[Hepatitis B immunization of patients with inherited bleeding disorders: personal experiences]. / Vlastní zkusenosti s ockováním proti virové hepatitidé B u pacientu s vrozenou poruchou koagulace.

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. The samples were tested for HBsAg, hepatitis B surface and core antibodies (anti-HBs, anti-HBc). Protective anti-HBs level (≥10 IU/l) after immunization was proved in 50 of 51 patients (98 %). Waning of protective anti-HBs antibodies was detected in 4 % and 24 % of patients within 5 and 10 years after vaccination. No HBsAg carrier status or anti-HBc seroconversion were detected. Subcutaneous vaccination against hepatitis B provides long-term protection in patients with inherited bleeding disorders.

[A rare complication of acute lymphoblastic leukaemia in young patients]. / Neobvyklá komplikace akutní lymfoblastické leukémie u detských pacientu.

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. The present study describes one of such rare complications, which appeared in two three-year-old girl patients, accidentally during the same time period: namely benign oesophagus stenosis. The condition was caused by several factors - first mycotic infection, histologically proved as Candida Albicans - which in one of the girls lead to septic states and the condition was generalised with more affected organs. Due to the location of the stenosis in the lower third of oesophagus, gastrooesophagus reflux played its role, too and last but not least there was a negative effect of one of the cytostatics - methotrexate - causing mycotic infections (here stomatitis and oesophagitis). For the proper development and overall well-being of a healthy organism, an optimal, sufficient and appropriate per oral reception of food is necessary. Satisfying this need becomes even more crucial in the case of young patient otherwise affected by an immunodeficient condition and an overall impoverishment of their organism. The oesophagus stenosis presents an obstacle manageable using either endoscopic methods or surgery. Although in the cases discussed the treatment was very demanding due to the age of the patients, continuous cytostatic therapy in progress, the primary disease, and the general anaesthesia - it was finally effective and successful.

[Consolidation of therapy of acute myeloid leukemia with the AML-BFM 93 protocol in children in the Czech Republic]. / Sjednocení lécby detí s akutní myeloidní leukémií v Ceské republice podle protokolu AML-BFM 93.

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. Management of children with AML was unified in the Czech Republic in 1993 according to AML-BFM 93 Study protocol. METHODS AND RESULTS: Treatment results were evaluated in 61 patients, of whom 45 (73.8%) achieved complete remission. Five-year event-free-survival (EFS) was found in 42.3%, and overall survival was 45.3%. Prognosis of the standard-risk patients was significantly better than in the high-risk group (EFS 62.5% vs. 29.7%, p = 0.03). The most important prognostic factor was the early treatment response. Compared to chemotherapy, allogeneic stem-cell transplantation did not significantly improve the outcome of high-risk patients. CONCLUSIONS: Treatment results of children with AML in the Czech Republic are comparable to those achieved by leading leukemia study groups in the world. The aim of the next study is to increase the complete-remission rate by reducing early deaths.

[Improved results in children with acute lymphoblastic leukemia treated with the ALL-BFM 90 protocol in the Czech Republic]. / Zlepsení výsledku lécby detí s akutní lymfoblastickou leukémií podle protokolu ALL-BFM 90 v Ceské republice.

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. Patients were classified into standard-risk (SR), medium-risk (MR) and high-risk (HR) group according to initial leukaemic burden, early treatment response, and genotype of leukaemia. Duration of the chemotherapy was two years. Treatment results were evaluated in 352 children. With a median follow-up of 7.3 years, event-free-survival (EFS) was 71.3% and overall survival 76.4%. EFS was 80.3%, 74% and 28.2% in SR, MR and HR group, respectively. Relapse was diagnosed in 17.8% of the patients. CONCLUSIONS: The treatment outcome of children with ALL improved significantly (p = 0.0045) compared to the previous study ALL-BFM 83 (EFS 62%). These results are comparable to those achieved by leading leukaemia study groups in the world.

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. In 11 patients, complex karyotypes have included common recurrent chromosomal abnormalities, such as translocation t(12;21) in seven cases, t(9;22) in two cases, one case with t(2;1;19) and another one with translocation involving 11q23. In 10 patients, miscellaneous abnormalities were detected. Five patients displayed hyperdiploidy (47 approximately 57 chromosomes), three patients complex karyotypes with deletions of 9p, one patient with two new complex translocations t(2;4;12;13) and t(7;11;20), and the last patient with dic(12;21). The evaluation of the frequency of the chromosomal breaks (>5 per chromosome) showed that chromosomes 2, 4, 5, 7, 9, 12, 13, and 21 were most frequently affected. Survival analysis revealed statistically significant unfavorable event-free survival (EFS) (P=0.013) and decreased overall survival in the group with complex karyotypes (n=21) compared with the other cases (n=58). The evaluation of overexpression profile revealed increased occurrence of double CD13/CD33 positivity in patients with common recurrent chromosomal abnormalities (in 70% of cases); no such cases were registered in the other group (P<0.01).

