ABSTRACT
Aim The Paediatric High Dependency Unit (PHDU) at University Hospital Limerick (UHL) operates as the only standalone unit outside of Dublin centres. The aim of this study was to describe a regional PHDU population, compare outcomes with international standards (PICANet) and ensure adequate clinical governance. Methods This is a retrospective analysis of 126 admission records from January - December 2019. Results There were 126 admissions to PHDU in 2019, of which respiratory (n=81, 64.3%) and neurological (n=23, 18.3%) subgroups represented the largest populations. Median length of stay was two days with mean age of admission 3.97 ± 4.5 years and slightly more male admissions (56%). Of the total, 65% required oxygen, 32.1% needed CPAP directly and 38% commenced high-flow, of whom 29% transitioned to CPAP. Transfer for tertiary care was required in 10.3%, of whom 7.9% needed PICU. Conclusion The data show UHL PHDU to have a patient population reflecting international trends as well as producing satisfactory patient outcomes. With a low rate of transfer for tertiary care and given that 15 other paediatric units exist in the Republic of Ireland outside Dublin, development of regional PHDU capacity would provide great opportunity to decrease strain on PICU bed capacity, particularly during busy Winter months.
Subject(s)
Intensive Care Units, Pediatric , Patient Admission , Child , Child, Preschool , Hospital Units , Hospitalization , Humans , Infant , Length of Stay , Male , Retrospective StudiesSubject(s)
COVID-19 , Pneumonia , Antibodies, Viral , Child , Humans , Ireland/epidemiology , SARS-CoV-2ABSTRACT
BACKGROUND: Positive expiratory pressure (PEP) devices are an important element of the management of cystic fibrosis, and of other respiratory diseases. Whereas there have been reports in the literature of contamination of airway clearance devices and their surfaces by microbial pathogens, there is little evidence available regarding such contamination and its contribution to respiratory infection. AIM: To establish whether pathogenic bacteria can contaminate PEP devices in the context of normal cleaning and maintenance practices. METHODS: Patients' home-use clearance devices were brought to a routine clinic appointment and collected for microbiology sampling and analysis. The patients were provided with replacement devices. Nineteen such devices were collected from 17 patients, reflecting use of multiple devices by some patients. Swabs were taken and cultured from each patient's used device, the patient's airway, as well as from new unopened and unused devices that acted as controls. RESULTS: Seven of 19 devices (37%) tested positive for presence of pathogenic bacteria. Device-cleaning methods varied among patients and non-sterilization methods were found to be ineffective at removing pathogens. Microbial species found on the devices did not correlate with those identified from airway swabs. CONCLUSION: This study demonstrates the presence of pathogens on positive expiratory pressure devices. The potential for transmission of these pathogens to the patient's airway and the risk of infection remains unclear and requires further study.
ABSTRACT
A gender gap exists in cystic fibrosis (CF). Here we investigate whether plasma microRNA expression profiles differ between the sexes in CF children. MicroRNA expression was quantified in paediatric CF plasma (n = 12; six females; Age range:1-6; Median Age: 3; 9 p.Phe508del homo- or heterozygotes) using TaqMan OpenArray Human miRNA Panels. Principal component analysis indicated differences in male versus female miRNA profiles. The miRNA array analysis revealed two miRNAs which were significantly increased in the female samples (miR-885-5p; fold change (FC):5.07, adjusted p value: 0.026 and miR-193a-5p; FC:2.6, adjusted p value: 0.031), although only miR-885-5p was validated as increased in females using specific qPCR assay (p < 0.0001). Gene ontology analysis of miR-885-5p validated targets identified cell migration, motility and fibrosis as processes potentially affected, with RAC1-mediated signalling featuring significantly. There is a significant increase in miR-885-5p in plasma of females versus males with CF under six years of age.
Subject(s)
Cystic Fibrosis/blood , MicroRNAs/blood , Sex Characteristics , Child , Child, Preschool , Cystic Fibrosis/genetics , Female , Gene Ontology , Humans , Infant , Male , Prognosis , Reverse Transcriptase Polymerase Chain ReactionABSTRACT
Introduction Quick, painless, cheap and reliable, the sweat test remains the gold standard diagnostic test for cystic fibrosis. We aimed to describe the pattern of testing in Ireland over a calendar year. Methods Information on sweat test practices was requested from each centre between 1st January 2011 and 31st December 2011, and the number of positive, negative, equivocal, and insufficient samples was recorded. Results In 2011 there were 2555 sweat tests performed in 15 centres, ranging from 35 to over 450 tests per centre. 35 (1.4%) were in the diagnostic range. The overall quantity not sufficient (QNS) rate was 10.3% (range 0-28.3%). Testing was performed across a wide age range (2.5 weeks to 75 years). The mean sweat chloride value was 16.5 mmol/L (SD 16.1 mmol/L). Discussion Our study demonstrates a high number of sweat tests performed in Ireland with significant variation in sweat testing practices across 15 different sites.
