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Ah-Fong Hoo,
Lena P Thia,
The Thanh Diem Nguyen,
Andrew Bush,
Jane Chudleigh,
Sooky Lum,
Deeba Ahmed,
Ian Balfour Lynn,
Siobhan B Carr,
Richard J Chavasse,
Kate L Costeloe,
John Price,
Anu Shankar, Colin Wallis,
Hilary A Wyatt,
Angela Wade,
Janet Stocks
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ABSTRACT: BACKGROUND: Long-term benefits of newborn screening (NBS) for cystic fibrosis (CF) have been established with respect to nutritional status, but effects on pulmonary health remain unclear. HYPOTHESIS: With early diagnosis and commencement of standardised treatment, lung function at ∼3 months of age is normal in NBS infants with CF. METHODS: Lung clearance index (LCI) and functional residual capacity (FRC) using multiple breath washout (MBW), plethysmographic (pleth) FRC and forced expirations from raised lung volumes were measured in 71 infants with CF (participants in the London CF Collaboration) and 54 contemporaneous healthy controls age ∼3 months. RESULTS: Compared with controls, and after adjustment for body size and age, LCI, FRC(MBW) and FRC(pleth) were significantly higher in infants with CF (mean difference (95% CI): 0.5 (0.1 to 0.9), p=0.02; 0.4 (0.1 to 0.7), p=0.02 and 0.9 (0.4 to 1.3), p<0.001, z-scores, respectively), while forced expiratory volume (FEV(0.5)) and flows (FEF(25-75)) were significantly lower (-0.9 (-1.3 to -0.6), p<0.001 and -0.7 (-1.1 to -0.2), p=0.004, z-scores, respectively). 21% (15/70) of infants with CF had an elevated LCI (>1.96 z-scores) and 25% (17/68) an abnormally low FEV(0.5) (below -1.96 z-scores). While only eight infants with CF had abnormalities of LCI and FEV(0.5), using both techniques identified abnormalities in 35% (24/68). Hyperinflation (FRC(pleth) >1.96 z-scores) was identified in 18% (10/56) of infants with CF and was significantly correlated with diminished FEF(25-75) (r=-0.43, p<0.001) but not with LCI or FEV(0.5). CONCLUSION: Despite early diagnosis of CF by NBS and protocol-driven treatment in specialist centres, abnormal lung function, with increased ventilation inhomogeneity and hyperinflation and diminished airway function, is evident in many infants with CF diagnosed through NBS by 3 months of age.
Thorax 06/2012; 67(10):874-881. · 6.84 Impact Factor
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ABSTRACT: Aortopexy is the treatment of choice for clinically significant tracheobronchomalacia from external vascular compression. When a marked chest depression is present, aortopexy may be less effective. We report 2 patients with pectus excavatum and vascular compression of the trachea who, despite their young age, benefited from combined Nuss bar insertion and aortopexy.
The Annals of thoracic surgery 01/2011; 91(1):e8-9. · 3.74 Impact Factor
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ABSTRACT: A 2-year-old boy with type 3 Gaucher disease (GD) on treatment with enzyme replacement therapy (ERT) was found dead in bed having been apparently well the night before. At the time of diagnosis, he had significant respiratory symptoms (severe and persistent bouts of coughing) that had been attributed to Gaucher lung infiltration and that were controlled by inhaled and orally administered steroids. These symptoms had begun to reappear just prior to death. Postmortem revealed extensive pulmonary hemorrhage and intra-alveolar collections of Gaucher cells. There was very little evidence of GD elsewhere. Death was ascribed to pulmonary hemorrhage secondary to GD. The pathogenesis was unclear. To the best of our knowledge, this is the first case of isolated pulmonary hemorrhage secondary to GD and may represent a hitherto unrecognized complication of this condition. Given the apparent temporal relationship, we propose that it represented a severe, terminal event in the course of Gaucher lung disease.
Journal of Inherited Metabolic Disease 08/2010; · 3.58 Impact Factor
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ABSTRACT: Osmotic agents, such as inhaled dry powder mannitol, may increase mucociliary clearance by rehydrating the airway surface liquid and thus act as disease-modifying treatments in cystic fibrosis (CF). This is the first therapeutic trial of inhaled mannitol in children with CF; it was compared with recombinant human deoxyribonuclease (rhDNase), the current best established mucolytic treatment.
