ORCID Profile
0000-0002-9348-2760
Current Organisations
UNSW Sydney
,
Sydney Children's Hospital
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Publisher: American Thoracic Society
Date: 07-2011
Publisher: Springer Science and Business Media LLC
Date: 11-03-2019
Publisher: Elsevier BV
Date: 04-2016
DOI: 10.1016/J.SLEEP.2016.12.005
Abstract: Myotonic dystrophy type 1 (DM1) is an autosomal dominant neuromuscular disease with variable severity that affects all ages. Sleepiness is an important co-morbidity affecting a large proportion of paediatric DM1 patients. The current study examined the relationship between sleepiness and quality of life in a paediatric DM1 cohort. A cross-sectional study was conducted in children and adolescents with DM1 attending a multi-disciplinary neuromuscular clinic in a tertiary paediatric centre. The modified Epworth sleepiness scale (ESS), the PedsQL™ quality of life (version 4.0) and neuromuscular modules (version 3.0) were used to measure sleepiness, generic quality of life and neuromuscular-specific quality of life, respectively. Seventeen current patients with DM1 completed all questionnaires and assessments. Of them, 35.5% had abnormal scores on the modified ESS, which is indicative of excessive daytime sleepiness (EDS). Higher ESS scores were highly significantly related to reduced quality of life in neuromuscular-specific (r = 0.77, p < 0.001) and generic measures (r = 0.78, p < 0.001). EDS was not significantly related to intellectual function or sleep disorders as detected on polysomnography. EDS is common in children and adolescents with DM1. It is associated with reduced quality of life and should be routinely assessed. Further studies to develop treatments of EDS in this population are required and may improve overall outcomes.
Publisher: Springer Science and Business Media LLC
Date: 02-07-2015
Publisher: Elsevier BV
Date: 11-2019
DOI: 10.1016/J.JCF.2019.03.010
Abstract: Children with CF are insulin deficient from infancy but very little is known about the impact of glucose abnormalities in early life. We aimed to identify and describe interstitial glucose levels in CF children 11.1 mmol/L in 39% of participants. The percentage neutrophil count on BAL was positively correlated with elevated SG (peak SG r Children with CF frequently demonstrate elevated SG levels before age 6 years, which are associated with increased pulmonary inflammation and Pseudomonas aeruginosa infection. Transient SG elevations into the diabetic range (≥11.1 mmol/L) were identified in children from 1 year of age.
Publisher: Wiley
Date: 08-08-2019
DOI: 10.1002/PPUL.24456
Publisher: Elsevier BV
Date: 03-2016
Publisher: Elsevier BV
Date: 05-2013
DOI: 10.1016/J.JCF.2012.09.005
Abstract: Diabetes has a deleterious effect on clinical status in children with Cystic Fibrosis (CF). We hypothesized that children with CF Related Diabetes (CFRD) or Impaired Glucose Tolerance (IGT) would have more rapidly progressive lung disease based on chest computed tomography (CT) than those with normal glucose tolerance (NGT). In a retrospective study we compared lung structure changes over time, as assessed by CT, in 34 CF children with CFRD, IGT or NGT. We then compared CT findings with changes in lung function. Percentage forced expiratory volume in 1s (%FEV1) remained stable over time with a mean (±SD) yearly change of -0.5% (±3.9), -0.4% (±2.3) and -0.85% (±2.8) (p=0.92) for the CFRD, IGT and NGT groups respectively. However, there was a mean (95%CI) increase in % CT score of 3.86%/year (1.77-5.95%), 1.59%/year (0.6-2.58%) and 1.09%/year (0.07-2.11%) (p=0.023). In patients with CFRD, there was a more rapid progression of structural lung disease, compared to those who had NGT that was not reflected by change in lung function.
Publisher: Informa UK Limited
Date: 13-06-2016
DOI: 10.1080/17476348.2016.1190646
Abstract: Cystic fibrosis-related diabetes (CFRD) is the end-point of a spectrum of glucose abnormalities in cystic fibrosis that begins with early insulin deficiency and ultimately results in accelerated nutritional decline and loss of lung function. Current diagnostic and management regimens are unable to entirely reverse this clinical decline. This review summarises the current understanding of the pathophysiology of CFRD, the issues associated with using oral glucose tolerance tests in CF and the challenges faced in making the diagnosis of CFRD. Medline database searches were conducted using search terms "Cystic Fibrosis Related Diabetes", "Cystic Fibrosis" AND "glucose", "Cystic Fibrosis" AND "insulin", "Cystic Fibrosis" AND "Diabetes". Additionally, reference lists were studied. Expert commentary: Increasing evidence points to early glucose abnormalities being clinically relevant in cystic fibrosis and as such novel diagnostic methods such as continuous glucose monitoring or 30 minute s led oral glucose tolerance test (OGTT) may play a key role in the future in the screening and diagnosis of early glucose abnormalities in CF.
