title: [Cystinuria - Cystine Stones: Recommendations for Diagnosis, Therapy and Follow-up].
abstract: Cystinuria, an autosomal-recessive disorder, is the cause of 1 - 2 % of all kidney stones observed in adults and about 10 % of those observed in infants. Despite increasing understanding of underlying pathomechanisms, patients still form recurrent stones and have to undergo repeated interventions with increasing risk of renal insufficiency. Dietary and medical metaphylaxis may lower the frequency of recurrent stones but are often not practiced. Regular follow-up examinations and optimal therapy significantly enlarge stone-free intervals. This review offers an overview of the underlying pathogenetic mechanisms as well as guidance for diagnosis, monitoring, metaphylaxis and therapy of cystinuria following the recommendations of the Deutsche Gesellschaft für Urologie (DGU) and the European Association of Urology (EAU).
authors.affiliation: Urologische Klinik, Universitätsklinikum Mannheim, Theodor-Kutzer-Ufer 1-3, 68135 Mannheim, Germany. thomas.knoll@uro.ma.uni-heidelberg.de
authors.forename: T
authors.initials: T
authors.lastname: Knoll
authors.forename: V
authors.initials: V
authors.lastname: Janitzky
authors.forename: M S
authors.initials: MS
authors.lastname: Michel
authors.forename: P
authors.initials: P
authors.lastname: Alken
authors.forename: K U
authors.initials: KU
authors.lastname: Köhrmann
mesh_terms: D000328:Adult; D000367:Age Factors; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000008:administration & dosage / Q000627:therapeutic use; D001205:Ascorbic Acid / Q000008:administration & dosage / Q000627:therapeutic use; D002216:Captopril / Q000008:administration & dosage / Q000627:therapeutic use; D002614:Chelating Agents / Q000008:administration & dosage / Q000627:therapeutic use; D002648:Child; D003555:Cystinuria / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D005500:Follow-Up Studies; D006801:Humans; D007669:Kidney Calculi / Q000209:etiology* / Q000517:prevention & control / Q000628:therapy; D008096:Lithotripsy; D010396:Penicillamine / Q000008:administration & dosage / Q000627:therapeutic use; D013997:Time Factors; D008625:Tiopronin / Q000008:administration & dosage / Q000627:therapeutic use; D018666:Ureteroscopy
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors; D002614:Chelating Agents; D002216:Captopril; D008625:Tiopronin; D010396:Penicillamine; D001205:Ascorbic Acid