Haemophilia - unexpected complication of rhinoplasty.

Authors report a case of unexpected postoperative bleeding after an aesthetic rhinoplasty that was due to previously undiagnosed asymptomatic haemophilia.

[Diagnosis and treatment of BCR/ABL-negative myeloproliferative diseases--principles and rationale of CZEMP recommendations]. / Diagnostika a lécba BCR/ABL-negativních myeloproliferativních onemocnení--principy a výchdiska doporuceni CZEMP.

In 2009, the recommendations of the Czech Collaborative Group for Ph- Myeloproliferative Diseases (CZEMP) for diagnosis and treatment of BCR/ABL-negative myeloproliferative diseases (MPD), i.e., essential thrombocythemia (ET), polycythaemia vera (PV) and primary myelofibrosis (PMF) were updated and extended. The present article gives the rationale of the recommendations in full detail. The CZEMP diagnostic criteria for ET and PMF are based on histopathological (HP) findings, which must unconditionally be in line with the given clinical and laboratory characteristics of ET or of a certain stage of PMF, respectively. The platelet count is not decisive for diagnosis. In cases lacking an adequately taken and read HP finding, the Polycythemia Vera Study Group (PVSG) criteria are recommended. The diagnosis of typical PV is based on demonstration of the V617F mutation of the JAK2 gene along with a significant increase of red cell parameters. If these are close to borderline, the demonstration of increased total red cell mass (RCM) is required. In atypical cases lacking polyglobulia or elevated RCM, the HP picture of PV (in accordance with WHO description) plus JAK2 V617F mutation is satisfactory for diagnosis, or, in cases lacking JAK2 V617F mutation, the HP picture of PV along with polyglobulia (or increased RCM) is sufficient. The treatment principles of ET and other MPDs with thrombocythemia (MPD-T; i.e., the early stages of PMF and PV) are identical. The patients are stratified by their thrombotic risk (preceding thrombosis, another thrombophilic state, jAK2 mutation), presence of disease symptoms (mainly microcirculatory), platelet count and age. Only patients up to 65 years lacking the above mentioned risks with a platelet count < 1000 x 10(9)/l are considered as low-risk and do not demand cytoreducing therapy. The others are high-risk ones and have an indication for thromboreduction. In patients older than 65 years, the potentially leukemogenic drug hydroxyurea (HU) may be used. In the younger ones, the choice is between anagrelide (ANG) or interferon-alpha (IFN). In high-risk patients, the treatment goal is to maintain platelet counts below 400, and in low-risk ones, below 600 x 10(9)/l. In PV, polycythemia itself is another thrombotic risk factor. The condition is treated by bloodletting or erythrocytaphereses. If hematocrit levels < or =45 are not achieved, cytoreductive therapy using HU in patients over 65 years, or IFN in younger individuals is required. All patients with thrombocythemia in PV are high-risk and have an indication for cytoreduction. Acetylsalicylic acid is given to all patients with MPD-T with platelets < 1000 x 10(9)/l (at higher counts, hemorrhage is imminent), and to all individuals with PV, unless contraindication is present. In case of platelet count normalization, it may be withdrawn in cases of low-risk ET or PMF, not in JAK2+ PV. The treatment of advanced stages of PMF is symptomatic, with substitution of blood derivatives being the basis. The only curative treatment is allogeneic stem cell transplantation, which should not be indicated too early seeing to its risks, but also not too late--we must not allow transition into acute leukemia, which is heralded by blasts in the blood picture. The indication is the presence of any of the following criteria: values of hemoglobin < 10 g/dl, WBC < 4 x 10(9)/l and platelets < 100 x 10(9)/l, any percentage of blasts or > or = 10% immature granulocytes in the differential picture, >1 erythroblast per 100 cells--all at repeated examinations within at least a 2-month interval, and in addition, rapid progression of hepato-/splenomegaly, presence of general symptoms of the disease, portal hypertension and extensive swellings.

[The results of patients with essentials thrombocythemia and other myeloproliferation-related thrombocythemia--a report of patients treated with Thromboreductin]. / Výsledky lécby nemocných s esentiální trombocytemií a dalsími myeloproliferacemi provázenými trombocytemií--zpráva z registru pacientu lécených Thromboreductinem.

