Comparação entre a evolução de pré-escolares com fibrose cística identificados por triagem neonatal ou por sintomatologia clínica
DOI:
https://doi.org/10.15448/1980-6108.2018.2.29566Palavras-chave:
fibrose cística, espirometria, triagem neonatal, triagem do recém-nascido, nutrição da criança.Resumo
OBJETIVOS: 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.
Downloads
Referências
Stocks J, Thia LP, Sonnappa S. Evaluation and use of childhood lung function tests in cystic fibrosis. Curr Opin Pulm Med. 2012;18(6):602-8. https://doi.org/10.1097/MCP.0b013e328358dfbe
Cantin AM, Hartl D, Konstan MW, Chmiel JF. Inflammation in cystic fibrosis lung disease: Pathogenesis and therapy. J Cyst Fibros. 2015;14(4):419-30. https://doi.org/10.1016/j.jcf.2015.03.003
Farrell PM, Rosenstein BJ, White TB, Accurso FJ, Castellani C, Cutting GR, Durie PR, Legrys VA, Massie J, Parad RB, Rock MJ, Campbell PW 3rd; Cystic Fibrosis Foundation. Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report. J Pediatr. 2008;153(2):S4-S14. https://doi.org/10.1016/j.jpeds.2008.05.005
Rosenfeld M. Overview of published evidence on outcomes with early diagnosis from large US observational studies. J Pediatr. 2005;147(3 Suppl):S11-4. https://doi.org/10.1016/j.jpeds.2005.08.010
Martin B, Schechter MS, Jaffe A, Cooper P, Bell SC, Ranganathan S. Comparison of the US and Australian cystic fibrosis registries: the impact of newborn screening. Pediatrics. 2012;129(2):e348-55. https://doi.org/10.1542/peds.2011-0567
Sims EJ, McCormick J, Mehta G, Mehta A; Steering Committee of the UK Cystic Fibrosis Database. Neonatal screening for cystic fibrosis is beneficial even in the context of modern treatment. J Pediatr. 2005;147(3 Suppl):S42-6. https://doi.org/10.1016/j.jpeds.2005.08.002
Dijk FN, McKay K, Barzi F, Gaskin KJ, Fitzgerald DA. Improved survival in cystic fibrosis patients diagnosed by newborn screening compared to a historical cohort from the same centre. Arch Dis Child. 2011;96(12):1118-23. https://doi.org/10.1136/archdischild-2011-300449
Hoo AF, Thia LP, Nguyen TT, Bush A, Chudleigh J, Lum S, Ahmed D, Balfour Lynn I, Carr SB, Chavasse RJ, Costeloe KL, Price J, Shankar A, Wallis C, Wyatt HA, Wade A, Stocks J; London Cystic Fibrosis Collaboration. Lung function is abnormal in 3-month-old infants with cystic fibrosis diagnosed by newborn screening. Thorax. 2012;67(10):874-81. https://doi.org/10.1136/thoraxjnl-2012-201747
Ranganathan S, Linnane B, Nolan G, Gangell C, Hall G. Early detection of lung disease in children with cystic fibrosis using lung function. Paediatr Respir Rev. 2008;9(3):160-7. https://doi.org/10.1016/j.prrv.2008.05.002
Shwachman H, Kulczycki LL. Long term study of one hundred five patients with cystic fibrosis. Am J Dis Child. 1958;96(1):6-15. https://doi.org/10.1001/archpedi.1958.02060060008002
Kozlowska WJ, Bush A, Wade A, Aurora P, Carr SB, Castle RA, Hoo AF, Lum S, Price J, Ranganathan S, Saunders C, Stanojevic S, Stroobant J, Wallis C, Stocks J; London Cystic Fibrosis Collaboration..Lung function from infancy to the preschool years after clinical diagnosis of cystic fibrosis. Am J Respir Crit Care Med. 2008;178(1):42-9. https://doi.org/10.1164/rccm.200710-1599OC
Lee TW, Brownlee KG, Conway SP, Denton M, Littlewood JM. Evaluation of a new definition for chronic Pseudomonas aeruginosa infection in cystic fibrosis patients. J Cyst Fibros. 2003;2(1):29-34. https://doi.org/10.1016/S1569-1993(02)00141-8
