Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 1.356
Filtrar
1.
Ann Hematol ; 100(3): 667-673, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33439304

RESUMO

The severe pneumonia caused by the human coronavirus (hCoV)-SARS-CoV-2 has inflicted heavy casualties, especially among the elderly and those with co-morbid illnesses irrespective of their age. The high mortality in African-Americans and males, in general, raises the concern for a possible X-linked mediated process that could affect the viral pathogenesis and the immune system. We hypothesized that G6PD, the most common X-linked enzyme deficiency, associated with redox status, may have a role in severity of pneumonia. Retrospective chart review was performed in hospitalized patients with COVID19 pneumonia needing supplemental oxygen. A total of 17 patients were evaluated: six with G6PD deficiency (G6PDd) and 11 with normal levels. The two groups (normal and G6PDd) were comparable in terms of age, sex, co-morbidities, and laboratory parameters-LDH, IL-6, CRP, and ferritin, respectively. Thirteen patients needed ventilatory support ; 8 in the normal group and 5 in the G6PDd group (72% vs.83%). The main differences indicating increasing severity in normal vs. G6PDd groups included G6PD levels (12.2 vs. 5.6, P = 0.0002), PaO2/FiO2 ratio (159 vs. 108, P = 0.05), days on mechanical ventilation (10.25 vs. 21 days P = 0.04), hemoglobin level (10 vs. 8.1 P = 0.03), and hematocrit (32 vs. 26 P = 0.015). Only one patient with G6PDd died; 16 were discharged home. Our clinical series ascribes a possible biological role for G6PDd in SARS-CoV2 viral proliferation. It is imperative that further studies are performed to understand the interplay between the viral and host factors in G6PDd that may lead to disparity in outcomes. KEY POINTS: • COVID19 studies show higher mortality in men, due to severe pneumonia and ARDS, indicating possible X-linked mediated differences • G6PD, the most common X-linked enzymopathy, highly prevalent in African Americans and Italians, maintains redox homeostasis. • Preclinical studies using G6PD deficient (G6PDd) cells infected with human coronavirus (hCoV), show impaired cellular responses, viral proliferation and worsening oxidative damage. • Retrospective chart review in hospitalized patients with COVID19 pneumonia needing supplemental oxygen shows differences between the two groups (Normal and G6PDd) in hematological indices; the G6PDdgroup demonstrated prolonged PaO2/FiO2 ratio, and longer days on mechanical ventilation indicating the severity of the pneumonia.


Assuntos
/complicações , Deficiência de Glucosefosfato Desidrogenase/complicações , /etiologia , Afro-Americanos , /diagnóstico , Contraindicações de Medicamentos , Cuidados Críticos , Feminino , Predisposição Genética para Doença , Deficiência de Glucosefosfato Desidrogenase/sangue , Deficiência de Glucosefosfato Desidrogenase/etnologia , Deficiência de Glucosefosfato Desidrogenase/fisiopatologia , Humanos , Hidroxicloroquina/efeitos adversos , Hidroxicloroquina/uso terapêutico , Masculino , Pessoa de Meia-Idade , Estresse Oxidativo , Respiração Artificial , /terapia , Estudos Retrospectivos , Índice de Gravidade de Doença , Distribuição por Sexo
2.
Cochrane Database Syst Rev ; 9: CD010458, 2020 09 06.
Artigo em Inglês | MEDLINE | ID: mdl-32892362

