Cystic fibrosis breakthrough reveals why females fare worse than males

June 8, 2012

(Medical Xpress) -- Cystic Fibrosis (CF) is the most common life-threatening inherited disease in Ireland with the highest incidence of this disease globally seen on this island. Females with CF have a poorer outcome as a result of serious bacterial infections in their respiratory tract. Collaborative research conducted in Dublin between the Royal College of Surgeons, Beaumont Hospital and the School of Medicine, Trinity College has furthered our understanding of this phenomenon and was recently published in the prestigious New England Journal of Medicine

“This is a fundamental breakthrough in understanding why females with cystic fibrosis have a poorer prognosis than males,” says Trinity’s Dr Stephen Smith, of the Department of Clinical Microbiology in the School of Medicine.  It shows for the first time that the female hormone oestrogen promotes the presence of a particular mucoid form of the bacterium Pseudomonas aeruginosa in the lungs of women with CF. When in the mucoid form, bacteria are surrounded by a layer of gelatinous material and this is poorly penetrated by antibiotics. In the clinic, the mucoid type of Pseudomonas aeruginosa is more difficult to treat and causes significant lung inflammation, explaining, in part, why with CF, have a worse outcome.  This study opens the way to new understanding and potential therapeutic approaches in CF.

Professor Gerry McElvaney, Director of the Respiratory Research Laboratory RCSI/Beaumont Hospital and a senior author on the paper stated: “This research study is among the first examples which shows the effects of gender hormones on infections and therefore has major implications for conditions beyond cystic fibrosis including other respiratory diseases such as asthma.”

Dr Sanjay Chotirmall, a Molecular Medicine Ireland (MMI) Clinician-Scientist fellow is the first author on the paper and Dr. Catherine Greene is the joint senior author.

The clinical research was carried out in the Cystic Fibrosis Unit, Beaumont Hospital and also utilised data from the National Registry of .

This paper is an excellent example of the translational research presently underway in RCSI and illustrates interdepartmental and inter-institutional links as co-authors on the paper include colleagues from the Departments of Medicine (Dr Sonya Cosgrove), General Practice (Dr Borislav Dimitrov) and Molecular Medicine (Professor Brian Harvey), RCSI; Beaumont Hospital/RCSI (Dr Cedric Gunaratnam, Professor Shane O’Neill); and the School of Medicine (Dr Stephen Smith), Trinity College, Dublin. 

The study’s results are being presented by Dr Greene at the American Thoracic Society meeting in San Francisco this month and by Dr Chotirmall at the European Respiratory Society meeting in Vienna later this year.

Explore further: Early cystic fibrosis lung disease detected by bronchoalveolar lavage and lung clearance index

More information: “Effect of Estrogen on Pseudomonas Mucoidy and Exacerbations in Cystic Fibrosis” New England Journal of Medicine

Related Stories

Early cystic fibrosis lung disease detected by bronchoalveolar lavage and lung clearance index

January 27, 2012
The lung clearance index (LCI) is a sensitive non-invasive marker of early lung disease in young children with cystic fibrosis (CF), according to a new study from Australian researchers.

Scientists develop first ever drug to treat 'Celtic gene' in cystic fibrosis sufferers

June 20, 2011
An international research team led by Queen's University have developed a ground breaking treatment for Cystic Fibrosis sufferers. The new drug will benefit sufferers who have the 'Celtic Gene', a genetic mutation which ...

Unraveling a new regulator of cystic fibrosis

September 19, 2011
Cystic fibrosis (CF), a chronic disease that clogs the lungs and leads to life-threatening lung infections, is caused by a genetic defect in a chloride channel called cystic fibrosis transmembrane conductase regulator (CFTR). ...

Recommended for you

Decrease in sunshine, increase in Rickets

November 17, 2017
A University of Toronto student and professor have teamed up to discover that Britain's increasing cloudiness during the summer could be an important reason for the mysterious increase in Rickets among British children over ...

Anti-malaria drug shows promise as Zika virus treatment

November 17, 2017
A new collaborative study led by researchers at Sanford Burnham Prebys Medical Discovery Institute (SBP) and UC San Diego School of Medicine has found that a medication used to prevent and treat malaria may also be effective ...

Scientists identify biomarkers that indicate likelihood of survival in infected patients

November 17, 2017
Scientists have identified a set of biomarkers that indicate which patients infected with the Ebola virus are most at risk of dying from the disease.

Research team unlocks secrets of Ebola

November 16, 2017
In a comprehensive and complex molecular study of blood samples from Ebola patients in Sierra Leone, published today (Nov. 16, 2017) in Cell Host and Microbe, a scientific team led by the University of Wisconsin-Madison has ...

Study raises possibility of naturally acquired immunity against Zika virus

November 16, 2017
Birth defects in babies born infected with Zika virus remain a major health concern. Now, scientists suggest the possibility that some women in high-risk Zika regions may already be protected and not know it.

A structural clue to attacking malaria's 'Achilles heel'

November 16, 2017
Researchers from The Scripps Research Institute (TSRI) and PATH's Malaria Vaccine Initiative (MVI) have shed light on how the human immune system recognizes the malaria parasite though investigation of antibodies generated ...

0 comments

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.