38 children were recruited to an open crossover study. Subjects underwent an initial bronchial provocation challenge with dry powder mannitol. Those children with a negative challenge were randomly allocated to one of three consecutive 12-week treatment blocks (inhaled mannitol alone, nebulised rhDNase alone and mannitol + rhDNase). The primary outcome was forced expiratory volume in 1 s (FEV(1)). A number of secondary outcome measures were also studied.
Twenty children completed the study. Bronchoconstriction and cough associated with mannitol administration contributed to the high attrition rate. The mean increase in FEV(1) following 12 weeks of treatment was 0.11 litres (6.7%) (p = 0.055) for mannitol alone, 0.12 litres (7.2%) (p = 0.03) for rhDNase alone and 0.03 litres (1.88%) (p = 0.67) for rhDNase and mannitol. None of the secondary clinical outcomes was statistically significantly different between treatments.
Inhaled mannitol was at least as effective as rhDNase after 3 months treatment. There was a marked individual variation in tolerance to mannitol and in response to treatment however. Children who do not respond to rhDNase many benefit from a trial of inhaled mannitol. The combination of mannitol and rhDNase was not useful.
Thorax 12/2009; 65(1):51-6. · 6.84 Impact Factor
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Victoria H Castleman,
Leila Romio,
Rahul Chodhari,
Robert A Hirst,
Sandra C P de Castro,
Keith A Parker,
Patricia Ybot-Gonzalez,
Richard D Emes,
Stephen W Wilson, Colin Wallis, [......],
Amelia Shoemark,
Andrew Bush,
Claire Hogg,
R Mark Gardiner,
Orit Reish,
Nicholas D E Greene,
Christopher O'Callaghan,
Saul Purton,
Eddie M K Chung,
Hannah M Mitchison
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ABSTRACT: Primary ciliary dyskinesia (PCD) is a genetically heterogeneous inherited disorder arising from dysmotility of motile cilia and sperm. This is associated with a variety of ultrastructural defects of the cilia and sperm axoneme that affect movement, leading to clinical consequences on respiratory-tract mucociliary clearance and lung function, fertility, and left-right body-axis determination. We performed whole-genome SNP-based linkage analysis in seven consanguineous families with PCD and central-microtubular-pair abnormalities. This identified two loci, in two families with intermittent absence of the central-pair structure (chromosome 6p21.1, Zmax 6.7) and in five families with complete absence of the central pair (chromosome 6q22.1, Zmax 7.0). Mutations were subsequently identified in two positional candidate genes, RSPH9 on chromosome 6p21.1 and RSPH4A on chromosome 6q22.1. Haplotype analysis identified a common ancestral founder effect RSPH4A mutation present in UK-Pakistani pedigrees. Both RSPH9 and RSPH4A encode protein components of the axonemal radial spoke head. In situ hybridization of murine Rsph9 shows gene expression restricted to regions containing motile cilia. Investigation of the effect of knockdown or mutations of RSPH9 orthologs in zebrafish and Chlamydomonas indicate that radial spoke head proteins are important in maintaining normal movement in motile, "9+2"-structure cilia and flagella. This effect is rescued by reintroduction of gene expression for restoration of a normal beat pattern in zebrafish. Disturbance in function of these genes was not associated with defects in left-right axis determination in humans or zebrafish.
The American Journal of Human Genetics 03/2009; 84(2):197-209. · 10.60 Impact Factor
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ABSTRACT: There has been recent interest in dry powder inhaled mannitol as a therapeutic agent in patients with cystic fibrosis (CF). It is has been shown to increase mucociliary clearance (MCC) by rehydrating the airway. To date there have been no studies exclusively in children with CF examining the effect of dry powder mannitol on the airways. The aim of this study was to determine acute tolerability of inhaled mannitol in children with CF.
Thirty-nine children (aged 8-18 years) with CF underwent a bronchial provocation challenge with incrementally increasing doses of dry powder mannitol (up to a maximum cumulative dose of 475 mg). A positive challenge was defined as a drop in FEV(1) of >or=15% from baseline.
Nine out of 38 subjects (24%; 95% confidence interval 10-38%) had a positive challenge. Only two of these nine subjects had a PD(15) (dose of mannitol required to cause a 15% reduction in FEV(1)) prior to the 315 mg dose, the remaining seven children dropping their FEV(1) by >or=15% on the 315 or 475 mg (final) cumulative dose. We found no association between patient characteristics and a positive challenge. Although cough was common during the challenge, other adverse events were infrequent.
We report that 24% of children with CF had a positive airway challenge with inhaled mannitol. This compares with a previously reported 12% of subjects in a study including both adults and children. We could not identify factors predictive of a positive mannitol challenge in our children.