RESUMO

BACKGROUND: Plasmodium vivax malaria has a persistent liver stage that causes relapse of the disease and continued P vivax transmission. Primaquine (PQ) is used to clear the liver stage of the parasite, but treatment is required for 14 days. Primaquine also causes haemolysis in people with glucose-6-phosphate dehydrogenase (G6PD) deficiency. Tafenoquine (TQ) is a new alternative to PQ with a longer half-life and can be used as a single-dose treatment. OBJECTIVES: To assess the effects of tafenoquine 300 mg (single dose) on preventing P vivax relapse. SEARCH METHODS: We searched the following up to 3 June 2020: the Cochrane Infectious Diseases Group Specialized Register; CENTRAL; MEDLINE; Embase; and three other databases. We also searched the WHO International Clinical Trial Registry Platform and the metaRegister of Controlled Trials for ongoing trials using "tafenoquine" and "malaria" as search terms up to 3 June 2020. SELECTION CRITERIA: Randomized controlled trials (RCTs) that gave TQ to prevent relapse in people with P vivax malaria. We planned to include trials irrespective of whether participants had been screened for G6PD enzyme deficiency. DATA COLLECTION AND ANALYSIS: All review authors independently extracted data and assessed risk of bias. As true relapse and reinfection are difficult to differentiate in people living in endemic areas, studies report "recurrences" of infection as a proxy for relapse. We carried out meta-analysis where appropriate, and gave estimates as risk ratios (RR) with 95% confidence intervals (CI). We assessed the certainty of the evidence using the GRADE approach. MAIN RESULTS: Three individually randomized RCTs met our inclusion criteria, all in endemic areas, and thus reporting recurrence. Trials compared TQ with PQ or placebo, and all participants received chloroquine (CQ) to treat the asexual infection). In all trials, pregnant and G6PD-deficient people were excluded. Tafenoquine 300 mg single dose versus no treatment for relapse prevention Two trials assessed this comparison. TQ 300 mg single dose reduces P vivax recurrences compared to no antihypnozoite treatment during a six-month follow-up, but there is moderate uncertainty around effect size (RR 0.32, 95% CI 0.12 to 0.88; 2 trials, 504 participants; moderate-certainty evidence). In people with normal G6PD status, there is probably little or no difference in any type of adverse events (2 trials, 504 participants; moderate-certainty evidence). However, we are uncertain if TQ causes more serious adverse events (2 trials, 504 participants; very low-certainty evidence). Both RCTs reported a total of 23 serious adverse events in TQ groups (One RCT reported 21 events) and a majority (15 events) were a drop in haemoglobin level by > 3g/dl (or >30% reduction from baseline). Tafenoquine 300 mg single dose versus primaquine 15 mg/day for 14 days for relapse prevention Three trials assessed this comparison. There is probably little or no difference between TQ and PQ in preventing recurrences (proxy measure for relapse) up to six months of follow-up (RR 1.04, 95% CI 0.8 to 1.34; 3 trials, 747 participants; moderate-certainty evidence). In people with normal G6PD status, there is probably little or no difference in any type of adverse events (3 trials, 747 participants; moderate-certainty evidence). We are uncertain if TQ can cause more serious adverse events compared to PQ (3 trials, 747 participants; very low-certainty evidence). Two trials had higher point estimates against TQ while the other showed the reverse. Most commonly reported serious adverse event in TQ group was a decline in haemoglobin level (19 out of 29 events). Some other serious adverse events, though observed in the TQ group, are unlikely to be caused by it (Hepatitis E infection, limb abscess, pneumonia, menorrhagia). AUTHORS' CONCLUSIONS: TQ 300 mg single dose prevents relapses after clinically parasitologically confirmed P vivax malaria compared to no antihypnozoite treatment, and with no difference detected in studies comparing it to PQ to date. However, the inability to differentiate a true relapse from a recurrence in the available studies may affect these estimates. The drug is untested in children and in people with G6PD deficiency. Single-dose treatment is an important practical advantage compared to using PQ for the same purpose without an overall increase in adverse events in non-pregnant, non-G6PD-deficient adults.


Assuntos
Aminoquinolinas/administração & dosagem , Antimaláricos/administração & dosagem , Malária Vivax/tratamento farmacológico , Primaquina/administração & dosagem , Prevenção Secundária , Adulto , Aminoquinolinas/efeitos adversos , Antimaláricos/efeitos adversos , Cloroquina/administração & dosagem , Cloroquina/efeitos adversos , Esquema de Medicação , Deficiência de Glucosefosfato Desidrogenase/complicações , Humanos , Parasitemia/tratamento farmacológico , Placebos , Primaquina/efeitos adversos , Ensaios Clínicos Controlados Aleatórios como Assunto , Recidiva
3.
PLoS Negl Trop Dis ; 14(9): e0008697, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32925910