The registry of patients treated with Thromboreductin (anagrelide) in the Czech Republic contains data concerning patients that have been treated using this drug since 2004. As of June 2009, the total number of patients was 549. The current analysis focused mainly on evaluation of anagrelide dosage needed to achieve a complete response in high-risk patients: reduction in platelet count to below 400 x 10(9)/l, which was also considered as reaching the therapeutic goal. The outcomes of the registry confirm that although anagrelide (Thromboreductin) is a very effective platelet-reducing agent, the administration of which is related to a low incidence of adverse effects and complications, the therapeutic goal is not achieved in all cases and or despite a quick treatment response, the therapeutic goal is achieved more slowly.

[What is the current treatment of patients with essential thrombocytopenia and other myeloproliferations accompanied with thrombocythemia [corrected] and what can be the predictive sign of the risk of thrombosis in such patients--a report from the registry of patients treated by Thromboreductine]. / Jak lécíme nemocné s esenciální trombocytemií a dalsími myeloproliferacemi provázenými trombocytemií a co muze být prediktivní známkou rizika trombózy u techto nemocných--zpráva z registru pacientu lécených Thromboreductinem.

The registry of patients treated with Thromboreductine (anagrelid) in the contributing centres in the Czech Republic has been updated with data on the patients receiving this medication since 2004. The original purpose of the registry was to record responses to Thromboreductine therapy and adverse drug reactions in patients with essential thrombocytopenia. However, data on additional Ph negative myeloproliferations, as well as data on cytoreductive therapies other than exclusively that using Thromboreductine has also been recorded in the course of its compilation, including data on combined regimes. At present, the database contains data on 421 patients, and valid conclusions can be drawn if the level of data filling is enhanced. Evaluation has been currently focused on the analysis of the risk of development of clinical symptoms of thrombosis and on the standards of treatment from the viewpoint of the achieved treatment response. Analyses of data from the registry corroborate the special importance of the proof of JAK2 mutation, and of the test for factor V Leiden mutation, and of protein of S for the assessment of the risk of thromboembolic complications. The output of the analysis confirms that anagrelid is a very efficient thromboreductive agent the administration of which is associated with a low incidence of non-serious adverse effects (10.9%). However, in spite of a fast response to therapy, the therapeutic goal consisting in the reduction of the platelet count below 400 (or below 600) x 10(9)/l, i.e. the complete (or partial) treatment response, is relatively slow to achieve. This is likely to be due to lack of radical corrections in the dosage of the drug for different reasons.

[Essential thrombocytemia and other myeloproliferations with thrombocytemia in the data of the register of patients treated with Thromboreductin till the end of 2006]. / Esenciálni trombocytemie a dalsí myeloproliferace s trombocytemií v údajích registru pacientu lécených Thromboreductinem do konce roku 2006.

Since 2005, registers of patients treated with Thromboreductin (anagrelid) kept by some centres in the Czech Republic have been supplied with data concerning patients whose treatment with this preparation started in 2004. The purpose of the register is to record responses to therapy by Thromboreductin and adverse events in patients with essential thrombocytemia and other myeloproliferations, and to subsequently analyse the data. Another objective is to detect predisposition to clinical symptomatology and disease complications. Apart from thrombocyte count, additional risk factors are monitored. The database currently contains data for 336 patients. Initial analyses of data from the register point to the fact that anagrelid is a highly effective thromboreductive agent the administration of which is associated with relatively low incidence of adverse events (11.8 %) of mild and usually transitory nature. The therapeutic objective is attained at a relatively slow rate (according to overall stratification under 400 or under 600 x 10(9)/l thrombocytes), which is probably due to insufficient dose adjustment.

[The use of nitric oxide in the treatment of acute respiratory failure]. / NO v lécbe respiracního selhání.

