods Patients PH-COPD (mean pulmonary arterial pressure ≥30 mmHg vascular resistance ≥4 WU) were enrolled a multicentre, randomised (1:1), double-blind, placebo-controlled,...
Leflunomide has been reported as an alternative therapy in sarcoidosis. However, the published data are limited. We performed a retrospective chart review of tolerance and effects leflunomide patients with 76 were included. The most common reasons for initiation progression disease or failure other immunomodulator therapy. Side-effects attributable to noted 34% subjects, prompting discontinuation 17%. lungs target 33 (44%) extrapulmonary organs 45 (59%). mean ± sd change forced vital...
Small fiber neuropathy commonly affects patients with sarcoidosis and is often refractory to standard immunosuppressive therapies used for systemic disease. The clinical features of sarcoidosis-associated small (SSFN) its response medical therapy have not been described in a large population.We performed retrospective review SSFN seen at the Cleveland Clinic over 4-year period.SSFN was identified 143 individuals although other causes were found 28 cases. Of remaining 115 patients, 100 (87%)...
Hereditary hemorrhagic telangiectasia (HHT, Osler-Weber-Rendu disease) is a rare multisystem vascular disorder causing chronic gastrointestinal bleeding, epistaxis, and severe anemia. Bevacizumab, an anti-vascular endothelial growth factor antibody, may be effective to treat bleeding in HHT. This international, multicenter, retrospective study evaluated the use of systemic bevacizumab HHT-associated anemia at 12 HHT treatment centers. Hemoglobin, epistaxis severity score, red cell units...
Abstract Pazopanib (Votrient) is an orally administered tyrosine kinase inhibitor that blocks VEGF receptors potentially serving as anti-angiogenic treatment for hereditary hemorrhagic telangiectasia (HHT). We report a prospective, multi-center, open-label, dose-escalating study [50 mg, 100 200 and 400 mg], designed proof-of-concept to demonstrate efficacy of pazopanib on HHT-related bleeding, measure safety. Patients, recruited at 5 HHT Centers, required ≥ 2 Curacao criteria AND [anemia OR...
Hereditary hemorrhagic telangiectasia (HHT) is characterized by extensive telangiectasias and arteriovenous malformations. The primary clinical manifestation epistaxis that results in iron-deficiency anemia reduced health-related quality of life.
Juvenile polyposis syndrome is a dominant GI defined by ≥ 5 juvenile polyps or 1 with family history of polyposis. Mutations in BMPR1A SMAD4 are found 50% individuals. Hereditary hemorrhagic telangiectasia disorder characterized epistaxis, visceral arteriovenous malformations, and telangiectasias. diagnosed when 3 criteria including clinical manifestations history, present. A polyposis-hereditary overlap has previously been reported 22% patients due to mutation.Our objective was determine...
Quantification of haemosiderin-laden macrophages in bronchoalveolar lavage fluid (BALF) has been used to diagnose diffuse alveolar haemorrhage (DAH) but not assessed patients with damage (DAD). The present study analysed BALF obtained from 21 DAD diagnosed by surgical lung biopsy. median age was 68 yrs (range 18-79 yrs); 14 (67%) were male and 12 (57%) immunocompromised. proportion 5% 0-90%), >or=20% seven (33%) patients, fulfilling the commonly criterion for DAH. There a trend toward...
Normal resting mean pulmonary artery pressure (PAP) is 8–20 mmHg. Pulmonary hypertension defined as PAP of ≥25 Borderline levels 21–24 mmHg are unclear significance. We sought to determine the clinical characteristics and survival subjects with examined 1,491 patients enrolled in Cleveland Clinic Hypertension Registry between February 1990 May 2012 baseline right heart catheterization. The relationship all‐cause mortality was assessed by Cox models a tree‐based analysis. Sixty‐three had...
Rationale: Lymphangioleiomyomatosis (LAM) is a progressive cystic lung disease that predominantly affects women and can worsen with pregnancy, estrogen treatment, the menstrual cycle, suggesting an important role for in pathogenesis.Objectives: To assess efficacy safety of aromatase inhibitor letrozole treatment LAM.Methods: Seventeen postmenopausal LAM were enrolled this phase II trial randomized to receive 2.5 mg daily (n = 9) or placebo 8) period 12 months. Five patients each group also...