RESUMO

The proportion of Plasmodium vivax malaria among all malarias is increasing worldwide. Treatment with 8-aminoquinolines remain the only radical cure. However, 8-aminoquinolines can cause severe hemolysis in glucose-6-phosphate dehydrogenase (G6PD) deficient patients. The population of the multi-ethnic Chittagong Hill Tracts (CHT) carry the highest malaria burden within Bangladesh. As in many countries the national treatment guidelines recommend 8-aminoquinoline based radical cure without routine G6PD deficiency (G6PDd) testing to guide treatment. Aim of this study was to determine the need for routine testing within a multi-ethnic population by assessing the prevalence of G6PDd among the local population. Participants from 11 ethnicities were randomly selected and malaria status was assessed by microscopy, rapid diagnostic test (RDT) and polymerase chain reaction (PCR). G6PD status was determined by spectrophotometry and G6PD genotyping. The adjusted male median (AMM) was defined as 100% G6PD activity, participants were categorized as G6PD deficient (<30% activity), G6PD intermediate (30% to 70% activity) or G6PD normal (>70% activity). Median G6PD activities between ethnicities were compared and the association between G6PD activity and malaria status was assessed. 1002 participants were enrolled and tested for malaria. G6PD activity was measured by spectrophotometry in 999 participants and host G6PD genotyping undertaken in 323 participants. Seven participants (0.7%) had peripheral parasitaemia detected by microscopy or RDT and 42 by PCR (4.2%). Among 106 participants (32.8%) with confirmed genotype, 99 (93.4%) had the Mahidol variant. The AMM was 7.03U/gHb with 90 (9.0%) G6PD deficient participants and 133 (13.3%) with intermediate G6PD activity. Median G6PD activity differed significantly between ethnicities (p<0.001), proportions of G6PD deficient individuals ranged from 2% to 26% but did not differ between participants with and without malaria. The high G6PDd prevalence and significant variation between ethnicities suggest routine G6PDd testing to guide 8-aminoquinoline based radical in the CHT and comparable settings.


Assuntos
Grupos Étnicos/genética , Deficiência de Glucosefosfato Desidrogenase/epidemiologia , Deficiência de Glucosefosfato Desidrogenase/genética , Glucosefosfato Desidrogenase/genética , Glucosefosfato Desidrogenase/metabolismo , Adulto , Aminoquinolinas/efeitos adversos , Aminoquinolinas/uso terapêutico , Bangladesh/epidemiologia , Testes Diagnósticos de Rotina , Feminino , Deficiência de Glucosefosfato Desidrogenase/complicações , Deficiência de Glucosefosfato Desidrogenase/diagnóstico , Humanos , Malária Vivax/complicações , Malária Vivax/tratamento farmacológico , Malária Vivax/epidemiologia , Masculino , Pessoa de Meia-Idade , Reação em Cadeia da Polimerase , Prevalência , Adulto Jovem
5.
Clin Immunol ; 219: 108544, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32707089

RESUMO

Glucose 6-phosphate dehydrogenase (G6PD) deficiency facilitates human coronavirus infection due to glutathione depletion. G6PD deficiency may especially predispose to hemolysis upon coronavirus disease-2019 (COVID-19) infection when employing pro-oxidant therapy. However, glutathione depletion is reversible by N-acetylcysteine (NAC) administration. We describe a severe case of COVID-19 infection in a G6PD-deficient patient treated with hydroxychloroquine who benefited from intravenous (IV) NAC beyond reversal of hemolysis. NAC blocked hemolysis and elevation of liver enzymes, C-reactive protein (CRP), and ferritin and allowed removal from respirator and veno-venous extracorporeal membrane oxygenator and full recovery of the G6PD-deficient patient. NAC was also administered to 9 additional respirator-dependent COVID-19-infected patients without G6PD deficiency. NAC elicited clinical improvement and markedly reduced CRP in all patients and ferritin in 9/10 patients. NAC mechanism of action may involve the blockade of viral infection and the ensuing cytokine storm that warrant follow-up confirmatory studies in the setting of controlled clinical trials.


