Long-term pulmonary function in children with recessive polycystic kidney disease.

Autor:	Jahnukainen T; Department of Pediatric Nephrology and Transplantation, Children's Hospital, Helsinki University and Helsinki University Central Hospital, Helsinki, Finland., Kirjavainen T; Department of Pediatrics, Children's Hospital, Helsinki University and Helsinki University Central Hospital, Helsinki, Finland., Luoto T; Department of Pediatric Surgery, Children's Hospital, Helsinki University and Helsinki University Central Hospital, Helsinki, Finland., Ylinen E; Department of Pediatric Nephrology and Transplantation, Children's Hospital, Helsinki University and Helsinki University Central Hospital, Helsinki, Finland., Linkosalo L; Department of Pediatrics, University of Tampere and Tampere University Hospital, Tampere, Finland., Arikoski P; Department of Pediatrics, Kuopio University Hospital and University of Eastern Finland, Kuopio, Finland., Pakarinen M; Department of Pediatric Surgery, Children's Hospital, Helsinki University and Helsinki University Central Hospital, Helsinki, Finland., Jalanko H; Department of Pediatric Nephrology and Transplantation, Children's Hospital, Helsinki University and Helsinki University Central Hospital, Helsinki, Finland.
Jazyk:	angličtina
Zdroj:	Archives of disease in childhood [Arch Dis Child] 2015 Oct; Vol. 100 (10), pp. 944-7. Date of Electronic Publication: 2015 Jul 10.
DOI:	10.1136/archdischild-2015-308451
Abstrakt:	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. (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)
Databáze:	MEDLINE
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