Lung function is abnormal in 3-month-old infants with cystic fibrosis diagnosed by newborn screening

Hoo, Ah‐Fong; Thia, Lena P; Bush, Andrew; Chudleigh, Jane; Lum, Sooky; Ahmed, Deeba; Lynn, I B; Carr, S.B.; Chavasse, Richard; Costeloe, Kate; Price, John; Shankar, Anu; Wallis, Colin; Wyatt, Hilary; Wade, Angela; Stocks, Janet

doi:10.1136/thoraxjnl-2012-201747

Cited by 139 publications

(181 citation statements)

References 52 publications

(51 reference statements)

Supporting

Mentioning

147

Contrasting

Unclassified

Order By: Relevance

“…Cross-sectional data in infants and children suggest that those with evidence of bacterial infection are more likely to have abnormal LCI (36,38,40). In a preschool crosssectional study, children who had "ever cultured" Pseudomonas aeruginosa from airway secretions had higher LCI than those who had not (32).…”

Section: Change In Lci With Microbiological Statusmentioning

confidence: 99%

“…In contrast to older groups, at least half of infants with CF have normal LCI (28,(40)(41)(42). Specifically, some infants appear to have normal LCI in the presence of abnormal infant spirometry (measured by the raised volume rapid thoracoabdominal compression technique) (40) or abnormal computed tomography (28).…”

Section: Observational Studies Lci Correlation With Other Pulmonary Pmentioning

confidence: 99%

“…Specifically, some infants appear to have normal LCI in the presence of abnormal infant spirometry (measured by the raised volume rapid thoracoabdominal compression technique) (40) or abnormal computed tomography (28). A multicenter study from London (43), tracking children from preschool years to early school age, demonstrated (1) that LCI is far more likely to be abnormal than spirometry in preschool years, and (2) abnormal LCI in preschool children predicts both abnormal LCI and abnormal spirometry during school-age years.…”

Section: Observational Studies Lci Correlation With Other Pulmonary Pmentioning

confidence: 99%

See 2 more Smart Citations

Multiple-Breath Washout as a Lung Function Test in Cystic Fibrosis. A Cystic Fibrosis Foundation Workshop Report

et al. 2015

View full text Add to dashboard Cite

The lung clearance index (LCI) is a lung function parameter derived from the multiple-breath washout (MBW) test. Although first developed 60 years ago, the technique was not widely used for many years. Recent technological advances in equipment design have produced gains in popularity for this test among cystic fibrosis (CF) researchers and clinicians, particularly for testing preschool-aged children. LCI has been shown to be feasible and sensitive to early CF lung disease in patients of all ages from infancy to adulthood. A workshop was convened in January 2014 by the North American Cystic Fibrosis Foundation to determine the readiness of the LCI for use in multicenter clinical trials as well as clinical care. The workshop concluded that the MBW text is a valuable potential outcome measure for CF clinical trials in preschool-aged patients and in older patients with FEV 1 in the normal range. However, gaps in knowledge about the choice of device, gas, and standardization across systems are key issues precluding its use as a clinical trial end point in infants. Based on the current evidence, there are insufficient data to support the use of LCI or MBW parameters in the routine clinical management of patients with CF.

show abstract

Section: Change In Lci With Microbiological Statusmentioning

confidence: 99%

Section: Observational Studies Lci Correlation With Other Pulmonary Pmentioning

confidence: 99%

Section: Observational Studies Lci Correlation With Other Pulmonary Pmentioning

confidence: 99%

See 1 more Smart Citation

Multiple-Breath Washout as a Lung Function Test in Cystic Fibrosis. A Cystic Fibrosis Foundation Workshop Report

et al. 2015

View full text Add to dashboard Cite

show abstract

“…We have previously reported that at 3 months, Forced Expired Volume in 0.5 seconds (FEV 0.5 ) was reduced in 25%(17/68) of infants while Lung Clearance Index (LCI) was elevated in 21%(15/70) [3]. ILF at 1yr was predicted by that at 3mth and impressively FEV 0.5 improved over the first year of life with standard UK treatment [4].…”

Section: Introductionmentioning

confidence: 98%

Pulmonary function deficits in newborn screened infants with cystic fibrosis managed with standard UK care are mild and transient

Davies

Stocks²,

Thia³

et al. 2017

Eur Respir J

Self Cite

View full text Add to dashboard Cite

With the advent of novel designer molecules for cystic fibrosis (CF) treatment, there is huge need for early-life clinical trial outcomes, such as infant lung function (ILF). We investigated the degree and tracking of ILF abnormality during the first 2 years of life in CF newborn screened infants.Forced expiratory volume in 0.5 s (FEV0.5), lung clearance index (LCI) and plethysmographic functional residual capacity were measured at ∼3 months, 1 year and 2 years in 62 infants with CF and 34 controls.By 2 years there was no significant difference in FEV0.5 z-score between CF and controls, whereas mean LCI z-score was 0.81 (95% CI 0.45–1.17) higher in CF. However, there was no significant association between LCI z-score at 2 years with either 3-month or 1-year results. Despite minimal average group changes in any ILF outcome during the second year of life, marked within-subject changes occurred. No child had abnormal LCI or FEV0.5 on all test occasions, precluding the ability to identify “high-risk” infants in early life.In conclusion, changes in lung function are mild and transient during the first 2 years of life in newborn screened infants with CF when managed according to a standardised UK treatment protocol. Their potential role in tracking disease to later childhood will be ascertained by ongoing follow-up.

