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1.
Rev. MED ; 25(2): 7-21, jul.-dic. 2017. tab, graf
Article Dans Espagnol | LILACS | ID: biblio-977031

Résumé

RESUMEN La deficiencia de la Glucosa 6 Fosfato Deshidrogenasa es un desorden hereditario del metabolismo ligado al cromosoma X, considerada como la enfermedad metabólica de mayor frecuencia a nivel mundial con más de 400 millones de individuos afectados, es la causa más común de anemia hemolítica no autoinmune de origen metabólico. La prevalencia calculada para Colombia según la organización mundial de la salud se encuentra entre 3 - 7%, pero los reportes de estudios poblacionales son escasos, creando la necesidad de incluir esta deficiencia en un programa de tamizaje neonatal, más cuando aproximadamente un 11 % de la población es afrodescendiente, que sin contar con el mestizaje, puede ofrecer una frecuencia mayor de la deficiencia en ciertas regiones del país. El presente estudio brinda entonces, una panorámica general de la prevalencia de esta enzimopatía eritrocitaria, en población con anemia hemolítica crónica o episódica de naturaleza no inmune, procedentes de diferentes lugares del territorio nacional. Comprende la valoración de 3837 muestras durante el periodo 1998 al 2016, un total de 1801 controles y 2036 individuos (982 hombres/1054 mujeres, Rango de edad: 1 semana a 91 años) con hallazgos sugerentes de esta condición metabólica. Los resultados encontrados, ofrecen una prevalencia del 7 % de la deficiencia con grados variables de actividad residual. El rango general de los grupos control osciló entre 3,31 - 9,98 Ul/gr HB, contrastante contra el grupo de afectados (n=144) que ofreció un valor de 0,0 - 2,97, el 67 % (n=96) correspondiente a varones afectados. Estos hallazgos evidencian la importancia de incluir dentro de un programa de detección neonatal, esta enzimo-patía eritrocitaria.


ABSTRACT Deficiency of Glucose-6 -Phosphate Dehydrogenase is an inherited disorder of X-linked metabolism, as the metabolic disease of the world's largest frequency with more than 400 million affected individuals, is the most common cause of non-autoimmune hemolytic anemia of metabolic origin. The prevalence calculated for Colombia according to the world health organization is between 3 and 7%, but the reports of population studies are scarce, creating the need to include this deficiency in a neonatal screening program, closer to 11% population is Afro-descendant that without the miscegenation, can offer a greater frequency of deficiency in certain regions of the country. The present study provides an overview of the prevalence of erythrocyte enzyme disease in the population with chronic or episodic hemolytic anemia of non-immune nature from different parts of the national territory. lt comprises the evaluation of 3837 samples during the period 1998 to 2016, a total of 1801 controls and 2036 individuals (982 men / 1054 women, age range: 1 week to 91 years) with suggestive findings of this metabolic condition. The results found, offer a prevalence of 7% of the deficiency with variable degrees of residual activity. The overall range of the control groups ranged from 3.31 - 9.98 lU / g HB, contrasting against the group of affected (n = 144) who offered a value of 0.0 - 2.97, 67% = 96 ) corresponds to affected males. These findings highlight the importance of including this erythrocyte enzyme disease in a neonatal screening program.


RESUMO A deficiência de glucose-6-fosfato desidrogenase é uma desordem hereditária do metabolismo ligado ao X, como a doença metabólica da maior freqüência do mundo com mais de 400 milhões de indivíduos afetados, é a causa mais comum de anemia hemolítica não autoimune de origem metabólica. A prevalência calculada para a Colômbia de acordo com a organização mundial de saúde é entre 3 e 7%, mas os relatórios de estudos populacionais são escassos, criando a necessidade de incluir esta deficiência em um programa de triagem neonatal, perto de 11% a população é afro-descendente que sem a miscigenação, pode oferecer uma maior frequência de deficiência em certas regiões do país. O presente estudo fornece uma visão geral da prevalência de doença enzimática de eritrócitos na população com anemia hemolítica crônica ou episódica de natureza não imune de diferentes partes do território nacional. Compreende a avaliação de 3837 amostras durante o período de 1998 a 2016, um total de 1801 controles e 2036 indivíduos (982 homens / 1054 mulheres, faixa etária: 1 semana a 91 anos) com achados sugestivos desta condição metabólica. Os resultados encontrados, oferecem uma prevalência de 7% da deficiência com graus variáveis de atividade residual. O intervalo geral dos grupos de controle variou de 3,31 a 9,98 Ul / g de HB, contrastando com o grupo de afetados (n = 144) que ofereceu um valor de 0,0-297, 67% = 96) corresponde a machos afetados. Esses achados destacam a importância de incluir esta doença enzimática de eritrócitos em um programa de triagem neonatal.


