. "Transplantace hematopoetick\u00FDch bun\u011Bk u p\u011Bti pacient\u016F s chronickou granulomat\u00F3zn\u00ED nemoc\u00ED v \u010Cesk\u00E9 republice"@cs . "174969" . "Transplantation of hematopoietic cells in five patients with chronic granulomatous disease in the Czech Republic"@en . . "Janda, A." . . "Transplantace hematopoetick\u00FDch bun\u011Bk u p\u011Bti pacient\u016F s chronickou granulomat\u00F3zn\u00ED nemoc\u00ED v \u010Cesk\u00E9 republice"@cs . "Chronic granulomatous disease (CGD) is a primary immunodeficiency caused by defective phagocytosis. Conservative treatment is applied in most of the patients. Currently, the only available curative treatment is allogeneic hematopoietic stem cell transplantation (HSCT). Thirty-seven patients were diagnosed with CGD in the Czech and Slovak Republic between 1957 and 2011. Calculated incidence of the disease in the Czech Republic is 1:280,000 live births. Five patients with serious course of the disease underwent allogeneic HSCT in 2007\u20132010. Median age of CGD diagnosis in these patients was 6 months (6\u201317 months), median age at transplantation was 21 months (1\u201317 years). One patient was transplanted in myeloablative regimen, 4 patients in regimen with reduced intensity. All of the grafts were from HLA-matched unrelated donors. The procedure was complicated with graft versus host disease \u2013 GvHD (3 patients), infections (4 patients), autoimmunity (3 patients) and toxic side effects (3 patients). Median follow-up after the transplantation was 12 months (6\u201344 months). In all of the patients normalization of granulocyte function was documented within 2 months after the transplantation. Mixed chimerismwas present in all of them. The oldest patient (transplanted at the age of 17 years, 4months) died 6 months after transplantation due to intracranial haemorrhage after operational revision of healed brain abscess. All the other patients (4) are in a good clinical condition, without signs of CGD or GvHD. In concordance with other transplantation centres we show that early allogeneic HSCT is a suitable treatment of patients with severe CGD."@en . . "Keslov\u00E1, P." . "Sedl\u00E1\u010Dek, P." . "10"^^ . "Chronick\u00E1 granulomat\u00F3zn\u00ED nemoc (CGD) pat\u0159\u00EDmezi vrozen\u00E9 imunodeficience zp\u016Fsoben\u00E9 poruchou fagocyt\u00F3zy. V\u011Bt\u0161ina pacient\u016F je l\u00E9\u010Dena konzervativn\u011B. V sou\u010Dasn\u00E9 dob\u011B je jedinou dostupnou kurativn\u00ED terapi\u00ED alogenn\u00ED transplantace hematopoetick\u00FDch bun\u011Bk (HSCT). V letech 1957 a\u017E 2011 bylo v \u010Cesk\u00E9 a Slovensk\u00E9 republice diagnostikov\u00E1no 37 pacient\u016F s CGD. Odhadovan\u00E1 incidence onemocn\u011Bn\u00ED v \u010Cesk\u00E9 republice je 1:280 000 \u017Eiv\u011B narozen\u00FDch d\u011Bt\u00ED. P\u011Bt pacient\u016F se z\u00E1va\u017En\u00FDm pr\u016Fb\u011Bhem onemocn\u011Bn\u00ED podstoupilo v letech 2007\u20132010 alogenn\u00ED HSCT. Medi\u00E1n stanoven\u00ED diagn\u00F3zy byl u t\u011Bchto pacient\u016F 6 m\u011Bs\u00EDc\u016F (6\u201317 m\u011Bs\u00EDc\u016F), medi\u00E1n v\u011Bku p\u0159i transplantaci 21 m\u011Bs\u00EDc\u016F (1 rok a\u017E 17 let). Jeden pacient byl transplantov\u00E1n v myeloablativn\u00EDm re\u017Eimu, 4 pacienti s pou\u017Eit\u00EDm redukovan\u00E9ho re\u017Eimu, v\u017Edy \u0161t\u011Bpy od HLA-shodn\u00FDch nep\u0159\u00EDbuzn\u00FDch d\u00E1rc\u016F. V\u00FDkon byl komplikov\u00E1n GvHD II. stupn\u011B (3 pacienti), infekc\u00ED (4 pacienti), autoimmunitou (3 pacienti) a vedlej\u0161\u00EDmi \u00FA\u010Dinky l\u00E9\u010Dby (3 pacienti). Medi\u00E1n sledov\u00E1n\u00ED po transplantaci byl 12 m\u011Bs\u00EDc\u016F (6\u201344 m\u011Bs\u00EDc\u016F). U v\u0161ech pacient\u016F do\u0161lo v pr\u016Fb\u011Bhu 2 m\u011Bs\u00EDc\u016F po transplantaci k normalizaci funkce granulocyt\u016F, u v\u0161ech byl p\u0159\u00EDtomen sm\u00ED\u0161en\u00FD chimerismus. Nejstar\u0161\u00ED pacient (transplantov\u00E1n ve v\u011Bku 17 let, 4 m\u011Bs\u00EDce) zem\u0159el 6 m\u011Bs\u00EDc\u016F po transplantaci na intrakrani\u00E1ln\u00ED krv\u00E1cen\u00ED po opera\u010Dn\u00ED revizi zhojen\u00E9ho mozkov\u00E9ho abscesu. Ostatn\u00ED pacienti (4) jsou v dobr\u00E9m klinick\u00E9m stavu, bez zn\u00E1mek CGD a GvHD. Ve shod\u011B se zahrani\u010Dn\u00EDmi pracovi\u0161ti ukazuj\u00ED na\u0161e v\u00FDsledky, \u017Ee \u010Dasn\u00E1 alogenn\u00ED transplantace hematopoetick\u00FDch bun\u011Bk p\u0159edstavuje vhodnou alternativu l\u00E9\u010Dby pacient\u016F se z\u00E1va\u017Enou formou chronick\u00E9 granulomat\u00F3zn\u00ED nemoci."