Acute immune trobocytopenic purpura in pregnant adolescent.

OBJECTIVE: The article reviews the causes of thrombocytopenia in pregnancy and the basic examination scheme. Further, it deals in more details with diagnosis of immune thromocytopenic purpura (ITP) and presents a case report of a rare case of acute severe ITP in pregnant adolescent. DESIGN: Review article and case report. SETTINGS: Department Obstet/Gynecol Uni J. A. Purkyně and Masaryk´s Hospital, Ústí nad Labem; Children and adolescent dep. Hospital Most; Institute of Hematology and Blood Transfusion Prague; Department of Children Med. Uni J. A. Purkyně and Masaryk´s Hospital, Ústí nad Labem; Department of Clinic Hematology Masaryk´s Hospital, Ústí nad Labem; Department of Neonatology Uni J. A. Purkyně and Masaryk´s Hospital, Ústí nad Labem. METHODS: Based on a review of the published papers, general rules for diagnosis, treatment, fetal risks, pregnancy management and labor in women with immune thrombocytopenic purpura (ITP) are given. Further we present the case of a 16-year-old pregnant girl with an acute severe form of ITP not responding to corticosteroid treatment who underwent twice critical decline of platelets up to 1×109 and repeatedly it was necessary to access the application of IVIG and Azathioprim (Imuran) was also used in her treatment. Pregnancy was unplanned terminated for dg. placental abortion in gestational age 32+4.The girl of 1740 g/42 cm was born with Apgar score 10-10-10 with no signs of thrombocytopenia. Surgery and postoperative course in the mother without complications. The newborn was discharged from the hospital into home care at a gestational age of 36+1 along with his mother. RESULTS: We present a case of acute severe form of ITP in pregnancy with a successful though somewhat dramatic end. CONCLUSIONS: These rare cases are demanding in terms of logistical, personnel and material provision. The aim of the treatment is to achieve a satisfactory maturity of the fetus, the treatment is financially demanding and is not without risks. Therefore, these cases should only be dealt with at Perinatology Centers in close cooperation with hematologists and neonatologists.

Antioxidant and prooxidant properties of flavonoids.

The interest in possible health benefits of flavonoids has increased owing to their potent antioxidant and free radical scavenging activities observed in vitro. Nevertheless, the antioxidant efficacy of flavonoids in vivo is less documented and their prooxidant properties have been actually described in vivo. Due to their prooxidant properties, they are able to cause oxidative damage by reacting with various biomolecules, such as lipids, proteins and DNA. Hence, the aim of this review is to discuss both the antioxidant and prooxidant effects of flavonoids.

Wilson's disease: monocentric experiences over a period of 10 years [corrected].

BACKGROUND: Wilson's disease (WD) is an autosomal recessive disorder of copper metabolism. The objective of this study is to present diagnostic pitfalls and long time follow-up data in Wilson disease. PATIENTS/METHODS: We studied 21 WD patients and 14 heterozygote carriers aged 2-43 years, retrospectively. 18 WD patients presented liver disease, three had mixed neurological and hepatic involvement and 9 patients underwent orthotopic liver transplantation (OLT). RESULTS: The median age at diagnosis of WD children without OLT was 10.16+/-3.8 (range, 5-16). All of females and younger age categories of patients prevailed in acute liver failure group. Serum ceruloplasmine levels were below 0.2 g/l in about (1/3) of WD carriers (X =0.27+/-0.09 g/l) and nearly (2/3) of children with WD (X = 0.21+/-0.13 g/l). A statistically significant difference (p<0.05) in the 24-h excretion of copper in urine was noticed between healthy controls, children with WD and WD heterozygote carriers. As diagnostic important proved the copper content of more than 250 microg/g hepatic dry weight. The Kayser-Fleischer?s ring was not observed in children. Ceruloplasmine, haemoglobin, ALT, ALP and plasma albumin were significantly different between fulminant and non-fulminant WD and could be used as indirect markers in evaluation of urgent OLT. CONCLUSION: Detection of WD in children remains very difficult. The most important investigation is liver biopsy with the assessment of liver copper. Genetic analysis may help in doubtful cases.