Pediatric Pulmonology 11/2008; 43(11):1078-84. · 2.53 Impact Factor
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Wanda J Kozlowska,
Andrew Bush,
Angela Wade,
Paul Aurora,
Siobhán B Carr,
Rosie A Castle,
Ah-Fong Hoo,
Sooky Lum,
John Price,
Sarath Ranganathan,
Clare Saunders,
Sanja Stanojevic,
John Stroobant, Colin Wallis,
Janet Stocks
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ABSTRACT: After recent standardization of forced expiratory maneuvers for both infants and preschool children, longitudinal measurements are now possible from birth.
The aim of this study was to investigate the evolution of lung function during the first 6 years of life after a clinical diagnosis of cystic fibrosis (CF) in infancy in children with CF and in healthy control subjects.
The raised volume technique was used during infancy and incentive spirometry during the preschool years.
Forty-eight children with CF and 33 healthy control subjects had up to seven (median, 3) measurements. Over these early years, the diagnosis of CF itself accounted for a significant mean reduction of 7.5% (95% confidence interval, 0.9 - 13.6%) in FEV(0.75) and 15.1% (95% confidence interval, 3.6 - 25.3%) in FEF(25-75). Wheeze on auscultation, recent cough, and Pseudomonas aeruginosa (PsA) infection (even if apparently effectively treated) were all independently associated with further reductions in lung function. Premorbid lung function did not predict infection with PsA.
This is the first study to describe physiologic measurements from infancy through the preschool years in subjects with CF and healthy control subjects, the understanding of which is critical for future intervention trials. Airflow obstruction in uncomplicated CF persists through the preschool years despite treatment, with PsA acquisition being associated with further deterioration in lung function, even when apparently eradicated. This suggests that new therapies are needed to treat the airflow obstruction of uncomplicated CF, and rigorous strategies to prevent PsA acquisition.
American Journal of Respiratory and Critical Care Medicine 08/2008; 178(1):42-9. · 11.08 Impact Factor
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ABSTRACT: The current practice of slide tracheoplasty for children with long-segment tracheal stenosis is reviewed.
In our own series, the mortality for children with long-segment tracheal stenosis managed by slide tracheoplasty in the 5-year period between 1995 and 2000 was 43% (3/7), consistent with other series in the literature. In 2001, we developed a multidisciplinary approach with aggressive postoperative surveillance, intermittent balloon dilatation and selective stenting for salvage. Mortality fell to 11% over the next 5 years (2/18). Since 2005, 25 cases have been treated and hospital mortality has been eliminated (0%, 0/25). There has been one late death due to renal disease. Quality of life for survivors is good.
The outlook has changed substantially in recent years for children with long-segment tracheal stenosis. Previous operations have been superseded by the advent of slide tracheoplasty in combination with a multidisciplinary approach with balloon dilatation and, occasionally, stenting in cases of recurrent stenosis. Survival rates have risen dramatically.
Current opinion in otolaryngology & head and neck surgery 03/2008; 16(1):75-82.
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Sooky Lum,
Per Gustafsson,
Henrik Ljungberg,
Georg Hülskamp,
Andrew Bush,
Siobhán B Carr,
Rosemary Castle,
Ah-Fong Hoo,
John Price,
Sarath Ranganathan,
John Stroobant,
Angie Wade, Colin Wallis,
Hilary Wyatt,
Janet Stocks
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ABSTRACT: Lung clearance index (LCI), a measure of ventilation inhomogeneity derived from the multiple-breath inert gas washout (MBW) technique, has been shown to detect abnormal lung function more readily than spirometry in preschool children with cystic fibrosis, but whether this holds true during infancy is unknown.
To compare the extent to which parameters derived from the MBW and the raised lung volume rapid thoraco-abdominal compression (RVRTC) techniques identify diminished airway function in infants with cystic fibrosis when compared with healthy controls.
Measurements were performed during quiet sleep, with the tidal breathing MBW technique being performed before the forced expiratory manoeuvres.
Measurements were obtained in 39 infants with cystic fibrosis (mean (SD) age 41.4 (22.0) weeks) and 21 controls (37.0 (15.1) weeks). Infants with cystic fibrosis had a significantly higher respiratory rate (38 (10) vs 32 (5) bpm) and LCI (8.4 (1.5) vs 7.2 (0.3)), and significantly lower values for all forced expiratory flow-volume parameters compared with controls. Girls with cystic fibrosis had significantly lower forced expiratory volume (FEV(0.5) and FEF(25-75 )) than boys (mean (95% CI girls-boys): -1.2 (-2.1 to -0.3) for FEV(0.5) Z score; FEF(25-75): -1.2 (-2.2 to -0.15)). When using both the MBW and RVRTC techniques, abnormalities were detected in 72% of the infants with cystic fibrosis, with abnormalities detected in 41% using both techniques and a further 15% by each of the two tests performed.