Publisher: Wiley
Date: 09-2019
DOI: 10.1002/PPUL.24491
Abstract: Children with cystic fibrosis (CF) are routinely managed in a multidisciplinary clinic at tertiary pediatric centers. However, children with bronchiectasis may not be managed in the same way. We sought to compare the management model and clinical outcomes of children with bronchiectasis with children diagnosed with CF, in a single pediatric center. We identified patients with bronchiectasis from hospital medical records at an urban tertiary pediatric hospital and identified a sex- and age-matched CF patient at the same center to compare lung function, nutritional status, frequency of physiotherapy and respiratory physician visits, and number of microbiological s les taken for bacterial culture. Twenty-two children with bronchiectasis were identified, mean (standard deviation [SD]) age was 11 (3) years. The most common known etiology for bronchiectasis was postinfective (6 of 22) but was unknown in 8 of 22. The cohort with bronchiectasis had poorer lung function (FEV1 mean [SD] percent predicted 78.6 [20.5] vs 94.5 [14.7], P = .005) and had less outpatient reviews by the respiratory physician (P < .001) and respiratory physiotherapist (P < .001) when compared to those with CF. Nutritional parameters did not differ between the groups. Many children (10 of 22, 45%) with bronchiectasis did not have any microbiological respiratory tract s les taken for evaluation. Children with bronchiectasis at this institution have poorer lung function than children with CF, and are deserving of improved multidisciplinary care.
Publisher: Elsevier BV
Date: 2018
DOI: 10.1016/J.JCF.2017.07.011
Abstract: The pathogenesis of gut inflammation, bacterial dysbiosis and increased rates of malignancy in CF is unclear. Fecal M2-pyruvate kinase (M2-PK) is a biomarker indicative of cellular proliferation that may be raised in intestinal malignancy and inflammation. Biomarkers, including M2-PK, may be useful in assessing effects of novel therapies on the gastrointestinal tract. M2-PK was measured in stools collected from patients with CF and HC (0-10years). Linear mixed model analysis was used. M2-PK levels did not significantly change in children with CF (36 patients, 77 s les) (P=0.998) or HC (45 patients, 45 s les) (P=0.21), over the age range 0-10years. Patients with CF had elevated M2-PK compared to HC (median [IQR range]: 10.7 [5.7-28.6 1.0-239.1] (n=77) vs. 1.0 [1.0-1.0 1.0-50.0] (n=45) U/mL, respectively P=0.001). Fecal M2-PK was elevated in children with CF compared with HC during infancy and throughout childhood suggesting abnormalities in the CF gut exist in early life.
Publisher: Wiley
Date: 12-02-2010
DOI: 10.1007/S10545-009-9042-5
Abstract: Classic galactosaemia is caused by a recessively inherited deficiency of the enzyme galactose 1 phosphate uridyl transferase (GALT). Patients with classical galactosaemia are at increased risk of developing cataracts. We sought to retrospectively review the incidence and severity of cataracts in the cohort of galactosaemia patients attending our national treatment centre and to assess a possible effect of dietary compliance on cataract formation and the benefits of regular ophthalmic follow-up. We retrospectively reviewed the clinical notes of all patients currently attending our centre with classic galactosaemia and identified all those in whom cataracts had been diagnosed by an ophthalmologist. Compliance to diet was also reviewed and compared with a matched control group. Of 100 active patient charts, 14 had cataracts diagnosed at some stage. Six of these persisted whereas eight regressed. Three occurred soon after birth. Age at cataract formation varied from soon after birth to 19 years of age. There was no significant difference in the cataract group between those who were compliant and those who were noncompliant with diet (p = 0.09). There was no difference in compliance between the cataract group and the control group (p = 0.16). None of the cataracts found were affecting vision. Cataracts affecting vision were not found in our cohort. A direct relationship between dietary compliance and cataract formation was not demonstrated. On the basis of our data, regular life-long ophthalmic exam of patients with classic galactosemia seems to be unnecessary. Cataracts which develop in patients with classical Galactosaemia do not usually affect vision and may be unrelated to compliance to diet.
