Abstract
Three children ages 6 through 10 years with Jeune Syndrome (asphyxiating thoracic dystrophy) and end stage renal failure underwent successful kidney transplantation using parents as donors. They were evaluated by pulmonary function studies 5, 14 and 30 months following their renal transplantation. The patients, 2 males and 1 female, exhibited the typical physical and radiologic findings of the syndrome consisting of congenital narrowing of the thorax, shortened ribs and limbs, and abnormalities of the bones of the pelvis and extremities. The patients weighed 14, 16 and 22kg at the time of transplantation. Renal function, post-transplantation, was good to excellent with creatinine clearances ranging from 70cc to 190cc/min/1.73 meters2 and serum creatinines ranging from 0.4 to 1.2mg%. Pulmonary function studies including lung volumes and flow volume loops were consistent with a pure restrictive pulmonary defect. The patients were followed serially with pulmonary function studies and as somatic growth occurred lung volumes increased proportionately to growth in height. Preliminary data would indicate that renal transplantation is practical and desirable in this syndrome since the restrictive pulmonary disease appears not to be a limiting factor in children surviving infancy.
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Fennell, R., Loughlin, G., Garin, E. et al. 1061 PULMONARY FUNCTION STUDIES IN CHILDREN WITH JEUNE SYNDROME POST-RENAL TRANSPLANTATION. Pediatr Res 12 (Suppl 4), 540 (1978). https://doi.org/10.1203/00006450-197804001-01067
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DOI: https://doi.org/10.1203/00006450-197804001-01067