These findings support the view that inflammatory and/or structural changes in the airways of children with cystic fibrosis start early in life, and have important implications regarding early detection and interventions. Monitoring of early lung disease and functional status in infants and young children with cystic fibrosis may be enhanced by using both MBW and the RVRTC.
Thorax 05/2007; 62(4):341-7. · 6.84 Impact Factor
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ABSTRACT: Forced expiratory volume in 1s (FEV(1)) is the usual primary outcome variable in clinical trials in cystic fibrosis (CF). Usually, several secondary outcomes are also measured. We assessed which secondary outcomes are likely to give additional clinically useful information about treatment effects, in order to inform the design of future studies. The study was performed as part of a trial comparing daily rhDNase with alternate day rhDNase and hypertonic saline in CF. The primary outcome was FEV(1). Secondary outcomes were forced vital capacity (FVC), forced expiratory flow at 25-75% of forced vital capacity (FEF(25-75)), number of pulmonary exacerbations, weight gain, quality of life (QOL), and exercise tolerance. The usefulness of each secondary outcome was investigated by assessing if the change in that outcome over the treatment period could be predicted from the primary outcome. Change in FEV(1) correlated with changes in FVC (r(2)=0.76, P=0.001), FEF(25-75) (r(2)=0.64, P=0.001), weight (r(2)=0.08, P=0.001), and change in oxygen saturation with exercise (r(2)=0.08, P=0.001). However, it did not correlate with changes in visual analogue score (VAS) with exercise, QOL, nor with the occurrence of pulmonary exacerbations. Only the outcomes QOL and VAS with exercise actually provided additional information to FEV(1) in this study.
Respiratory Medicine 03/2007; 101(2):254-60. · 2.47 Impact Factor
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ABSTRACT: A 6-year-old Asian girl was diagnosed with cystic fibrosis at 3 months of age, following investigations for failure to thrive. She had intrauterine growth retardation and continued to have restricted postnatal growth, despite adequate caloric intake and enzyme replacement therapy. Further investigations were initiated when she was 5 years old, as her growth was not responding to the usual treatment measures. These tests revealed that she had maternal isodisomy of chromosome 7.
Pediatric Pulmonology 09/2005; 40(2):166-8. · 2.53 Impact Factor
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Developmental Medicine & Child Neurology 06/2005; 47(5):347-52. · 2.92 Impact Factor
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ABSTRACT: Sensitive measures of lung function applicable to young subjects are needed to detect early cystic fibrosis (CF) lung disease. Forty children with CF aged 2 to 5 years and 37 age-matched healthy control subjects performed multiple-breath inert gas washout, plethysmography, and spirometry. Thirty children in each group successfully completed all measures, with success on first visit being between 68 and 86% for all three measures. Children with CF had significantly higher lung clearance index (mean [95% CI] difference for CF control 2.7 [1.9, 3.6], p < 0.001) and specific airway resistance (1.65 z-scores [0.96, 2.33], p < 0.001), and significantly lower forced expired volume in 0.5 seconds (-0.49 z-scores [-0.95, -0.03], p < 0.05). Abnormal lung function results were identified in 22 (73%) of 30 children with CF by multiple-breath washout, compared with 14 (47%) of 30 by plethysmography, and 4 (13%) of 30 by spirometry. Children with CF who were infected with Pseudomonas aeruginosa had significantly higher lung clearance index, but no significant difference in other lung function measures, when compared with noninfected children. Most preschool children can perform multiple-breath washout, plethysmography, and spirometry at first attempt. Multiple-breath washout detects abnormal lung function in children with CF more readily than plethysmography or spirometry.
American Journal of Respiratory and Critical Care Medicine 03/2005; 171(3):249-56. · 11.08 Impact Factor
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Ergin Kocyildirim,
Mazyar Kanani,
Derek Roebuck, Colin Wallis,
Clare McLaren,
Clair Noctor,
Nick Pigott,
Quen Mok,
Ben Hartley,
Catherine Dunne,
Savjeet Uppal,
Martin J Elliott
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ABSTRACT: Long-segment tracheal stenosis is rare, life-threatening, difficult, and expensive to treat. Management remains controversial. A multidisciplinary tracheal team was formed in 2000 to deal with a large number of children with airway problems referred for management. We review the effect of that service, comparing the era before and after the establishment of the multidisciplinary tracheal team.
