title: Imaging medullary cystic kidney disease with magnetic resonance.
abstract: Medullary cystic kidney disease is characterized by multiple renal cysts at the corticomedullary boundary area, by autosomal dominant inheritance, and by onset of chronic renal failure in the third decade of life. Its clinical manifestations are often insignificant and nonspecific. Furthermore, its diagnosis may be difficult in sporadic forms where genetic linkage analysis cannot be performed. The authors report the case of a patient presenting with a sporadic form of medullary cystic kidney disease whose diagnosis was confirmed using computerized tomography with 3-dimensional reconstruction at the nephrography-excretion time and magnetic resonance imaging (MRI) with magnetic resonance angiography and urography after the injection of gadolinium, a nonnephrotoxic compound. Both imaging techniques showed normal-sized, normal-shaped kidneys containing multiple cysts from 1 to 30 mm in diameter in the medulla and at the corticomedullary junction. A characteristic medullary nephrogram appeared after injection of iodinated contrast medium or gadolinium corresponding to contrast-filled dilated collecting ducts. This report shows that MRI with gadolinium injection can substitute for computerized tomography in azotemic patients. MRI seems particularly promising for the diagnosis of cystic diseases of the kidney and must also be considered when investigating a patient with chronic renal failure of unknown origin.
authors.affiliation: Department of Nephrology, Hôpital Tenon (Assistance Publique-Hôpitaux de Paris) and University Paris 6, Paris, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Meier
authors.forename: Maria Teresa
authors.initials: MT
authors.lastname: Farres
authors.forename: Béatrice
authors.initials: B
authors.lastname: Mougenot
authors.forename: Laurent
authors.initials: L
authors.lastname: Jacob
authors.forename: Françoise
authors.initials: F
authors.lastname: Le Goas
authors.forename: Corinne
authors.initials: C
authors.lastname: Antignac
authors.forename: Pierre
authors.initials: P
authors.lastname: Ronco
mesh_terms: D000328:Adult; D001706:Biopsy; D005260:Female; D005682:Gadolinium; D006801:Humans; D007676:Kidney Failure, Chronic / Q000209:etiology; D007679:Kidney Medulla / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D016891:Polycystic Kidney, Autosomal Dominant / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D019275:Radiopharmaceuticals
chemical_list: D019275:Radiopharmaceuticals; D005682:Gadolinium