Abstract: Aims : To demonstrate the origin and the diagnostic significance of non-cholesterol sterols (NCSs) in healthy pregnant women with gestational hypercholesterolemia. Patients and Methods: Based on a total of 21,000 clinical biochemistry tests of healthy pregnant women with hypercholesterolemia observed during pregnancy, a group of 84 women with TC (total cholesterol) >7.0 mmol/L was recruited to analyze their NCSs using Gas Chromatography–Mass Spectrometry. The NCSs under examination comprised lathosterol (Lat) and desmosterol (Des) as markers of endogenous cholesterol synthesis, and campesterol (Cam) and sitosterol (Sit) as markers for intestinal absorption. Results : In the total of 21,000 pregnant women, the median values were: TC 6.8 mol/l, LDL-C 4.6 mmol/L, and HDL-C 2.2 mmol/L. In the testing group of 84 women, the average values were: Lat 7.8+/-1.7 I¼mol/L, Des 4.7+/-0.9 I¼mol/L, Cam 9.8+/-2.6 I¼mol/L, and Sit 9.6 +/-3.8 I¼mol/L. Lat was found to correlate with TC (r = 0.53), LDL-C (r = 0.36), and non-HDL-C (r = 0.35). No such correlations were observed for Sit (r = 0.162) or Cam (r = 0.153). Conclusion : Our findings show that the high incidence of hypercholesterolemia during pregnancy is caused by increased endogenous cholesterol synthesis via lathosterol. The enormous rise of TC levels during pregnancy can be effectively used to detect familial hypercholesterolemia in women.

OBJECTIVE:Analysis of gestational hypercholesterolemia incidence in Prague population of healthy pregnant women. Diagnostic significance of non-cholesterol sterols as suitable markers of endogenous synthesis and intestinal absorption in etiology of gestational hypercholesterolemia.DESIGN:Retrospective study.PATIENTS AND METHODS:From 21 000 healthy pregnant women set of 84 patients with blood level of total cholesterol >7.0 mmol/l where noncholesterol sterols had been analyzed by use of GC/MS method on Finnigan MAT 120b. Lathosterol and desmosterol as markers of endogenous syntesis and campesterol and sitosterol as markers for intestinal absorbtion. Classical lipid parameters have been analyzed on Beckman Coulter and Cobas analyzators.RESULTS:The median of total cholesterol values in the set of 21 000 healthy pregnant women - 6,8 mmol/l was observed; median of LDLCh - 4.6 mmol/l and HDLCh - 2.2 mmol/l. The frequency of hypercholesterolemia values > 8.0 mmol/l 1:132 (!) was observed. The average values were for lathosterol 7.8 ± 1.7 µmol/l; desmosterol 4.7 ± 0.9 µmol/l; campesterol 9.8 ± 2.6 µmol/l; sitosterol 9.6 ± 3.8 µmol/l. The correlations of lathosterol with total cholesterol r = 0.524 as well as with non-HDLCh r = 0.35 and LDLCh r = 0.36 were observed. In campesterol or sitosterol as well as HDLCh or TAG no significant correlations have been observed.DISCUSSION:Pilot study for gestational hypercholesterolemias in Czech population of healthy pregnant women proved the high frequency of increased levels of total cholesterol (> 8.0 mmol/l) 1:132. Increased levels of lathosterol values could explain the hypercholesterolemia in pregnancy as result of higher endogene synthesis of cholesterol.CONCLUSION:Relatively high frequency of hypercholesterolemia in pregnancy is caused according to our findings by increased endogenous synthesis of cholesterol via lathosterol. The highly increased values of cholesterolemia during pregnancy could be efectivelly used for detection and after ending of lactation period for further differential diagnostic and treatment of previously undiagnosed familial hypercholesterolemias.