Sujets)
Humains , Anémie hémolytique , Colombie , Stress oxydatif , Glucose-6-phosphate , Acetoine dehydrogenase
2.
Anesthesia and Pain Medicine ; : 243-246, 2017.
Article Dans Anglais | WPRIM | ID: wpr-145724

Résumé

The authors describe the successful perioperative management of a 3-year-old boy from Dubai with glucose-6-phosphate dehydrogenase (G6PD) deficiency, who underwent robot-assisted laparoscopic pyeloplasty for complete right ureteropelvic junction obstruction. G6PD deficiency is a genetic disorder predisposing patients to hemolytic anemia from oxidative stress. Important considerations in anesthetic management include avoiding oxidative stress, which can be caused by various conditions, and monitoring for hypercapnia, which can cause acidosis and hemolysis. Laparoscopic surgery is usually associated with hypercapnia and therefore an increased risk for respiratory acidosis. During surgery in this particular case, efforts were made to avoid carbon dioxide retention and to keep the patient warm. General anesthesia was induced with thiopental sodium, rocuronium, and fentanyl, and maintained with sevoflurane. There were no signs of hemolysis in the perioperative period and he was discharged owing to his improved condition.


Sujets)
Enfant d'âge préscolaire , Humains , Mâle , Acidose , Acidose respiratoire , Anémie hémolytique , Anesthésie générale , Dioxyde de carbone , Fentanyl , Glucose-6-phosphate , Déficit en glucose-6-phosphate-déshydrogénase , Glucose 6-phosphate dehydrogenase , Hémolyse , Hypercapnie , Laparoscopie , Stress oxydatif , Période périopératoire , Thiopental
3.
Korean Journal of Pediatrics ; : 106-111, 2017.
Article Dans Anglais | WPRIM | ID: wpr-50098

Résumé

PURPOSE: This study aimed to determine the prevalence of glucose-6-phosphate dehydrogenase (G6PD) deficiency among infants with neonatal indirect hyperbilirubinemia (NIH); compare G6PD-deficient and G6PD-normal patients regarding hyperbilirubinemia and need for exchange transfusions (ET); and assess risk factors for ET and kernicterus. METHODS: This is a case-control retrospective study. Medical records of NIH patients admitted to the Pediatric Department, Salmaniya Medical Complex, Bahrain, between January 2007 and June 2010 were reviewed. Data on sex, age at presentation, hospitalization duration, need for ET, hemoglobin (Hb) level, reticulocyte count, direct Coombs test, serum total and indirect bilirubin levels, thyroid function, blood and urine cultures, G6PD status, and blood groups were collected and compared between the G6PD-deficent and G6PD-normal patients. RESULTS: Of 1,159 NIH patients admitted, 1,129 were included, of whom 646 (57%) were male. Among 1,046 patients tested, 442 (42%) were G6PD deficient, 49 (4%) needed ET, and 11 (1%) had suspected Kernicterus. The G6PD-deficient patients were mainly male (P<0.0001), and had lower Hb levels (P<0.0001) and higher maximum bilirubin levels (P=0.001). More G6PD-deficient patients needed ET (P<0.0001). G6PD deficiency (P=0.006), lower Hb level (P=0.002), lower hematocrit count (P=0.02), higher bilirubin level (P<0.0001), higher maximal bilirubin level (P<0.0001), and positive blood culture result (P<0.0001) were significant risk factors for ET. Maximal bilirubin level was a significant risk factor for kernicterus (P=0.021) and independently related to ET (P=0.03). CONCLUSION: G6PD deficiency is an important risk factor for severe NIH. In G6PD-deficent neonates, management of NIH should be hastened to avoid irreversible neurological complications.