ABSTRACT
Pulmonary aspiration of gastric refluxate (PAGR) has been demonstrated in association with pulmonary inflammation in school aged children with Cystic Fibrosis (CF). We sought to determine if similar findings were present in preschool children. Pepsin was measured in Broncho-alveolar lavage (BAL) fluid collected from clinically stable preschool children with CF and controls. Elevated pepsin levels were found in a subgroup of children with CF, but this was not found to be associated with pulmonary infection, pulmonary inflammation or respiratory or gastrointestinal symptoms.
Subject(s)
Bronchoalveolar Lavage Fluid , Cystic Fibrosis/diagnosis , Cystic Fibrosis/physiopathology , Respiratory Aspiration/diagnosis , Respiratory Aspiration/physiopathology , Bronchoalveolar Lavage , Bronchoscopy/methods , Child , Child, Preschool , Cystic Fibrosis/epidemiology , Female , Gastroesophageal Reflux/diagnosis , Gastroesophageal Reflux/physiopathology , Humans , Infant , Male , Prospective Studies , Respiratory Aspiration/epidemiologySubject(s)
Bacteria/isolation & purification , Candida/isolation & purification , Equipment and Supplies/microbiology , Bacteria/classification , Candida/classification , Endoscopy , Gastrostomy , Humans , Microbiological Techniques , Printing, Three-Dimensional , Spectrometry, Mass, Matrix-Assisted Laser Desorption-IonizationABSTRACT
Significant gaps have been identified in parental understanding of CF newborn screening and the consequences of carrying an altered CF gene. Seven potential causes of psychosocial adversity arising from false positive newborn screening for CF have been identified. The current study aimed to increase parents understanding of CF, reduce their levels of stress, and investigate psychosocial adversity arising from false-positive screening. This national study was run over one year in the Republic of Ireland. Parents were recruited for the study following a diagnostic sweat test confirming their child carried a single altered CF gene. Parents were randomly assigned into a control and intervention group, with those in the intervention group receiving a carefully designed information pack. All parents took part in semi-structured interviews. Parents (n = 16) who received an information pack had significantly higher CF knowledge scores than parents (n = 16) in the control group. 66% of parents in the control group misunderstood the health implications of carrying an altered CF gene, no parents in the intervention group had the same misunderstanding. There was no significant difference in stress scores between the groups. Parents of infants who had more than one sweat test due to insufficient sweat quantity had higher overall stress percentiles (50%), than parents of infants who had one sweat test (30%), indicating greater parental stress. The combination of written and audio-visual information contained in the information pack successfully increased parents comprehension of CF. The study also evaluates the potential for psychosocial adversity following false positive newborn screening for CF.
Subject(s)
Cystic Fibrosis/psychology , Genetic Carrier Screening/methods , Genetic Counseling/methods , Health Knowledge, Attitudes, Practice , Neonatal Screening/methods , Parents/psychology , Adaptation, Psychological , Child , Cystic Fibrosis/genetics , Cystic Fibrosis/prevention & control , Cystic Fibrosis Transmembrane Conductance Regulator/genetics , Female , Humans , Infant , Infant, Newborn , Ireland , Male , Neonatal Screening/psychology , Parents/education , Sweat/chemistryABSTRACT
BACKGROUND: There is a paucity of research examining the impact of informal caregiving on parents of young children with cystic fibrosis (CF). The aim of this study was to examine caregiver burden and identify risk factors associated with high caregiver burden in mothers and fathers of young children with CF. METHODS: This was a cross-sectional study of parents of young children with CF. A total of 213 families were invited to complete the CarerQoL questionnaire, a validated tool composed of two parts: (i) the CarerQol-7D which describes the care situation in terms of the negative and positive effects of caregiving and (ii) the visual analogue scale (VAS) which measures happiness on a scale from 0 to 10 (0=completely unhappy and 10=completely happy). The utility score (US) is a weighted average of the subjective burden derived from the CarerQol-7D (0-100); higher US indicates reduced burden. Differences in mother-father dyad median utility scores were examined using Wilcoxon signed rank test. Generalised linear mixed models were used to identify factors associated with high caregiver burden. RESULTS: At least one parent from 195 families completed the questionnaire (130 mother-father dyads, 189 mothers and 137 fathers). Fathers had a significantly higher median utility score than mothers [(89.2 (IQR 79.6-96.5) vs. 84.7 (74.5-88.0) p<0.001]. Factors found to be significantly associated with higher caregiver burden were increasing child age (OR 1.02; CI: 1.00-1.04), having a child ever positive for Pseudomonas aeruginosa (Pa) (OR 2.48; CI: 1.30-4.73) and being a mother (OR 1.65; CI: 1.02-2.65). CONCLUSIONS: This study contributes new findings to the sparse literature on caregiver burden of parents of young children with CF. Increasing child age and infection with Pa, associated with higher morbidity, were linked to greater parental burden.
