scholarly journals Meteorological Factors Influence the Presence of Fungi in the Air; A 14-Month Surveillance Study at an Adult Cystic Fibrosis Center

2021 ◽  
Vol 11 ◽  
Author(s):  
Norman van Rhijn ◽  
James Coleman ◽  
Lisa Collier ◽  
Caroline Moore ◽  
Malcolm D. Richardson ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Elevated Temperatures ◽  
Meteorological Data ◽  
Fungal Spores ◽  
Outdoor Environment ◽  
Lung Damage ◽  
Lower Airway ◽  
Inherited Disease ◽  
Survey Period ◽  
Wind Speeds

BackgroundCystic fibrosis is an inherited disease that predisposes to progressive lung damage. Cystic fibrosis patients are particularly prone to developing pulmonary infections. Fungal species are commonly isolated in lower airway samples from patients with cystic fibrosis. Fungal spores are prevalent in the air.MethodsWe performed environmental air sampling surveillance at the Manchester Adult Cystic Fibrosis Centre, UK (MACFC) over a 14-month period to assess fungal growth inside and outside the CF center.ResultsAirborne counts of fungal spores peaked from May to October, both in outdoor and indoor samples. Collection of meteorological data allowed us to correlate fungal presence in the air with elevated temperatures and low wind speeds. Additionally, we demonstrated patient rooms containing windows had elevated fungal counts compared to rooms not directly connected to the outdoors.ConclusionsThis study suggests that airborne Aspergillus fumigatus spores were more abundant during the summer months of the survey period, which appeared to be driven by increased temperatures and lower wind speeds. Indoor counts directly correlated to outdoor A. fumigatus levels and were elevated in patient rooms that were directly connected to the outdoor environment via an openable window designed for ventilation purposes. Further studies are required to determine the clinical implications of these findings for cystic fibrosis patients who are predisposed to Aspergillus related diseases, and in particular whether there is seasonal influence on incidence of Aspergillus related conditions and if screening for such complications such be increased during summer months and precautions intensified for those with a known history of Aspergillus related disease.

scholarly journals The lower airway microbiota in early cystic fibrosis lung disease: a longitudinal analysis

Thorax ◽  
2017 ◽  
Vol 72 (12) ◽  
pp. 1104-1112 ◽  
Author(s):  
Katherine B Frayman ◽  
David S Armstrong ◽  
Rosemary Carzino ◽  
Thomas W Ferkol ◽  
Keith Grimwood ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Longitudinal Analysis ◽  
Cystic Fibrosis Lung ◽  
Lower Airway ◽  
Cystic Fibrosis Lung Disease

Cystic Fibrosis Manifested as Undescended Testis and Absence of Vas Deferens

PEDIATRICS ◽  
1992 ◽  
Vol 90 (6) ◽  
pp. 982-983
Author(s):  
RAN GOSHEN ◽  
EITAN KEREM ◽  
TZIPORA SHOSHANI ◽  
BAT-SHEVA KEREM ◽  
ELAD FEIGIN ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Autosomal Recessive ◽  
Undescended Testis ◽  
Vas Deferens ◽  
Pancreatic Insufficiency ◽  
European Ancestry ◽  
Inherited Disease ◽  
Musculoskeletal Problems ◽  
Male Patients ◽  
Almost All

Cystic fibrosis (CF) is the most common autosomal recessive inherited disease in whites. Among whites of European ancestry, approximately 1 in 2000 live births are affected, implying a carrier frequency of 1:25.1 The disease is characterized by chronic lung disease, which usually leads to the patient's death. Furthermore, patients with CF suffer from pancreatic insufficiency and other less common manifestations, such as meconium ileus, hepatobiliary abnormalities, diabetes mellitus, and musculoskeletal problems.1 Almost all males with CF are infertile. Absence of the vas deferens has been reported in 70% to more than 90% of male patients affected by CF.2 Although 4% of full-term male neonates have un-descended testes at birth, 0.8% to 1.0% of 1-year-old boys have cryptorchism and may be subjected to a later surgical intervention.3

scholarly journals Upper and lower airway microbiology in cystic fibrosis

Thorax ◽  
2010 ◽  
Vol 65 (3) ◽  
pp. 278-278
Author(s):  
T Daniels
Keyword(s):  
Cystic Fibrosis ◽  
Lower Airway

Meteorological study for Gangotri Glacier and its comparison with other high altitude meteorological stations in central Himalayan region