Sujets)
Humains , Nourrisson , Nouveau-né , Mâle , Bahreïn , Bilirubine , Antigènes de groupe sanguin , Études cas-témoins , Test de Coombs , Glucose-6-phosphate , Déficit en glucose-6-phosphate-déshydrogénase , Glucose 6-phosphate dehydrogenase , Hématocrite , Hospitalisation , Hyperbilirubinémie , Hyperbilirubinémie néonatale , Ictère nucléaire , Dossiers médicaux , Prévalence , Numération des réticulocytes , Études rétrospectives , Facteurs de risque , Glande thyroide
4.
Clinical and Experimental Reproductive Medicine ; : 193-198, 2016.
Article Dans Anglais | WPRIM | ID: wpr-54503

Résumé

OBJECTIVE: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common human enzyme defect. G6PD plays a key role in the pentose phosphate pathway, which is a major source of nicotinamide adenine dinucleotide phosphate (NADPH). NADPH provides the reducing equivalents for oxidation-reduction reductions involved in protecting against the toxicity of reactive oxygen species such as H₂O₂. We hypothesized that G6PD deficiency may reduce the amount of NADPH in sperms, thereby inhibiting the detoxification of H₂O₂, which could potentially affect their motility and viability, resulting in an increased susceptibility to infertility. METHODS: Semen samples were obtained from four males with G6PD deficiency and eight healthy males as a control. In both groups, motile sperms were isolated from the seminal fluid and incubated with 0, 10, 20, 40, 60, 80, and 120 µM concentrations of H2O2. After 1 hour incubation at 37℃, sperms were evaluated for motility and viability. RESULTS: Incubation of sperms with 10 and 20 µM H₂O₂ led to very little decrease in motility and viability, but motility decreased notably in both groups in 40, 60, and 80 µM H₂O₂, and viability decreased in both groups in 40, 60, 80, and 120 µM H₂O₂. However, no statistically significant differences were found between the G6PD-deficient group and controls. CONCLUSION: G6PD deficiency does not increase the susceptibility of sperm to oxidative stress induced by H₂O₂, and the reducing equivalents necessary for protection against H₂O₂ are most likely produced by other pathways. Therefore, G6PD deficiency cannot be considered as major risk factor for male infertility.


Sujets)
Humains , Mâle , Glucose-6-phosphate , Déficit en glucose-6-phosphate-déshydrogénase , Glucose 6-phosphate dehydrogenase , Infertilité , Infertilité masculine , NADP , Oxydoréduction , Stress oxydatif , Voie des pentoses phosphates , Espèces réactives de l'oxygène , Facteurs de risque , Sperme , Spermatozoïdes
5.
Journal of Experimental Hematology ; (6): 1108-1111, 2015.
Article Dans Chinois | WPRIM | ID: wpr-274084

Résumé

<p><b>OBJECTIVE</b>To investigate the diagnostic value and clinical significance of the glucose 6-phosphate dehy-drogenase (G6PD) activity for mediterranean anemia (MA), so as to provide the reference for early clinical diagnosis and treatment of MA.</p><p><b>METHODS</b>The peripheral blood was collected from 100 healthy persons and 168 patients with MA, then the agarose gel electrophoresis, MA gene detection, blood routine examination, serum ferrium levels and G6PD activity assay were performed, and the results of evaluating MA were comparatively analyzed.</p><p><b>RESULTS</b>The G6PD activity in all type MA patients was obviously higher than that in healthy controls (P < 0.01), the MCV value in all type MA patients was significantly lower than that in healthy controls (P < 0.01). The detection of G6PD activity showed that the sensitivity, specificity, positive and negative likelihood ratio, diagnostic index and Youden index of G6PD for MA patients were 85.12%, 68%, 2.66, 0.219, 1.53 and 0.53 respectively, which suggest the better efficacy of G6PD value for diagnosis of MA.</p><p><b>CONCLUSION</b>The G6PDS activity of patients with MA in different subtypes is higher than that of healthy persons, the G6PD level has a certain diagnostic value for MA, but there is an optimal range.</p>