Assuntos
Acetilcisteína/uso terapêutico , Antioxidantes/uso terapêutico , Betacoronavirus/patogenicidade , Infecções por Coronavirus/tratamento farmacológico , Síndrome da Liberação de Citocina/tratamento farmacológico , Deficiência de Glucosefosfato Desidrogenase/tratamento farmacológico , Pneumonia Viral/tratamento farmacológico , Adulto , Antirreumáticos/uso terapêutico , Biomarcadores/sangue , Proteína C-Reativa/metabolismo , Infecções por Coronavirus/sangue , Infecções por Coronavirus/complicações , Infecções por Coronavirus/virologia , Síndrome da Liberação de Citocina/sangue , Síndrome da Liberação de Citocina/complicações , Síndrome da Liberação de Citocina/virologia , Esquema de Medicação , Ferritinas/sangue , Produtos de Degradação da Fibrina e do Fibrinogênio/metabolismo , Deficiência de Glucosefosfato Desidrogenase/sangue , Deficiência de Glucosefosfato Desidrogenase/complicações , Deficiência de Glucosefosfato Desidrogenase/virologia , Humanos , Hidroxicloroquina/uso terapêutico , Inflamação/prevenção & controle , Masculino , Pandemias , Pneumonia Viral/sangue , Pneumonia Viral/complicações , Pneumonia Viral/virologia , Resultado do Tratamento
6.
Am J Case Rep ; 21: e925788, 2020 Jul 22.
Artigo em Inglês | MEDLINE | ID: mdl-32697769

RESUMO

BACKGROUND Beta-hemoglobinopathies and glucose-6-phosphate dehydrogenase (G6PD) deficiency are genetic disorders that cause hemolytic anemia when exposed to oxidative stress. Their co-existence is, however, not proven to enhance the severity of anemia. CASE REPORT We report the case of a young man with no known co-morbidities, who came with fever and cough and was diagnosed with COVID-19 pneumonia. He was found to have hemoglobin D thalassemia and G6PD deficiency during further evaluation. Hydroxychloroquine therapy started initially, was discontinued after 3 doses once the G6PD deficiency was diagnosed. His hospital course showed a mild drop in hemoglobin with evidence of hemolysis on peripheral smear. However, the hemoglobin improved without any need for transfusion. CONCLUSIONS Hydroxychloroquine therapy can induce hemolytic crises in patients with underlying G6PD deficiency or hemoglobinopathies and should be avoided or closely monitored. Immediate intervention to stop hydroxychloroquine after 3 doses saved our patient from a major hemolytic crisis. The significance of this case report is that it is the first report that outlines the clinic course of COVID-19 pneumonia in a patient with underlying hemoglobin D disease and G6PD deficiency.


Assuntos
Betacoronavirus , Infecções por Coronavirus/complicações , Deficiência de Glucosefosfato Desidrogenase/complicações , Hemoglobinas/metabolismo , Pneumonia Viral/complicações , Talassemia/complicações , Doenças Assintomáticas , Infecções por Coronavirus/virologia , Deficiência de Glucosefosfato Desidrogenase/sangue , Humanos , Masculino , Pandemias , Pneumonia Viral/virologia , Talassemia/sangue , Adulto Jovem
7.
Ann Biol Clin (Paris) ; 78(4): 411-416, 2020 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-32576539

RESUMO

BACKGROUND AND OBJECTIVES: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common human enzymopathy worldwide associated with hemolysis as well as neonatal jaundice, kernicterus, and even death. The goal of this study is to determinate the prevalence of G6PD deficiency in icteric neonates and to investigate its biochemical, hematological and molecular characteristics. PATIENTS AND METHODS: This cross sectional study was carried out on 154 icteric newborns admitted to the Bechir Hamza Children's Hospital in Tunisia. Laboratory evaluations included complete blood count, total serum bilirubin level (TSB), and erythrocyte G6PD activity. The G6PD activity was determined using a quantitative assay, which allowed us to divide the total population into two groups: normal and deficient population. The common G6PD Tunisian mutations (GdA - and GdMed) were determined using the amplification refractory mutation system (ARMS-PCR) method. RESULTS: The prevalence of G6PD deficiency among total population (154 icteric newborns) was 18.83%. Male neonates showed a higher incidence of G6PD deficiency of 11.03% compared to females (7.79%). There was no statistical difference between the two groups (normal and deficient), in relation to the sex, peak TSB level, age at peak TSB, hemoglobin level, and hematocrit. However, there was a significant difference in gestational age. In the deficient group, 48.28% neonates presented the peak TSB level between 3 to 7 days and 55% of the cases show a peak TSB level greater than 250 µmol/L. The G6PD G202A variant was found in 41.37% of cases. CONCLUSION: This study shows a higher prevalence of G6PD deficiency in icteric newborns of Tunisia (18.83%). This emphasizes the necessity of neonatal screening for G6PD deficiency to prevent the exposure of these newborns to known hemolytic agents and, subsequently, to prevent kernicterus or other serious complications.


