Renal lesion in Jeune's syndrome

In three children, presenting in chronic renal failure at the ages of six, four and three years radiology suggsested the diagnosis of Jeune's syndrome (asphyxiating thoracic dysplasia). In each case the skeletal survey showed widespread diagnostic features of this condition. The syndrome is characterized by varying degrees of repiratory distress in infancy and early childhood, with osseous dl three children in this study died from juvenile nephronopthisis. The correct diagnosis is essential for patient management and prognosis and also for genetic counselling. This report emphasizes the importance of the renal lesions in older children with Jeune's syndrome.