BACKGROUND: Nitric oxide was used as an important selective vasodilator in the treatment of acute respiratory failure accompanied with high pulmonary resistance in children and adults since late 80's. METHODS AND RESULTS: Paper includes remarks about nitric oxide physiology in organism. Group of 33 patients is presented (group I 26 newborns, group II 7 children) in which selective pulmonary vasodilation with nitric oxide was used. According to response to NO subject were classified into subgroups of responders, non-responders. In evaluation of oxygenation status OI (oxygenation index, A-a DO2 (alveoloarterial difference) and paO2/FiO2 were used. CONCLUSIONS: Significant differences of above mentioned values were revealed between responders and non-responders in group I (newborns). Significant differences were not revealed in group II (children). Results are in accordance with other papers.

[Anagrelide in the treatment of essential thrombocythemia (ET) and other myeloproliferative disorders with thrombocythemia based on data from patient register in the CR]. / Anagrelid v lécbe esenciální trombocytemie a dalsích myeloproliferací s trombocytemií sledovaných v registru pacientu v CR.

Anagrelide hydrochloride is an effective drug used in patients with ET and other myeloproliferative disorders with thrombocythemia to selectively decrease the number of thrombocytes. Indications for use of anagrelide were described in detail in Czech medical literature. Since 2005 data concerning treatment with anagrelide in some medical clinics have been collected in patient register showing course of treatment from 2004, when the medicament obtained marketing authorization from State Institute for Drug Control to be used in the treatment of thrombocythemia in myeloproliferative disorders. Aim of patient register is to monitor medical effect of anagrelide therapy and incidence of adverse effects in patients with ET and other myeloproliferative disorders and subsequent analysis of collected data. At the moment patient register contains data from 154 patients.

[Sodium nitrite poisoning in a neonate]. / Otrava novorozence dusitanem sodným.

Natrium nitrosum intoxication is usually associated with a subsequent methemoglobinemia. Beside it, nitrates can cause also some other pathological states. Treatment with the toluidine blue may have various adverse side effects. Newborn intoxication by natrium nitrosum developing after the intoxication of the bearing mother before the parturition has not been described yet. Our own observation is referred.

[Relation between levels of vitamins C, E, A and beta-carotene and activity of antioxidant enzymes in the blood]. / Vztah hladín vitamínov C, E, A a beta-karoténu k aktivite antioxidacných enzýmov v krvi.

UNLABELLED: Antioxidant protection is procured by many enzymatic and non-enzymatic factors which maintain the physiological level of reactive forms of oxygen. The aim of this study was to determine the levels and relationship between concentrations of the selected non-enzymatic antioxidants in healthy people (vitamin C--Vit C, vitamin E--Vit E, beta-carotene-beta-Car and vitamin A--Vit A) in the serum, antioxidant enzymes, CuZn-superoxide dismutase--SOD, catalase CAT, glutathione peroxidase--GPx) in erythrocytes and malondialdehyde (MDA) in the serum. We have examined 128 volunteers (57 men and 71 women) at the age ranging between 20 and 72. The average levels of vitamins and beta-Carotene were detected near the lower level of normal values or below it, whilst the highest interindividual differences were detected in Vit C and Vit E. The level of Vit E indirectly correlated with SOD, GPx, MDA, but directly with CAT. On the contrary, the levels of Vit A directly correlated with SOD, GPx, MDA and indirectly with CAT. The levels of Vit C and beta-Carotene had no relation with the activities of antioxidant enzymes and the level of MDA. The direct correlation was between the levels of Vit C and Vit E, and Vit C and beta-Carotene. CONCLUSIONS: 1. A decrease in average levels of Vit A, C, E and beta-Carotene in the blood of the investigated group of healthy persons indicates their lower antioxidant protection. 2. The relation between Vit E, MDA, activities of GPx and SOD indicates the presumed mutually supplementing effect of these antioxidants in liquidation of products of lipoperoxidations. (Tab. 2, Fig. 3, Ref. 17.)

[Biochemical and hematologic indicators in the blood of young vegetarians]. / Niektoré biochemické a hematologické ukazovatele v krvi mladých vegetariánov.

