. . "CZ - \u010Cesk\u00E1 republika" . "Adam, Zden\u011Bk" . . . "RIV/00209805:_____/11:#0000207!RIV12-MZ0-00209805" . . "Erdheim-Chester disease; juvenile xanthogranuloma; PET-CT imagination; bone scan; 2-chlorodeoxadenosine; cladribine; cyclophosphamide; dexamethasone; fewer of unknown origin; osteosclerosis; hyperostosis; osteoporosis; retroperitoneal fibrosis; Ormonds disease"@en . . . . "\u00DAsp\u011B\u0161n\u00E1 l\u00E9\u010Dba Erdheimovy-Chesterovy nemoci chemoterapi\u00ED obsahuj\u00EDc\u00ED 2-chlorodeoxyadenozin. Popis dvou p\u0159\u00EDpad\u016F a p\u0159ehled literatury"@cs . . . "2"^^ . . . "\u00DAvod: Erdheimova-Chesterova nemoc je velmi vz\u00E1cn\u00E1, a proto nen\u00ED optim\u00E1ln\u00ED l\u00E9\u010Dba t\u00E9to nemoci zn\u00E1ma. Na na\u0161em pracovi\u0161ti byly v letech 2008 a 2009 nov\u011B diagnostikov\u00E1ny 2 p\u0159\u00EDpady. Oba pacienti m\u011Bli diabetes insipidus, B symptomy (subfebrilie a\u017E febrilie) a bolesti v dlouh\u00FDch kostech doln\u00EDch kon\u010Detin. Popisy p\u0159\u00EDpad\u016F: Zobrazovac\u00ED vy\u0161et\u0159en\u00ED prok\u00E1zala vysokou akumulaci fluorodeoxygluk\u00F3zy, ale i Tc-pyrofosf\u00E1tu v dlouh\u00FDch kostech doln\u00EDch, ale i horn\u00EDch kon\u010Detin, zes\u00EDlen\u00ED st\u011Bny aorty s periaort\u00E1ln\u00ED fibr\u00F3zou a periren\u00E1ln\u00ED fibr\u00F3zu. U jednoho z pacient\u016F byla nav\u00EDc zji\u0161t\u011Bna v\u00EDce\u010Detn\u00E1 lo\u017Eiska choroby v mozku. Pro l\u00E9\u010Dbu obou pacient\u016F jsme zvolili 2-chlorodeoxyadenosin 5 mg/m2 s.c. a cyklofosfamid 150 mg/m2, aplikovan\u00E9 1.\u20135. den ve 28denn\u00EDch cyklech. U 2. pacienta jsme p\u0159idali k t\u00E9to l\u00E9\u010Db\u011B je\u0161t\u011B dexametazon 24 mg/den po dobu 5 dn\u016F. Celkem jsme pl\u00E1novali 6 cykl\u016F t\u00E9to l\u00E9\u010Dby. Oba pacienti dost\u00E1vaj\u00ED bisfosfon\u00E1ty, jeden zoledronat, druh\u00FD klodronat. L\u00E9\u010Debn\u00FD \u00FA\u010Dinek jsme vyhodnocovali na z\u00E1klad\u011B PET-CT a MR vy\u0161et\u0159en\u00ED. U 1. pacienta se po ukon\u010Den\u00ED v\u00FD\u0161e uveden\u00E9 chemoterapie infiltr\u00E1ty v mozku v\u00FDrazn\u011B zmen\u0161ily na mal\u00E9 reziduum a pacienta p\u0159estaly bolet doln\u00ED kon\u010Detiny. Nedo\u0161lo v\u0161ak k poklesu akumulace fluorodeoxygluk\u00F3zy v kostn\u00EDch lo\u017Eisc\u00EDch nemoci, a proto l\u00E9\u010Debnou odpov\u011B\u010F hodnot\u00EDme jako parci\u00E1ln\u00ED remisi. Tento pacient podstupuje l\u00E9\u010Dbu dal\u0161\u00ED linie, doba sledov\u00E1n\u00ED je 26 m\u011Bs\u00EDc\u016F od stanoven\u00ED diagn\u00F3zy. U 2. pacienta prok\u00E1zala opakovan\u00E1 PET-CT vy\u0161et\u0159en\u00ED signifikantn\u00ED pokles akumulace fluorodeoxygluk\u00F3zy ve v\u0161ech patologick\u00FDch lo\u017Eisc\u00EDch. Po l\u00E9\u010Db\u011B zmizely vysok\u00E9 teploty, bolesti dlouh\u00FDch kost\u00ED i patologick\u00E1 \u00FAnava (fatigue). Normalizovala se vstupn\u011B zv\u00FD\u0161en\u00E1 hodnota CPR i fibrinogenu. Stav hodnot\u00EDme jako kompletn\u00ED remisi nemoci. Doba sledov\u00E1n\u00ED tohoto pacienta od stanoven\u00ED diagn\u00F3zy je 16 m\u011Bs\u00EDc\u016F."