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). Among the 351 women studied, a subgroup of 27 (8%) women demonstrated CRDs during their study. Patients with CRDs were more likely to report the symptom of strain voiding (48% of patients with CRDs, 18% of patients without CRDs; chi(2)((1)) = 17.6, P < 0.001). Patients with CRDs were also more likely to have undergone prior urethral dilation (15% of patients with CRDs versus 2% of patients without CRDs, chi(2)((1)) = 9.5, P < 0.01). Patients with CRDs were similar to patients without CRDs with respect to symptoms of incontinence and history of hysterectomy or surgery for incontinence. Ten patients who had urethral CRDs underwent surgery for their GSI with a 90% success rate. The duration of post-operative catheterization did not differ from that of our general population of patients undergoing similar procedures. Urethral CRDs appear to be a non-specific finding, and it is premature to alter the approach to patients with lower urinary tract dysfunction for this reason.

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. Statistical correlations were made among prednisolone (PRED) and/or dexamethasone (DEX) LCS50 and absolute number of blast cells (ANB) on day 0/8 and a new parameter named blast cells clearance (BCC, BCC8 [%] = ANB8: ANB0 x 100) on day 8. Despite the previously published results of Ito et al. (J. Clin. Oncol. 14: 2370-2376, 1996) and Kaspers et al. (MPO 27: 114-121, 1996) on a positive correlation of DEX versus PRED LCS50 (p < 0.002), in our study, we identified 30% of children (21/69) with differential in vitro responsiveness to PRED and DEX. 16% of patients (11/69) were highly sensitive to DEX and resistant to PRED, while 14% of them (10/69) were resistant to DEX and highly sensitive to PRED. The major difference found in our and the other studies was in the processing of leukemic cells. These results were confirmed in a model experiment using the CCRF-CEM line, where we showed that sensitivity to PRED and DEX, but not to other anti-cancer drugs critically depends on manipulation with tumor cells (cryopreservation). Correlation of PRED/DEX in vitro sensitivity values with parameters of in vivo patient's response to PRED monotherapy identified significant association of PRED LCS50 with BCC8 (p < 0.02). It indicates strong linkage of in vitro sensitivity to PRED with percentage of blast cells eliminated from patient blood within the first 8 days of PRED monotherapy.

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. The association of Evans' syndrome with other immune disorders is discussed.

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. Tissue sections of malignant and normal squamous epithelium, tumors of nonsquamous origin, and normal and malignant cytological specimens were tested with an immunoperoxidase assay. Seventy-nine of 81 (98%) SCC of the head and neck, 26 of 26 (100%) SCC of the cervical and female gynecological tract, 29 of 30 (97%) SCC of the lung, 19 of 19 (100%) SCC of the oral cavity, and 17 of 17 (100%) SCC-involved neck lymph nodes reacted strongly. Various carcinomas from breast, colon, ovary, and others were unreactive. In normal squamous epithelial tissues, monoclonal antibody 17.13. reacts only with basal cells but not the cells above the basal layers. Normal tissues from heart, liver, spleen, kidney, bladder, colon, ovary, stomach, pancreas, breast, lung, prostate, thyroid, and lymph nodes were unreactive with the exception of myoepithelial cells. Monoclonal antibody 17.13. may be useful in the diagnosis and management of SCC.

Visemes observed by hearing-impaired and normal-hearing adult viewers.

A series of VCV nonsense syllables formed with 23 consonants and the vowels /a/, /i/, /u/, and / / was presented on videotape without sound to 5 hearing-impaired adults and 5 adults with normal hearing. The two-fold purpose was (a) to determine whether the two groups would perform the same in their identification of visemes and (b) to observe whether the identification of visemes is influenced by vowel context. There were no differences between the two groups either with respect to the overall percentage of items correct or to the visemes identified. Noticeable differences occurred in viseme identification between the /u/ context and the other 3 vowel contexts; visemes with /a/ differed slightly from those with / / and /i/; and there were no differences in viseme identification for / / and /i/ contexts. Findings were in general agreement with other studies with respect to the visemes identified, provided it is acknowledged that changes can occur depending on variables such as talkers, stimuli, recording and viewing conditions, training procedures, and statistical criteria. A composite grouping consists of /p,b,m/; /f,v/; /theta sigma/; /w,r/; /tS,d3S,3/; and /t,d,s,k,n,g,l/.

The effects of self-care instruction on locus of control in children.

This study investigated the effects of self-care instruction on locus of control in fifth grade children. Forty-two fifth graders attending an upper middle-class Southwestern school were randomly assigned to either a control group or an experimental group. Children in the experimental group participated in a program adapted from the Health PACT program developed by Igoe and designed to show children how to become managers in their own health care. Children in the control group attended a health discussion. The results of this study suggest that participation in self-care instruction can increase the extent to which children view health event outcomes as being due to their actions. This research provides direct implications that schools should adopt classes encouraging children to become managers in their own health care.