Haemolytic anaemia and acute liver failure--the initial manifestations of Wilson's disease.

We describe a case of a 16-year-old girl with Wilson disease, which was initially presented as Coombs-negative haemolytic anaemia and acute liver failure. The diagnosis was based on the findings of low ceruloplasmin serum level and high copper levels both in serum and 24-hour urinary excretion. The patient underwent orthotopic liver transplantation. A DNA-based diagnostic tool confirmed Wilson's disease: the patient was p.H1069Q homozygote. Based on further molecular-genetic examinations in the family, Wilson disease was diagnosed seven days later in one of the patient's asymptomatic brothers. The proband's cousin was confirmed as a carrier of the p.H1069Q mutation (Fig. 1, Ref. 24).

The capacity of antioxidant protection during modulated ageing of bean (Phaseolus vulgaris L.) cotyledons. 2. The low-molecular weight antioxidants.

The aim of the present work was to evaluate non-enzymic antioxidants during natural and artificially modulated senescence. Senescence of bean (Phaseolus vulgaris L. cv. Jantar) cotyledons was modulated by UV C irradiation or by the decapitation of plants apices. The content of beta-carotene and zeaxanthin decreased in control and decapitated plants but in UV C irradiated plants these contents increased. The degree of de-epoxidation increased in all cultivations with age. The content of total glutathione (sum of reduced and oxidized) sharply decreased in bean cotyledons grown in all conditions. Interestingly, the content of total ascorbate increased at the end of cotyledon life span of control and decapitated plants but decreased in UV plants. Decrease of reduced/oxidized ratio of ascorbate and glutathione during cotyledon ageing confirmed increasing oxidative stress during senescence in all cultivations.

The capacity of antioxidant protection during modulated ageing of bean (Phaseolus vulgaris L.) cotyledons. 1. The antioxidant enzyme activities.

Reactive oxygen species are known to increase in plant senescence. We investigated the participation of antioxidative enzymes in initiation of cotyledon senescence. Senescence of bean (Phaseolus vulgaris L.) cotyledons was modulated by UV C irradiation and by the decapitation of plant apices. Senescence was accompanied by a decrease of protein content and by a decrease of photochemical efficiency. A drop in activity of antioxidative enzymes preceded the onset of senescence in control plants. In cotyledons with prolonged life span, the decrease of antioxidant activities and the markers of senescence onset appeared at a similar age as in controls. Thus we presumed that the period from senescence initiation to cotyledon abscission was extended. On the other hand, in UV C irradiated plants we did not observe actual senescence initiation, and antioxidant enzymes although elevated, did not effectively play their role. The decrease of antioxidant enzymes activity and the markers of senescence appeared at a similar age both in control and in decapitated (D) plants, so we can presume that we prolonged mainly the period from senescence onset to cotyledon abscission in D plants. In UV C irradiated plants the antioxidative enzymes were probably destroyed before the process of senescence could begin.

The effect of plant cytokinin hormones on the production of ethylene, nitric oxide, and protein nitrotyrosine in ageing tobacco leaves.

Transgenic plants with genetically increased or decreased levels of cytokinins were used to investigate the effect of cytokinin level on the production of ethylene, a plant hormone with suggested role in senescence, and the production of nitric oxide, potentially important signalling and regulatory molecule. The production of these gases was followed during the course of leaf development and senescence. The production of ethylene and nitric oxide is under genetic control of genes other than those involved in regulation of senescence. The difference in basic ethylene and NO levels in different tobacco cultivars was higher than their changes in senescence. The results of this study did not indicate a direct link between ethylene production and cytokinin levels. However, there was a decreased production of NO in senescent leaves. Low cytokinins level was associated with increased NO production during leaf development. Protein nitrotyrosine proved to be a better indicator of the reactive nitrogen species than measuring of the NO production. Higher nitrotyrosine concentrations were found in insoluble proteins than in the soluble ones, pointing to membrane proteins as the primary targets of the reactive nitrogen species. In plants with elevated cytokinin levels the content of nitrated proteins decreased both in soluble and insoluble fractions. This finding indicates an antioxidative function of cytokinins against reactive nitrogen species.

