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Diagnosis was based on clinical history and thoracic computed tomography &#40;CT&#41; findings&#46; All patients followed for two consecutive years &#40;2018-19&#41; were included&#46; Children with otherwise etiological diagnosis like CF or PCD were excluded&#46; The frequency &#40;average per year&#41; of respiratory physician visits&#44; physiotherapy prescription &#40;including education in the management of airway clearance&#41;&#44; number of spirometric evaluations&#44; anthropometric assessments and number of microbiological samples taken for bacterial culture were registered&#46;</p><p id="para0006" class="elsevierStylePara elsevierViewall">At our centre&#44; spirometry is performed in cooperative patients older than 4 years of age in accordance with ERS&#47;ATS guidelines&#46; Analysis of height&#44; weight&#44; and BMI z&#8208;scores &#40;standard scores&#41; taken from the spirometry records and standardized using WHO reference values was completed&#46; The best FEV<span class="elsevierStyleInf">1</span> and FEV<span class="elsevierStyleInf">1</span>&#47;FVC results for the calendar year were collected for all patients who performed good quality tests and averaged for analysis&#46;</p><p id="para0007" class="elsevierStylePara elsevierViewall">Respiratory microbiology surveillance &#40;frequency of testing&#41; was determined from medical record as was referral rates to physiotherapy services&#46; The number of samples &#40;sputum or cough swabs&#41; analysed in a calendar year was counted and averaged&#46;</p><p id="para0008" class="elsevierStylePara elsevierViewall">A descriptive analysis for continuous variables was done and described as median &#40;min and max&#41;&#46; Linear regression analysis between average number of physician visits per year and FEV<span class="elsevierStyleInf">1</span> z-score was performed&#46;</p><p id="para0009" class="elsevierStylePara elsevierViewall">During the study period&#44; 28 children were observed &#40;<a class="elsevierStyleCrossRef" href="#tbl0001">Table 1</a>&#41;&#46; Only four had respiratory samples collected &#40;all with bronchiectasis&#41;&#46; During this period&#44; three non-residents in Portugal were observed&#44; seven patients initiated follow-up&#44; four were transferred and three lost to follow-up&#46;</p><elsevierMultimedia ident="tbl0001"></elsevierMultimedia><p id="para0010" class="elsevierStylePara elsevierViewall">Fourteen &#40;50&#37;&#41; had been prescribed mucus clearance devices or educated on respiratory clearance manoeuvres&#46; Three patients with PIBO &#40;plus bronchiectasis in two&#41; are currently on long term oxygen &#40;FEV<span class="elsevierStyleInf">1</span> z-score ranged from -5&#46;0 and -5&#46;7&#41;&#46;</p><p id="para0011" class="elsevierStylePara elsevierViewall">No association between clinic visits&#44; clearance methods or other follow-up measures described and FEV<span class="elsevierStyleInf">1</span> z-score was found&#46;</p><p id="para0012" class="elsevierStylePara elsevierViewall">This study shows that patients with PIBO and&#47;or bronchiectasis had an average of 2&#46;7 physician appointments and performed 2&#47;1&#46;5 spirometries per year&#46; Respiratory microbiology surveillance was low and only half had some record for respiratory rehabilitation or use of mucus clearance devices&#46;</p><p id="para0013" class="elsevierStylePara elsevierViewall">In this sample&#44; lung function alone did not affect follow-up&#46; However&#44; the lack of association may be due to small sample size&#44; presence of outliers and shared follow-up with local hospitals&#46;</p><p id="para0014" class="elsevierStylePara elsevierViewall">Despite the limitations and biases of our retrospective analysis&#44; we can assume that the management plan of these children is heterogeneous&#44; with regular physician appointments and spirometry&#44; but low respiratory microbiology surveillance and no standard physiotherapy or nutritional consultations&#46;</p><p id="para0015" class="elsevierStylePara elsevierViewall">In our setting&#44; patients with PCD&#44; PIBO and&#47;or bronchiectasis are managed by respiratory physicians in a general respiratory clinic without a formal multidisciplinary team&#46; Allied health services are available on request and require a separate appointment&#46; There are no internal or national guidelines for the management of these conditions concerning frequency of visits&#44; lung function testing&#44; collection of respiratory samples for bacterial culture&#44; nutritional assessment&#44; timing of imaging by CT or referral to respiratory physiotherapists&#46; By comparison&#44; at the same centre&#44; patients with CF are seen at least every three months by a multidisciplinary team according to international recommendations&#46;</p><p id="para0016" class="elsevierStylePara elsevierViewall">The clinical course of children with PIBO and&#47;or bronchiectasis can be less predictable and differs from CF and PCD&#46; While some children have serious structural and functional lung disease&#44; others experience more subtle effects&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">5-7</span></a> These heterogeneous characteristics are due to aetiology and age at diagnosis&#47;referral to tertiary centres&#46; Furthermore&#44; some of these children tend to stabilize or even improve their overall status&#44; including lung function results&#44; over time&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">5</span></a><span class="elsevierStyleSup">&#44;</span><a class="elsevierStyleCrossRef" href="#bib0006"><span class="elsevierStyleSup">6</span></a></p><p id="para0017" class="elsevierStylePara elsevierViewall">For adult patients with Chronic Obstructive Pulmonary Disease&#44; routine follow-up is essential&#46; It focuses on symptoms&#44; exacerbations&#44; objective measures of airflow limitations and identifying complications and&#47;or comorbidities&#46;<a class="elsevierStyleCrossRef" href="#bib0008"><span class="elsevierStyleSup">8</span></a> However&#44; for children it can be on reversing the disease when possible and halting its progress&#46;<a class="elsevierStyleCrossRef" href="#bib0003"><span class="elsevierStyleSup">3</span></a> Furthermore&#44; integrated care needs to be individualized to the developmental stage of the child and the family&#39;s health literacy&#46;</p><p id="para0018" class="elsevierStylePara elsevierViewall">Extrapolating from standards of care for other complex chronic lung diseases could preserve lung function&#44; reduce exacerbations&#44; improve quality of life&#44; prevent nutritional decline&#44; and enhance survival for patients with PIBO and&#47;or bronchiectasis&#46; Since these are rare entities&#44; there is little evidence to advise on their management&#46; Future directions should dictate more precise standards of care tailored by severity&#44; rate of exacerbations&#44; decline of lung function and minimal follow-up requirements&#46;</p></span>"
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Letter to the Editor
Should there be a tailored guided management plan for children with post-infectious bronchiolitis obliterans and bronchiectasis?