Subject(s)
Burnout, Psychological , Cost of Illness , Cystic Fibrosis/psychology , Parents/psychology , Quality of Life , Adaptation, Psychological , Adult , Age Factors , Burnout, Psychological/etiology , Burnout, Psychological/psychology , Caregivers/psychology , Child , Cross-Sectional Studies , Cystic Fibrosis/diagnosis , Cystic Fibrosis/epidemiology , Family Health , Female , Humans , Ireland/epidemiology , Male , Pseudomonas aeruginosa/isolation & purification , Risk Factors , Surveys and QuestionnairesSubject(s)
Cross Infection/microbiology , Cystic Fibrosis/therapy , Equipment and Supplies/microbiology , Gram-Negative Bacterial Infections/microbiology , Pneumonia, Bacterial/microbiology , Stenotrophomonas maltophilia/isolation & purification , Child , Cross Infection/transmission , Cystic Fibrosis/complications , Disease Transmission, Infectious , Gram-Negative Bacterial Infections/transmission , Humans , MaleABSTRACT
The pathogenesis of CF lung disease may start in infancy. Therefore, it is important to monitor the early stages of its progress. The Exhalyzer D is the first commercially available device designed to measure lung ventilation inhomogeneity at any age. This study was conducted to assess the performance and feasibility of using the Exhalyzer D in a paediatric CF clinic. A total of 91 subjects were recruited (23 controls, and 68 patients with CF). The majority of CF patients (79%) and controls (78%) completed at least two successful washouts. A strong linear correlation was noted between LCI and FEV1. Children with CF under six years of age struggled to perform the washout in a technically correct manner. A clear learning effect was observed, with improved technique and shorter testing times on repeated visits.
Subject(s)
Cystic Fibrosis/diagnosis , Pulmonary Ventilation/physiology , Age Factors , Ambulatory Care Facilities , Case-Control Studies , Child , Child, Preschool , Cohort Studies , Cystic Fibrosis/etiology , Cystic Fibrosis/physiopathology , Disease Progression , Feasibility Studies , Forced Expiratory Volume , Humans , InfantABSTRACT
BACKGROUND: The Republic of Ireland has one of the highest reported incidences of cystic fibrosis (CF) in the world (1/1353) with an estimated carrier rate of 1/20. No cure exists, however there have been significant advances in available treatments. Newborn bloodspot screening (NBS) for CF was added to the NBS programme in Ireland in July 2011. Little is known about antenatal or postnatal women's knowledge about CF. METHODS: This was a cross-sectional study of 662 antenatal (≥36weeks gestation) and 480 postnatal women (post NBS). Women were asked to self-complete a questionnaire including 14 CF knowledge questions. RESULTS: Among the respondents significantly more postnatal than antenatal women were aware that CF is included on the NBS (81.8% vs 63.5%; p<0.001). 92.7% believe that there are health consequences to being a carrier and 33.6% believe there is a cure for CF. In the multivariate analysis, lower educational status (OR 2.13; 95% CI 1.31, 3.46) being an antenatal mother (OR 1.51; 95% CI 1.04, 2.18), having no family history of CF (OR 5.82; 95% CI 1.62, 20.90) were associated with poor CF knowledge, while increasing age was found to be protective against poor CF knowledge (OR 0.96; 95% CI 0.92, 0.99). CONCLUSIONS: Results from this study provide a useful insight into women's preexisting knowledge about CF, which could be used to inform initial discussions with parents about their child's diagnosis.