@cs . . "Vnit\u0159n\u00ED l\u00E9ka\u0159stv\u00ED" . "[64018592C223]" . "\u0158eh\u00E1k, Zden\u011Bk" . . "Successful treatment of Erdheim-Chester disease by 2-chlorodeoxyadenosine-based chemotherapy. Two case studies and a literature review."@en . . "\u00DAvod: Erdheimova-Chesterova nemoc je velmi vz\u00E1cn\u00E1, a proto nen\u00ED optim\u00E1ln\u00ED l\u00E9\u010Dba t\u00E9to nemoci zn\u00E1ma. Na na\u0161em pracovi\u0161ti byly v letech 2008 a 2009 nov\u011B diagnostikov\u00E1ny 2 p\u0159\u00EDpady. Oba pacienti m\u011Bli diabetes insipidus, B symptomy (subfebrilie a\u017E febrilie) a bolesti v dlouh\u00FDch kostech doln\u00EDch kon\u010Detin. Popisy p\u0159\u00EDpad\u016F: Zobrazovac\u00ED vy\u0161et\u0159en\u00ED prok\u00E1zala vysokou akumulaci fluorodeoxygluk\u00F3zy, ale i Tc-pyrofosf\u00E1tu v dlouh\u00FDch kostech doln\u00EDch, ale i horn\u00EDch kon\u010Detin, zes\u00EDlen\u00ED st\u011Bny aorty s periaort\u00E1ln\u00ED fibr\u00F3zou a periren\u00E1ln\u00ED fibr\u00F3zu. U jednoho z pacient\u016F byla nav\u00EDc zji\u0161t\u011Bna v\u00EDce\u010Detn\u00E1 lo\u017Eiska choroby v mozku. Pro l\u00E9\u010Dbu obou pacient\u016F jsme zvolili 2-chlorodeoxyadenosin 5 mg/m2 s.c. a cyklofosfamid 150 mg/m2, aplikovan\u00E9 1.\u20135. den ve 28denn\u00EDch cyklech. U 2. pacienta jsme p\u0159idali k t\u00E9to l\u00E9\u010Db\u011B je\u0161t\u011B dexametazon 24 mg/den po dobu 5 dn\u016F. Celkem jsme pl\u00E1novali 6 cykl\u016F t\u00E9to l\u00E9\u010Dby. Oba pacienti dost\u00E1vaj\u00ED bisfosfon\u00E1ty, jeden zoledronat, druh\u00FD klodronat. L\u00E9\u010Debn\u00FD \u00FA\u010Dinek jsme vyhodnocovali na z\u00E1klad\u011B PET-CT a MR vy\u0161et\u0159en\u00ED. U 1. pacienta se po ukon\u010Den\u00ED v\u00FD\u0161e uveden\u00E9 chemoterapie infiltr\u00E1ty v mozku v\u00FDrazn\u011B zmen\u0161ily na mal\u00E9 reziduum a pacienta p\u0159estaly bolet doln\u00ED kon\u010Detiny. Nedo\u0161lo v\u0161ak k poklesu akumulace fluorodeoxygluk\u00F3zy v kostn\u00EDch lo\u017Eisc\u00EDch nemoci, a proto l\u00E9\u010Debnou odpov\u011B\u010F hodnot\u00EDme jako parci\u00E1ln\u00ED remisi. Tento pacient podstupuje l\u00E9\u010Dbu dal\u0161\u00ED linie, doba sledov\u00E1n\u00ED je 26 m\u011Bs\u00EDc\u016F od stanoven\u00ED diagn\u00F3zy. U 2. pacienta prok\u00E1zala opakovan\u00E1 PET-CT vy\u0161et\u0159en\u00ED signifikantn\u00ED pokles akumulace fluorodeoxygluk\u00F3zy ve v\u0161ech patologick\u00FDch lo\u017Eisc\u00EDch. Po l\u00E9\u010Db\u011B zmizely vysok\u00E9 teploty, bolesti dlouh\u00FDch kost\u00ED i patologick\u00E1 \u00FAnava (fatigue). Normalizovala se vstupn\u011B zv\u00FD\u0161en\u00E1 hodnota CPR i fibrinogenu. Stav hodnot\u00EDme jako kompletn\u00ED