Akutní lymfoblastická leukemie (ALL) tvoří jednu čtvrtinu dětských nádorů a je tak nejčastějším maligním nádorovým onemocněním v dětském věku. Jedná se o heterogenní onemocnění, jehož biologie je odlišná od ALL dospělých. Medián věku při diagnóze dětské ALL je 5 let, nejčastějšími chromozomálními abnormitami jsou fúzní gen TEL-AML a hyperdiploidie nad 50 chromozomů. Pravděpodobnost přežití bez selhání (EFS) je při aplikaci kombinované chemoterapie lišící se dle rizika nepříznivého průběhu více než 75 % a pravděpodobnost vyléčení je 85 %. Relaps postihuje 15–20 % dětí s ALL a v jeho léčbě hraje významnou roli transplantace kostní dřeně. Akutní myeloidní leukemie (AML) tvoří 15 % leukemií v dětství. Nejčastějším genetickým podtypem AML je monocytární leukemie s přestavbou MLL genu na dlouhém raménku 11. chromozomu (20–25 % dětí s AML), která má intermediární prognózu. Prognosticky příznivé podtypy akutní promyelocytární leukemie s t (15; 17), myeloidní leukemie s t (8; 21) a myelomonocytární leukemie s inv (16) se nachází u 30–40 % dětí s AML s šancí na vyléčení 70 %. Celkové výsledky léčby AML se u dětí stále zlepšují s šancí na dosažení remise více než 90 % a pravděpodobností přežití více než 60 %., Acute lymphoblastic leukemia (ALL) represents 25 % of childhood tumors being the most frequent childhood cancer. ALL is a heterogeneous disease, biologically different from the same disease in adults. Median age at diagnosis is 5 years, the most frequent chromosomal abnormalities are fusion gene TEL-AML1 and hyperdiploidy over 50 chromosomes. Event-free-survival is over 75 % and overall survival 85 % in children treated with combined chemotherapy intensified according to the risk group. The incidence of relapse is 15–20 % and bone marrow transplantation is the important treatment modality in such situation. Acute myeloid leukemia (AML) represents 15 % of childhood leukemias. The most frequent genetic subtype is monocytic leukemia with MLL rearrangement at the long arm of chromosome 11 (11q23) (20–25 % of all AML) with intermediate prognosis. Subtypes with good prognosis – acute promyelocytic leukemia with t (15; 17), myeloid leukemia with t (8; 21) and myelomonocytic leukemia with inv (16) represent 30–40 % of AML with overall survival 70 %. Total treatment results are steadily improving with a chance to achieve remission over 90 % and overall survival over 60 %., Jan Starý, and Lit.: 20
Hemolytic uremic syndrome (HUS) is a type of thrombotic microangiopathy, in the course of which some patients may develop chronic kidney disease (CKD). It is clinically important to investigate the markers of a poor prognosis. The levels of angiotensinogen (AGT) and interleukin-18 (IL-18) in serum and urine were evaluated. Study was conducted in 29 children with a history of HUS. Serum and urine AGT concentration was significantly higher in children after HUS as compared to the control group. No differences depending on the type of HUS and gender were noted. The serum concentration of IL-18 in children after HUS was significantly lower, whereas in urine did not differ significantly between the sick and healthy children. A negative correlation between the concentration of AGT in serum and albuminuria in patients after HUS was detected. The results indicate that the concentration of AGT in serum and urine in children after HUS increases, which may indicate the activation of the intrarenal renin-angiotensin-aldosterone system. The statement, that AGT may be a good biomarker of CKD after acute kidney injury due to HUS requires prospective studies with follow-up from the acute phase of the disease on a larger group of patients. Reduced IL-18 serum concentration in children after HUS with no difference in its urine concentration may indicate a loss of the protective effects of this cytokine on renal function due to previously occurred HUS., K. Lipiec, P. Adamczyk, E. Świętochowska, K. Ziora, M. Szczepańska., and Obsahuje bibliografii
A recently discussed cardiovascular risk factor, asymmetric dimethylarginine (ADMA), is known to act as an endogenous inhibitor of endothelial nitric oxide synthase. The aim of this study was to establish 1) the relationship between ADMA and ultrasonographically or biochemically determined endothelial dysfunction in children, and 2) the effect of folate supplementation on these parameters. The study cohort included 32 children with familial hypercholesterolemia (FH), 30 with diabetes mellitus type 1 (DM1) and 30 age-matched healthy children as the control group. Furthermore, twenty-eight randomly selected FH and DM1 children were re-examined after 3-months supplementation with folic acid. Baseline levels of ADMA and oxidized low density lipoproteins (oxLDL) were significantly higher in FH group than in DM1 and healthy children. Children in DM1 group had significantly lower concentration of homocysteine, but ADMA levels were normal. Folic acid supplementation significantly lowered homocysteine and hsCRP levels in both FH and DM1 group; however, ADMA and oxLDL concentrations remained unaltered. In conclusion, ADMA and oxLDL appear to be associated with endothelial dysfunction in children with FH. Administration of folic acid did not influence these markers in both FH and DM1 children., P. Jehlička ... [et al.]., and Obsahuje seznam literatury