Publications by authors named "Dominic Hughes"

7 Publications

  • Page 1 of 1

A Short extension to multiple breath washout provides additional signal of distal airway disease in people with CF: A pilot study.

J Cyst Fibros 2021 Jul 15. Epub 2021 Jul 15.

Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom; National Heart and Lung Institute, Imperial College London, Manresa Rd, London, United Kingdom; European Cystic Fibrosis Society Lung Clearance Index Core Facility, London, United Kingdom.

Background: Adding a slow vital capacity (SVC) to multiple breath washout (MBW) allows quantification of otherwise overlooked signal from under/un-ventilated lung units (UVLU) and may provide a more comprehensive assessment of airway disease than conventional lung clearance index (LCI).

Methods: We conducted a pilot study on people undergoing MBW tests: 10 healthy controls (HC) and 43 cystic fibrosis (CF) subjects performed an SVC after the standard end of test. We term the new outcome LCI with Short extension (LCI). We assessed (i) CF/ HC differences, (ii) variability (iii) effect of pulmonary exacerbation (PEx)/treatment and (iv) relationship with CF computed tomography (CFCT) scores.

Results: HC/ CF group differences were larger with LCI than LCI (P<0.001). Within the CF group UVLU was highly variable and when abnormal it did not correlate with corresponding LCI. Signal showed little variability during clinical stability (n = 11 CF; 2 visits; median inter-test variability 2.6% LCI 2.5% LCI). PEx signal was significantly greater for LCI both for onset and resolution. Both MBW parameters correlated significantly with total lung CT scores and hyperinflation but only LCI correlated with mucus plugging.

Conclusions: UVLU captured within the LCI varies between individuals; the lack of relationship with LCI demonstrates that new, additional information is being captured. LCI repeatability during clinical stability combined with its larger signal around episodes of PEx may lend it superior sensitivity as an outcome measure. Further studies will build on this pilot data to fully establish its utility in monitoring disease status.
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http://dx.doi.org/10.1016/j.jcf.2021.06.013DOI Listing
July 2021

Clinical characteristics of Pseudomonas and Aspergillus co-infected cystic fibrosis patients: A UK registry study.

J Cyst Fibros 2021 May 3. Epub 2021 May 3.

National Heart & Lung Institute, Imperial College London, UK; Royal Brompton and Harefield Hospitals, London, UK.

Background: Pseudomonas aeruginosa (Pa) and Aspergillus species (Asp) are the most common bacterial and fungal organisms respectively in CF airways. Our aim was to examine impacts of Asp infection and Pa/Asp co-infection.

Methods: Patients on the UK CF Registry in 2016 were grouped into: absent (Pa-), intermittent (Pai) or chronic Pa (Pac), each with Asp positive (Asp+) or negative (Asp-). Primary outcome was best percentage predicted FEV (ppFEV) that year. Secondary outcomes were intravenous (IV) antibiotic courses, growth (height, weight, BMI) and additional disease complications. Associations between outcomes and infection-status were assessed using regression models adjusting for significant confounders (age, sex, Phe508del homozygosity and CF-related diabetes (CFRD)).

Results: 9,270 patients were included (median age 19 [IQR 9-30] years, 54% male, 50% Phe508del/F508del). 4,142 patients (45%) isolated Pa, 1,460 (16%) Asp. Pa-/Asp+ subjects had an adjusted ppFEV that was 5.9% lower than Pa-/Asp- (p < 0.0001). In patients with Pai or Pac, there was no additional impact of Asp on ppFEV. However, there was a higher probability that Pac/Asp+ patients had required IV antibiotics than Pac/Asp- group (OR 1.23 [1.03-1.48]). Low BMI, ABPA, CF-liver disease and CFRD were all more frequent with Asp alone than Pa-/Asp-, though not more common in Pac/Asp+ than Pac/Asp-.

Conclusions: Co-infection with Pa and Asp was not associated with reduced lung function compared with Pa alone, but was associated with additional use of IV antibiotics. Asp infection itself is associated with several important indicators of disease severity. Longitudinal analyses should explore the impact of co-infection on disease progression.
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http://dx.doi.org/10.1016/j.jcf.2021.04.007DOI Listing
May 2021

Eradication success for non-tuberculous mycobacteria in children with cystic fibrosis.

Eur Respir J 2021 05 27;57(5). Epub 2021 May 27.

National Heart and Lung Institute, Imperial College London, London, UK.

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http://dx.doi.org/10.1183/13993003.03636-2020DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8280568PMC
May 2021

in the Cystic Fibrosis Airway: Does It Deserve Its Reputation as a Predatory "Bully"?

Am J Respir Crit Care Med 2021 04;203(8):1027-1030

Royal Brompton and Harefield NHS Foundation Trust London, United Kingdom and.