Subject(s)
Cystic Fibrosis Transmembrane Conductance Regulator/genetics , Cystic Fibrosis , Health Literacy , Neonatal Screening , Perinatal Care , Adult , Cross-Sectional Studies , Cystic Fibrosis/diagnosis , Cystic Fibrosis/epidemiology , Cystic Fibrosis/genetics , Cystic Fibrosis/psychology , Educational Status , Female , Genetic Testing/methods , Health Knowledge, Attitudes, Practice , Health Literacy/methods , Health Literacy/statistics & numerical data , Humans , Infant, Newborn , Ireland/epidemiology , Medical History Taking , Needs Assessment , Neonatal Screening/methods , Neonatal Screening/psychology , Perinatal Care/methods , Perinatal Care/organization & administration , Pregnancy , Surveys and QuestionnairesABSTRACT
Sleep related breathing disorders (SRBD) have historically been under-recognised and under-treated. Obstructive sleep apnoea (OSA) affects approximately 3% of children. In line with the increased recognition of SRBD there has been an increase in demand for diagnostic services. We determined the awareness of SRBD amongst Irish paediatricians, examined the provision of sleep services to children throughout the country between 2007 and 2011 and audited diagnostic sleep services in a tertiary centre in 2011. Amongst respondents there was an awareness of SRBD but a poor understanding of diagnostic evaluation with 31/46 (67) referring to inappropriate services. There has been a sharp increase in both diagnostic sleep tests (433-1793 [414]) and in the use of non-invasive ventilation (NIV) (31-186 [627]) for treatment of SRBD between 2007 and 2011. Paediatric sleep services are organized in an ad-hoc manner nationally with significant service variation. The use of domiciliary overnight oximetry reduced the requirement for more formal polysomnography by 70%.
Subject(s)
Diagnostic Services/statistics & numerical data , Disease Management , Sleep Apnea Syndromes , Child , Child Health Services/methods , Child Health Services/statistics & numerical data , Diagnostic Techniques, Respiratory System , Health Care Surveys , Health Services Needs and Demand , Humans , Ireland/epidemiology , Polysomnography/statistics & numerical data , Prevalence , Sleep Apnea Syndromes/diagnosis , Sleep Apnea Syndromes/epidemiology , Sleep Apnea Syndromes/etiology , Sleep Apnea Syndromes/therapyABSTRACT
The prevalence of Methicillin Resistant Staphylococcus Aureus (MRSA) in patients with Cystic Fibrosis (CF) has risen dramatically over the past 10 years. The clinical significance of MRSA in CF patients remains undetermined. We conducted a review of patients with CF infected with MRSA over a 10 year period at Our Lady's Children's Hospital, Crumlin between 1999 and 2009. We collected data from 24 patients infected with MRSA and 24 control patients without MRSA There was a significant difference between the two groups in the rate of decline in percentage FEV1 two years after MRSA infection (Difference: -17.4, 95% CI: -30.48, -4.31, p = 0.01). A similar trend was seen for FVC% and FEF25-75% predicted. This study suggests that persistent MRSA infection in the airways of children with CF is associated with diminished lung function two years post acquisition, when compared to a matched control cohort without MRSA.
Subject(s)
Cystic Fibrosis , Methicillin-Resistant Staphylococcus aureus , Respiratory System , Staphylococcal Infections/epidemiology , Adolescent , Case-Control Studies , Child , Child, Preschool , Cystic Fibrosis/complications , Cystic Fibrosis/microbiology , Cystic Fibrosis/physiopathology , Female , Humans , Male , Methicillin-Resistant Staphylococcus aureus/isolation & purification , Methicillin-Resistant Staphylococcus aureus/pathogenicity , Prevalence , Respiratory Function Tests , Respiratory System/microbiology , Respiratory System/physiopathology , Staphylococcal Infections/complications , Staphylococcal Infections/physiopathology , TimeABSTRACT
AIMS: To review the effect of total splenectomy on lung function and nutrition in children with cystic fibrosis related liver disease (CFLD) and associated portal hypertension. The stated indications for surgery and the short and long term risks of the procedure were also documented. METHOD: Over a 25 year period from January 1980 to June 2005, approximately 650 patients with cystic fibrosis (CF) were treated at the Royal Children's Hospital, Melbourne, Australia. Nine patients with CFLD who underwent a splenectomy during that time were identified and their medical records were reviewed. RESULTS: FEV1% predicted dropped by -16+/-11% in the two years pre-splenectomy. This contrasts with the increase in FEV1% predicted of 2+/-16% in the two years post-splenectomy (p = 0.05). The cumulative gain in WAZ score (DeltaWAZ pre) over the two years prior to splenectomy of 0.045+/-0.69 was not significantly different from the cumulative gain in WAZ score (DeltaWAZ post) for the two years after splenectomy of 0.15+/-0.36 (p = 0.65). The average age at splenectomy was 14.8 years (SD = 3 years). The average weight of an excised spleen was 983 g (SD = 414 g). There were no deaths associated with splenectomy. The median length of follow up post-splenectomy was 6.0 years (range 0.7-15.8). There were no episodes of bacterial peritonitis or overwhelming sepsis. CONCLUSIONS: Splenectomy may have a beneficial effect on lung function although this may not manifest itself until the second year post-splenectomy. Splenectomy in patients with CFLD appears to be a safe procedure.