Effect of Glucose-6-Phosphate Dehydrogenase Deficiency on Neutrophil Function

Ardati, Kasim O.; Bajakian, Koharik M.; Tabbara, Khaled S.

doi:10.1159/000203685

Cited by 26 publications

(16 citation statements)

References 11 publications

(14 reference statements)

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…A deficiency of this enzyme may result in the premature destruction of red blood cells (an acute hemolytic anemia or a chronic spherocytic type) when an affected individual is exposed to certain medications or chemicals, experiences certain viral or bacterial infections, and/or inhales the pollen of, or consumes, fava beans (favism). Glucose-6-Phosphate Dehydrogenase Deficiency is inherited as an X-linked genetic trait 5 . It is a common inborn error of metabolism among humans.…”

Section: Discussionmentioning

confidence: 99%

Glucose-6-phosphate Dehydrogenase Deficiency: A Case Report

Hassan¹,

Saha²,

Kundu³

et al. 2017

Faridpur Med Coll J

View full text Add to dashboard Cite

Abstract:Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common hereditary enzyme disorder and more than 200 million people have a deficiency in this enzyme. G6PD deficiency is an X-linked enzyme defect, and one of its main signs is the presence of hemolytic anemia. It is a worldwide important cause of neonatal jaundice and causes life threatening hemolytic crisis in childhood. At later ages, certain drugs such as anti-malarial drugs and fava beans cause hemolysis among G6PD deficiency patients. The frequency and severity is influenced by genetic and cultural factors. It is common in Mediterranean, African and some East Asian populations but rare in Bangladeshi peoples. Genetic counseling may be of benefit for patients and their families. Other treatment is symptomatic and supportive.

show abstract

Section: Discussionmentioning

confidence: 99%

Glucose-6-phosphate Dehydrogenase Deficiency: A Case Report

Hassan¹,

Saha²,

Kundu³

et al. 2017

Faridpur Med Coll J

View full text Add to dashboard Cite

show abstract

“…We used a NBT dye reduction assay to study the metabolic function of the cells since the respiratory burst-dependent NBT reduction in phagocytes occur by a chemical reaction between the dye and the O −2 that is generated during the burst, which in turn is related to hexose monophosphate shunt and NADPH production (Ardati et al, 1997). Therefore, the dye may be used to measure the ability of the neutrophils to kill microbes because of the close association between microbicidal function and dye reduction.…”

Section: Discussionmentioning

confidence: 99%

Endotoxins of Enteric Pathogens Are Chemotactic Factors for Human Neutrophils

Islam¹,

Nabi²,

Ahmed³

et al. 2002

BMB Reports

View full text Add to dashboard Cite

Early activation of human peripheral blood polymorphonuclear neutrophils is characterized by their morphological changes from spherical to polarized shapes. The endotoxins from enteric pathogens (S. dysenteriae type 1, V. cholerae Inaba 569B, S. typhimurium, and K. pneumoniae) were assessed by their ability to induce morphological polarization of the neutrophils as measures of early activation. Phagocytic activity, adhesion, chemokinetic locomotion, and nitroblue tetrazolium (NBT) dyereduction ability measured the later activation of the cells. Neutrophils showed distinct morphological polarization in suspension over a wide range of concentrations of these endotoxins when were compared with those that were induced by the standard chemotactic factor, N-formyl-Lmethionyl-L-leucyl-L-phenylalanine (FMLP). It was discovered that all of the endotoxins induced locomotor responses in neutrophils in suspension that were dose-and time-dependent. The optimum concentration for the endotoxins of S. dysenteriae, V. cholerae, and K. pneumoniae was 1 mg/ml in which 71, 69, and 66% of the neutrophils were polarized. However, the S. typhimurium dose was 2 mg/ml in which 50% of the cells responded. Neutrophils that were stimulated with endotoxins also showed increased random locomotion (p<0.005) through cellulose nitrate filters, but an enhanced adhesion of the cells to glass surfaces (p<0.03). These are important functions of these cells to reach and phagocytose damaged cells, as well as invading microorganisms. Interestingly, the endotoxins had a highly-significant inhibitory effect upon the proportions of neutrophils phagocytosing opsonized yeast (p<0.01) with a small number of yeast that were engulfed by the cells (p<0.02). Further, endotoxin-treated cells showed an enhanced ability to reduce NBT dye (p<0.03). Therefore, we concluded that endotoxins of enteric pathogens are neutrophil chemotactic factors.