Selected ion flow tube mass spectrometry, SIFT-MS. has been used to investigate if absolute levels of trace compounds in the headspace of ethanol/water vapour mixture can be quantified. This case study was directed towards the analysis of methylamine in distilled ethanol of agricultural origin because of its relevance to quality control legislation in the distillery industry. This has required a detailed study of the ion chemistry occurring - initiated by H3O+ precursor ions - when ethanol/water vapour mixtures are introduced into the H3O+/helium carrier gas swarm and has resulted in the construction of a full scheme of the complex ionic reactions that occur. It has been found that under the SIFT-MS flow reactor conditions (He pressure 130 Pa and temperature 299 K) the terminating ions of the several parallel and sequential reactions that occur are the proton bound ethanol clusters ions. C2H5OH2+(C2H5OH)(n). with n = 1,2,3, proton bound trimer (n = 2) being the dominant species. These ethanol cluster ions can be used as precursor (reagent) ions for the chemical ionisation of the methylamine present in the ethanol/water vapour, which produces two characteristic product ions CH3NH2H+(C2H5OH)(1,2) that are used for the methylamine analysis. The ratio of the product ion count rate to the precursor ion count rate is used in an analogous way to the routinely used for SIFT-MS analyses to quantify the methylamine concentration. The results of calibration experiments show that using SIFT-MS it is possible to quantify methylamine in liquid ethanol/water mixtures at levels of 0.1 mg/L or greater. (C) 2009 Elsevier B.V. All rights reserved.

BACKGROUND:Leukotrienes (LTs) are increased in exhaled breath condensate (EBC) in patients with asthma. So far no data have been reported about LT levels in nonasthmatic patients with seasonal allergic rhinitis (SAR). The aim of the study was to find out whether the LT levels in EBC were increased in the nonasthmatic adult patients with SAR both during and after the pollen season in comparison with healthy controls and to assess the changes of the LT levels after the pollen season.METHODS:Twenty-nine nonasthmatic adult patients with SAR underwent measurement of exhaled LTs in the EBC during and after the pollen season. Leukotrienes B(4), C(4), D(4) and E(4) were analysed by a specific and sensitive gas chromatography/mass spectrometry (GC/MS) assay and compared with 50 healthy nonsmoking controls. Spirometry, skin prick tests and nonspecific IgE were evaluated.RESULTS:Leukotrienes concentrations (B(4), E(4) but not D(4)) were significantly increased in and after the pollen season in patients with SAR in comparison with healthy controls. In most of the samples, LT C(4) was undetectable. The values of all exhaled LTs were significantly decreased after the pollen season compared with the seasonal baseline: LTB(4) (P = 0.023), LTD(4) (P = 0.020), LTE(4) (P = 0.047).CONCLUSIONS:Levels of exhaled LTB(4) and LTE(4) were higher in SAR patients than in healthy controls and decreased after the pollen season as compared with levels in season. The SAR patients with the highest in season LT levels had also the post-season levels elevated and this may be an early marker of inflammatory process in the lower airways despite the absence of clinical symptoms of asthma.

Background: Leukotriene-like immunoreactivity has been detected in exhaled breath condensate (EBC), but definitive evidence for the presence of leukotrienes (LTs) in this biological fluid is not available. A study was undertaken to determine whether LTC4, LTD4, LTE4, and LTB4 are measurable in EBC by gas chromatography/mass spectrometry and to quantify exhaled LTs in adults and children with asthma and in control subjects. Methods: Twenty eight adults and 33 children with mild to moderate persistent asthma treated with inhaled corticosteroids and age matched healthy controls (50 adults and 50 children) were studied. LTB4, LTC4, LTD4, and LTE4 in EBC were measured by gas chromatography/mass spectrometry. Results: LTD4, LTE4, and LTB4 were detectable in all samples. Concentrations of LTC4 in EBC were either close to or below the detection limit of 1 pg/ml. Median exhaled LTD4, LTE4, and LTB4 concentrations in asthmatic adults were increased 4.1-fold (p<0.001), 1.8-fold (p<0.01), and 2.6-fold (p<0.001), respectively, compared with values in healthy adults. Median exhaled LTD4, LTE4, and LTB4 concentrations in asthmatic children were increased 2.8-fold (p<0.001), 1.3-fold (p<0.001), and 1.6-fold (p<0.001), respectively, compared with those in healthy children. In patients with asthma there was a correlation between exhaled LTD4 and LTE4 in both adults (r = 0.87, p<0.0001) and children (r = 0.78, p<0.0001). Conclusions: Gas chromatography/mass spectrometry can be used to accurately quantify exhaled LTs which are increased in asthmatic adults and children compared with controls.

