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Prikaz primera: patološki jetrni[!] testi, fotosenzitivnost in steatohepatitis - kronična hepatična porfirija (porfirija cutanea tarda) = Case report: pathological liver tests, photosensitivity and steatohepatitis - chronical hepatic porphiria (porphiria cutanea tarda)
Zavrtanik, Mark ; Janša, Rado
Porfirija cutanea tarda je bolezen, ki je posledica motene presnove hema. Vzrok je znižana aktivnost[!] encima uroporfinogen dekarboksilaze, kar vodi v kopičenje karboksiliranih presnovkov in ...posledično simptomatiko. Najpogosteje se kaže s fotosenzitivnostjo, ter kožno simptomatiko z vezikulami[!] in posledičnimi hiperpigmentacijami. Prizadetost jeter pri porfiriji cutaneai tardai ni redka, pogosto je spregledana. Kaže se lahko z le nekoliko povišanimi transaminazami, resnejše okvare jeter pa so steatoza, steatohepatitis, sideroza, in posledična ciroza. Predstavljamo 65-letno bolnico s porfirijo cutaneao tardao, s hudo prizadetostjo jeter zaradi steatohepatitisa. Ob ustrezni terapiji je prišlo do popolnega regresa bolezni.
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