Assuntos
Deficiência de Glucosefosfato Desidrogenase/epidemiologia , Icterícia Neonatal/epidemiologia , Análise Química do Sangue , Estudos Transversais , Análise Mutacional de DNA , Feminino , Idade Gestacional , Glucosefosfato Desidrogenase/análise , Glucosefosfato Desidrogenase/sangue , Glucosefosfato Desidrogenase/genética , Deficiência de Glucosefosfato Desidrogenase/sangue , Deficiência de Glucosefosfato Desidrogenase/complicações , Deficiência de Glucosefosfato Desidrogenase/genética , Testes Hematológicos , Humanos , Recém-Nascido , Icterícia Neonatal/sangue , Icterícia Neonatal/complicações , Icterícia Neonatal/genética , Masculino , Prevalência , Tunísia/epidemiologia
8.
Infect Dis (Lond) ; 52(9): 659-661, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32496938

RESUMO

While the COVID-19 epidemic occurred since December 2019, as of end April 2020, no treatment has been validated or invalidated by accurate clinical trials. Use of hydroxychloroquine has been popularised on mass media and put forward as a valid treatment option without strong evidence of efficacy. Hydroxychloroquine (HCQ) has its own side effects, some of which are very serious like acute haemolysis in glucose-6-phosphate dehydrogenase (G6PD) deficient patients. Side effects may be worse than the disease itself. Belgian national treatment guidelines recommend the use of HCQ in mild to severe COVID-19 disease. As opinions, politics, media and beliefs are governing COVID-19 therapy, performance of randomised controlled blinded clinical trials became difficult. Results of sound clinical trials are eagerly awaited. We report a case of acute haemolysis leading to admission in intensive care unit and renal failure in a patient with uncovered G6PD deficiency.


Assuntos
Infecções por Coronavirus/complicações , Infecções por Coronavirus/tratamento farmacológico , Inibidores Enzimáticos/efeitos adversos , Deficiência de Glucosefosfato Desidrogenase/complicações , Hemólise , Hidroxicloroquina/efeitos adversos , Pneumonia Viral/complicações , Pneumonia Viral/tratamento farmacológico , Idoso , Azitromicina/uso terapêutico , Betacoronavirus/genética , Betacoronavirus/isolamento & purificação , Transfusão de Sangue , Terapia de Substituição Renal Contínua , Quimioterapia Combinada , Inibidores Enzimáticos/uso terapêutico , Haptoglobinas/análise , Humanos , Hidroxicloroquina/uso terapêutico , Hipóxia/induzido quimicamente , Hipóxia/complicações , Masculino , Nasofaringe/virologia , Pandemias , Vírus da SARS/genética , Vírus da SARS/isolamento & purificação
9.
Am J Med Sci ; 360(1): 72-74, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32448501

RESUMO

Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency is an X-linked genetic disorder associated with intravascular hemolysis. Rhabdomyolysis with myoglobinuria in a patient with G6PD deficiency is a very rare manifestation, in fact, to the best of our knowledge, only a few case reports have been published in the literature to date. Herein, we report an unusual presentation of a 33-year-old male with G6PD deficiency with multiple episodes of severe rhabdomyolysis with no significant concurrent hemolysis. This case supports the hypothesis that rhabdomyolysis may be a rare manifestation of G6PD deficiency, though the exact causation still remains unclear.


Assuntos
Deficiência de Glucosefosfato Desidrogenase/complicações , Rabdomiólise/etiologia , Rabdomiólise/patologia , Adulto , Hidratação , Deficiência de Glucosefosfato Desidrogenase/terapia , Humanos , Masculino
10.
Am J Trop Med Hyg ; 103(1): 394-403, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32372747