C. Constanta,b,c,*, L. Pereiraa,c, A. Saiandaa, R. Ferreiraa,c, A.M. Silvab, A. Descalçob, L. Lobod, T. Bandeiraa,b,c
a Paediatric Respiratory Unit, Department of Paediatrics, Hospital de Santa Maria, CHULN, Lisbon, Portugal
b Paediatric Lung Function Laboratory, Department of Paediatrics, Hospital de Santa Maria, CHULN, Lisbon, Portugal
c Lisbon Academic Medical Centre, University of Lisbon, Lisbon, Portugal
d General Radiology Service, Hospital de Santa Maria, CHULN, Lisbon, Portugal
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="para0002" class="elsevierStylePara elsevierViewall">Current guidelines for chronic lung diseases such as cystic fibrosis &#40;CF&#41;&#44; Primary Ciliary Dyskinesia &#40;PCD&#41; and Bronchiectasis in children recommend regular and multidisciplinary monitoring to delay long&#8208;term pulmonary complications&#46;<a class="elsevierStyleCrossRefs" href="#bib0001"><span class="elsevierStyleSup">1-3</span></a></p><p id="para0003" class="elsevierStylePara elsevierViewall">Post-infectious Bronchiolitis Obliterans &#40;PIBO&#41; with or without Bronchiectasis is heterogeneous with diverse clinical expression and severity&#44;<a class="elsevierStyleCrossRefs" href="#bib0004"><span class="elsevierStyleSup">4-6</span></a> which contributes to the lack of specific guidelines for monitorization of disease progression and precludes the design of management plans after diagnosis is established&#46;</p><p id="para0004" class="elsevierStylePara elsevierViewall">We aimed to describe the management plans of children with PIBO and&#47;or Bronchiectasis in a tertiary care hospital and analyse whether the variability of care depends on the severity of the obstructive ventilatory defect as determined by FEV<span class="elsevierStyleInf">1</span>&#46;</p><p id="para0005" class="elsevierStylePara elsevierViewall">A retrospective chart review of children with PIBO and&#47;or bronchiectasis followed in a tertiary care paediatric hospital was undertaken&#46; Diagnosis was based on clinical history and thoracic computed tomography &#40;CT&#41; findings&#46; All patients followed for two consecutive years &#40;2018-19&#41; were included&#46; Children with otherwise etiological diagnosis like CF or PCD were excluded&#46; The frequency &#40;average per year&#41; of respiratory physician visits&#44; physiotherapy prescription &#40;including education in the management of airway clearance&#41;&#44; number of spirometric evaluations&#44; anthropometric assessments and number of microbiological samples taken for bacterial culture were registered&#46;</p><p id="para0006" class="elsevierStylePara elsevierViewall">At our centre&#44; spirometry is performed in cooperative patients older than 4 years of age in accordance with ERS&#47;ATS guidelines&#46; Analysis of height&#44; weight&#44; and BMI z&#8208;scores &#40;standard scores&#41; taken from the spirometry records and standardized using WHO reference values was completed&#46; The best FEV<span class="elsevierStyleInf">1</span> and FEV<span class="elsevierStyleInf">1</span>&#47;FVC results for the calendar year were collected for all patients who performed good quality tests and averaged for analysis&#46;</p><p id="para0007" class="elsevierStylePara elsevierViewall">Respiratory microbiology surveillance &#40;frequency of testing&#41; was determined from medical record as was referral rates to physiotherapy services&#46; The number of samples &#40;sputum or cough swabs&#41; analysed in a calendar year was counted and averaged&#46;</p><p id="para0008" class="elsevierStylePara elsevierViewall">A descriptive analysis for continuous variables was done and described as median &#40;min and max&#41;&#46; Linear regression analysis between average number of physician visits per year and FEV<span class="elsevierStyleInf">1</span> z-score was performed&#46;</p><p id="para0009" class="elsevierStylePara elsevierViewall">During the study period&#44; 28 children were observed &#40;<a class="elsevierStyleCrossRef" href="#tbl0001">Table 1</a>&#41;&#46; Only four had respiratory samples collected &#40;all with bronchiectasis&#41;&#46; During this period&#44; three non-residents in Portugal were observed&#44; seven patients initiated follow-up&#44; four were transferred and three lost to follow-up&#46;</p><elsevierMultimedia ident="tbl0001"></elsevierMultimedia><p id="para0010" class="elsevierStylePara elsevierViewall">Fourteen &#40;50&#37;&#41; had been prescribed mucus clearance devices or educated on respiratory clearance manoeuvres&#46; Three patients with PIBO &#40;plus bronchiectasis in two&#41; are currently on