@cs . . "Rozs\u00EDval, P." . "\u010Cesko-slovensk\u00E1 pediatrie" . . . "0069-2328" . . "chronic granulomatous disease; primary immunodeficiency; hematopoietic stem cell transplantation"@en . . "I, V, Z(MSM0021620812), Z(MZ0FNM2005)" . "Pa\u0159\u00EDzkov\u00E1, E." . . . . . "RIV/00159816:_____/12:#0000845" . "8"^^ . . . "\u0160ediv\u00E1, A." . "2" . "Litzman, Ji\u0159\u00ED" . "Form\u00E1nkov\u00E1, R." . . "RIV/00159816:_____/12:#0000845!RIV13-MZ0-00159816" . . "CZ - \u010Cesk\u00E1 republika" . "Transplantation of hematopoietic cells in five patients with chronic granulomatous disease in the Czech Republic"@en . . "Transplantace hematopoetick\u00FDch bun\u011Bk u p\u011Bti pacient\u016F s chronickou granulomat\u00F3zn\u00ED nemoc\u00ED v \u010Cesk\u00E9 republice" . . "1"^^ . "Chronick\u00E1 granulomat\u00F3zn\u00ED nemoc (CGD) pat\u0159\u00EDmezi vrozen\u00E9 imunodeficience zp\u016Fsoben\u00E9 poruchou fagocyt\u00F3zy. V\u011Bt\u0161ina pacient\u016F je l\u00E9\u010Dena konzervativn\u011B. V sou\u010Dasn\u00E9 dob\u011B je jedinou dostupnou kurativn\u00ED terapi\u00ED alogenn\u00ED transplantace hematopoetick\u00FDch bun\u011Bk (HSCT). V letech 1957 a\u017E 2011 bylo v \u010Cesk\u00E9 a Slovensk\u00E9 republice diagnostikov\u00E1no 37 pacient\u016F s CGD. Odhadovan\u00E1 incidence onemocn\u011Bn\u00ED v \u010Cesk\u00E9 republice je 1:280 000 \u017Eiv\u011B narozen\u00FDch d\u011Bt\u00ED. P\u011Bt pacient\u016F se z\u00E1va\u017En\u00FDm pr\u016Fb\u011Bhem onemocn\u011Bn\u00ED podstoupilo v letech 2007\u20132010 alogenn\u00ED HSCT. Medi\u00E1n stanoven\u00ED diagn\u00F3zy byl u t\u011Bchto pacient\u016F 6 m\u011Bs\u00EDc\u016F (6\u201317 m\u011Bs\u00EDc\u016F), medi\u00E1n v\u011Bku p\u0159i transplantaci 21 m\u011Bs\u00EDc\u016F (1 rok a\u017E 17 let). Jeden pacient byl transplantov\u00E1n v myeloablativn\u00EDm re\u017Eimu, 4 pacienti s pou\u017Eit\u00EDm redukovan\u00E9ho re\u017Eimu, v\u017Edy \u0161t\u011Bpy od HLA-shodn\u00FDch nep\u0159\u00EDbuzn\u00FDch d\u00E1rc\u016F. V\u00FDkon byl komplikov\u00E1n GvHD II. stupn\u011B (3 pacienti), infekc\u00ED (4 pacienti), autoimmunitou (3 pacienti) a vedlej\u0161\u00EDmi \u00FA\u010Dinky l\u00E9\u010Dby (3 pacienti). Medi\u00E1n sledov\u00E1n\u00ED po transplantaci byl 12 m\u011Bs\u00EDc\u016F (6\u201344 m\u011Bs\u00EDc\u016F). U v\u0161ech pacient\u016F do\u0161lo v pr\u016Fb\u011Bhu 2 m\u011Bs\u00EDc\u016F po transplantaci k normalizaci funkce granulocyt\u016F, u v\u0161ech byl p\u0159\u00EDtomen sm\u00ED\u0161en\u00FD chimerismus. Nejstar\u0161\u00ED pacient (transplantov\u00E1n ve v\u011Bku 17 let, 4 m\u011Bs\u00EDce) zem\u0159el 6 m\u011Bs\u00EDc\u016F po transplantaci na intrakrani\u00E1ln\u00ED krv\u00E1cen\u00ED po opera\u010Dn\u00ED revizi zhojen\u00E9ho mozkov\u00E9ho abscesu. Ostatn\u00ED pacienti (4) jsou v dobr\u00E9m klinick\u00E9m stavu, bez zn\u00E1mek CGD a GvHD. Ve shod\u011B se zahrani\u010Dn\u00EDmi pracovi\u0161ti ukazuj\u00ED na\u0161e v\u00FDsledky, \u017Ee \u010Dasn\u00E1 alogenn\u00ED transplantace hematopoetick\u00FDch bun\u011Bk p\u0159edstavuje vhodnou alternativu l\u00E9\u010Dby pacient\u016F se z\u00E1va\u017Enou formou chronick\u00E9 granulomat\u00F3zn\u00ED nemoci." . "[4192C4EA9576]" . "67" . "Hou\u0161\u0165kov\u00E1, H." . "Transplantace hematopoetick\u00FDch bun\u011Bk u p\u011Bti pacient\u016F s chronickou granulomat\u00F3zn\u00ED nemoc\u00ED v \u010Cesk\u00E9 republice" . "Star\u00FD, J." .