RESUMO

Tafenoquine has been licensed for the single-dose radical cure of Plasmodium vivax in adults; however, it is only recommended in patients with > 70% of normal glucose-6-phosphate dehydrogenase (G6PD) activity. Because this may hinder widespread use, we investigated sex-based treatment strategies in which all adult patients are tested with a qualitative G6PD rapid diagnostic test (RDT). Glucose-6-phosphate dehydrogenase normal males are prescribed tafenoquine in all three strategies, whereas G6PD normal females are prescribed either a low-dose 14-day primaquine regimen (PQ14, total dose 3.5 mg/kg) or a high-dose 7-day primaquine regimen (PQ7, total dose 7 mg/kg), or referred to a healthcare facility for quantitative G6PD testing before prescribing tafenoquine. Patients testing G6PD deficient are prescribed a weekly course of primaquine for 8 weeks. We compared the cost-effectiveness of these three strategies to usual care in four countries using a decision tree model. Usual care in Ethiopia does not include radical cure, whereas Afghanistan, Indonesia, and Vietnam prescribe PQ14 without G6PD screening. The cost per disability-adjusted life-year (DALY) averted was expressed through incremental cost-effectiveness ratios (ICERs). Compared with usual care, the ICERs for a sex-based treatment strategy with PQ7 for females from a healthcare provider perspective were $127 per DALY averted in Vietnam, $466 in Ethiopia, $1,089 in Afghanistan, and $4,443 in Indonesia. The PQ14 and referral options cost more while averting fewer DALYs than PQ7. This study provides an alternative cost-effective mode of rolling out tafenoquine in areas where initial testing with only a G6PD RDT is feasible.


Assuntos
Aminoquinolinas/efeitos adversos , Anemia Hemolítica/induzido quimicamente , Antimaláricos/efeitos adversos , Deficiência de Glucosefosfato Desidrogenase/diagnóstico , Malária Vivax/tratamento farmacológico , Primaquina/efeitos adversos , Adulto , Afeganistão , Aminoquinolinas/administração & dosagem , Anemia Hemolítica/etiologia , Antimaláricos/administração & dosagem , Cloroquina/administração & dosagem , Análise Custo-Benefício , Etiópia , Feminino , Deficiência de Glucosefosfato Desidrogenase/complicações , Hemizigoto , Heterozigoto , Homozigoto , Humanos , Indonésia , Masculino , Programas de Rastreamento , Adesão à Medicação , Plasmodium vivax , Primaquina/administração & dosagem , Anos de Vida Ajustados por Qualidade de Vida , Recidiva , Fatores Sexuais , Vietnã
11.
Eur J Haematol ; 105(3): 357-359, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32324284

RESUMO

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an inherited genetic disorder caused by red cell enzymatic defects and is associated with haemolytic crisis when patients are exposed to oxidative agents (fava beans, drugs, infections). Hydroxychloroquine is suspected to trigger haemolytic crisis in G6PD-deficient patients, and off-label administration of this drug to patients infected with the novel coronavirus (SARS-CoV-2) could cause concern. We report here the first case of severe haemolytic crisis in a patient with G6PD deficiency, initiated by severe COVID-19 infection and hydroxychloroquine use. With worldwide spread of COVID-19, especially in regions with a high prevalence of G6PD deficiency, our case should alert physicians to this possible correlation.


Assuntos
Betacoronavirus , Infecções por Coronavirus/tratamento farmacológico , Deficiência de Glucosefosfato Desidrogenase/complicações , Hemólise/efeitos dos fármacos , Hidroxicloroquina/efeitos adversos , Pneumonia Viral/tratamento farmacológico , Idoso , Comorbidade , Infecções por Coronavirus/complicações , Infecções por Coronavirus/epidemiologia , Deficiência de Glucosefosfato Desidrogenase/epidemiologia , Humanos , Hidroxicloroquina/uso terapêutico , Masculino , Pandemias , Pneumonia Viral/epidemiologia
14.
Turk J Med Sci ; 50(1): 103-109, 2020 02 13.
Artigo em Inglês | MEDLINE | ID: mdl-31731336