2007 ◽  
Vol 38 (1) ◽  
pp. 59-77 ◽  
Author(s):  
Pratap Singh ◽  
Umesh K. Haritashya ◽  
Naresh Kumar
Keyword(s):  
High Altitude ◽  
Meteorological Data ◽  
Weather Conditions ◽  
Meteorological Station ◽  
Himalayan Region ◽  
Valley Wind ◽  
Maximum Rainfall ◽  
Garhwal Himalayas ◽  
Wind Speeds ◽  
Gangotri Glacier

In spite of the vital role of high altitude climatology in melting of snow and glaciers, retreat or advancement of glaciers, flash floods, erosion and sediment transport, etc., weather conditions are not much studied for the high altitude regions of Himalayas. In this study, a comprehensive meteorological analysis has been made for the Gangotri Meteorological Station (Bhagirathi Valley, Garhwal Himalayas) using data observed for four consecutive melt seasons (2000–2003) covering a period from May to October for each year. The collected meteorological data includes rainfall, temperature, wind speed and direction, relative humidity, sunshine hours and evaporation. The results and their distribution over the different melt seasons were compared with available meteorological records for Dokriani Meteorological Station (Dingad Valley, Garhwal Himalayas) and Pyramid Meteorological Station (Khumbu Valley, Nepal Himalayas). The magnitude and distribution of temperature were found to be similar for different Himalayan regions, while rainfall varied from region to region. The influence of the monsoon was meagre on the rainfall in these areas. July was recorded to be the warmest month for all the regions and, in general, August had the maximum rainfall. For all the stations, daytime up-valley wind speeds were 3 to 4 times stronger than the nighttime down-valley wind speeds. It was found that the Gangotri Glacier area experienced relatively low humidity and high evaporation rates as compared to other parts of the Himalayas. Such analysis reveals the broad meteorological characteristics of the high altitude areas of the Central Himalayan region.

scholarly journals Deletion of cftr leads to an excessive neutrophilic response and defective tissue repair in a zebrafish model of sterile inflammation

2019 ◽  
Author(s):  
Audrey Bernut ◽  
Catherine A. Loynes ◽  
R. Andres Floto ◽  
Stephen A. Renshaw
Keyword(s):  
Cystic Fibrosis ◽  
Tissue Repair ◽  
Cell Function ◽  
Immune Cell ◽  
Genetic Disorder ◽  
Innate Immune ◽  
Lung Damage ◽  
Tanshinone Iia ◽  
Sterile Inflammation ◽  
Inflammatory Status

AbstractInflammation-related progressive lung destruction is the leading causes of premature death in cystic fibrosis (CF), a genetic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. However, therapeutic targeting of inflammation has been hampered by a lack of understanding of the links between a dysfunctional CFTR and the deleterious innate immune response in CF. Herein, we used CFTR-depleted zebrafish larvae as an innovative in vivo vertebrate model, mimicking aspects of the inflammatory pathology of CF-related lung, to understand how CFTR dysfunction leads to abnormal inflammatory status in CF.We show that impaired CFTR-mediated inflammation correlates with an exuberant neutrophilic response after injury: CF zebrafish exhibit enhanced and sustained accumulation of neutrophils at wounds. Excessive epithelial oxidative responses drive enhanced neutrophil recruitment towards wounds. Persistence of neutrophils at inflamed sites is associated with impaired reverse migration of neutrophils and reduction in neutrophil apoptosis. As a consequence, the increased number of neutrophils at wound sites causes tissue damage and abnormal tissue repair. Importantly, the pro-resolution molecule Tanshinone IIA successfully re-balances inflammation both by accelerating inflammation resolution and by improving tissue repair in CFTR-deficient animal.Larval zebrafish giving a unique insight into innate immune cell function in CFTR deficiency, our findings bring important new understanding of the mechanisms underlying the inflammatory pathology in CF, which could be addressed therapeutically to prevent inflammatory lung damage in CF patients with potential improvements in disease outcomes.

scholarly journals Can newborn screening be cost benefit procedure if preventing serious complications of cystic fibrosis?

2021 ◽  
pp. 020-023
Author(s):  
Šubat-Dežulović Mirna ◽  
Pelčić Gordana ◽  
Valković Ana ◽  
Flajšman-Raspor Sanja ◽  
Pelčić Goran ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Newborn Screening ◽  
Metabolic Alkalosis ◽  
Cost Benefit ◽  
Inherited Disease ◽  
Eu Countries ◽  
Life Threatening ◽  
Long Term Prognosis ◽  
The Cost

Cystic Fibrosis (CF) is a common inherited disease with reported mean prevalence of 0.737/10,000 in 27 EU countries (Farrell J Cyst Fibros. 2008). Still, many EU countries have not implemented CF in the Newborn Screening (NBS) programme, including our country. We report the case of a 7-month-old boy whose presenting signs of CF were life-threatening neurological symptoms caused by severe metabolic alkalosis and hypoelectrolytemia. By presenting this case, we argue hoping to persuade the authorities in any country that the available newborn screening for CF is the cost benefit procedure in preventing life treating consequences with the obvious impact on the long-term prognosis of this chronic disease.

