Abstract
There are few reports of cystic fibrosis (CF) diagnosed in premature infants. We describe the clinical course of three patients, from our neonatal intensive care units, who were diagnosed with CF, and discuss the existing literature and treatment considerations.
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References
Cystic Fibrosis Foundation Patient Registry Annual Data Report. Bethesda, Maryland: Cystic Fibrosis Foundation; 2008. http://www.cff.org/UploadedFiles/research/ClinicalResearch/2008-Patient-Registry-Report.pdf.
Walters S, Mehta A . Epidemiology of cystic fibrosis. In: Hodson M, Geddes DM, Bush A (eds). Cystic Fibrosis, 3rd edn. Edward Arnold Ltd: London, 2007, pp 21–45.
Sharples P, Colditz P, Wilkinson A . Lethal respiratory failure in preterm infant due to cystic fibrosis. Acta Paediatr Scand 1989; 78: 641–643.
Macek Jr M, Macek M, Stuhrmann M, Kulovaný E, Dolanská M, KoukolÃk F et al. The direct early diagnosis of cystic fibrosis by the detection of the delta F508 CFTR gene mutation in a prematurely delivered boy. Clin Genet 1991; 39: 219–222.
Festini F, Taccetti G, Repetto T, Reali MF, Campana S, Mergni G et al. Gestational and neonatal characteristics of children with cystic fibrosis: a cohort study. J Pediatr 2005; 147: 316–320.
Reiter PD, Townsend SF, Velasquez R . Dornase alfa in premature infants with severe respiratory distress and early bronchopulmonary dysplasia. J Perinatol 2000; 20: 530–534.
El Hassan NO, Chess PR, Huysman MW, Merkus PJ, de Jongste JC . Rescue use of DNase in critical lung atelectasis and mucus retention in premature neonates. Pediatrics 2001; 108: 468–470.
Diot P, Palmer LB, Smaldone A, DeCelie-Germana J, Grimson R, Smaldone GC . RhDNase I aerosol deposition and related factors in cystic fibrosis. Am J Respir Crit Care Med 1997; 156: 1662–1668.
Wagener JS, Rock MJ, McCubbin MM, Hamilton SD, Johnson CA, Ahrens RC . Aerosol delivery and safety of recombinant human deoxyribonuclease in young children with cystic fibrosis: a bronchoscopic study. J Pediatr 1998; 133: 486–491.
Salim A, Martin M . High-frequency percussive ventilation. Crit Care Med 2005; 33: S241–S245.
Rusakow LS, Abman SH, Sokol RJ, Seltzer W, Hammond K, Accurso FJ . Immunoreactive trypsinogen levels in infants with cystic fibrosis complicated by meconium ileus. Screening 1993; 2: 13–17.
Eng W, LeGrys V, Schechter M, Laughon M, Barker P . Sweat testing in pre-term and full-term infants less than 6 weeks of age. Pediatr Pulmonol 2005; 40: 64–67.
Cade A, Walters MP, McGinley N, Firth J, Brownlee KG, Conway SP et al. Evaluation of fecal pancreatic elastase-1 as a measure of pancreatic exocrine function in children with cystic fibrosis. Pediatr Pulmonol 2000; 29: 172–176.
Parad RB . Buccal cell DNA mutation analysis for diagnosis of cystic fibrosis in newborns and infants inaccessible to sweat chloride measurement. Pediatrics 1998; 101: 851–855.
Bennett LC, Kraemer R, Liechti-Gallati S . Buccal cell DNA analysis in premature and term neonates: screening for mutations of the complete coding region for the cystic fibrosis transmembrane conductance regulator. Eur J Pediatr 2000; 159: 99–102.
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Lu, K., Engmann, C., Moya, F. et al. Cystic fibrosis in premature infants. J Perinatol 31, 504–506 (2011). https://doi.org/10.1038/jp.2010.198
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DOI: https://doi.org/10.1038/jp.2010.198
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