Sujets)
Humains , Glucose-6-phosphate , Glucose 6-phosphate dehydrogenase , bêta-Thalassémie
6.
Annals of Dermatology ; : S8-S10, 2011.
Article Dans Anglais | WPRIM | ID: wpr-190425

Résumé

Hypohidrotic ectodermal dysplasia (HED) is a syndrome characterized by hypodontia, hypotrichosis, and partial or total ecrine sweat gland deficiency. The most prevalent form of HED is inherited as an X linked pattern. Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency is an X-linked recessive disease, which leads to hemolytic anemia and jaundice. It is expressed in males, while heterozygous females are usually clinically normal. A 12-year-old boy with the complaints of hair and eyebrow disturbances, teeth disfigurement, decreased sweating, and xerosis presented to the outpatient clinic. Dermatological examination revealed sparse hair and eyebrows, conical-shaped teeth, xerosis, syndactylia, transverse grooves, and discoloration of nails. Laboratory findings indicated anemia. His 3-year-old sister also had sparse hair and eyebrows, xerosis, and syndactylia. We learned that the patient had a previous history of neonatal jaundice and a diagnosis of G-6-PD deficiency. Although it has been shown that loci of ectodermal dysplasia and G-6-PD deficiency genes are near to one another, we did not find any case study reporting on occurrence of these two genetic diseases together. With the aspect of this rare and interesting case, the relationship between HED and G-6-PD deficiency was defined.


Sujets)
Enfant , Femelle , Humains , Nouveau-né , Mâle , Établissements de soins ambulatoires , Anémie , Anémie hémolytique , Anodontie , Ectoderme , Dysplasie ectodermique , Sourcils , Glucose-6-phosphate , Glucose 6-phosphate dehydrogenase , Déficit en glucose-6-phosphate-déshydrogénase , Poils , Hypotrichose , Ictère , Ictère néonatal , Ongles , Enfant d'âge préscolaire , Fratrie , Sueur , Glandes sudoripares , Sudation , Syndactylie , Dent
7.
Acta Pharmaceutica Sinica ; (12): 1173-1177, 2011.
Article Dans Chinois | WPRIM | ID: wpr-233016

Résumé

Nuclear receptors pregnane X receptor (PXR) and constitutive androstane receptor (CAR) are originally characterized as transcription factors regulating many target genes. Recent works have revealed that these nuclear receptors play critical roles in regulating genes that encode drug metabolism enzymes and modulating hepatic energy metabolism, such as down-regulating gluconeogenesis, fatty acid oxidation, and ketogenesis, as well as up-regulating lipogenesis. Studies on PXR and CAR have important implication on drug-drug interaction (DDI) and potential disease treatment targets.


Sujets)
Animaux , Humains , Interactions médicamenteuses , Métabolisme énergétique , Glucose , Métabolisme , Glucose-6-phosphate , Métabolisme , Inflammation , Métabolisme , Métabolisme lipidique , Foie , Métabolisme , Facteur de transcription NF-kappa B , Métabolisme , Récepteurs cytoplasmiques et nucléaires , Physiologie , Récepteurs aux stéroïdes , Physiologie
8.
Laboratory Animal Research ; : 279-286, 2010.
Article Dans Coréen | WPRIM | ID: wpr-133078