Considerable interest of specialists all over the world has focused on the measurement of the markers of inflammation and oxidative stress in the exhaled breath condensate in patients with asthma or chronic obstructive pulmonary diseases recently. Use of exhaled condensate is based on the hypothesis that aerosol particles exhaled in human breath reflect the composition of the bronchoalveolar extracellular lining fluid. The standard collection of the material requires condensation of exhaled air and the samples have to be kept in biologically inert containers. Measurement of the very low concentrations of selected substances requires very sensitive analytical methods. The examination of exhaled breath condensate is absolutely non-invasive method, which can be repeated as often as needed and it is extremely well tolerated both by children and seniors. Markers in the condensate enable detection and quantification of the inflammation process, the disease monitoring, and assessment of the response to the treatment. The breath condensate diagnostics is a new progressive method and in the patients with asthma and chronic obstructive pulmonary disease it can bring complementary information to the very sensitive method of determination of exhaled nitric oxide.

Propionyl-CoA is a common product of metabolism of essential AA (Val, Thr, Leu, Ile), fatty acids and cholesterol and is further metabolised enzymatically to methyl-malonyl CoA, which is further isomerised to succinyl CoA. Because of the common cofactor (cobalamin) methylmalonic acid (Mma) can serve as an early marker of functional cobalamin deficiency. Purpose of the study: To evaluate possible hidden cobalamin deficiency and the effect of treatment with iv. vit B12 on Mma and tot. homocystein (tHcy) levels in dialysis patients (pts). Methods: 48 HD treated pts, in routine medication folic acid 5 mg/d, pyridoxin 20 mg/d. Measurement of serum levels of Mmk, folic acid, tHcy, cobalamin, blood counts. Healthy patients served as controls for Mma. Vit B12 therapy – cyanocobalamin 1 mg iv. after HD every week for 6 weeks, after 6 weeks 1mg monthly. Results: HD patients: before B12 treatment after B12 Methylmalonic acid 0,904 +/− 0,389 0,554 +/− 0,222 Total homocystein 21,653 +/− 6,851 16,055 +/− 4,896 Mma in EPO treated pts: 0,842 +/− 0,325 0,536 +/− 0,234 Mma in EPO non-treated: 1,028 +/− 0,482 0,590 +/− 0,199 Healthy controls: Mma = 0,305 +/− 0,158 umol/l (max. norm. 0,22) Conclusions: We did not find overt vitamin B12 deficiency in HD pts, however elevated levels of Mma and tHcy raise a question about functional B12 deficiency. Vitamin B12 supplementation improved (decreased) the levels of both methylmalonic acid and homocystein in this subset of patients. Influence of EPO treatment merits further study.

Journal of Inherited Metabolic DiseaseVolume 14, Issue 2 p. 265-266 Case Report Mevalonic aciduria V. Kožich, V. Kožich Centre for Inherited Metabolic Disorders, Charles University, Prague, CzechoslovakiaSearch for more papers by this authorK. M. Gibson, K. M. Gibson Baylor Medical Center, Dallas, Texas, USASearch for more papers by this authorJ. Zeman, J. Zeman Department of Paediatrics, Charles University, Prague, CzechoslovakiaSearch for more papers by this authorJ. Němeček, J. Němeček Institute of Microbiology, Czechoslovak Academy of Sciences, Prague, CzechoslovakiaSearch for more papers by this authorG. F. Hoffman, G. F. Hoffman Children's Hospital, University of Göttingen, FRGSearch for more papers by this authorF. Pehal, F. Pehal Centre for Inherited Metabolic Disorders, Charles University, Prague, CzechoslovakiaSearch for more papers by this authorJ. Hyánek, J. Hyánek Centre for Inherited Metabolic Disorders, Charles University, Prague, CzechoslovakiaSearch for more papers by this authorA. Grosmanová, A. Grosmanová Department of Child Neurology, Medical Postgraduate Institute, Prague, CzechoslovakiaSearch for more papers by this authorP. Verner, P. Verner Centre for Inherited Metabolic Disorders, Charles University, Prague, CzechoslovakiaSearch for more papers by this author V. Kožich, V. Kožich Centre for Inherited Metabolic Disorders, Charles University, Prague, CzechoslovakiaSearch for more papers by this authorK. M. Gibson, K. M. Gibson Baylor Medical Center, Dallas, Texas, USASearch for more papers by this authorJ. Zeman, J. Zeman Department of Paediatrics, Charles University, Prague, CzechoslovakiaSearch for more papers by this authorJ. Němeček, J. Němeček Institute of Microbiology, Czechoslovak Academy of Sciences, Prague, CzechoslovakiaSearch for more papers by this authorG. F. Hoffman, G. F. Hoffman Children's Hospital, University of Göttingen, FRGSearch for more papers by this authorF. Pehal, F. Pehal Centre for Inherited Metabolic Disorders, Charles University, Prague, CzechoslovakiaSearch for more papers by this authorJ. Hyánek, J. Hyánek Centre for Inherited Metabolic Disorders, Charles University, Prague, CzechoslovakiaSearch for more papers by this authorA. Grosmanová, A. Grosmanová Department of Child Neurology, Medical Postgraduate Institute, Prague, CzechoslovakiaSearch for more papers by this authorP. Verner, P. Verner Centre for Inherited Metabolic Disorders, Charles University, Prague, CzechoslovakiaSearch for more papers by this author First published: 01 March 1991 https://doi.org/10.1007/BF01800602Citations: 13AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume14, Issue2March 1991Pages 265-266 RelatedInformation

