Nephrogenic fibrosing dermatopathy, cardiac calcification and pulmonary hypertension in an adolescent on chronic hemodialysis.

Nephrogenic fibrosing dermatopathy (NFD) is a systemic disorder of unknown etiology. Recent reports have associated the development of NFD with the use of gadolinium-enhanced magnetic resonance imaging (MRI). Here, we present the case of an adolescent with end-stage renal disease who died of biopsy-proven NFD and also developed cardiac calcification and clinical manifestations of pulmonary fibrosis with pulmonary hypertension. Only five cases of NFD have been reported in children, all of which were prior to the information regarding the consequences of using gadolinium. Here, we report a patient with NFD who received gadolinium while on chronic hemodialysis, 16 months prior to the onset of symptoms. Because he succumbed to this disease, we stress on the importance of eliminating the use of gadolinium-enhanced MRI examinations in children with impaired kidney function until the etiology of NFD is clarified.