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Annual General Meeting of the Irish Thoracic Society - IJMS | Irish ...

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1<br />

SESSION ONE<br />

1<br />

ANNUAL MEETING OF THE IRISH THORACIC SOCIETY • 11 - 12 November 2005 • WESTWOOD HOUSE HOTEL, GALWAY ANNUAL MEETING OF THE IRISH THORACIC SOCIETY • 11 - 12 November 2005 • WESTWOOD HOUSE HOTEL, GALWAY<br />

SESSION ONE<br />

1.4<br />

1.5<br />

Influence <strong>of</strong> CFTR genotype on pulmonary disease in adult<br />

Cystic Fibrosis patients from Belfast and Dublin referral centres<br />

Background<br />

In referring to differences in survival between Cystic<br />

Fibrosis (CF) patients in <strong>the</strong> Republic and Nor<strong>the</strong>rn<br />

Ireland, <strong>the</strong> recent Pollock Report 1 also cautions<br />

on <strong>the</strong> interpretation <strong>of</strong> available data, citing<br />

variation in diagnosis and coding as factors that can<br />

complicate comparisons. Ano<strong>the</strong>r factor that can<br />

greatly influence survival in CF is CFTR genotype.<br />

The aim <strong>of</strong> this study was to evaluate differences in<br />

CFTR genotype between CF patients attending adult<br />

referral centres in Dublin and Belfast and to assess<br />

<strong>the</strong> influence <strong>of</strong> <strong>the</strong>se differences on pulmonary<br />

function, a major indicator <strong>of</strong> pulmonary prognosis<br />

and survival in CF.<br />

Methods<br />

CFTR genotypes on 205 patients attending <strong>the</strong><br />

Dublin centre and 115 patients attending <strong>the</strong> Belfast<br />

centre were collected. Pulmonary function was<br />

assessed on all patients when clinically stable with<br />

no evidence <strong>of</strong> acute infection.<br />

Results<br />

Just over half (50.4%) <strong>of</strong> <strong>the</strong> Belfast group had CFTR<br />

Introduction<br />

The management <strong>of</strong> acute pulmonary exacerbations<br />

in Cystic Fibrosis has changed in recent years with<br />

<strong>the</strong> increasing usage <strong>of</strong> home intravenous <strong>the</strong>rapy.<br />

Method<br />

A retrospective examination <strong>of</strong> hospital admission<br />

<strong>of</strong> patients with acute pulmonary exacerbations<br />

<strong>of</strong> Cystic Fibrosis in 2004 was carried out. Total<br />

admission figures were derived from <strong>the</strong> HIPE<br />

system. A review <strong>of</strong> all home IV prescriptions during<br />

this time was performed. This was accompanied<br />

by review <strong>of</strong> FEV1 values and sputum culture and<br />

sensitivity corresponding with time <strong>of</strong> exacerbation.<br />

Results<br />

In total <strong>the</strong>re were over 300 episodes <strong>of</strong> acute<br />

exacerbations in 2004. There were 298 admissions<br />

to SVUH with exacerbations in 2004. In this year<br />

131 courses <strong>of</strong> home IV’s were prescribed to 77<br />

patients. Of <strong>the</strong>se 104 were initiated and completed<br />

genotypes that are associated with mild disease<br />

compared only 25% <strong>of</strong> Dublin patients (p

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