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RCSI
MATTERS
39
He says: "Our study was the rst to
reveal the mechanisms by which a
lack of the alpha-1 protein causes an
increase in the release of white blood
cell proteins into the blood stream. is
leads to an autoimmune process in the
body that mistakenly recognises these
proteins as foreign and activates its own
white blood cells to produce harmful
oxidants.
"Our research also reveals how a
treatment known as augmentation
therapy, where alpha-1 protein puri ed
from blood is given intravenously, leads
to a decrease in the abnormal protein
release, thereby, alleviating the disease-
associated autoimmunity."
is study was published in
Science
Translational Medicine, and was
awarded Research Paper of the Year at
the 2015 Irish Healthcare Awards.
CYSTIC FIBROSIS
Another major area of study, which
Professor McElvaney and his team are
involved in, is CF research.
CF is a multisystem disorder
caused by mutations of the CF
transmembrane regulator gene. is
results in a defective salt channel,
which ultimately reduces the water
content and increases the thickness
of secretions in the lungs, pancreas,
liver, intestines and reproductive tract.
Lung disease remains the leading
cause of morbidity and mortality in
patients with CF.
"Today, the median life expectancy
age of patients with CF is 38 years old.
is is still unacceptable and this is
why research is so important. By 2020,
my aim is to prolong this life expectancy
to well into the late 50s and 60s age
range by developing new therapies," says
Professor McElvaney.
Women tend to be more susceptible
to infection because of the levels of
oestrogen in their bodies, he says.
In 2012, his team was the rst group,
again, to show that if you take bacteria
from the lung and expose it to
oestrogen, this bacteria becomes more
virulent, leaving the patient with CF
more susceptible to in ammation and
infection. is research was published in
the prestigious
New England Journal of
Medicine.
e study demonstrated that women
with CF are at increased risk for mucoid
conversion of
Pseudomonas aeruginosa,
which contributes to a sexual dichotomy
in disease severity. His research
group also led to a breakthrough in
understanding the mechanisms behind
impaired bacterial killing by white
blood cells in individuals with CF.
is innate impairment was a direct
result of a lack of CF transmembrane
conductance regulator (CFTR) function,
and treatment of patients with the ion
channel potentiator Ivaca or resulted
in normalised neutrophil degranulation
and extracellular bacterial killing.
Can this bad gene that causes lung
diseases in CF be eliminated?
" is is ongoing research, and in the
future, a better understanding of the
pathogenesis of the condition may
unlock the secret to eradicating many
hereditary diseases that exist today."
MAKING A DIFFERENCE
WORLDWIDE
r an ay aresh hotirma
edicine ass o
Ph
has been recent y
a ointed as ssistant Pro essor
at ee Kon hian choo o
edicine in a ore rin
his time wor in with Pro essor
c vaney r hotirma was
awarded a resti io s o ec ar
edicine re and inician cientist
e owshi
and
com eted a Ph investi atin the
ro e o oestro en in cystic fibrosis
As a recognised clinician-scientist, Dr Chotirmall has returned home to Singapore
to join the faculty at LKC Medicine, where he has established a Translational
Respiratory Research Group, with focus on infection, in ammation, and
immunity, in the context of chronic in ammatory respiratory diseases that a ect
Asian populations. Research in the Chotirmall lab is described as `sharing a
core translational respiratory focus with a common goal to improve patient care
through advancing the understanding of disease from a scienti c basis'.
Dr Chotirmall cites Professor Gerry McElvaney his mentor, teacher, trainer,
and now close friend as a major in uence in his current research interests.
" roughout my translational research and clinical career to date, Professor
McElvaney always reiterated that `research exists for our patients, not vice versa'.
A er countless summers in his lab working and learning as a medical student,
undergoing an intern post with him, clinical specialist training under his guidance
and completing a PhD in his lab, I am above all most grateful to him for teaching
me the key values to being the best clinician-scientist possible."
Dr Chotirmall says these values include empathy for patients, mentorship towards
junior colleagues, and a determination to attempt to answer the di cult clinical
questions through research.
oday the median
i e ex ectancy a e o
atients with cystic
fibrosis is years o d
his is nacce tab e and
this is why research is so
im ortant y
my
aim is to ro on this i e
ex ectancy to we into
the ate s and s a e
ran e by deve o in new
thera ies
RCSI RE
SEARCH
PROFESSOR GERR
Y MCEL
V
ANEY
RCSI MattersSRH.indd 39
03/03/2016 11:56