From January 1998 through January 2004, 34 patients with long-segment tracheal stenosis (21 patients with cardiovascular anomalies) underwent surgical intervention. Cardiopulmonary bypass was used in all operations. Before the multidisciplinary tracheal team, pericardial patch tracheoplasty with or without an autograft technique was the preferred method of repair. After the multidisciplinary tracheal team, an integrated care plan preferring slide tracheoplasty was initiated, correcting cardiac lesions simultaneously.
Before the establishment of the multidisciplinary tracheal team, pericardial patch tracheoplasty was performed in 15 of 19 patients. Twelve patients had a suspended pericardial patch tracheoplasty, 2 (17%) of whom died late after the operation. Of 3 patients who had had a simple unsuspended patch, 2 (67%) died early after the operation. Four patients were operated on with the tracheal autograft technique, 2 (50%) dying early in the postoperative period. After multidisciplinary tracheal team formation, in the era between 2001 and 2004, 15 patients were operated on with slide tracheoplasty, and there were 2 (13%) early postoperative deaths. A significant reduction in cost and duration of stay has been shown both in the intensive care unit and the hospital.
Our data suggest that a formalized multidisciplinary team approach and a policy of primary slide tracheoplasty are beneficial in the management of children with long-segment tracheal stenosis.
Journal of Thoracic and Cardiovascular Surgery 01/2005; 128(6):876-82. · 3.41 Impact Factor
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Sarath C Ranganathan,
Janet Stocks,
Carol Dezateux,
Andrew Bush,
Angie Wade,
Siobhán Carr,
Rosemary Castle,
Robert Dinwiddie,
Ah-Fong Hoo,
Sooky Lum,
John Price,
John Stroobant, Colin Wallis
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ABSTRACT: This study aimed to investigate the evolution of airway function in infants newly diagnosed with cystic fibrosis (CF). FEV(0.5) was measured soon after diagnosis (median age of 28 weeks) and 6 months later in subjects with CF and on two occasions 6 months apart (median ages of 7.4 and 33.7 weeks) in healthy infants, using the raised-volume technique. Repeated measurements were successful in 34 CF and 32 healthy subjects. After adjustment for age, length, sex, and exposure to maternal smoking, mean FEV(0.5) was significantly lower in infants with CF both shortly after diagnosis and at the second test, with no significant difference in rate of increase in FEV(0.5) with growth between the two groups. When compared with published reference data, FEV(0.5) was reduced by an average of two z scores on both test occasions in those with CF, with 72% of individuals having an FEV(0.5) of less than 1.64 z-scores (i.e., less than the fifth percentile) on one or both test occasions. On longitudinal analysis, subjects with CF experienced a mean (95% confidence interval) reduction in FEV(0.5) of 20% (11, 28). Airway function is diminished soon after diagnosis in infants with CF and does not catch up during infancy and early childhood. These findings have important implications for early interventions in CF.
American Journal of Respiratory and Critical Care Medicine 05/2004; 169(8):928-33. · 11.08 Impact Factor
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ABSTRACT: Daily recombinant human deoxyribonuclease (rhDNase) is an established but expensive treatment in cystic fibrosis (CF). Alternate-day rhDNase and hypertonic saline (HS) represent potential cheaper alternative therapies. However, not all patients improve on treatment. To assess response, many CF centers have developed formal n-of-1 trials of treatment to find out who benefits. Response to daily rhDNase at 3 months has been shown to be a good predictor of response at 1 year. There are no data correlating individual response at a shorter time period with 3-month response. We assessed whether individual responses to daily rhDNase, alternate-day rhDNase, and HS could be predicted from lung function response at 6 weeks, thus shortening the n-of-1 trial, or from baseline patient characteristics, therefore avoiding the need for an n-of-1 trial. In a randomized crossover trial, 48 CF children were allocated consecutively to 12 weeks of once-daily 2.5-mg rhDNase, alternate-day 2.5-mg rhDNase, and twice-daily 5 ml of 7% HS. Forced expiratory volume in 1 sec (FEV1) and forced vital capacity (FVC) were measured at baseline and then at 6 and 12 weeks into each treatment period. Lung function response to the drugs at 6 weeks was highly predictive of response at 3 months. There was some evidence that response to HS was worse in patients with lower baseline lung function. However, there was no association between response to alternate-day or daily rhDNase and baseline characteristics. In conclusion, response to rhDNase and HS at 6 weeks was highly predictive of response at 3 months. For daily and alternate-day rhDNase, at least, the drug needs to be administered for at most 6 weeks initially to assess long-term response to treatment. Response to treatment could not be reliably predicted from baseline characteristics.
