ABSTRACT
Paciente de 7 anos, sexo masculino, portador de miocardiopatia restritiva relacionada ao gene BAG3 e histórico de hipertensão pulmonar com insuficiência tricúspede importante, admitido para avalição de transplante cardíaco. Após a cirurgia deu entrada na UTI estável mas evoluiu no 1º PO com acidose metabólica, choque refratário ao volume e hipoxemia refratária a manobras de fisioterapia. Os níveis de metahemoglobina foram dosados e o óxido nítrico foi imediatamente suspenso. Iniciou-se administração endovenosa de azul de metileno na dose 3mg/kg com melhora progressiva do quadro clínico. Os níveis de metahemoglobina eram 29,05 e 5,2 ao início e final da administração da droga, respectivamente. Conclusão: Metahemoglobinemia deve ser suspeitada em pacientes que utilizam NO. Níveis elevados de metahemoglobina no sangue podem ser incompatíveis com a vida. O tratamento deve ser instituído de forma rápida e eficaz.
A 7-year-old boy was admitted for evaluation to heart transplantation with a restrictive cardiopathy congenital associated to BAG3 gene. During childhood, he developed high pulmonary hipertension with a significant tricuspid insufficiency. At first post-operative, patient has evoluted with metabolic acidosis, shock refractory to volume and refractory hypoxemia presented at maneuvers physiotherapy. The levels of methemoglobin were checked on laboratory and the nitric oxide was immediately suspended. It started the administration of methylene blue in 3 mg/kg with progressive improvement of clinical situation. Levels of metheboglobin were 29,05 before the treatment and 5,2 after methylene blue administration. Conclusion: Metahemoglobinemia must be suspected in patients using NO. Elevated levels of metahemoglobin may be incompatible with life. Treatment should be instituted quickly and effectively.
Subject(s)
Humans , Male , Child , Child , Heart Transplantation , Hemoglobins , Methemoglobinemia , Methemoglobin/analysis , Nitric Oxide , PediatricsABSTRACT
Abstract Objective To determine eight parameters of oxidative stress markers in erythrocytes from children with sickle cell disease and compare with the same parameters in erythrocytes from healthy children, since oxidative stress plays an important role in the pathophysiology of sickle cell disease and because this disease is a serious public health problem in many countries. Methods Blood samples were obtained from 45 children with sickle cell disease (21 males and 24 females with a mean age of 9 years; range: 3–13 years) and 280 blood samples were obtained from children without hemoglobinopathies (137 males and 143 females with a mean age of 10 years; range: 8–11 years), as a control group. All blood samples were analyzed for methemoglobin, reduced glutathione, thiobarbituric acid reactive substances, percentage of hemolysis, reactive oxygen species, and activity of the enzymes glucose 6-phosphate dehydrogenase, superoxide dismutase, and catalase. Data were analyzed using Student's t-test and were expressed as the mean ± standard deviation. A p-value of <0.05 was considered significant. Results Significant differences were observed between children with sickle cell disease and the control group for the parameters methemoglobin, thiobarbituric acid reactive substances, hemolysis, glucose 6-phosphate dehydrogenase activity, and reactive oxygen species, with higher levels in the patients than in the controls. Conclusions Oxidative stress parameters in children's erythrocytes were determined using simple laboratory methods with small volumes of blood; these biomarkers can be useful to evaluate disease progression and outcomes in patients.
Resumo Objetivo Determinar parâmetros de estresse oxidativo em eritrócitos de crianças com doença falciforme e compará-los com os mesmos parâmetros em eritrócitos de crianças saudáveis, pois o estresse oxidativo desempenha um importante papel na fisiopatologia da doença falciforme, considerada um sério problema de saúde pública em muitos países. Métodos Foram obtidas amostras de sangue de 45 crianças com doença falciforme (21 meninos e 24 meninas com média de 9 anos, variação de 3 a 13) e 280 amostras de sangue de crianças sem hemoglobinopatias (137 meninos e 143 meninas com média de 10 anos, variação de 8 a 11), como grupo controle. Em todas as amostras foram determinados meta-hemoglobina, glutationa reduzida, substâncias reativas ao ácido tiobarbitúrico, porcentagem de hemólise, espécies reativas de oxigênio e atividade das enzimas glucose6-fosfato desidrogenase, superóxido dismutase e catalase. Os dados foram analisados com o teste t de Student e foram expressos como média ± desvio padrão. Um valor de p < 0,05 foi considerado significativo. Resultados Foram observadas diferenças significativas entre as crianças com doença falciforme e o grupo controle para os parâmetros meta-hemoglobina, substâncias reativas ao ácido tiobarbitúrico, porcentagem de hemólise, espécies reativas de oxigênio e atividade da enzima glucose6-fosfato desidrogenase, com níveis aumentados nos pacientes. Conclusões Foi possível determinar parâmetros de estresse oxidativo em eritrócitos de crianças, com técnicas laboratoriais simples e pequenos volumes de sangue. Esses biomarcadores podem ser úteis na avaliação da progressão e dos resultados de tratamentos da doença.
Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , Oxidative Stress/physiology , Erythrocytes/metabolism , Anemia, Sickle Cell/blood , Reference Values , Superoxide Dismutase/blood , Methemoglobin/analysis , Biomarkers/blood , Catalase/blood , Case-Control Studies , Reactive Oxygen Species/blood , Statistics, Nonparametric , Glucosephosphate Dehydrogenase/blood , Glutathione/blood , Hemolysis/physiology , Anemia, Sickle Cell/physiopathologyABSTRACT
Introduction: Hemoglobin S (HbS) is unstable hemoglobin that easily oxidizes, causing methemoglobin (MetHb) increased production in patients with sickle-cell anemia (SCA). Objectives: To determine MetHb levels and the influence of hydroxyurea (HU) therapy on this marker in patients with SCA. Materials and methods: Blood samples from 53 patients with SCA at the steady-state, with and without HU therapy, and 30 healthy individuals were collected to evaluate MetHb levels. The MetHb measurement was performed by spectrophotometry. Complete blood count, HU measurements, and fetal hemoglobin (HbF) and HbS concentrations were taken from medical records. Results: MetHb levels were statically higher in patients with SCA when compared to control group (p < 0.001). There was no statistical difference in MetHb level between SCA patients, either using or not HU. We obtained a positive correlation between MetHb measurements and HbS concentration (r = 0.2557; p = 0.0323). Conclusion: HbS presence favored hemoglobin breaking down, and consequently increased MetHb production. Treatment with HU, however, did not influence the levels of this marker...
Introdução: A hemoglobina S (HbS) é uma hemoglobina instável que facilmente se oxida, causando aumento da produção de metemoglobina (MetHb) em pacientes com anemia falciforme (AF). Objetivos: Determinar os níveis de MetHb e verificar a influência do tratamento com a hidroxiureia (HU) sobre as dosagens desse marcador em pacientes com AF. Materiais e métodos: Amostras de sangue de 53 pacientes adultos com AF em estado basal, em uso ou não de HU, e 30 indivíduos saudáveis foram coletadas para avaliar os níveis de MetHb. A dosagem de MetHb foi realizada pelo método espectrofotométrico. Os parâmetros hematológicos, a dosagem de HU e a concentração de hemoglobina F (HbF) e HbS foram retirados dos prontuários médicos. Resultados: Níveis de MetHb apresentaram-se mais elevados estatisticamente em pacientes com AF em relação ao grupo-controle (p < 0,0001). Não foi verificada diferença estatística nos níveis de MetHb entre pacientes em uso ou não de HU. Observou-se correlação positiva entre as dosagens de MetHb e a concentração de HbS (r = 0,2557; p = 0,0323). Conclusão: A presença da HbS favoreceu a degradação da hemoglobina, causando elevação da produção de MetHb. Tratamento com HU, entretanto, não influenciou nos níveis desse marcador...
Subject(s)
Humans , Adult , Anemia, Sickle Cell/drug therapy , Antineoplastic Agents/therapeutic use , Hydroxyurea/therapeutic use , Methemoglobin/administration & dosage , Case-Control Studies , Methemoglobin/analysisABSTRACT
Methemoglobin (Hb-M) is a rare hemoglobinopathy in China. We hereby report on a family living in Yantai, East China, with congenital cyanosis due to Hb-M mutation. The proband, a 65-year-old female, presented 63 percent oxygen saturation. Both Hb-M concentration and arterial oxygen saturation remained unchanged, even following intravenous treatment with methylene blue. There was also no change in blood-color (chocolate-brown) after adding 0.1 percent KCN. A fast-moving band (Hb-X) in hemolysates was found by cellulose acetate electrophoresis, the Hb-X/Hb-A ratio exceeding 10 percent. GT transition at 131nt of exon 2, although present in one of the alfa2-globin alleles, was not found in alfa1-globin alleles as a whole. This mutation leads to the aspartic acid to tyrosine substitution (Asp76Tyr). In this family, the novel mutation in the alfa2-globin gene resulted in a rare form of congenital cyanosis due to Hb-M. This hemoglobin was named Hb-M Yantai.
