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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)

Long-term pulmonary morbidity in survivors of congenital diaphragmatic hernia.

Our objective was to study long-term respiratory outcomes of congenital diaphragmatic hernia (CDH) treated in the perinatal period. This was a cohort study with 26 adolescent survivors and age- and gender-matched controls. Medical histories were retrieved from hospital charts and questionnaires. Pulmonary function testing included measurement of maximum inspiratory and expiratory pressures (MIPS and MEPS) and maximum voluntary ventilation (MVV). Unpaired two-tailed t-test and nonlinear regression were used for statistical analysis. Significant differences were found in forced expiratory volume in one second (FEV(1)) (79% +/- 16% vs. 94% +/- 10%, P < 0.001), FEF(25-75) (62% +/- 24% vs. 84% +/- 15%, P < 0.001), FRC (114% +/- 20% vs. 95% +/- 13%, P < 0.001), RV/TLC (31% +/- 10% vs. 22% +/- 6%, P < 0.001), MVV (74% +/- 16% vs. 90% +/- 13%, P < 0.001), and MIPS (69% +/- 19% vs. 84% +/- 16%, P < 0.01), with numbers indicating percent predicted of reference values +/- SD. Reduction of MVV was not independent from FEV1 (r = 0.83). Forty-eight percent of patients vs. 4% of controls showed significant improvement of FEV1 after bronchodilators (86% +/- 15 vs. 98% +/- 10, P < 0.01). Forty-six percent of patients had abnormalities of the chest wall or spinal column such as pectus excavatum, pectus carinatum, and scoliosis, mostly mild or moderate. In conclusion, long-term respiratory outcome in adolescent CDH is associated with mild to moderate airway obstruction, a high prevalence of response to bronchodilators, and decreased inspiratory muscle strength. This should guide follow-up scheduling and should be taken into account for perioperative and critical care management.[1]


  1. Long-term pulmonary morbidity in survivors of congenital diaphragmatic hernia. Trachsel, D., Selvadurai, H., Bohn, D., Langer, J.C., Coates, A.L. Pediatr. Pulmonol. (2005) [Pubmed]
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