Pediatric Pulmonology 05/2004; 37(4):305-10. · 2.53 Impact Factor
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ABSTRACT: Primary ciliary dyskinesia is an autosomal recessive disorder characterized by chronic upper and lower respiratory tract symptoms. We report the diagnosis of primary ciliary dyskinesia associated with a circular ciliary beat pattern in three siblings. This beat pattern is consistent with a ciliary transposition defect, where a peripheral microtubule doublet is transposed to the center of the ciliary axoneme to replace the absent central microtubule pair. However, in these siblings, ultrastructural analysis of the cilia revealed an absence of the central microtubule pair only. This variant of transposition with a circular ciliary beat pattern has not been described previously. In addition, this defect, together with the transposition defect, may help explain the mechanism of the circular beat pattern and also the absence of situs inversus in these patients.
American Journal of Respiratory and Critical Care Medicine 04/2004; 169(5):634-7. · 11.08 Impact Factor
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Martin Elliott,
Derek Roebuck,
Clair Noctor,
Clare McLaren,
Ben Hartley,
Quen Mok,
Catherine Dunne,
Nick Pigott,
Chirag Patel,
Alpesh Patel, Colin Wallis
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ABSTRACT: This paper reviews current concepts and results in the management of congenital tracheal stenosis (CTS). Diagnostic options are considered and the requirements for successful management defined. Chief amongst these is a multi-disciplinary approach with individualised patient management. Severe long-segment CTS represents the biggest challenge to clinicians and the worst problems for affected families. Near-death episodes are frequent in affected infants and some cannot be ventilated and require ECMO. Associated cardiovascular anomalies are frequent. Patients require immediate resuscitation and transfer to a specialist unit. After careful assessment, accurate diagnosis and discussion, primary resection and end-to-end repair with a slide technique should always be the first option, with concomitant repair of associated cardiac anomalies. If this is impossible because of the severity of the lesion, some form of patch tracheoplasty will be indicated. Cardiopulmonary bypass is often required. Patches include pericardium, autograft trachea, carotid artery, cartilage, and allograft trachea. Mortality ranges from 0 to 30% in the literature, which largely comprises single-centre long-term experience. Recurrence is common and can be managed by stenting and tracheal homograft implantation. Long-term quality of life of survivors is little reported but seems good. Physiological data are lacking. To improve results, we suggest a treatment algorithm to rationalise care.
International Journal of Pediatric Otorhinolaryngology 01/2004; 67 Suppl 1:S183-92. · 1.17 Impact Factor
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American Journal of Respiratory and Critical Care Medicine 07/2003; 167(11):1577; author reply 1577-8. · 11.08 Impact Factor
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ABSTRACT: We assessed the safety and use of induced sputum (IS) in children with cystic fibrosis (CF). Forty-eight children (19 males) with CF, mean age 12.6 (range, 7.3-17.0) years and median forced expired volume in 1 sec (FEV(1)) 48% (range, 14-77%) predicted were recruited. Patients spontaneously expectorated sputum and then performed sputum induction by inhalation of nebulized 7% hypertonic saline. Samples were sent for bacteriological culture, and for measurement of the following inflammatory mediators: interleukin-8, myeloperoxidase, eosinophil cationic protein, and neutrophil elastase activity. FEV(1) was performed before and after inhalation of hypertonic saline. There was no increase in mediator levels in IS compared to expectorated sputum (ES) samples. Only 3 patients demonstrated significant bronchoconstriction following inhalation of hypertonic saline, by the method used. From the ES samples, Pseudomonas aeruginosa was isolated in 13 patients, Staphylococcus aureus in 7 patients, Stenotrophomonas maltophilia in 1 patient, and both Pseudomonas aeruginosa and Staphylococcus aureus in 5 patients. All these organisms were found in the IS samples. However, in 2 patients whose ES grew no organisms, one patient's IS grew Pseudomonas aeruginosa, and the other patient's IS grew Staphylococcus aureus. In our study, sputum induction was safe, with no proinflammatory effect.
Pediatric Pulmonology 05/2003; 35(4):309-13. · 2.53 Impact Factor
