Article Text

other Versions

PDF
Long-term pulmonary function in children with recessive polycystic kidney disease
  1. Timo Jahnukainen1,
  2. Turkka Kirjavainen2,
  3. Topi Luoto3,
  4. Elisa Ylinen1,
  5. Laura Linkosalo4,
  6. Pekka Arikoski5,
  7. Mikko Pakarinen3,
  8. Hannu Jalanko1
  1. 1Department of Pediatric Nephrology and Transplantation, Children's Hospital, Helsinki University and Helsinki University Central Hospital, Helsinki, Finland
  2. 2Department of Pediatrics, Children's Hospital, Helsinki University and Helsinki University Central Hospital, Helsinki, Finland
  3. 3Department of Pediatric Surgery, Children's Hospital, Helsinki University and Helsinki University Central Hospital, Helsinki, Finland
  4. 4Department of Pediatrics, University of Tampere and Tampere University Hospital, Tampere, Finland
  5. 5Department of Pediatrics, Kuopio University Hospital and University of Eastern Finland, Kuopio, Finland
  1. Correspondence to Dr Timo Jahnukainen, Department of Pediatric Nephrology and Transplantation, Children's Hospital, Stenbäckinkatu 11, Box 281, Helsinki 00029, Finland; timo.jahnukainen{at}hus.fi

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.

  • Nephrology
  • Respiratory
  • Outcomes research

Statistics from Altmetric.com

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.