Suzanne Crowley
A5056354729- Cystic Fibrosis Research Advances
- Tracheal and airway disorders
- Neonatal Respiratory Health Research
- Asthma and respiratory diseases
- Pediatric health and respiratory diseases
- Iron Metabolism and Disorders
- Respiratory and Cough-Related Research
- Sinusitis and nasal conditions
- Hemoglobinopathies and Related Disorders
- Adrenal Hormones and Disorders
- Respiratory viral infections research
- Virology and Viral Diseases
- Nasal Surgery and Airway Studies
- Growth Hormone and Insulin-like Growth Factors
- Ear Surgery and Otitis Media
- Dysphagia Assessment and Management
- Genetic and Kidney Cyst Diseases
- Stress Responses and Cortisol
- Microtubule and mitosis dynamics
- Inhalation and Respiratory Drug Delivery
- Pharmaceutical studies and practices
- Bone health and osteoporosis research
- Congenital Diaphragmatic Hernia Studies
- Hormonal and reproductive studies
- Hormonal Regulation and Hypertension
Oslo University Hospital
2014-2024
Cancer Registry of Norway
2020
University of Bergen
2017
Innlandet Hospital Trust
2017
Smerteklinikken
2010-2016
Med-Storm Innovation (Norway)
2010
St George's Hospital
2005
St George's Hospital
2003-2004
Harefield Hospital
2002
Royal Brompton Hospital
1999
Data on primary ciliary dyskinesia (PCD) epidemiology is scarce and published studies are characterised by low numbers. In the framework of European Union project BESTCILIA we aimed to combine all available datasets in a retrospective international PCD cohort (iPCD Cohort). We identified eligible performing systematic review containing clinical information PCD, contacting members past current Respiratory Society Task Forces PCD. compared contents datasets, clarified definitions pooled them...
Tubulin, one of the most abundant cytoskeletal building blocks, has numerous isotypes in metazoans encoded by different conserved genes. Whether these distinct form cell type– and context-specific microtubule structures is poorly understood. Based on a cohort 12 patients with primary ciliary dyskinesia as well mouse mutants, we identified characterized variants TUBB4B isotype that specifically perturbed centriole cilium biogenesis. Distinct differentially affected dynamics cilia formation...
ABSTRACT We have investigated the adrenal response, in eight healthy adult men, to low doses of ACTH(1–24) order define a dose which will elicit response similar that obtained with short Synacthen test. The studies were performed at 14.00 h and blood samples withdrawn 5-min intervals after an i.v. bolus injection ACTH(1–24). sampling interval was crucial determining timing peak response. Using times 0, 10, 15, 20, 25 30 min ensured detection 47 out 48 peaks. A dose-dependent rise plasma...
Primary ciliary dyskinesia (PCD) has been considered a relatively mild disease, especially compared to cystic fibrosis (CF), but studies on lung function in PCD patients have few and small.This study from spirometry of normal reference values published data CF patients. We calculated z-scores % predicted for forced expiratory volume 1 s (FEV1) vital capacity (FVC) using the Global Lung Function Initiative 2012 991 international Cohort. then assessed associations with age, sex, country,...
ABSTRACT We compared the reproducibility and repeatability of acute adrenal response to low doses (90 500 ng/1·73 m 2 ) Synacthen (ACTH(1–24)) with that standard dose (250 μg/1·73 ). also examined effect basal cortisol levels on peak values achieved after stimulation a dose. ACTH(1–24) was given six male volunteers: 90 twice at 90-min intervals day 1, once 250 3. The rise in serum concentration repeated ACTH not attenuated (161 ± 49 ( s.d. nmol/l initial vs 150 41 repeat stimulation; P =...
Pulmonary exacerbations are a cause of significant morbidity in patients with primary ciliary dyskinesia (PCD) and frequently used as an outcome measure clinical research into chronic lung diseases. So far, there has been no consensus on the definition pulmonary PCD. 30 multidisciplinary experts developed for children adults Following systematic review, panel modified Delphi process combination face-to-face meetings e-surveys to develop that can be settings A exacerbation was defined by...
Chronic respiratory disease can affect growth and nutrition, which influence lung function. We investigated height, body mass index (BMI), function in patients with primary ciliary dyskinesia (PCD). In this study, based on the international PCD (iPCD) Cohort, we calculated z-scores for height BMI using World Health Organization (WHO) national references, assessed associations age, sex, country, diagnostic certainty, age at diagnosis, organ laterality multilevel regression models that...