Subject(s)
Humans , Female , Aged , Cyanosis/congenital , Hemoglobin M , Methemoglobinemia/congenital , China , Globins , Methemoglobin/analysis , Polymerase Chain Reaction/methodsABSTRACT
Background. Pulse oximeters are frequently used in the clinical practice and we must known their precision and accuracy. The objective was to evaluate the precision and accuracy of a "pocket" pulse oximeter at an altitude of 2,240 m above sea level. Methods. We tested miniature pulse oximeters (Onyx 9,500, Nonin Finger Pulse Oximeter) in 96 patients sent to the pulmonary laboratory for an arterial blood sample. Patients were tested with 5 pulse oximeters placed in each of the fingers of the hand oposite to that used for the arterial puncture. The gold standard was the oxygen saturation of the arterial blood sample. Results. Blood samples had SaO2 of 87.2 ± 11.0 (between 42.2 and 97.9%). Pulse oximeters had a mean error of 0.28 ± 3.1%. SaO2 - (1.204 x SpO2) - 17.45966 (r = 0.92, p < 0.0001). Intraclass correlation coefficient between each of five pulse oximeters against the arterial blood standard ranged between 0.87 and 0.99. HbCO (2.4 ± 0.6) did not affect the accuracy. Conclusions. The miniature oximeter Nonin is precise and accurate at 2,240 m of altitude. The observed levels of HbCO did not affect the performance of the equipment. The oximeter good performance, small size and low cost enhances its clinical usefulness.
Introducción. Los oxímetros de pulso son ampliamente utilizados en la práctica clínica y su precisión y exactitud deben conocerse especialmente en altitudes moderadas como en la ciudad de México. El objetivo principal fue evaluar la precisión y exactitud de un oxímetro de pulso "de bolsillo" (Onyx 9500 de Nonin®) en la ciudad de México. Métodos. Se realizaron mediciones de la saturación de oxígeno estimada por el oxímetro de pulso (SpO2) y se compararon contra la medida espectrofotométricamente en sangre arterial (SaO2) en 96 pacientes que acudieron al laboratorio de fisiología pulmonar del Instituto Nacional de Enfermedades Respiratorias. A cada paciente se le colocaron cinco oxímetros de pulso en los dedos de una mano obteniéndose un promedio de SpO2 para cada uno de los oxímetros y para los cinco oxímetros a la vez, mediciones que se compararon de manera simultánea contra el estándar. Resultados. Las muestras sanguíneas de los 96 pacientes que participaron tuvieron una SaO2 de 87.2 ± 11.0 (entre 42.2-97.9%). Los oxímetros (promedio de los cinco) tuvieron un error de medición promedio de 0.28 ± 3.1%. SaO2 = (1.204 x SpO2) - 17.45966 (r = 0.92, p < 0.0001). Los coeficientes de correlación intraclase que se obtuvieron al comparar los oxímetros entre sí y contra el estándar (SaO2) estuvieron entre 0.87 y 0.99. El nivel de carboxihemoglobina (HbCO) fue de 2.4 ± 0.6% y no afectó significativamente la exactitud del oxímetro. Conclusiones. El oxímetro digital de pulso "de bolsillo" es preciso y exacto en la estimación de la SaO2 a 2,240 metros de altitud. Los niveles observados de HbCO (2.4%) no afectaron su exactitud. Se observó un adecuado desempeño del oxímetro digital de pulso en la ciudad de México.
Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Oximetry/instrumentation , Oxygen/blood , Altitude , Hypoxia/blood , Hypoxia/diagnosis , Hypoxia/epidemiology , Carboxyhemoglobin/analysis , Environmental Exposure , Fingers , Hemoglobinometry/methods , Hemoglobins/analysis , Methemoglobin/analysis , Mexico/epidemiology , Oximetry/economics , Oxyhemoglobins/analysis , Reproducibility of Results , Sensitivity and Specificity , Smoke , Spectrophotometry , Smoking/blood , Smoking/epidemiology , Tobacco Smoke Pollution , Urban PopulationABSTRACT
Erythrocyte glucose-6-phosphate dehydrogenase (G6PD) and pyruvate kinase (PK) activities were studied in hemoglobin H (HbH) patients by spectrophotometric method, cytochemical method and the methemoglobin reduction (MR) test for the detection of heterozygous G6PD deficiency. G6PD deficiency was found in 7 of 64 cases (10.9%), including 3 cases of genotype alpha 1/alpha 2 and 4 cases of genotype alpha 1/CS. None of the HbH patients was found to be PK-deficient. Spectrophotometrically determined G6PD and PK activities were significantly higher in HbH patients than in normals (p less than 0.001), whereas the MR test yielded a significantly lower percentage of residual methemoglobin in HbH patients than in normals (p less than 0.05). All three methods were efficient in the detection of hemizygous G6PD deficiency in HbH patients, but not in G6PD-deficient females.