Glucose-6-phosphate dehydrogenase deficiency and the use of primaquine: top-down and bottom-up estimation of professional costs.

The aim of this study has been to study whether the top-down method, based on the average value identified in the Brazilian Hospitalization System (SIH/SUS), is a good estimator of the cost of health professionals per patient, using the bottom-up method for comparison. The study has been developed from the context of hospital care offered to the patient carrier of glucose-6-phosphate dehydrogenase (G6PD) deficiency with severe adverse effect because of the use of primaquine, in the Brazilian Amazon. The top-down method based on the spending with SIH/SUS professional services, as a proxy for this cost, corresponded to R$60.71, and the bottom-up, based on the salaries of the physician (R$30.43), nurse (R$16.33), and nursing technician (R$5.93), estimated a total cost of R$52.68. The difference was only R$8.03, which shows that the amounts paid by the Hospital Inpatient Authorization (AIH) are estimates close to those obtained by the bottom-up technique for the professionals directly involved in the care.

Glucose-6-phosphate dehydrogenase deficiency and the use of primaquine: top-down and bottom-up estimation of professional costs / Deficiência de glicose-6-fosfato desidrogenase e uso de primaquina: estimativa de custos de profissionais por macrocusteio e microcusteio

ABSTRACT The aim of this study has been to study whether the top-down method, based on the average value identified in the Brazilian Hospitalization System (SIH/SUS), is a good estimator of the cost of health professionals per patient, using the bottom-up method for comparison. The study has been developed from the context of hospital care offered to the patient carrier of glucose-6-phosphate dehydrogenase (G6PD) deficiency with severe adverse effect because of the use of primaquine, in the Brazilian Amazon. The top-down method based on the spending with SIH/SUS professional services, as a proxy for this cost, corresponded to R$60.71, and the bottom-up, based on the salaries of the physician (R$30.43), nurse (R$16.33), and nursing technician (R$5.93), estimated a total cost of R$52.68. The difference was only R$8.03, which shows that the amounts paid by the Hospital Inpatient Authorization (AIH) are estimates close to those obtained by the bottom-up technique for the professionals directly involved in the care.

RESUMO A pesquisa teve por objetivo estudar se o macrocusteio, baseado no valor médio identificado no Sistema de Internação Hospitalar (SIH/SUS), constitui um bom estimador do custo de profissionais de saúde por paciente, tendo como comparação o método de microcusteio. O estudo foi desenvolvido no contexto da assistência hospitalar oferecida ao portador da deficiência de glicose-6-fosfato desidrogenase (dG6PD) do sexo masculino com evento adverso grave devido ao uso da primaquina, na Amazônia Brasileira. O macrocusteio baseado no gasto em serviços profissionais do SIH/SUS, como proxy desse custo, correspondeu a R$60,71, e o microcusteio, baseado nos salários do médico (R$30,43), do enfermeiro (R$16,33) e do técnico de enfermagem (R$5,93), estimou um custo total de R$52,68. A diferença foi de apenas R$8,03, mostrando que os valores pagos pela Autorização de Internação Hospitalar (AIH) são estimadores próximos daqueles obtidos por técnica de microcusteio para os profissionais envolvidos diretamente no cuidado.

Glucose-6-phosphate dehydrogenase deficiency A- variant in febrile patients in Haiti.

Haiti is one of two remaining malaria-endemic countries in the Caribbean. To decrease malaria transmission in Haiti, primaquine was recently added to the malaria treatment public health policy. One limitation of primaquine is that, at certain doses, primaquine can cause hemolytic anemia in individuals with glucose-6-phosphate dehydrogenase (G6PD) deficiency (G6PDd). In this study, we genotyped two mutations (A376G and G202A), which confer the most common G6PDd variant in West African populations, G6PDd A-. We estimated the frequency of G6PDd A- in a sample of febrile patients enrolled in an on-going malaria study who represent a potential target population for a primaquine mass drug administration. We found that 33 of 168 individuals carried the G6PDd A- allele (includes A- hemizygous males, A- homozygous or heterozygous females) and could experience toxicity if treated with primaquine. These data inform discussions on safe and effective primaquine dosing and future malaria elimination strategies for Haiti.

High frequency of diabetes and impaired fasting glucose in patients with glucose-6-phosphate dehydrogenase deficiency in the Western brazilian Amazon.

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is one of the most common human genetic abnormalities, and it has a significant prevalence in the male population (X chromosome linked). The purpose of this study was to estimate the frequency of impaired fasting glucose and diabetes among G6PD-deficient persons in Manaus, Brazil, an area in the Western Brazilian Amazon to which malaria is endemic. Glucose-6-phosphate dehydrogenase-deficient males had more impaired fasting glucose and diabetes. This feature could be used as a screening tool for G6PD-deficient persons who are unable to use primaquine for the radical cure of Plasmodium vivax malaria.

Performance of the CareStart glucose-6-phosphate dehydrogenase (G6PD) rapid diagnostic test in Gressier, Haiti.

