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Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
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<a href="http://doi.org/10.1002/ppul.21291" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/ppul.21291</a>
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Title
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The feasibility and validity of forced spirometry in ataxia telangiectasia
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Pediatric Pulmonology
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2010
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Adolescent; Ataxia Telangiectasia/di [Diagnosis]; Ataxia Telangiectasia/pp [Physiopathology]; Case-Control Studies; Child; Preschool; Feasibility Studies; Female; Humans; Male; Reproducibility of Results; Spirometry; Young Adult; breathing difficulties; Ataxia Telangiectasia; trajectory; characteristics; lung deterioration
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Vilozni D; Berkun Y; Levi Y; Weiss B; Jacobson J M; Efrati O
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OBJECTIVES: To explore the feasibility and validity of forced spirometry in patients with ataxia telangiectasia (A-T). STUDY DESIGN: Twenty-eight patients (aged 3.7-19.3 years) performed spirometry on 47 occasions. Parameters studied were technical quality and relation to: predicted values, pulmonary illness. RESULTS: Start of test criteria for correct expiratory effort was significantly prolonged (183 +/- 115 ms; P < 0.001). The rise-time to peak flow in children free of respiratory symptoms (Group-FRS; n = 8) increased by 16.2 +/- 12.5 ms/year above recommended and in children having recurrent infections (n = 8) 30.4 +/- 16.1 ms/year, P < 0.01. Expiration-time was significantly shorter than requested (1.21 +/- 0.47 sec) and was ended abruptly in 57% of the patients. FEV(1) could not be established by 8/20 patients. The intra-subject reproducibility met criteria (4.4 +/- 2.7%, 5.2 +/- 2.8%, 2.9 +/- 3.2%, 6.3 +/- 5.3%, for FVC, FEV(0.5), PEF, FEF(25-75), respectively). Group-FRS showed yearly deterioration in FVC of 2.2%, while patients with hyper-reactive airways (Group-HRA; n =12) had a deterioration rate of 3.6%/year. FEV(0.5) deterioration rate was similar in both groups (2.2 and 2.0, respectively), but baseline values in Group-HRA were significantly lower than those of Group-FRS (P = 0.029) in similar young ages, indicating airway obstruction at early ages in Group-HRA. FEV(0.5) values deterioration also correlated with body mass index (P< 0.017). CONCLUSION: Forced spirometry in A-T patients is reproducible and has a distinct pattern, although curves do not meet other recommendations for acceptable criteria. The study insinuates that a rapid deterioration in lung function occurs in A-T patients with recurrent respiratory infection, suggesting that early intervention may prevent further deterioration or improve their lung function. Further studies are needed to confirm our results.Copyright © 2010 Wiley-Liss, Inc.
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<a href="http://doi.org/10.1002/ppul.21291" target="_blank" rel="noreferrer noopener">10.1002/ppul.21291</a>
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Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2010
Adolescent
ataxia telangiectasia
ataxia telangiectasia/di [Diagnosis]
Ataxia Telangiectasia/pp [Physiopathology]
Berkun Y
breathing difficulties
Case-Control Studies
characteristics
Child
Efrati O
Feasibility Studies
Female
Humans
Jacobson J M
Levi Y
lung deterioration
Male
Pediatric Pulmonology
Preschool
Reproducibility of Results
Spirometry
Trajectory
Vilozni D
Weiss B
Young Adult