For the purpose of judgement of the vegetarian population state of health and nutrition 41 vegetarians were examined, namely university students in the age of 18-24 years, 36% of which were lacto-vegetarians and 64% lacto-ovo-vegetarians, 22 men and 19 women. Low values of cholesterol and triacylglycerols, content of HDL-cholesterol to 32% (close to the value 1.4 mmol per litre), low atherogenic index, low values of blood pressure and no case of obesity are favourable factors in the prevention of atherosclerosis. The only exception is represented by low values of vitamin C which neither in autumn period achieved the bottom value of the physiological range (low intake of fruit and vegetables). Saturation of organism with proteins is good (intake of full-value mixture of aminoacids in the form of plant and milk proteins), the values of glycemia were on the level of the bottom border of the physiological range. Low values of hematological parameters were detected. The erythrocyte count and levels of hemoglobin and hematocrit were on the bottom border of the physiological range; the values of serum iron in the bottom half of the physiological standard. The vegetarians consumed less fruit and vegetables (vitamin C facilities the conversion of the ferri-form into ferro-form), more cerials (linkage of iron with the phytic acid), and only scarcely the foliage vegetables. The incidence of anemias was equal with that in non-vegetarians in the age of 17-18 years (epidemiologic study of the institute). The supply of organism by vitamins B1,B2 and B6 were within the physiological standard.(ABSTRACT TRUNCATED AT 250 WORDS)

Plasma lipids, lipoproteins and atherogenic index in men and women administered vitamin C.

The aim of the study was to establish whether it is possible, in a group of deliberately selected subjects with hyperlipidaemia, to modulate cholesterol levels by ascorbic acid administered at a dose of 500 mg/day. The authors assessed the levels of vitamin C, total and HDL cholesterol, triacylglycerols in the blood serum of 140 probands assigned to an 83-member experimental group, and to a 57-member control group. The experimental group was provided Celaskon effervescens Spofa at a dose of 500 mg/day/person. The experiment lasted for 18 months. Blood collections were made in the whole cohort at six-month intervals. Administration of L-ascorbic acid led to a highly significant decrease in the levels of total and LDL cholesterol. After 12 months of study, a highly significant decrease in atherogenic index and an increase in HDL cholesterol levels were found persisting until the end of the experiment.

[Hearing and treatment of neonates with gentamycin]. / Sluch a lécba novorozencu Gentamycinem.

The authors examined two groups of children treated during neonatal period with high doses of Gentamycin. The reasons for Gentamycin treatment at the intensive care unit were infection of the CNS, pneumopaties, intracranial haemorrhagiae, cerebral oedema and other causes. The first group of children was treated in 1981, the second one in 1986. Both groups were examined in 1987. All children were evaluated to objective hearing tests, impedance audiometry by acoustically evoked potentials of the brain stem. Children of the first group examined during their fifth or sixth year were also submitted to a hearing test. The control group was formed by children born in 1986 treated at the intensive care unit with similar indications but who were not given Gentamycin. A total of 51 children were examined. Twenty-two children examined at the age of five had no hearing impairment. Acoustically evoked potentials of the brain stem have a wide variability which may be the manifestation of the disease which led to admission to the intensive care unit. Therefore it should be conceived as a screening examination and the revealed pathological findings should be verified by repeated examinations. Possible hearing disorders should be confirmed at a later age by conventional examinations.

Hypocholesterolemic effect of ascorbic acid in maturity-onset diabetes mellitus.

A significantly lower vitamin C concentration has been found in the blood and particularly in the leukocytes of hypercholesterolemic diabetic patients than of healthy blood donors. Ascorbic acid administered in a dose of 500 mg per day for 12 months to metabolically stabilized hypercholesterolemic subjects with maturity-onset diabetes mellitus (diabetic diet without insulin or diabetic drugs) brought about a striking decline of cholesterolemia and a moderate decline of triglyceridemia. The serum lipid level in the control group given placebo remained unaltered. A daily administration of 500 mg of ascorbic acid for six months failed to affect the fasting level of serum immunoreactive insulin. It is assumed that the long-term administration of ascorbic acid to maturity-onset diabetics removed the tissue ascorbate deficiency and improved the liver ability to compensate the increased endogenous synthesis of cholesterol by its enhanced transformation to bile acids.