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http://dx.doi.org/10.1164/rccm.202009-3639LEDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8048747PMC
April 2021

Selective Sampling of the Lower Airway in Children with Cystic Fibrosis: What Are We Missing?

Am J Respir Crit Care Med 2020 03;201(6):747-748

Royal Brompton and Harefield NHS Foundation TrustLondon, United Kingdomand.

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http://dx.doi.org/10.1164/rccm.201911-2134LEDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7068833PMC
March 2020

King's Variceal Prediction Score: A Novel Noninvasive Marker of Portal Hypertension in Pediatric Chronic Liver Disease.

J Pediatr Gastroenterol Nutr 2017 04;64(4):518-523

Paediatric Liver, Gastroenterology and Nutrition Centre, King's College Hospital, London, UK.

Background And Aims: Variceal haemorrhage can be a life-threatening complication of chronic liver disease in children. There is limited evidence about the optimal prophylactic management and selection criteria of children who will benefit from upper gastrointestinal endoscopy.

Methods: Children presenting in our centre with suspected portal hypertension or gastrointestinal bleeding and undergoing their first oesophagogastroduodenoscopy between 2005 and 2012 were included. Clinical, biochemical, and radiological data were collected. A separate validation cohort from May 2013 to October 2014 was obtained.

Results: Data on 124 treatment-naïve patients were collected; 50% had biliary atresia. Thirty-five (28%) children presented with gastrointestinal bleeding and overall 79 (64%) had clinically significant (grade II-III) varices. Clinical prediction rule, aspartate aminotransferase-platelet ratio index, and varices prediction rule had at optimal cut-off sensitivity and specificity of 76% and 59%, 60% and 55%, and 80% and 59%, respectively. Logistic regression yielded a new prediction rule of (3 × albumin ([g/dL]) -  (2 - equivalent adult spleen size [cm]). This King's variceal prediction score had a favourable areas under the curve of 0.772 (0.677-0.867) compared to clinical prediction rule 0.732 (0.632-0.832). At the optimal cut-off of 76 this yielded a sensitivity and specificity of 72% and 73% and a positive and negative predictive value of 82% and 60%, respectively. In the validation cohort areas under the curve was 0.818 (0.654-0.995) with sensitivity and specificity of 78% and 73%, respectively.

Conclusions: Our new prediction score may be a useful tool in the selection of children with clinically significant varices eligible for a screening endoscopy.
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http://dx.doi.org/10.1097/MPG.0000000000001423DOI Listing
April 2017

Delayed hepato-spleno-renal bypass for renal salvage following malposition of an infrarenal aortic stent-graft.

J Endovasc Ther 2010 Jun;17(3):326-31

Department of Vascular Surgery, Hammersmith Hospitals, London, UK.

Purpose: To report a salvage maneuver for accidental coverage of both renal arteries during endovascular aneurysm repair (EVAR) of an infrarenal aortic aneurysm (AAA) and survey our surgical colleagues in the UK for their use of this bypass procedure.

Methods: A 74-year-old woman who had an EVAR complicated by renal failure secondary to malposition of the stent-graft underwent successful delayed renal revascularization with hepatorenal and splenorenal bypasses. This case prompted a literature review and preparation of an online 6-part questionnaire regarding the incidence and management of renal impairment following EVAR. The survey invitation was sent to all listed members of the Vascular Society of Great Britain and Ireland.

Results: Responses from 68 (10.5%) of the 650 vascular surgeons invited to participate in the survey were analyzed. The combined experience of those who completed the survey was >1500 EVAR procedures per annum. Forty percent (27/68) of the respondents had experienced a case of bilateral renal artery occlusion during EVAR. Two thirds (67%, 18/27) of these surgeons stated a preference for revascularizing the kidneys endovascularly, 7 surgeons would convert to open repair, 1 surgeon favored iliorenal bypass, and another suggested splenorenal bypass. Following intervention, 15 (56%) of 27 surgeons achieved revascularization that resulted in a return to baseline serum creatinine, 7 (26%) achieved partial recovery of the patient's serum creatinine, 3 (11%) had a patient on permanent dialysis, and 2 (7%) had patients who died (after open repair and endovascular procedure, respectively).

Conclusion: Bilateral renal artery occlusion caused by malposition of a stent-graft is probably underreported. If revascularization of the kidneys by endovascular techniques fails, there is no consensus as to the optimal approach. Delayed revascularization should be considered if the kidneys show concentration of imaging contrast. Hepato-spleno-renal bypass, which has not heretofore been indicated for renal salvage post EVAR, can provide a good functional result in this situation.
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http://dx.doi.org/10.1583/09-3011.1DOI Listing
June 2010
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