Administering primaquine (PQ) to treat malaria patients with glucose-6-phosphate dehydrogenase (G6PD) deficiency can pose a serious risk of drug-induced hemolysis (DIH). New easy to use point-of-care rapid diagnostic tests are being developed as an alternative to labor-intensive spectrophotometric methods, but they require field testing before they can be used at scale. This study screened 456 participants in Gressier, Haiti using the Access Bio CareStart qualitative G6PD rapid detection test compared with the laboratory-based Trinity Biotech quantitative spectrophotometric assay. Findings suggest that the CareStart test was 90% sensitive for detecting individuals with severe deficiency and 84.8% sensitive for detecting individuals with moderate and severe deficiency compared with the Trinity Biotech assay. A high negative predictive value of 98.2% indicates excellent performance in determining those patients able to take PQ safely. The CareStart G6PD test holds much value for screening malaria patients to determine eligibility for PQ therapy.

Efecto hemolítico de la cloroquina en estudiantes deficientes de glucosa-6-fosfato deshidrogenasa / Hemolytic effect of chloroquine in students with glucose-6-phosphate dehydrogenase deficiency

Se estudió la presencia de estudiantes portadores de la deficiencia en la enzima glucosa-6-fosfato deshidrogenasa eritrocitaria en el Instituto de Ciencias Básicas y Preclínicas "Victoria de Girón" mediante el método del azul de metileno, la actividad enzimática y la electroforesis en geles de almidón. Luego se estudió el efecto hemolítico de la administración profiláctica de fosfato de cloroquina en estos sujetos, estudiando las variaciones con significación clínica en la concentración de hemoglobina y el hematócrito, antes y después del tratamiento con este fármaco. Se descartó la presencia de enfermedades asociadas a cuadros hemolíticos en sujetos portadores de esta deficiencia. De los 994 estudiantes latinoamericanos que arribaron al instituto en el año 2001, 8 fueron confirmados como deficientes de G6PD y el tratamiento de cloroquina suministrado no provocó hemólisis(AU)

Hemólisis y tratamiento con primaquina. Informe Preliminar / Hemolysis and treatment with primaquine. A preliminary report

Se estudiaron 8 pacientes con el diagnóstico de paludismo por Plasmodium vivax y deficiencia de glucosa 6 fosfato deshidrogenasa, que debían recibir tratamiento radical antipalúdico con primaquina. Se determinó que el 87,5 por ciento de los paciente presentó hemólisis, pero la relación de ésta con la actividad enzimática no fue significativa (p < 0,05) el 50 por ciento de los pacientes no pudo concluir el tratamiento por la aparición de hemólisis importante. Concluimos que en los pacientes con déficit de glucosa 6 fosfato deshidrogenasa no debe hacerse uso indiscriminado de la primaquina(AU)

Hemólisis y tratamiento con primaquina. Informe Preliminar / Hemolysis and treatment with primaquine. A preliminary report

Se estudiaron 8 pacientes con el diagnóstico de paludismo por Plasmodium vivax y deficiencia de glucosa 6 fosfato deshidrogenasa, que debían recibir tratamiento radical antipalúdico con primaquina. Se determinó que el 87,5 por ciento de los paciente presentó hemólisis, pero la relación de ésta con la actividad enzimática no fue significativa (p < 0,05) el 50 por ciento de los pacientes no pudo concluir el tratamiento por la aparición de hemólisis importante. Concluimos que en los pacientes con déficit de glucosa 6 fosfato deshidrogenasa no debe hacerse uso indiscriminado de la primaquina

Improved erythrocyte survival with combined vitamin E and selenium therapy in children with glucose-6-phosphate dehydrogenase deficiency and mild chronic hemolysis.

To study the antioxidant effect of high-dose vitamin E alone and in combination with selenium in patients with glucose-6-phosphate dehydrogenase deficiency with mild chronic hemolysis, 36 male children with such manifestations were enrolled consecutively into two equal groups. Group 1 received 800 IU vitamin E daily, and group 2 received 800 IU vitamin E in combination with 25 micrograms selenium. Hematologic status before and 2 months after treatment was evaluated. After treatment there was a significant change toward normal in both groups. The mean red cell half-life increased in group 1 from 16.9 to 22.8 days (P less than 0.01), and in group 2 from 15.6 to 24.3 days (P less than 0.01). A comparison of the mean difference of paired values in the two groups revealed a more significant increase in hemoglobin (0.9 +/- 0.1 gm/dl vs 1.2 +/- 0.2 gm/dl, P less than 0.05), hematocrit (2.4% +/- 0.4% vs 3.8% +/- 0.3%, P less than 0.05), and red cell half-life (5.9 +/- 3.0 days vs 9.1 +/- 4.4 days, P less than 0.01), and more significant reduction in reticulocytes (-0.7% +/- 0.2% vs -1.5% +/- 0.4%, P less than 0.01) in group 2. Clinical assessment and follow-up indicated no side effects related to the drugs.

Studies in type I glycogenosis: the paradoxical effect of ethanol on lactate.

Paradoxically, ethanol, which raises lactate in normal individuals, lowers the elevated levels of lactate in patients with Type I glycogenosis. We found that, although lactate levels fell, pyruvate proportionately declined even more, resulting in an increased L/P ratio which indicates that, as in the normal, the oxidation of ethanol had generated NADH. In type I glycogenosis, the increased level of pyruvate-lactate derives from glycogenolysis. We found that, despite continued glycogenolysis, ethanol had caused less pyruvate-lactate to form. The effect of an increased NADH/NAD+ ratio on the flow of carbon through the Embden-Meyerhof pathway could account for the finding, presumably by its effect on the oxidation-reduction couples with diversion of carbon toward formation of triglyceride rather than pyruvate-lactate.