[The prospects for children with acute lymphoblastic leukemia of being cured has increased in the Czech Republic in the 21st century to 90% - outcome of the ALL-IC BFM 2002 trial].

Background: Acute lymphoblastic leukemia (ALL) is the most frequent childhood malignancy. Treatment has been unified in the middle of 1980 in the Czech Republic. In 2002-2007 children and adolescents with acute lymphoblastic leukemia were treated in an international randomized trial ALL-IC BFM 2002 in the Czech Republic.

Inhibitors incidence rate in Czech previously untreated patients with haemophilia A has not increased since introduction of recombinant factor VIII treatment in 2003.

Our objective was to assess the incidence of inhibitors development in Czech Republic since the introduction of recombinant factor VIII (rFVIII) and to look for the factors potentially influencing this parameter. It is to be expected that inhibitors risk may be increased after the introduction of recombinant products. Data of Czech National Haemophilia Programme registry entered from 2003 till 2013 were analysed.

Human immunoglobulin (KIOVIG®/GAMMAGARD LIQUID®) for immunodeficiency and autoimmune diseases: an observational cohort study.

Aim: To document the therapeutic efficacy and safety of Human Normal Immunoglobulin 10% Liquid (KIOVIG(®)/GAMMAGARD LIQUID(®) [IVIG 10%]) under clinical routine conditions.

Patients & Methods: Subjects received IVIG 10% according to the prescribing information and were followed for 6 ± 1 weeks to 12 ± 2 months depending on indication. Efficacy, adverse events, infusion rates and duration and dose were recorded.

Combination of prednisolone and low dosed dexamethasone exhibits greater in vitro antileukemic activity than equiactive dose of prednisolone and overcomes prednisolone drug resistance in acute childhood lymphoblastic leukemia.

Introduction: Glucocorticoids, particularly prednisone/ prednisolone and dexamethasone, play a prominent role in the treatment of pediatric patients with acute lymphoblastic leukemia due to their ability to induce apoptosis in susceptible cells. Current therapeutic protocols use prednisone for both the prophase and the induction phase of the therapy because the greater antileukemic activity of dexamethasone is compromised by its high frequency of serious adverse reactions.

Aim: To compare, for the first time, the in vitro antileukemic activity of prednisolone alone to that of a combination of prednisolone and dexamethasone using dexamethasone at a very low and presumably safe dosage (1/50 w/w).

Minimal residual disease in peripheral blood at day 15 identifies a subgroup of childhood B-cell precursor acute lymphoblastic leukemia with superior prognosis.

Background: Most minimal residual disease-directed treatment interventions in current treatment protocols for acute lymphoblastic leukemia are based on bone marrow testing, which is a consequence of previous studies showing the superiority of bone marrow over peripheral blood as an investigational material. Those studies typically did not explore the prognostic impact of peripheral blood involvement and lacked samples from very early time points of induction.

Design And Methods: In this study, we employed real-time quantitative polymerase chain reaction analysis to examine minimal residual disease in 398 pairs of blood and bone marrow follow-up samples taken from 95 children with B-cell precursor acute lymphoblastic leukemia treated with the ALL IC-BFM 2002 protocol.

Dysfibrinogenemia in childhood: two cases of congenital dysfibrinogens.

A 2-year-old asymptomatic boy and his relatives were investigated for a suspected fibrinogen mutation after coagulation tests revealed a decreased functional fibrinogen level (family A). Eight-year-old and 1-year-old asymptomatic brothers were investigated for a suspected fibrinogen mutation after coagulation tests revealed a decreased functional fibrinogen level and prolonged thrombin time (family B). To identify whether genetic mutations were responsible for these dysfibrinogens, DNA extracted from the blood was analyzed.

[Hepatitis B immunization of patients with inherited bleeding disorders: personal experiences].

Hepatitis B immunization of patients with inherited bleeding disorders: personal experiences Hepatitis B vaccination was initiated in 55 patients with inherited bleeding disorders in 1994-2009. Patients received three doses of subcutaneous recombinant vaccine containing 20 mg HBsAg (hepatitis B surface antigen) at 0, 1 and 6 months. Blood samples were obtained at the starting of vaccination, 1-3 months after immunization, and biennially thereafter.

[A rare complication of acute lymphoblastic leukaemia in young patients].

The paper presents one of the rather rare complications related to the malignant blood disease in young patients. The treatment of this extremely serious disease should be accompanied not only with number of already described complications which are thus expected but also with rare ones, whose control and treatment is demanding and rather long-term. The immense progress in medical research makes it possible to cope with a variety of serious diseases including leukaemia in young patients with one-year intensive therapy and an overall exhaustion of their organisms, where in spite of a perfect therapeutic protocol new challenges need to be met.

Obstetrics-Gynecology resident satisfaction.

Objective: The objective of the study was to identify factors associated with satisfaction in the Indiana University School of Medicine Obstetrics-Gynecology residency program and the residents' and faculty's perception of whether these factors were extant in our program.

