ABSTRACT
We describe a successful perioperative management of a case of 38-year-old male, presented with chronic jaundice with severe mitral stenosis and moderate tricuspid regurgitation; upon evaluation, he was found to have severe glucose-6-phosphate dehydrogenase (G6PD) deficiency. Usually, patients deficient in G6PD exhibit increased hemolysis andtherefore increased need for blood transfusion after cardiac surgery as well as impaired oxygenation in the postoperative period leading to prolonged ventilation. On reperfusion after a period of ischemia, the antioxidant system recruits all of its components in an attempt to neutralize the overwhelming oxidative stress of free radicals, as the free radical scavenging system is deficient in these patients, the chances of free-radical-induced injury is more. Our patient underwent mitral valve replacement and tricuspid annuloplasty under cardiopulmonary bypass with necessary precautions to reduce the formation of free radicals. Treatment was targeted toward theprevention of free radical injuryin the G6PD-deficient patient. He had an uneventful intraoperative and postoperative course.
Subject(s)
Adult , Cardiac Surgical Procedures , Cardiopulmonary Bypass , Glucosephosphate Dehydrogenase Deficiency/metabolism , Humans , Male , Preoperative CareABSTRACT
Influx of the purine nucleoside, adenosine, was assessed in erythrocytes from both normal subjects and from subjects with a range of genetically determined erythrocyte disorders from Myanmar. The latter included alpha-thalassemia major (Myanmar variant), beta-thalassemia major (Myanmar variant), beta-thalassemia trait, HbEE and HbAE erythrocytes and two variants of glucose-6-phosphate dehydrogenase (G6PDH) deficiency. Significant reductions (p < 0.01) of adenosine influx were observed in erythrocytes from individuals with alpha- and beta-thalassemia major and severe G6PDH deficiency. Abnormal erythrocytes infected with the malarial parasites, Plasmodium falciparum or Plasmodium vivax, demonstrated a reduction in adenosine transport which correlated with the proportion of abnormal erythrocytes present in the samples obtained. The effect of nitrobenzylthioinosine (NBMPR) on adenosine influx was explored in normal and abnormal erythrocytes. In all these cases, NBMPR completely inhibited the transport of adenosine. However, transport of adenosine into P. falciparum and P. vivax-infected normal erythrocytes and abnormal cells was only inhibited 50-60% by NBMPR. The combination of tubercidin and NBMPR completely blocked adenosine transport into both normal and abnormal erythrocytes infected with either P. falciparum or P. vivax.
Subject(s)
Adenosine/blood , Adult , Affinity Labels/pharmacology , Child , Erythrocytes/metabolism , Erythrocytes, Abnormal/metabolism , Female , Glucosephosphate Dehydrogenase Deficiency/metabolism , Hemoglobinopathies/blood , Humans , Malaria, Falciparum/blood , Malaria, Vivax/blood , Male , Myanmar , Thioinosine/analogs & derivatives , Tubercidin/pharmacologyABSTRACT
Na presente pesquisa, o objetivo foi avaliar de forma mais precisa a secreçäo da insulina através da quantificaçäo do peptideo C plasmático após estímulo glicídico, assim como, após a L-arginina, outro estimulante da secreçäo de insulina, além da sensibilidade periférica à insulina. Constou dos objetivos avaliar a secreçäo de cortisol após estímulo com ACTH. Para tanto, foram estudados 11 indivíduos com deficiência de G-6-PD e 11 indivíduos controle que formaram o grupo I e foram submetidos aos testes para avaliar a funçäo secretória da célula pancreática (teste endovenoso de tolerância à glicose - TETG e teste de arginina) e a sensibilidade periférica à insulina (teste de tolerância à insulina - TTI). E, o grupo II composto com 12 indivíduos com deficiência de G-6-PD e 16 indivíduos com deficiência de G-6-PD e 16 indivíduos controle que foram submetidos à estimulaçäo das adrenais com ACTH (Teste de cortisol). Para os dois grupos de estudo os procedimentos experimentais foram realizados após jejum de 12-14 h. Os resultados demonstram que pacientes com deficiência de G-6-PD apresentam diminuiçäo na secreçäo de insulina (fase rápida) após estímulo com glicose e L-arginina, sensibilidade periférica à insulina semelhante a de indivíduos controle e secreçäo de cortisol diminuida durante a primeira hora após estimulaçäo com ACTH
Subject(s)
Humans , Male , Female , Glucosephosphate Dehydrogenase Deficiency/metabolism , Insulin/pharmacokinetics , Insulin/metabolismABSTRACT
In this article, the authors presente a review about the behaviour of G6PD in relation to pathologies other than hemolytic anemias, which are the many diagnostic use of the enzyme