remisi nemoci. Doba sledov\u00E1n\u00ED tohoto pacienta od stanoven\u00ED diagn\u00F3zy je 16 m\u011Bs\u00EDc\u016F." . . . "\u00DAsp\u011B\u0161n\u00E1 l\u00E9\u010Dba Erdheimovy-Chesterovy nemoci chemoterapi\u00ED obsahuj\u00EDc\u00ED 2-chlorodeoxyadenozin. Popis dvou p\u0159\u00EDpad\u016F a p\u0159ehled literatury" . "\u00DAsp\u011B\u0161n\u00E1 l\u00E9\u010Dba Erdheimovy-Chesterovy nemoci chemoterapi\u00ED obsahuj\u00EDc\u00ED 2-chlorodeoxyadenozin. Popis dvou p\u0159\u00EDpad\u016F a p\u0159ehled literatury" . . . "237453" . "http://www.prolekare.cz/pdf?id=35478" . . "RIV/00209805:_____/11:#0000207" . . . . "57" . . "14"^^ . . "Koukalov\u00E1, Renata" . "0042-773X" . "Summary: Introduction: Erdheim-Chester disease is an extremely rarely occuring condition and thus an optimal treatment is not known. Two new cases have been diagnosed in our centre in 2008 and 2009. Both patients had diabetes insipidus, B symptoms (subfebrile to febrile states) and pain in long bones of lower limbs. Case studies: Imaging showed high accumulation of fluorodeoxyglucose as well as Tc-pyrophosphate in long bones of lower as well as upper limbs, aortic wall thickening with periaortic fibrosis and perirenal fibrosis. In addition, one of the patients had multiple lesions in the brain. 2-chlorodeoxyadenosine 5 mg/m2 s.c. and cyclophosphamide 150 mg/m2 administered on days 1 to 5 in 28-day cycles were selected for the treatment of both patients. Dexamethasone 24 mg/day for 5 days was added to this treatment in the second patient. Six cycles of the treatment were planned. Both patients were prescribed bisphosphonates \u2013 zoledronate and clodronate, respectively. Treatment effect was assessed with PET-CT and MR. Following treatment completion, brain infiltrates were reduced to a small residuum in the first patient whot did not anymore complain of leg pain. However, there was no reduction in fluorodeoxyglucose accumulation in bone lesions and thus treatment response was assessed as partial remission. This patient is currently receiving a second line treatment and treatment follow-up is 26 months from the diagnosis. Repeated PET-CTs in the second patient showed a significant reduction in accumulation of fluorodeoxyglucose in all pathological lesions. Febrile states and pain in long bones as well as pathological fatigue ceased after the treatment. Increased CPR and fibrinogen gradually returned to their normal levels. This response is assessed as complete remission. This patient\u2019s follow-up is 16 months from the diagnosis."@en . "\u00DAsp\u011B\u0161n\u00E1 l\u00E9\u010Dba Erdheimovy-Chesterovy nemoci chemoterapi\u00ED obsahuj\u00EDc\u00ED 2-chlorodeoxyadenozin. Popis dvou p\u0159\u00EDpad\u016F a p\u0159ehled literatury"@cs . . "Successful treatment of Erdheim-Chester disease by 2-chlorodeoxyadenosine-based chemotherapy. Two case studies and a literature review."@en . "6" . "13"^^ . . "Z(MZ0MOU2005)" . .