Résumé

This study was carried out to investigate an enhancing effect of black ginseng extract (BGE) on exercise capacity in an endurance exercising animal model. Fifty Sprague-Dawley rats were assigned to 5 experimental groups including non-training control, training control, and 3 treated groups (BGE at doses of 75, 150 and 300 mg/kg). The animals were treated with BGE for 6 weeks and their exercise ability in the maximal running distance test was determined using a treadmill every week. The blood lactic acid (LA) level and the activity of citrate synthase (CS) in the muscle were also measured after the exercise. The levels of glucose and glucose-6-phosphate (G-6-P) in the liver and muscle were determined using commercial assay kits. BGE treatments at the doses of 150 and 300 mg/kg significantly increased the exercise capacity compared with the non-training control or training control groups (P<0.05). The level of blood LA was decreased but the activity of CS was increased by the treatment of BGE at the dose of 300 mg/kg compared with the training control group. The level of G-6-P in the liver was elevated by the treatment of BGE at the dose of 300 mg/kg, compared to the training group. As compared with non-training control group, the treatments of BGE increased the levels of glucose and G-6-P in the liver and soleus muscle of rats. These results indicate that BGE have a potential for promoting exercise capacity by increasing CS activity in the muscle and decreasing LA in the serum of rats. These results also suggested that BGE can be used as a candidate supplement of health food products for promoting endurance exercise capacity in human athletes.


Sujets)
Animaux , Humains , Rats , Athlètes , Citrate (si)-synthase , Exercice physique , Glucose , Glucose-6-phosphate , Nourriture biologique , Acide lactique , Foie , Modèles animaux , Muscles squelettiques , Muscles , Panax , Rat Sprague-Dawley , Course à pied
9.
Laboratory Animal Research ; : 279-286, 2010.
Article Dans Coréen | WPRIM | ID: wpr-133075

Résumé

This study was carried out to investigate an enhancing effect of black ginseng extract (BGE) on exercise capacity in an endurance exercising animal model. Fifty Sprague-Dawley rats were assigned to 5 experimental groups including non-training control, training control, and 3 treated groups (BGE at doses of 75, 150 and 300 mg/kg). The animals were treated with BGE for 6 weeks and their exercise ability in the maximal running distance test was determined using a treadmill every week. The blood lactic acid (LA) level and the activity of citrate synthase (CS) in the muscle were also measured after the exercise. The levels of glucose and glucose-6-phosphate (G-6-P) in the liver and muscle were determined using commercial assay kits. BGE treatments at the doses of 150 and 300 mg/kg significantly increased the exercise capacity compared with the non-training control or training control groups (P<0.05). The level of blood LA was decreased but the activity of CS was increased by the treatment of BGE at the dose of 300 mg/kg compared with the training control group. The level of G-6-P in the liver was elevated by the treatment of BGE at the dose of 300 mg/kg, compared to the training group. As compared with non-training control group, the treatments of BGE increased the levels of glucose and G-6-P in the liver and soleus muscle of rats. These results indicate that BGE have a potential for promoting exercise capacity by increasing CS activity in the muscle and decreasing LA in the serum of rats. These results also suggested that BGE can be used as a candidate supplement of health food products for promoting endurance exercise capacity in human athletes.


Sujets)
Animaux , Humains , Rats , Athlètes , Citrate (si)-synthase , Exercice physique , Glucose , Glucose-6-phosphate , Nourriture biologique , Acide lactique , Foie , Modèles animaux , Muscles squelettiques , Muscles , Panax , Rat Sprague-Dawley , Course à pied
10.
Korean Journal of Pediatrics ; : 1383-1387, 2009.
Article Dans Anglais | WPRIM | ID: wpr-55856

Résumé

We report a case of an 18-month-old girl with glycogen storage disease type Ib (GSD Ib). Her neutrophil counts had gradually decreased to less than 500/microL by the age of 3 years. However, there were no recurrent bacterial infections. Mutation analysis of the glucose-6-phosphate translocase (G6PT) gene revealed a compound heterozygous missense mutation (Ala148Val/Gly273Asp).