RESUMO

Background/aim: Severe neonatal hyperbilirubinemia is an important cause of morbidity and mortality in developing countries. The aim was to assess etiologic reasons for development of severe hyperbilirubinemia and define risk factors for exchange transfusion and acute bilirubin encephalopathy (ABE) in Sanliurfa located in the southeast region of Turkey. Materials and methods: An observational cohort study included 115 infants with ≥35 weeks of gestation admitted with diagnosis of severe hyperbilirubinemia in a period of 18 months. Potential risk factors associated with exchange transfusion and development of ABE were analyzed. Results: Among 115 infants, 67 (58.3%) received exchange transfusion and 45 (39.1%) developed ABE. Rh isoimmunization (OR: 24.6, 95% CI = 2.2­271, P = 0.009), glucose-6-phosphate dehydrogenase deficiency (G6PD) (OR: 21.1, 95% CI = 1.8­238.4, P = 0.01), early discharge (OR: 14.4, 95% CI = 4.2­48.9, P ≤ 0.001), and male sex (OR: 4.3, 95% CI = 1.3­14.1, P = 0.02) were independently associated with an increased risk for exchange transfusion. Being a refugee (OR: 6.8, 95% CI = 1.8­25.8, P = 0.005) and G6PD deficiency (OR: 9.9, 95% CI = 1.3­71.9, P = 0.02) were associated with development of ABE. Conclusion: Early discharge, Rh isoimmunization, and G6PD deficiency are significant risk factors for severe hyperbilirubinemia and exchange transfusion. Prevention of early hospital discharges, family education to increase awareness for hazardous effects of hyperbilirubinemia, and early follow-up visits after discharge would reduce the disease burden.


Assuntos
Hiperbilirrubinemia Neonatal/etiologia , Doença Aguda , Adulto , Transfusão Total , Feminino , Deficiência de Glucosefosfato Desidrogenase/complicações , Humanos , Hiperbilirrubinemia Neonatal/mortalidade , Recém-Nascido , Kernicterus/etiologia , Masculino , Gravidez , Isoimunização Rh/complicações , Fatores de Risco
15.
J Oncol Pharm Pract ; 26(2): 492-495, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31260379

RESUMO

INTRODUCTION: Glucose 6-phosphate dehydrogenase (G6PD) is a basic antioxidant pathway for erythrocytes, being its deficiency the most common gene mutation worldwide. As breast cancer is one of the most frequent tumors, many of these patients may present with G6PD deficiency prior treatment without notice. CASE REPORT: We present the case of a woman deficient for G6PD with the diagnosis of Stage IIIB (cT4d cN1 cM0) HER2-enriched early breast cancer. MANAGEMENT AND OUTCOME: The patient underwent neoadjuvance with trastuzumab and anthracycline-free chemotherapy, based on docetaxel (75 mg/m2, 120 mg) and carboplatin (AUC 5, 560 mg). She did not present hemolytic crisis and no blood transfusions were needed. She achieved a good pathologic response and completed one-year adjuvant trastuzumab without incidences. DISCUSSION: Although the role of HER2 and trastuzumab in oxidative stress is not yet completely understood, we suggest that trastuzumab may be a suitable agent for treatment in patients with HER2-enriched breast cancer in a non-oxidative chemotherapy scheme, with acceptable responses and no triggering hemolytic crisis.


Assuntos
Neoplasias da Mama/tratamento farmacológico , Deficiência de Glucosefosfato Desidrogenase/tratamento farmacológico , Terapia Neoadjuvante/métodos , Pós-Menopausa/efeitos dos fármacos , Receptor ErbB-2 , Trastuzumab/administração & dosagem , Idoso , Antineoplásicos Imunológicos/administração & dosagem , Neoplasias da Mama/complicações , Neoplasias da Mama/diagnóstico , Neoplasias da Mama/genética , Feminino , Deficiência de Glucosefosfato Desidrogenase/complicações , Deficiência de Glucosefosfato Desidrogenase/diagnóstico , Humanos , Pós-Menopausa/genética , Receptor ErbB-2/genética , Resultado do Tratamento
16.
PLoS One ; 14(12): e0226927, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31860695

