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Lung function in young adults with congenital diaphragmatic hernia; a longitudinal evaluation

Authors

  • Marjolein Spoel MD,

    Corresponding author
    1. Intensive Care and Department of Pediatric Surgery, Erasmus Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands
    • Erasmus MC, Sophia Children's Hospital, Room Sk-3142, P.O. Box 2060, 3000 CB, Rotterdam, the Netherlands.
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  • Monique H.M. van der Cammen-van Zijp PhD,

    1. Intensive Care and Department of Pediatric Surgery, Erasmus Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands
    2. Department of Rehabilitation Medicine and Physical Therapy, Erasmus Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands
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  • Wim C.J. Hop PhD,

    1. Department of Biostatistics, Erasmus Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands
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  • Dick Tibboel MD, PhD,

    1. Intensive Care and Department of Pediatric Surgery, Erasmus Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands
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  • Johan C. de Jongste MD, PhD,

    1. Department of Pediatrics, Division of Pediatric Respiratory Medicine, Erasmus Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands
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  • Hanneke IJsselstijn MD, PhD

    1. Intensive Care and Department of Pediatric Surgery, Erasmus Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands
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  • Conflict of interest: None.

Abstract

Background

Survival rates of patients with congenital diaphragmatic hernia (CDH) have improved to up to 80%. Little is known about long-term consequences of the disease and its treatment. We evaluated lung function and respiratory symptoms longitudinally in a previously studied cohort of CDH patients and age-matched non-CDH patients who underwent similar neonatal intensive care treatment.

Study Design

We tested 27 young adults [mean (SD) age: 26.8 years (2.9)] with CDH and 30 non-CDH patients. Dynamic and static lung volumes, midexpiratory flows, and diffusion capacity were expressed as mean (SD) standard deviation scores. Prevalence of respiratory symptoms was evaluated with the European Community Respiratory Health Survey.

Results

All data are expressed as mean (SD). FEF25–75 in CDH patients had slightly deteriorated since childhood (CDH: −0.7 (1.4) vs. −1.6 (1.5), P < 0.001; non-CDH patients: 0.2 (1.4) vs. −0.3 (1.6), P = 0.038, ns). Diffusion capacity decreased in both groups (CDH: DLCOc 0.2 (1.1) vs. −1.5 (1.1), P <0.001; non-CDH: DLCOc 0.1 (0.4) vs. −1.1 (1.1), P < 0.001). Lung volumes were normal in both groups. The prevalence of asthma was higher than in the normal population (27.6% in patients and 30% in controls, P < 0.001).

Conclusions

Airflow obstruction and diffusion capacity deteriorated mildly from childhood into adulthood in survivors of CDH. The improved survival of patients with more severe forms of diaphragmatic hernia calls for long-term follow-up of lung function. Pediatr Pulmonol. 2013; 48:130–137. © 2012 Wiley Periodicals, Inc.

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