Sujets)
Humains , Nourrisson , Infections bactériennes , Glucose-6-phosphate , Glycogène , Glycogénose , Mutation faux-sens , Neutropénie , Granulocytes neutrophiles
11.
Indian J Biochem Biophys ; 2007 Apr; 44(2): 88-93
Article Dans Anglais | IMSEAR | ID: sea-26405

Résumé

The conventional method of Fiske and Subba Row for the estimation of inorganic phosphate (Pi) is although rapid, but suffers from the disadvantage that the color is unstable and hence the optical density (OD) measurements have to be carried out within a short time span of 8-12 min. This poses a restriction on the number of samples, which can be handled in a batch. Although, modified procedures involving use of alternate reducing agents/or increasing the concentration of H2SO4 in conventional method have been subsequently developed, but the problem of color stability could not be solved. In addition, the use of higher concentrations H2SO4 has rendered the methods unsuitable in enzyme assays, especially if the acid labile phosphate containing substrates have been used. In the present study, attempts have been made to suitably modify the method to improve the stability of the color and sensitivity and also for its applicability in enzyme assays, especially when acid labile phosphate containing substrates such as ATP is used. We used the higher concentrations (0.625, 0.8 and 1.0 N) of H2SO4 rather than 0.5 N used in the conventional assay procedures. Under these conditions, the reagent blanks do not develop color for up to 24 h, whereas the intensity of the molybdenum blue color in the standard and/or experimental tubes increased with time reaching optimum value at 24 h. Simultaneously, the absorption maximum shifts from 660 nm to 820 nm. The highest concentration of H2SO4 (1.0 N) is found to be the most effective in the process of color development. The sensitivity of the method is from 1.7 to 2.1 times higher, as compared to the conventional Fiske and Subba Row method for the measurements carried out at the end of 15 min at 820 nm and with the highest concentration of H2SO4 (1.0 N); the sensitivity increased 4.8-fold at the end of 24 h. Presence of glucose and sucrose (1-10 mM), NaCl and KCI (5-100 mM), MgCl2 (1-10 mM) and BSA (10 to 500 microg per assay tube) do not interfere either with color development or with OD measurements. The extent of ATP hydrolysis is 1.6 to 3.4% for up to 1 hi, depending upon the concentration of H2SO4 used. Only negligible hydrolysis of G6P is observed under these conditions. These results suggest that the presently modified method is suitable for Pi analysis in the enzyme assays, in the presence of labile phosphate containing substrates.


Sujets)
Adénosine triphosphate/composition chimique , Glucides/composition chimique , Réactifs chromogènes/composition chimique , Glucose-6-phosphate/analyse , Phosphates/analyse , Sels/composition chimique , Sérumalbumine bovine/composition chimique , Acides sulfuriques/composition chimique
12.
The Journal of the Korean Rheumatism Association ; : 340-344, 2007.
Article Dans Coréen | WPRIM | ID: wpr-162140

Résumé

OBJECTIVE: Anti-glucose-6-phosphate isomerase (GPI) antibody (Ab) is known to be arthritogenic in K/BxN mice. Anti-GPI Ab is present in some patients with rheumatoid arthritis (RA), but their clinical manifestations are not clearly elucidated. The purpose of this study was to evaluate whether GPI serves as a specific autoantigen in patients with RA and to investigate the relationship of anti-GPI Ab with clinical parameters of RA. METHODS: Sera were collected from 54 patients with RA, 15 patients with osteoarthritis (OA) and 28 healthy controls. The samples were tested by enzyme-linked immunosorbent assay (ELISA) using human recombinant GPI as antigen. Patients with RA were classified according to rheumatoid factor (RF) positivity, the presence of RA shared epitope (SE), the presence of extraarticular manifestations, and evidence of bony erosive changes. RESULTS: Serum levels of anti-GPI Ab were higher in patients with RA than controls (1599.46+/-1022.48 versus 344.82+/-223.16 AU, p<0.001), and the levels of patients with OA were also higher than controls (1161.47+/-917.44 versus 344.82+/-223.16 AU, p<0.01). In RA, there were no significant difference in anti-GPI Ab levels according to RF positivity, the presence of RA SE, the presence of extraarticular manifestations, and evidence of bony erosive changes. CONCLUSION: Our results suggest that anti-GPI Ab may not be RA specific Ab and not related to the severity of RA.