Publisher: Public Library of Science (PLoS)
Date: 13-09-2012
Publisher: American Thoracic Society
Date: 07-2022
Publisher: Elsevier BV
Date: 07-2017
Publisher: Elsevier BV
Date: 03-2019
DOI: 10.1016/J.JCF.2019.05.005
Abstract: Screening for Cystic Fibrosis-related diabetes is recommended in patients with CF <10 years old when there are concerns about growth and lung function. The Oral Glucose Tolerance Test (OGTT) is recommended but has not been validated in this cohort. We sought to determine whether the 2-h OGTT, the gold standard diagnostic test for CFRD, detects clinical decline in children with CF <10 years old. We analysed blood glucose(BG) levels collected every 30 min during OGTT in 27 children with CF < 10 years old, comparing the 2-hour BG (BG The BG Children with CF < 10 years of age with higher BG
Publisher: Springer Science and Business Media LLC
Date: 25-02-2014
DOI: 10.1007/S11325-014-0960-6
Abstract: The aim of this study was to assess the impact of changes in respiratory support (RS) settings recommended after a titration polysomnography (PSG), in terms of daytime symptoms and quality of life. A retrospective chart review of all RS (CPAP and bi-level ventilation) titration studies was carried out at our tertiary paediatric sleep laboratory in the past 5 years. All patients with at least two studies in the past 5 years were included in the analysis. Parents completed the obstructive sleep apnoea (OSA)-18 and Paediatric Daytime Sleepiness Scale (PDSS) questionnaires on the night of each PSG. Results are presented as means (SD). A total of 42 patients (25 on CPAP and 17 on bi-level ventilation, age 11 (6) years) had 71 pairs of titration studies (41 CPAP and 30 bi-level). Changes in RS settings were recommended in 27 of 41 (65%) CPAP studies and 11 of 30 (36%) bi-level studies. Overall, changes were fully implemented by the treating physician in 55% of cases. There was an improvement in total OSA-18 score between studies in 48% of the paired CPAP studies and 65% of bi-level studies. OSA-18 scores improved in 47% of the studies where any recommended change had been implemented versus 0% of those where none of the recommended changes had been made (p=0.1). Titration studies frequently led to recommendations for a change in RS settings in these patients on long-term RS. Symptom scores were more likely to improve if recommendations for change were implemented by the time of the follow-up study.
Publisher: Daedalus Enterprises
Date: 25-09-2018
Abstract: Cough augmentation techniques are taught by health-care providers to improve secretion clearance and to help prevent respiratory infections in children with neuromuscular disease. There is some evidence of the effectiveness of a manually assisted cough when applied by health-care providers. However, it is unknown whether parents and caregivers may also be effective in applying manually assisted cough. The aim of this study was to evaluate whether parents and caregivers are effective at applying a manually assisted cough to a child with neuromuscular disease after being taught by a health-care provider. For this prospective cohort study, children and their parents or caregivers were recruited from neuromuscular clinics in the Sydney Children's Hospitals Network. Cough peak flow was the outcome measure for the strength of the child's cough. Children were eligible to participate if their unassisted cough peak flow at baseline was <270 L/min. Parents and caregivers were taught a manually assisted cough by a physiotherapist before being measured. The cough peak flow was measured in the following order: (1) during an unassisted cough as baseline, (2) during a manually assisted cough performed by a physiotherapist, (3) during a manually assisted cough performed by a parent or caregiver, and (4) during an unassisted cough after intervention. Twenty-eight children (24 boys, 4 girls mean ± SD age, 12 ± 3 y) completed the study. No clinically or statistically significant changes were found in the cough peak flow after the application of a manually assisted cough by parents or caregivers (95% CI -11 to 11 L/min) or by physiotherapists (95% CI -6 to 14 L/min). Parents and caregivers and health-care providers were ineffective at increasing cough peak flow in children with neuromuscular weakness when applying a manually assisted cough. A single training session was insufficient for a parent or caregiver to be able to apply a manually assisted cough effectively on his or her child with neuromuscular weakness. Further research is warranted to guide recommendations on how best to equip parents and caregivers with the skills to help manage children with neuromuscular disease.
Publisher: Elsevier BV
Date: 10-2016
No related grants have been discovered for John Widger.