[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.

[Amplification of 2p13-16 in a case of extranodal large cell diffuse B-cell lymphoma]. / Amplifikace 2p13-16 u prípadu extranodálního velkobunecného difuzního B-lymfomu.

Comparative genomic hybridisation (CGH) allows to detect genetic abnormalities associated with poor prognosis in subset of patients with diffuse large B-cell lymphoma (DLBCL). Amplification of 2p13-16 represents an adverse genetic sign especially in extranodal DLBCL. In the present case, 2p13-16 amplification was revealed by CGH in extranodal DLBCL. It was localized in the mesentery and remained resistant to the chemotherapy (CHOP). A patient, 72-year-old female, died 10 weeks after the diagnosis had been made. Rapid lethal course of the disease confirms poor outlook for patients with 2p13-16 and supports a role of CGH as a sensitive method in prognosis for patients with DLBCL.

[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.

[Retinoblastoma in monozygotic twins]. / Retinoblastom u monozygotních dvojcat.

Retinoblastoma is one of the most frequent ophthalmological tumours in children. It is an embryonic tumour originating in the retina. It is caused by abnormalities in the RB1 gene and deletions in the region 13q14. The authors present a bilateral non-hereditary retinoblastoma in monozygotic twins, associated with deletion in the region 13q14, stigmatization, psychomotor and somatic retardation.

[Molecular analysis of Wilson disease]. / Molekulární analýza Wilsonovy choroby.

BACKGROUND: Wilson disease is an autosomal recessive disorder, characterized by cooper accumulation and intoxication of the organism. Molecular basis of the disease represent mutations in the gene for the copper-transporting ATPase (ATP7B). METHODS AND RESULTS: The submitted paper deals with results of molecular-genetic examination in 130 unrelated families in which Wilson disease was diagnosed. By denaturing gradient gel electrophoresis (DGGE), the exons with abnormal sequences were detected. Followed by sequencing, 17 causal mutations and 9 silent polymorphism were found. Five novel mutations were detected. After analysis of 260 mutant alleles, 214 (82.3%) were identified. The most frequent mutation, H1069Q, occurred in our population with the frequency of 65.8%. Incidence of other mutations, however, did not exceed 5%. CONCLUSIONS: DNA analysis of the Wilson disease offers prompt and reliable results in affected families. It can help to identify asymptomatic and heterozygote siblings at genetic counselling.

Theoretical background of environmental monitoring and its conception in the Czech Republic.

This paper deals with the subject aspects connected with environmental monitoring. It summarizes the survey as to the level of knowledge of the environment and the main tasks that must be solved to ensure the sustainable development of the environment. State-of-art of the knowledge and character of the data sets that are susceptible to the environmental stage description show that it is useful to apply a system approach using the methodology of expert systems to solve the relevant problems of environmental policy. The present concept of environmental monitoring takes into consideration the level of knowledge and main tasks that must be solved to obtain the relevant information for decision-making. Therefore, a reliable set must be established that helps us to introduce expert methods during the preparation of bases for decision-making, i.e. [Symbol: see text] data base of stage variables of the environment, i.e. structural units of the environment with mutual relations; [Symbol: see text] data base of quantities describing a set of flows through the component parts; [Symbol: see text] data base of quantities describing process-response mechanisms, i.e. mechanisms that determine a dynamic behaviour of the environment. The problem is studied with reagard to the situation in the Czech Republic because the author's aim is to compile a state environmental monitoring; its concept is briefly described in the latter part of the paper. A very pragmatic approach to the problem is chosen for its realization.

[The microcomputer in the office of the community pediatrician]. / Mikropocítac v ordinaci obvodního pediatra.

The material submitted is a draft model system for the use of a microcomputer in the daily working activity of the community paediatrician. His work comprises preventive and therapeutic care of children and he has specific tasks and specific orientation on different age groups and the ensuing administrative work. The introduction of the information system in the surgery gives an idea of its asset for the user.