Sujets)
Animaux , Humains , Souris , Polyarthrite rhumatoïde , Autoanticorps , Test ELISA , Glucose 6-phosphate isomerase , Glucose-6-phosphate , Arthrose , Facteur rhumatoïde
13.
Korean Journal of Perinatology ; : 89-93, 2006.
Article Dans Coréen | WPRIM | ID: wpr-210612

Résumé

The most devastating clinical consequence of G-6-PD deficiency is neonatal hyperbilirubinemia which can be severe and result in kernicterus or even death, although glucose-6-phosphate dehydrogenase deficiency is responsible for two clinical syndromes, an episodic hemolytic anemia induced by infections or certain drugs and spontaneous chronic nonspherocytic hemolytic anemia. In the pathogenesis of neonatal hyperbilirubinemia associated with G-6-PD deficiency, decreased elimination of bilirubin has been suspected to be a key factor, because these neonates usually do not develop frank anemia even in the presence of severe hyperbilirubinemia. But, we experienced a glucose-6-phosphate dehydrogenase deficient male patient who showed jaundice and severe hemolytic anemia appearing within the first 24 hour of life. The patient had resolution of symptoms after phototherapy and transfusion. We report this case with a brief review of the related literatures.


Sujets)
Humains , Nouveau-né , Mâle , Anémie , Anémie hémolytique , Bilirubine , Glucose-6-phosphate , Déficit en glucose-6-phosphate-déshydrogénase , Glucose 6-phosphate dehydrogenase , Hyperbilirubinémie , Hyperbilirubinémie néonatale , Ictère , Ictère nucléaire , Photothérapie
14.
Journal of Southern Medical University ; (12): 1041-1043, 2006.
Article Dans Chinois | WPRIM | ID: wpr-334997

Résumé

<p><b>OBJECTIVE</b>To observe the therapeutic effect of glucose-6-phosphate polyclonal antibody (G-6-P pAb) on vasogenic brain edema (VBE) in rats.</p><p><b>METHODS</b>Sixty Wistar rats were randomly divided into normal control group, VBE group, mannitol-treated edema group, and G-6-P pAb-treated edema group. After establishment of rat models of VBE by intraperitoneal injection of phenylephrine in the latter 3 groups, mannitol was injected through the femoral vein in mannitol group and G-6-P pAb injected intraperitoneally in G-6-P pAb group. The permeability of the blood-brain barrier (BBB) was determined by Evans blue (EB) extravasation method, and the brain water content in the gray and white matter measured with a moisture analyzer.</p><p><b>RESULTS</b>G-6-P pAb administration significantly reduced the permeability of BBB as well as the water content in the white matter in comparison with mannitol treatment (P<0.01), but the two treatments showed no obvious difference in reducing the water content in the gray matter (P>0.05).</p><p><b>CONCLUSION</b>Changes in G-6-P activity results in BBB permeability alteration in the condition of VBE, and G-6-P pAb has a selective therapeutic effect against VBE, especially white matter edema.</p>


Sujets)
Animaux , Femelle , Mâle , Rats , Anticorps monoclonaux , Allergie et immunologie , Utilisations thérapeutiques , Barrière hémato-encéphalique , Oedème cérébral , Traitement médicamenteux , Perméabilité capillaire , Glucose-6-phosphate , Allergie et immunologie , Phényléphrine , Répartition aléatoire , Rat Wistar
16.
The Journal of the Korean Rheumatism Association ; : 12-17, 2005.
Article Dans Coréen | WPRIM | ID: wpr-203093

