title: Long-term pulmonary function in children with recessive polycystic kidney disease.
abstract: BACKGROUND
  Neonatal autosomal-recessive polycystic kidney disease (ARPKD) is associated with pulmonary hypoplasia and severe respiratory distress. There is no published information on long-term lung function in ARPKD survivors.
  
  
  METHODS
  Pulmonary function tests, including spirometry and diffusion capacity, were performed in a nationwide cohort of Finnish paediatric patients with ARPKD. The annual incidence of respiratory infections and the need for permanent asthma medication were also evaluated in this population.
  
  
  RESULTS
  Pulmonary function in 11 children surviving the neonatal period was good when measured after a median follow-up time of 10.4 years (range 5.4-16.1 years). None of the patients required oxygen supplementation, and only one patient had asthma. Patients who had received ventilator therapy during infancy had significantly lower maximal instantaneous forced expiratory flow (MEF%) (66%; 43-93% vs 105%; 63-110%; p=0.048) and forced expiratory volume/forced vital capacity (0.76; 0.7-0.81 vs 0.89; 0.77-0.91; p=0.03) than patients without a history of mechanical ventilation, suggesting tendency for airway obstruction in the former group of patients. The frequency of respiratory infections did not differ from Finnish paediatric population in general.
  
  
  CONCLUSIONS
  The results of pulmonary function tests were within reference values for most patients with ARPKD, which suggested good long-term lung prognosis. Lung function tests should be considered for patients with ARPKD with a history of mechanical ventilation during infancy.
authors.affiliation: Department of Pediatric Nephrology and Transplantation, Children's Hospital, Helsinki University and Helsinki University Central Hospital, Helsinki, Finland.
authors.forename: Timo
authors.initials: T
authors.lastname: Jahnukainen
authors.affiliation: Department of Pediatrics, Children's Hospital, Helsinki University and Helsinki University Central Hospital, Helsinki, Finland.
authors.forename: Turkka
authors.initials: T
authors.lastname: Kirjavainen
authors.affiliation: Department of Pediatric Surgery, Children's Hospital, Helsinki University and Helsinki University Central Hospital, Helsinki, Finland.
authors.forename: Topi
authors.initials: T
authors.lastname: Luoto
authors.affiliation: Department of Pediatric Nephrology and Transplantation, Children's Hospital, Helsinki University and Helsinki University Central Hospital, Helsinki, Finland.
authors.forename: Elisa
authors.initials: E
authors.lastname: Ylinen
authors.affiliation: Department of Pediatrics, University of Tampere and Tampere University Hospital, Tampere, Finland.
authors.forename: Laura
authors.initials: L
authors.lastname: Linkosalo
authors.affiliation: Department of Pediatrics, Kuopio University Hospital and University of Eastern Finland, Kuopio, Finland.
authors.forename: Pekka
authors.initials: P
authors.lastname: Arikoski
authors.affiliation: Department of Pediatric Surgery, Children's Hospital, Helsinki University and Helsinki University Central Hospital, Helsinki, Finland.
authors.forename: Mikko
authors.initials: M
authors.lastname: Pakarinen
authors.affiliation: Department of Pediatric Nephrology and Transplantation, Children's Hospital, Helsinki University and Helsinki University Central Hospital, Helsinki, Finland.
authors.forename: Hannu
authors.initials: H
authors.lastname: Jalanko
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005387:Finland; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D007223:Infant; D008168:Lung / Q000503:physiopathology*; D017044:Polycystic Kidney, Autosomal Recessive / Q000503:physiopathology*; D012129:Respiratory Function Tests; D012140:Respiratory Tract Diseases / Q000503:physiopathology; D012189:Retrospective Studies
keywords: Nephrology; Outcomes research; Respiratory