Study Design: Residents and faculty at the Indiana University School of Medicine Obstetrics-Gynecology program were surveyed using an instrument based on prior primary care specialty investigations. Multivariate regression evaluated the impact of various factors on resident satisfaction.

Long-term serial echocardiographic examination of late anthracycline cardiotoxicity and its prevention by dexrazoxane in paediatric patients.

Unlabelled: The authors conducted an 8-year prospective non-randomised study to determine whether dexrazoxane (ICRF-187) would reduce late anthracycline-induced cardiotoxicity in patients treated in childhood for haematological malignancy. The authors examined prospectively 75 patients (40 male/35 female) aged 2-17 years (median 6.5 years) at the time of diagnosis.

The analysis of correlations between drug resistance and clinical/laboratory measures found in a group of children with all treated by ALL-BFM 90 protocol.

This study was designed to assess the predictive value of an MTT (3-[4,5-dimethylthiazol-2,5-diphenyl] tetrazolium bromide) in vitro assay for the evaluation of leukemic cell resistance/sensitivity to cytotoxic drugs, and to compare these results with clinical and laboratory parameters in cases of childhood acute lymphoblastic leukemia (ALL).The chemoresistance of leukemic cells was ascertained by means of an MTT assay in 32 previously untreated children with ALL. The children were treated using the protocol ALL-BFM 90.

[Consolidation of therapy of acute myeloid leukemia with the AML-BFM 93 protocol in children in the Czech Republic].

Background: Acute myeloid leukemia (AML) in children is rare. Although more resistant to chemotherapy than acute lymphoblastic leukemia, its responsiveness and survival rates have considerably improved during the last 15 years by virtue of intensification of chemotherapy and due to the better supportive care. Relapses still remain the main cause of treatment failure.

[Improved results in children with acute lymphoblastic leukemia treated with the ALL-BFM 90 protocol in the Czech Republic].

Background: Prognosis of children with acute lymphoblastic leukaemia (ALL)--the most common cancer in childhood, has improved remarkably over the last 40 years. The authors report the treatment outcome in children with ALL cured according to ALL-BFM 90 Study protocol in the Czech Republic during the first half of nineties.

Methods And Results: Children aged 0-18 years were included into the study in 10 centers between 1990 to 1996.

Complex karyotypes in childhood acute lymphoblastic leukemia: cytogenetic and molecular cytogenetic study of 21 cases.

Cytogenetic and molecular cytogenetic analysis of 79 childhood acute lymphoblastic leukemias (ALL) revealed chromosomal abnormalities in 76 (96%). Complex karyotypes (a finding of three and more chromosomal aberrations in a karyotype) were identified in 21 (26.6%) out of 79 patients.

Complex repetitive discharges during urethral sphincter EMG: clinical correlates.

Complex repetitive discharges (CRDs) are a form of abnormal needle electromyographic (EMG) activity associated with a variety of neuromuscular disorders, including chronic denervation. Urethral CRDs have also been associated with symptoms of voiding dysfunction. We reviewed the findings of 351 consecutive urethral sphincter EMG studies to characterize the patients with CRDs in our patient population and further to characterize the peri-operative course of any patient with urethral CRDs who underwent surgery for genuine stress incontinence (GSI).

Differential antileukemic activity of prednisolone and dexamethasone in freshly isolated leukemic cells.

This study was designed to compare the antileukemic activity of prednisolone and dexamethasone in childhood acute lymphoblastic leukemia (ALL) under in vitro conditions. The chemoresistance of leukemic cells was ascertained by means of a MTT assay in 69 ALL children at diagnosis and the concentration killing 50% of leukemic cells (LCS50) was determined. The children were treated using the protocol ALL-BFM 90/95.

Evans' syndrome in a child with diabetes mellitus.

A patient suffering from infantile-onset insulin-dependent diabetes mellitus is reported in whom immune pancytopenia (Evans' syndrome) developed at the age of 2 1/2 years. Hepatosplenomegaly, chronic lymphadenopathy, and elevated levels of immunoglobulins G and M were also present. The course of Evans' syndrome was fatal in this patient.

Hb Nottingham or alpha 2 beta 2 98 (FG5) Val-->Gly in a Czech child.

We report a fourth case of Hb Nottingham [alpha 2 beta 2 98 (FG5) Val-->Gly] observed in an 8-year-old girl in the Czech Republic with clinical and laboratory symptoms of severe hemolytic anemia. The unstable hemoglobin probably represents a de novo mutation, since the parents of the patient and the two siblings do not exhibit any hematological abnormalities. Splenectomy had a beneficial effect on the degree of hemolysis, as well as on the Hb level.

Reactivity of monoclonal antibody 17.13. with human squamous cell carcinoma and its application to tumor diagnosis.

Monoclonal antibody 17.13., derived from a fusion of splenocytes of a BALB/c mouse immunized with a surgically resected poorly differentiated human laryngeal recurrent squamous cell carcinoma (SCC) with mouse Sp2/0 cells, is an IgM-K which recognizes a cytoplasmic component of basal cells.