Résumé

OBJECTIVE: Autoantibody against glucose-6-phosphate isomerase (GPI) has been shown to be present in both the serum and synovial fluid (SF) of patients with rheumatoid arthritis (RA). The purpose of this study was to evaluate whether GPI serves as a specific autoantigen in the SF of patients with RA and to investigate the relationship of anti-GPI antibody with clinical parameters of RA. METHODS: SF was collected from 34 patients with RA and 34 patients with osteoarthritis (OA). The samples were tested by enzyme-linked immunosorbent assay (ELISA) using human recombinant GPI as antigen. Patients with RA were classified according to rheumatoid factor (RF) positivity, the presence of RA shared epitope, the presence of extraarticular manifestations, and evidence of bony erosive changes. RESUTLS: SF levels of anti-GPI antibody were higher in patients with RA than in patients with OA (631.12+/-534.02 AU versus 112.38+/-90.45 AU, p<0.001). In RA, there was no significant difference in SF anti-GPI antibody levels according to RF positivity, the presence of extraarticular manifestations, and evidence of bony erosive changes. CONCLUSION: Autoantibodies to GPI in SF have more related with patients with RA compared with those with OA. In patients with RA, autoantibodies to GPI in SF are not associated with the poor prognostic factors and disease activity of RA.


Sujets)
Humains , Polyarthrite rhumatoïde , Autoanticorps , Test ELISA , Glucose 6-phosphate isomerase , Glucose-6-phosphate , Arthrose , Facteur rhumatoïde , Synovie
18.
Korean Journal of Pediatrics ; : 210-213, 2004.
Article Dans Coréen | WPRIM | ID: wpr-125110

Résumé

Glucose-6-phosphate dehydrogenase(G-6-PD) deficiency is a disease that shows hemolytic anemia and jaundice caused by injury of erythrocytes. The gene of G-6-PD has 13 exons and locates in Xq28, and over 150 mutations of this gene have been reported. We experienced a G-6-PD deficienct male patient who was suffering hemolytic anemia and jaundice confirmed by measuring low G-6-PD activity in the erythrocytes. We found point mutation at 1159th nucleotide in 10th exon, cytosine was changed to thymidine, and was confirmed as G-6-PD Guadalajara.


Sujets)
Humains , Mâle , Anémie hémolytique , Cytosine , Érythrocytes , Exons , Glucose-6-phosphate , Ictère , Mutation ponctuelle , Thymidine
19.
J. bras. med ; 83(3): 24-30, set. 2002.
Article Dans Portugais | LILACS | ID: lil-322001

Résumé

Os autores enfocam os aspectos fisiológicos e fisiopatológicos do eritrócito que se relacionam com a malária. Esta revisäo visa compreender a açäo seletiva da malária sobre as doenças hereditárias do glóbulo vermelho (anemia falciforme e hemoglobinopatias associadas, talassemias, deficiência de glicose-6-fosfato desidrogenase, ovalocitose hereditária) e do sistema Duffy, focalizando a discussäo nos aspectos associados ao ciclo evolutivo do parasita no inseto e no homem. Descrevem a fisiopatologia da deficiência de G6PD, talassemias, hemoglobinopatias C e E ovalocitose, sistema Duffy e as inter-relações com a malária


Sujets)
Humains , Hémopathies/génétique , Érythrocytes , Hémoglobinopathies , Paludisme , alpha-Thalassémie , Drépanocytose , Hémoglobinose C/étiologie , Glucose-6-phosphate
20.
El-Minia Medical Bulletin. 2002; 13 (2): 147-164
Dans Anglais | IMEMR | ID: emr-59321

Résumé

The aim of the present work was to evaluate the effects of verrucarin J-toxin on serum biochemistry and on light and electron microscopic structures of the liver of adult male mice. Treated mice was injected intraperitonealy by toxin in a single dose of 0.9 mg/kg [group A] and 0.5 mg/kg body weight [group B]. The animals were sacrificed after 2, 4, 6 weeks [10 each] and compared to toxin free control [C] group. Results indicated that glucose level was significantly different in the treated groups [A, B], which were injected for 2 and 4 weeks than control [C] group. But there was no significant difference in 6 weeks. Plasma levels of triglycerides or cholesterol were significantly higher in both treated mice than control. Many histological changes occurred in the liver cells of both treated groups [A and B] after 2 and 4 weeks. The hepatocytes were engorged with glycogen. The nuclei and cell organelles are markedly affected. After 6 weeks from treatment many hepatocytes were, somewhat similar to the control


Sujets)
Animaux de laboratoire , Foie , Histologie , Microscopie électronique , Souris , Glucose-6-phosphate , Pyruvate kinase , Glycémie
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