Journal of Inherited Metabolic DiseaseVolume 10, Issue 4 p. 399-399 Case Report A new case of 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency E. Marklová, E. Marklová Department of Pediatrics, Faculty Hospital, Hradec Králové, CS-500 36Search for more papers by this authorP. Verner, P. Verner Diagnostic Centre of Inherited Metabolic Diseases, Prague, CS-121 11 CzechoslovakiaSearch for more papers by this authorF. Pehal, F. Pehal Diagnostic Centre of Inherited Metabolic Diseases, Prague, CS-121 11 CzechoslovakiaSearch for more papers by this authorM. Brátová, M. Brátová First Department of Internal Medicine, Faculty Hospital, Hradec Králové, CS-500 36Search for more papers by this authorJ. Polák, J. Polák Department of Pediatrics, Faculty Hospital, Hradec Králové, CS-500 36Search for more papers by this author E. Marklová, E. Marklová Department of Pediatrics, Faculty Hospital, Hradec Králové, CS-500 36Search for more papers by this authorP. Verner, P. Verner Diagnostic Centre of Inherited Metabolic Diseases, Prague, CS-121 11 CzechoslovakiaSearch for more papers by this authorF. Pehal, F. Pehal Diagnostic Centre of Inherited Metabolic Diseases, Prague, CS-121 11 CzechoslovakiaSearch for more papers by this authorM. Brátová, M. Brátová First Department of Internal Medicine, Faculty Hospital, Hradec Králové, CS-500 36Search for more papers by this authorJ. Polák, J. Polák Department of Pediatrics, Faculty Hospital, Hradec Králové, CS-500 36Search for more papers by this author First published: 01 December 1987 https://doi.org/10.1007/BF01799984Citations: 5AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL No abstract is available for this article.Citing Literature Volume10, Issue4December 1987Pages 399-399 RelatedInformation

The modified apparatus for the isolation and concentration of organic compounds from water by steam distillation-extraction is described. The modified construction of the microapparatus is based on the theoretical aspects of the method and practical experiences. High recoveries and good detection limits obtained by applying the method can be used in trace water analysis.

Journal of High Resolution ChromatographyVolume 7, Issue 9 p. 540-541 Short Communication Isolation, concentration, and gas chromatographic determination of C4C12 fatty acids in water and sludge V. Janda, V. Janda Prague Institute of Chemical Technology, Department of Water Technology and Environmental Engineering, 16628 Prague 6, CzechoslovakiaSearch for more papers by this authorF. Pehal, F. Pehal Prague Institute of Chemical Technology, Department of Water Technology and Environmental Engineering, 16628 Prague 6, CzechoslovakiaSearch for more papers by this authorJ. Hrivňák, J. Hrivňák SVšt Bratislava, Department of Sanitary Engineering, 81368 Bratislava, CzechoslovakiaSearch for more papers by this author V. Janda, V. Janda Prague Institute of Chemical Technology, Department of Water Technology and Environmental Engineering, 16628 Prague 6, CzechoslovakiaSearch for more papers by this authorF. Pehal, F. Pehal Prague Institute of Chemical Technology, Department of Water Technology and Environmental Engineering, 16628 Prague 6, CzechoslovakiaSearch for more papers by this authorJ. Hrivňák, J. Hrivňák SVšt Bratislava, Department of Sanitary Engineering, 81368 Bratislava, CzechoslovakiaSearch for more papers by this author First published: September 1984 https://doi.org/10.1002/jhrc.1240070912Citations: 13AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume7, Issue9September 1984Pages 540-541 RelatedInformation