Search results for "Bloom syndrome"

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Different patterns of in vivo pro-oxidant states in a set of cancer- or aging-related genetic diseases

2008

A comparative evaluation is reported of pro-oxidant states in 82 patients with ataxia telangectasia (AT), Bloom syndrome (BS), Down syndrome (DS), Fanconi anemia (FA), Werner syndrome (WS), and xeroderma pigmentosum (XP) vs 98 control donors. These disorders display cancer proneness, and/or early aging, and/or other clinical features. The measured analytes were: (a) leukocyte and urinary 8-hydroxy-2'-deoxyguanosine (8-OHdG), (b) blood glutathione (GSSG and GSH), (c) plasma glyoxal (Glx) and methylglyoxal (MGlx), and (d) some plasma antioxidants [uric acid (UA) and ascorbic acid (AA)]. Leukocyte 8-OHdG levels ranked as follows: WS>BS approximately FA approximately XP>DS approximately AT appr…

AdultMalemedicine.medical_specialtyDown syndromeXeroderma pigmentosumAdolescentmedicine.disease_causeBiochemistrychemistry.chemical_compoundAtaxia TelangiectasiaPhysiology (medical)Internal medicinemedicineHumansBloom syndromeChildAgedXeroderma PigmentosumMethylglyoxalDeoxyguanosineGlutathioneGlyoxalMiddle AgedAscorbic acidmedicine.diseasePyruvaldehydeGlutathioneEndocrinologyFanconi AnemiaantioxidantschemistryBiochemistry8-Hydroxy-2'-DeoxyguanosineUric acidOxidative streFemaleWerner SyndromeDown SyndromeReactive Oxygen SpeciesOxidative stressBloom SyndromeDNA Damage
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Oxidative stress biomarkers in four Bloom syndrome (BS) patients and in their parents suggest in vivo redox abnormalities in BS phenotype.

2007

Objective: To evaluate an association of Bloom syndrome (BS) phenotype with an in vivo prooxidant state. Methods: The following endpoints were measured in 4 BS patients, their 6 parents, and 78 controls: a) leukocyte and urinary 8-hydroxy-2′-deoxyguanosine (8-OHdG); b) blood glutathione (GSSG and GSH), c) plasma levels of some plasma antioxidants (uric acid, UA, ascorbic acid, AA, α- and γ-tocopherol), and of glyoxal (Glx) and methylglyoxal (MGlx). Results: Leukocyte 8-OHdG levels were significantly increased in the 4 BS patients vs. 40 controls (p = 0.04), while the urinary 8-OHdG levels were non-significantly increased in BS patients. Glutathione disulfide levels and GSSG/GSH ratio were s…

AdultMaleParentsmedicine.medical_specialtyglyoxalAdolescentClinical Biochemistrymedicine.disease_causechemistry.chemical_compoundIn vivoInternal medicinemedicinemethylglyoxalLeukocytesHumansBloom syndromeChildoxidative streGlutathione DisulfideMethylglyoxalDeoxyguanosineGeneral MedicineGlutathioneMiddle AgedAscorbic acidmedicine.diseaseGlutathioneOxidative StressEndocrinologyPhenotypechemistryBiochemistry8-Hydroxy-2'-DeoxyguanosineUric acidGlutathione disulfideBloom syndromeFemaleOxidation-ReductionOxidative stressBiomarkersBloom SyndromeClinical biochemistry
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