Primary ciliary dyskinesia (PCD) presents with symptoms early in life and the disease course may be progressive, but longitudinal data on lung function are scarce. This multinational cohort study describes trajectories children, adolescents young adults PCD. We analysed from 486 patients repeated measurements obtained between age of 6 24 years International PCD Cohort calculated z-scores for forced expiratory volume 1 s (FEV1), vital capacity (FVC) FEV1/FVC ratio using Global Lung Function...
To investigate the natural history and outcome of antenatally diagnosed congenital cystic adenomatoid malformation (CCAM) lung.This was a retrospective study all cases fetal CCAM lung antenatally. All were referred to tertiary center for further management. A computer search identified cases, records these patients examined determine pregnancy outcome.In 4-year period, 34 At presentation, noted be unilateral CCAMs majority (79%) microcystic in nature. The complicated by varying degrees...
<h3>Background</h3> Asthma and airway hyper-responsiveness are reportedly more common in children with sickle cell disease (SCD). <h3>Aim</h3> To determine responsiveness, inflammation clinical features of asthma SCD. <h3>Methods</h3> A prospective, single-centre study 50 SCD without overt pulmonary vascular controls. Exhaled nitric oxide (FeNO) total serum IgE were measured spirometry methacholine challenge performed. The dose–response slope (DRS) was calculated. <h3>Results</h3> Doctor...
In primary ciliary dyskinesia (PCD) impaired mucociliary clearance leads to recurrent airway infections and progressive lung destruction, concern over chronic infection patient-to-patient transmission is considerable. So far, there has been no defined consensus on how control across centres caring for patients with PCD. Within the BEAT-PCD network, COST Action ERS CRC together ERN-Lung PCD core a first initiative now taken towards creating such statement.A multidisciplinary international...
Background Primary ciliary dyskinesia (PCD) is a rare genetic disorder characterized by dysfunction of motile cilia. Symptoms include recurrent and chronic airway infections which can lead to deteriorating lung function inflammatory destructive disease in the form persistent atelectasis bronchiectasis. Routine blood testing may be used as tool for monitoring management. However, currently there are no consensus-based guidelines within field PCD. BEAT-PCD together with ERN-LUNG PCD-Clinical...
<h3>Background</h3> Adults with sickle cell disease (SCD) and pulmonary hypertension have high mortality but death in SCD children is rare. The authors hypothesised that may be secondary to anaemia-induced cardiac output rather than vascular disease. <h3>Methods</h3> Two independent, validated techniques were used estimate resistance (PVR) 50 matched controls. Tricuspid regurgitant jet velocity (TRV) right ventricular outflow tract time integral measured using Doppler echocardiography; PVR...
Background Sinonasal symptoms are a common feature of primary ciliary dyskinesia (PCD); however, literature about their severity and frequency, particularly during the life course, is scarce. Using baseline data from Ear, nose throat (ENT) Prospective International Cohort PCD patients, we describe sinonasal disease in PCD. Methods We included participants who had routine examination which they completed questionnaire. compared frequency reported findings among children adults, identified...
Otologic disease is common among people with primary ciliary dyskinesia (PCD), yet little known about its spectrum and severity.
Introduction Nearly all patients with primary ciliary dyskinesia (PCD) report ear–nose–throat (ENT) symptoms. However, scarce evidence exists about how ENT symptoms relate to pulmonary disease in PCD. We explored possible associations between upper and lower respiratory among PCD a multicentre study. Methods included from the Prospective International Cohort (EPIC-PCD). studied of several reported chronic rhinosinusitis (defined using patient-reported information examination findings) sputum...
Primary ciliary dyskinesia (PCD) is a rare disease causing motile cilia dysfunction, recurrent airway infection, and bronchiectasis. Airway infection management strategies are borrowed from cystic fibrosis. The aim of this study to describe the with Pseudomonas aeruginosa ( PA) in children adults PCD across European centers. An online survey questionnaire was sent electronically using SurveyMonkey® 55 centers 36 countries. Fifty-two responded 43 26 countries, response rate 70%. Most (89%)...
Abstract Cilia are small microtubule-based structures found on the surface of most mammalian cells, which have key sensory and sometimes motile functions. Primary ciliary dyskinesia (PCD) is a type ciliopathy caused by defects in cilia. The genetic basis PCD only partially understood. Studying cohort 11 human patients with PCD, we find that de novo mutations TUBB4B , beta tubulin isotype, cause three distinct classes ciliopathic disease. In vivo studies mice show Tubb4b plays specific role...