Stanojevic S, Wade A, Cole TJ, Lum S, Custovic A, Silverman M, Hall GL, Welsh L, Kirkby J, Nystad W, Badier M, Davis S, Turner S, Piccioni P, Vilozni D, Eigen H, Vlachos-Mayer H, Zheng J, Tomalak W, Jones M, Hankinson JL, Stocks J;Asthma UK Spirometry Collaborative Group. Spirometry centile charts for young Caucasian children: the Asthma UK Collaborative Initiative. Am J Respir Crit Care Med. 2009;180(6):547-52. https://doi.org/10.1164/rccm.200903-0323OC
Heinzmann-Filho JP, Pinto LA, Marostica PJ, Donadio MV. Variation in lung function is associated with worse clinical outcomes in cystic fibrosis. J Bras Pneumol. 2015;41(6):509-15. https://doi.org/10.1590/s1806-37562015000000006
Miller MR, Hankinson J, Brusasco V, Burgos F, Casaburi R, Coates A, Crapo R, Enright P, van der Grinten CP, Gustafsson P, Jensen R, Johnson DC, MacIntyre N, McKay R, Navajas D, Pedersen OF, Pellegrino R, Viegi G, Wanger J; ATS/ERS Task Force. Standardisation of Spirometry. Eur Resp J. 2005;26(2):319-38. https://doi.org/10.1183/09031936.05.00034805
Siret D, Bretaudeau G, Branger B, Dabadie A, Dagorne M, David V, de Braekeleer M, Moisan-Petit V, Picherot G, Rault G, Storni V, Roussey M. Comparing the clinical evolution of cystic fibrosis screened neonatally to that of cystic fibrosis diagnosed from clinical symptoms: a 10-year retrospective study in a French region (Brittany). Pediatr Pulmonol. 2003;35(5):342-9. https://doi.org/10.1002/ppul.10259
Chatfield S, Owen G, Ryley HC, Williams J, Alfaham M, Goodchild MC, Weller P. Neonatal screening for cystic fibrosis in Wales and the West Midlands: clinical assessment after five years of screening. Arch Dis Child. 1991;66 (1 Spec No):29-33.
Groves T, Robinson P, Wiley V, Fitzgerald DA. Long-term outcomes of children with intermediate sweat chloride values in infancy. J Pediatr. 2015;166(6):1469-74. https://doi.org/10.1016/j.jpeds.2015.01.052
Castro LV, Martins MC. Application of Schwachman's scoring system to patients with cystic fibrosis with focus on the nutritional aspect. Rev Pediatr Ceara. 2005;6:27-36.
Stollar F, Adde FV, Cunha MT, Leone C, Rodrigues JC. Shwachman-Kulczycki score still useful to monitor cystic fibrosis severity. Clinics (Sao Paulo). 2011;66(6):979-83. https://doi.org/10.1590/S1807-59322011000600010
Assis I, Camargos PA, Reis FJ, Sulmonett N, Carneiro AP. Assessing correlations between spirometry and Shwachman-Kulczycki score in children and adolescents. Pediatr Pulmonol. 2003;36(4):305-9. https://doi.org/10.1002/ppul.10350
Freire ID, Abreu E Silva FA, Araújo MA. Comparison among pulmonary function test results, the Shwachman-Kulczycki score and the Brasfield score in patients with cystic fibrosis. J Bras Pneumol. 2008;34(5):280-7. https://doi.org/10.1590/S1806-37132008000500006
Farrell PM, Li Z, Kosorok MR, Laxova A, Green CG, Collins J, Lai HC, Rock MJ, Splaingard ML. Bronchopulmonary disease in children with cystic fibrosis after early or delayed diagnosis. Am J Respir Crit Care Med. 2003;168(9):1100-8. https://doi.org/10.1164/rccm.200303-434OC
Aurora P, Stocks J, Oliver C, Saunders C, Castle R, Chaziparasidis G, Bush A; London Cystic Fibrosis Collaboration. Quality control for spirometry in preschool children with and without lung disease. Am J Respir Crit Care Med. 2004;169(10):1152-9. https://doi.org/10.1164/rccm.200310-1453OC
Li Z, Kosorok MR, Farrell PM, Laxova A, West SE, Green CG, Collins J, Rock MJ, Splaingard ML. Longitudinal development of mucoid Pseudomonas aeruginosa infection and lung disease progression in children with cystic fibrosis. JAMA. 2005;293(5):581-8. https://doi.org/10.1001/jama.293.5.581