show abstract

“…Entretanto, existem poucos dados relacionando o impacto do diagnóstico precoce nos desfechos clínicos e funcionais de crianças pré-escolares [5][6][7][8][9]. O objetivo deste estudo foi comparar os dados clínicos, laboratoriais e espirométricos de pacientes pré-escolares portadores de FC, entre o grupo cuja suspeita diagnóstica foi levantada a partir da pesquisa do IRT no TTN e o grupo…”

Section: Introductionunclassified

Comparação entre a evolução de pré-escolares com fibrose cística identificados por triagem neonatal ou por sintomatologia clínica

Lumertz¹,

Moura²,

Pinto³

et al. 2018

Sci Med

View full text Add to dashboard Cite

Este artigo está licenciado sob forma de uma licença Creative Commons Atribuição 4.0 Internacional, que permite uso irrestrito, distribuição e reprodução em qualquer meio, desde que a publicação original seja corretamente citada. RESUMOOBJETIVOS: Comparar dados clínicos, laboratoriais e espirométricos de pacientes pré-escolares (idade entre três e seis anos) com fibrose cística, identificados pela alteração do tripsinogênio imunorreativo no teste de triagem neonatal (grupo IRT) com os dados de pacientes cuja suspeita diagnóstica ocorreu a partir de sinais e sintomas clínicos (grupo DC). MÉTODOS: Estudo do tipo coorte retrospectiva, com amostra obtida a partir dos prontuários dos pacientes que faziam acompanhamento ambulatorial especializado e multidisciplinar. Todas as crianças incluídas tinham diagnóstico confirmado de fibrose cística por teste de eletrólitos no suor e/ou sequenciamento genético. Como variáveis incluíram-se sexo, peso, altura, índice de massa corporal, número de internações por ano, colonização por Pseudomonas aeruginosa, presença de insuficiência pancreática e escore de Shwachman. Este escore abrange quatro domínios: atividade geral, nutrição, exame radiológico e avaliação física, cada um pontuado entre 5 e 25 pontos, sendo que o escore ≤40 pontos indica estado grave e ≥86 pontos excelente estado. Para comparação da função pulmonar utilizaram-se os dados da última espirometria realizada antes dos seis anos de idade. Os desfechos foram comparados por qui-quadrado ou teste t de Student, com limite de significância de 0,05. RESULTADOS: Foram incluídos no estudo 24 pacientes, sete no grupo IRT e 17 no grupo DC. As crianças do grupo IRT iniciaram acompanhamento com média de idade de 1,51±2,04 meses e as do grupo DC com 3,77±1,80 meses (p=0,014). A média do escore de Shwachman foi 94,40±2,19 nos pacientes do grupo IRT vs. 87,67±8,00 no grupo DC (p=0,018). A média do escore Z para o índice de massa corporal foi 0,95±1,02 no grupo IRT vs. 0,51±1,05 no grupo DC (p=0,051). Foram encontrados valores ligeiramente superiores nas variáveis espirométricas no grupo IRT, sem diferença estatisticamente significativa entre os grupos. CONCLUSÕES: Os resultados sugerem que os pacientes diagnosticados com fibrose cística a partir da suspeita pelo teste de triagem neonatal beneficiaram-se de uma intervenção precoce, podendo iniciar os testes de função pulmonar e receber tratamento e orientações mais cedo. A avaliação de parâmetros clínicos com o escore de Shwachman mostrou que os benefícios puderam ser observados já na idade pré-escolar. DESCRITORES: fibrose cística; espirometria; triagem neonatal; triagem do recém-nascido; nutrição da criança. ABSTRACTAIMS: To compare clinical, laboratory and spirometric parameters of preschoolers (ages from three to six years old) with cystic fibrosis identified by abnormal newborn screening test (NS group), with data of patients whose diagnostic suspicion arose from characteristic clinical signs and symptoms (CS group). METHODS: Retrospective cohort study, with a sample obtained...

show abstract

Lung function is abnormal in 3-month-old infants with cystic fibrosis diagnosed by newborn screening

Cited by 139 publications

References 52 publications

Multiple-Breath Washout as a Lung Function Test in Cystic Fibrosis. A Cystic Fibrosis Foundation Workshop Report

Multiple-Breath Washout as a Lung Function Test in Cystic Fibrosis. A Cystic Fibrosis Foundation Workshop Report

Pulmonary function deficits in newborn screened infants with cystic fibrosis managed with standard UK care are mild and transient

Comparação entre a evolução de pré-escolares com fibrose cística identificados por triagem neonatal ou por sintomatologia clínica

Contact Info

Product

Resources

About