long term oxygen &#40;FEV<span class="elsevierStyleInf">1</span> z-score ranged from -5&#46;0 and -5&#46;7&#41;&#46;</p><p id="para0011" class="elsevierStylePara elsevierViewall">No association between clinic visits&#44; clearance methods or other follow-up measures described and FEV<span class="elsevierStyleInf">1</span> z-score was found&#46;</p><p id="para0012" class="elsevierStylePara elsevierViewall">This study shows that patients with PIBO and&#47;or bronchiectasis had an average of 2&#46;7 physician appointments and performed 2&#47;1&#46;5 spirometries per year&#46; Respiratory microbiology surveillance was low and only half had some record for respiratory rehabilitation or use of mucus clearance devices&#46;</p><p id="para0013" class="elsevierStylePara elsevierViewall">In this sample&#44; lung function alone did not affect follow-up&#46; However&#44; the lack of association may be due to small sample size&#44; presence of outliers and shared follow-up with local hospitals&#46;</p><p id="para0014" class="elsevierStylePara elsevierViewall">Despite the limitations and biases of our retrospective analysis&#44; we can assume that the management plan of these children is heterogeneous&#44; with regular physician appointments and spirometry&#44; but low respiratory microbiology surveillance and no standard physiotherapy or nutritional consultations&#46;</p><p id="para0015" class="elsevierStylePara elsevierViewall">In our setting&#44; patients with PCD&#44; PIBO and&#47;or bronchiectasis are managed by respiratory physicians in a general respiratory clinic without a formal multidisciplinary team&#46; Allied health services are available on request and require a separate appointment&#46; There are no internal or national guidelines for the management of these conditions concerning frequency of visits&#44; lung function testing&#44; collection of respiratory samples for bacterial culture&#44; nutritional assessment&#44; timing of imaging by CT or referral to respiratory physiotherapists&#46; By comparison&#44; at the same centre&#44; patients with CF are seen at least every three months by a multidisciplinary team according to international recommendations&#46;</p><p id="para0016" class="elsevierStylePara elsevierViewall">The clinical course of children with PIBO and&#47;or bronchiectasis can be less predictable and differs from CF and PCD&#46; While some children have serious structural and functional lung disease&#44; others experience more subtle effects&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">5-7</span></a> These heterogeneous characteristics are due to aetiology and age at diagnosis&#47;referral to tertiary centres&#46; Furthermore&#44; some of these children tend to stabilize or even improve their overall status&#44; including lung function results&#44; over time&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">5</span></a><span class="elsevierStyleSup">&#44;</span><a class="elsevierStyleCrossRef" href="#bib0006"><span class="elsevierStyleSup">6</span></a></p><p id="para0017" class="elsevierStylePara elsevierViewall">For adult patients with Chronic Obstructive Pulmonary Disease&#44; routine follow-up is essential&#46; It focuses on symptoms&#44; exacerbations&#44; objective measures of airflow limitations and identifying complications and&#47;or comorbidities&#46;<a class="elsevierStyleCrossRef" href="#bib0008"><span class="elsevierStyleSup">8</span></a> However&#44; for children it can be on reversing the disease when possible and halting its progress&#46;<a class="elsevierStyleCrossRef" href="#bib0003"><span class="elsevierStyleSup">3</span></a> Furthermore&#44; integrated care needs to be individualized to the developmental stage of the child and the family&#39;s health literacy&#46;</p><p id="para0018" class="elsevierStylePara elsevierViewall">Extrapolating from standards of care for other complex chronic lung diseases could preserve lung function&#44; reduce exacerbations&#44; improve quality of life&#44; prevent nutritional decline&#44; and enhance survival for patients with PIBO and&#47;or bronchiectasis&#46; Since these are rare entities&#44; there is little evidence to advise on their management&#46; Future directions should dictate more precise standards of care tailored by severity&#44; rate of exacerbations&#44; decline of lung function and minimal follow-up requirements&#46;</p></span>"
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Article information
ISSN: 25310437
Original language: English
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Pulmonology

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