Association of lower airway inflammation with physiologic findings in young children with cystic fibrosis

2009 ◽  
Vol 44 (5) ◽  
pp. 503-511 ◽  
Author(s):  
Stacey L. Peterson-Carmichael ◽  
William T. Harris ◽  
Ruchika Goel ◽  
Terry L. Noah ◽  
Robin Johnson ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Young Children ◽  
Airway Inflammation ◽  
Lower Airway

Cystic Fibrosis and Non-Cystic Fibrosis Bronchiectasis

2014 ◽  
Author(s):  
Michael J Stephen
Keyword(s):  
Cystic Fibrosis ◽  
Differential Diagnosis ◽  
Forced Expiratory Volume ◽  
Sweat Test ◽  
Inherited Disease ◽  
Injury Rates ◽  
Computed Tomographic ◽  
Sweat Chloride ◽  
Average Survival ◽  
Pancreatic Exocrine Deficiency

Cystic fibrosis (CF) is an autosomal recessive disease characterized by an elevated sweat chloride level, diffuse bronchiectasis, and pancreatic exocrine deficiency. It is the most common lethal inherited disease in whites. Most patients present at birth or early childhood, although later diagnoses are not infrequent. Once CF was uniformly fatal at an early age, but advances in nutrition, airway clearance, and infection management have led to an average survival of 37 years. The newest aspect of care is the advent of protein modulators, which may increase life expectancy even further. This chapter discusses the epidemiology, genetics, pathophysiology and pathogenesis, diagnosis, differential diagnosis, and treatment of CF. The definition, epidemiology, etiology, pathogenesis, diagnosis, management, and prognosis of non-CF bronchiectasis are also covered. Figures illustrate normal and abnormal CF transmembrane conductance regulators, the vicious cycle hypothesis of lung injury, rates of respiratory germs by age, the diagnosis of CF, the therapeutics pipeline for CF, forced expiratory volume in 1 second lung function percent predicted versus body mass index, and the median predicted survival age of patients with CF. A chest x-ray and chest computed tomographic scan of CF are also provided. Tables outline the most common CF mutations in 2011, class mutations of CF, a mnemonic for acute exacerbations of CF, the diagnosis of CF-related diabetes in a stable patient, sweat test values, and the differential diagnosis of bronchiectasis.This chapter contains 9 highly rendered figures, 6 tables, 143 references, 1 teaching slide set, and 5 MCQs.

Cystic Fibrosis and Non-Cystic Fibrosis Bronchiectasis

2014 ◽  
Author(s):  
Michael J Stephen
Keyword(s):  
Cystic Fibrosis ◽  
Differential Diagnosis ◽  
Forced Expiratory Volume ◽  
Sweat Test ◽  
Inherited Disease ◽  
Injury Rates ◽  
Computed Tomographic ◽  
Sweat Chloride ◽  
Average Survival ◽  
Pancreatic Exocrine Deficiency

Cystic fibrosis (CF) is an autosomal recessive disease characterized by an elevated sweat chloride level, diffuse bronchiectasis, and pancreatic exocrine deficiency. It is the most common lethal inherited disease in whites. Most patients present at birth or early childhood, although later diagnoses are not infrequent. Once CF was uniformly fatal at an early age, but advances in nutrition, airway clearance, and infection management have led to an average survival of 37 years. The newest aspect of care is the advent of protein modulators, which may increase life expectancy even further. This chapter discusses the epidemiology, genetics, pathophysiology and pathogenesis, diagnosis, differential diagnosis, and treatment of CF. The definition, epidemiology, etiology, pathogenesis, diagnosis, management, and prognosis of non-CF bronchiectasis are also covered. Figures illustrate normal and abnormal CF transmembrane conductance regulators, the vicious cycle hypothesis of lung injury, rates of respiratory germs by age, the diagnosis of CF, the therapeutics pipeline for CF, forced expiratory volume in 1 second lung function percent predicted versus body mass index, and the median predicted survival age of patients with CF. A chest x-ray and chest computed tomographic scan of CF are also provided. Tables outline the most common CF mutations in 2011, class mutations of CF, a mnemonic for acute exacerbations of CF, the diagnosis of CF-related diabetes in a stable patient, sweat test values, and the differential diagnosis of bronchiectasis.This chapter contains 9 highly rendered figures, 6 tables, 143 references, 1 teaching slide set, and 5 MCQs.

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