show abstract

“…A atividade bactericida dos neutrófilos para microorganismos catalase-positivos depende primariamente de intermediários reativos de oxigênio (ânion superóxido, oxigê-nio e peróxido de hidrogênio), liberados pela ativação da enzima NADPH oxidase, reação conhecida como metabolismo oxidativo dos neutrófilos 5 . O metabolismo oxidativo (burst oxidativo) é caracterizado por um aumento abrupto no consumo de oxigênio (O 2 ) e sua redução parcial a ânion superóxido (O 2-).…”

Section: Discussionunclassified

“…A G6PD é a primeira enzima desta via (hexose monophosphate shunt), onde a glicose-6-fosfato (Glucose-6-P) é convertida para 6 fosfogluconato (6-PG) ao mesmo tempo que NADP+ é reduzida para NADPH, substrato para metabolismo oxidativo 5 . Quando o nível de G6PD nos neutrófilos é menor que 5% do normal 3 , o shunt não é ativado, e a atividade bactericida dos fagócitos é bloqueada.…”

Section: Discussionunclassified

Deficiência da glicose-6-fosfato desidrogenase com infecções de repetição: relato de caso

Rosa-Borges¹,

Sampaio²,

Condino‐Neto³

et al. 2001

J. Pediatr. (Rio J.)

View full text Add to dashboard Cite

ResumoObjetivo: relatar a ocorrência de uma deficiência funcional de neutrófilos rara, com quadro clínico e laboratorial semelhante ao da doença granulomatosa crônica.Métodos: relato de caso de paciente com deficiência acentuada da glicose-6-fosfato desidrogenase e infecções de repetição. Realizada pesquisa bibliográfica utilizando as bases de dados Medline e Lilacs, abrangendo o período de 1972 a 2000.Resultados: paciente com nível da glicose-6-fosfato desidrogenase extremamente reduzido e quadro de infeções graves com melhora clínica após uso de cotrimoxazol contínuo. Os leucócitos do paciente apresentam defeito no metabolismo oxidativo, similar ao da doença granulomatosa crônica.Conclusões: o diagnóstico da deficiência da glicose-6-fosfato desidrogenase em neutrófilos deve ser considerado em qualquer paciente com anemia hemolítica não esferocítica congênita no qual o nível da glicose-6-fosfato desidrogenase esteja anormalmente baixo ou apresente infeções de repetição. É diagnóstico diferencial da doença granulomatosa crônica.J Pediatr (Rio J) 2001; 77 (4): 331-6:deficiência de glucosefosfato desidrogenase, síndromes de deficiência imunológica, neutrófi-los, doença granulomatosa crônica, anemia hemolítica congênita. AbstractObjective: to report a case of rare neutrophil functional disorder with clinical and laboratory findings similar to those of chronic granulomatous disease.Methods: patient with extremely reduced level of glucose-6-phosphate dehydrogenase and recurrent infections that improved after continuous use of cotrimoxazole. The patient presented leukocytes with defective respiratory burst, similar to what occurs in chronic granulomatous disease.Comments: the diagnosis of glucose-6-phosphate dehydrogenase deficiency in neutrophils should be considered in any patient with hemolytic anemia whose level of G6PD is extremely low or in any patient that presents recurrent infections as differential diagnosis of chronic granulomatous disease.J Pediatr (Rio J) 2001; 77 (4): 331-6: glucosephosphate dehydrogenase deficiency, immunodeficiency syndromes, chronic granulomatous disease, neutrophils, congenital hemolytic anemia.Deficiência da glicose-6-fosfato desidrogenase com infeções de repetição: relato de caso IntroduçãoA deficiência da enzima glicose-6-fosfato desidrogenase (G6PD) afeta mais de 200 milhões de pessoas no mundo. Apresenta freqüência relativamente alta em negros americanos (13%) e populações do mediterrâneo (5 a 40%) 1 . Possui herança ligada ao X e tem como principal manifestação a anemia hemolítica. A hemólise pode ser desencadeada por infeções e por algumas drogas com propriedades oxidativas como o ácido acetil salicílico, a vitamina K, o cloranfenicol e antimaláricos 2 . A G6PD presente nos neutrófilos e eritrócitos é codificada pelo mesmo gene 3 , localizado no cromossomo Xq28 e tende a sofrer freqüentes mutações, tendo sido relatadas mais de 200 variantes 4 .

show abstract

Effect of Glucose-6-Phosphate Dehydrogenase Deficiency on Neutrophil Function

Cited by 26 publications

References 11 publications

Glucose-6-phosphate Dehydrogenase Deficiency: A Case Report

Glucose-6-phosphate Dehydrogenase Deficiency: A Case Report

Endotoxins of Enteric Pathogens Are Chemotactic Factors for Human Neutrophils

Deficiência da glicose-6-fosfato desidrogenase com infecções de repetição: relato de caso

Contact Info

Product

Resources

About