RESUMO

Neonatal jaundice is a common and severe disease in premature infants with Glucose-6-Phosphate Dehydrogenase (G-6-PD) deficiency. The World Health Organization (WHO) has recommended screening for G-6-PD deficiency in newborns for early recognition as well as to prevent unwanted outcomes in a timely manner. The present study aimed to assess a point-of-care, careSTARTTM G6PD biosensor as a quantitative method for the diagnosis of G-6-PD deficiency. Factors influencing the evaluation of G-6-PD enzyme activity were examined in 40 adults, including ethylenediaminetetraacetic acid (EDTA) anticoagulant, hematocrit concentration, storage temperature and time. Analytic performance of the careSTARTTM G6PD biosensor was evaluated in 216 newborns and compared with fluorescent spot test (FST) and standard quantitative G-6-PD enzyme activity (SGT) assay. The results of factors affecting the G-6-PD enzyme activity showed that the activity determined from finger-prick was not statistically different from venous blood (p = 0.152). The G-6-PD value was highly dependent on the hematocrit and rose with increasing hematocrit concentration. Its activity was stable at 4°C for 3 days. Reliability analysis between the careSTARTTM G6PD biosensor and SGT assay showed a strong correlation with a Pearson's correlation coefficient of 0.82 and perfect agreement by intraclass correlation coefficient (ICC) of 0.90. Analysis of the area under the Receiver Operating Curve (AUC) illustrated that the careSTARTTM G6PD biosensor had 100% sensitivity, 96% specificity, 73% positive predictive value (PPV), 100% negative predictive value (NPV) and 97% accuracy at 30% of residual activity. While the diagnostic ability for identifying G-6-PD deficiency had 78% sensitivity, 89% specificity, 56% positive predictive value (PPV), 96% negative predictive value (NPV) and 88% accuracy when stratified by gender. The careSTARTTM G6PD biosensor is an attractive option as a point-of-care quantitative method for G-6-PD activity detection. Quantification of G-6-PD enzyme activity in newborns is the most effective approach for the management of G-6-PD deficiency to prevent severe jaundice and acute hemolysis.


Assuntos
Técnicas Biossensoriais/métodos , Ensaios Enzimáticos Clínicos/métodos , Deficiência de Glucosefosfato Desidrogenase/diagnóstico , Glucosefosfato Desidrogenase/análise , Testes Hematológicos/métodos , Triagem Neonatal/métodos , Sistemas Automatizados de Assistência Junto ao Leito , Adolescente , Doadores de Sangue , Confiabilidade dos Dados , Feminino , Deficiência de Glucosefosfato Desidrogenase/complicações , Humanos , Recém-Nascido , Icterícia Neonatal/etiologia , Masculino , Sensibilidade e Especificidade , Adulto Jovem
18.
Transfus Apher Sci ; 58(6): 102677, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31757665

RESUMO

A mild degree of hemolysis is commonly encountered complication in acute viral hepatitis patients which generally resolves as the disease recovers. Rarely, some patients might present with severe hemolysis associated with glucose-6-phosphate dehydrogenase (G6PD) deficiency. It has been hypothesized that the hemolysis is initially provoked by the viral infection itself; however, it may be aggravated due to the administration of certain drugs in patients with G6PD deficiency. We report a case highlighting the role of high-volume plasma exchange in a G6PD deficient patient presenting with hepatitis A related acute liver failure (ALF) and concomitant acute renal failure (ARF).


Assuntos
Lesão Renal Aguda/etiologia , Deficiência de Glucosefosfato Desidrogenase/complicações , Deficiência de Glucosefosfato Desidrogenase/terapia , Hepatite A/complicações , Falência Hepática Aguda/etiologia , Troca Plasmática , Lesão Renal Aguda/virologia , Adolescente , Cor , Humanos , Lactente , Falência Hepática Aguda/virologia , Masculino
19.
J Pak Med Assoc ; 69(10): 1553-1556, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31622316

RESUMO

Dengue is a mosquito-borne viral disease that has rapidly spread in the recent years, particularly in South Asia. While haematologic complications, such as cytopenia and bleeding, may occur in severe dengue infection, reports of haemolytic anaemia in dengue fever are scant. We report a patient who developed haemolytic anaemia following dengue fever. A 45 years old gentleman presented with five-day history of fever and was recently diagnosed with dengue fever. He developed jaundice and started vomiting on the third day of his clinical course. His laboratory investigations revealed deranged liver profile, Coombs negative haemolytic anaemia and G6PD deficiency. He was treated conservatively with fluids and blood transfusions. His liver functions and haemolytic anaemia gradually resolved. This case highlights the importance of recognising dengue fever-induced haemolytic anaemia in a G6PD deficient patient by physicians and pathologists, enabling better diagnosis and improved management of this life-threatening condition.


Assuntos
Anemia Hemolítica/etiologia , Dengue/complicações , Deficiência de Glucosefosfato Desidrogenase/complicações , Anemia Hemolítica/sangue , Anemia Hemolítica/patologia , Anemia Hemolítica/terapia , Transfusão de Sangue , Dengue/sangue , Hidratação , Deficiência de Glucosefosfato Desidrogenase/sangue , Deficiência de Glucosefosfato Desidrogenase/patologia , Humanos , Masculino , Pessoa de Meia-Idade
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...