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1.
Hematol., Transfus. Cell Ther. (Impr.) ; 45(supl.2): S11-S17, July 2023. tab, graf
Artigo em Inglês | LILACS | ID: biblio-1514201

RESUMO

ABSTRACT Introduction: During pregnancy, the iron requirement increases to meet the optimal growth of the fetus and prevent iron deficiency anemia-related complications in the mother. However, in sickle cell disease (SCD) primarily due to repeated blood transfusions and hemolysis-induced recycling of iron, its supplementation during pregnancy remains questionable and may be harmful. Methods: Twenty-five pregnant women with homozygous SCD and 25 pregnant women with normal hemoglobin variants were included as cases and control, respectively. Pregnancy and sickle cell anemia (SCA) were diagnosed using standard protocols. The serum iron, serum ferritin, total iron-binding capacity (TIBC), percentage transferrin saturation and C-reactive protein were estimated, as per the manufacturer's protocol. The complete blood count was performed. The unpaired 't-test' was performed using the SPSS v23.0 and the principal component analysis (PCA) was performed using the online software MetaboAnalyst for statistical analysis. Main Results: The studied cases had significantly lower mean hemoglobin and higher mean corpuscular volume (MCV), compared to controls. The mean serum-iron, serum-ferritin and percentage transferrin-saturation in the cases were significantly higher than that of the controls, while the TIBC was lower in the cases (p < 0.0001). The mean level of serum iron, ferritin, percentage transferrin saturation and TIBC were 309.44 ± 122.40mcg/dl, 860.36 ± 624.64ng/ml, 42.6 ± 17.30% and 241.32 ± 96.30 mcg/dl, respectively, in the cases and 95.36 ± 41.90mcg/dl, 122.28 ± 49.70ng/ml, 15.83 ± 3.10% and 492.6 ± 149.40mcg/dl in the controls, respectively. Higher MCV, mean corpuscular hemoglobin (MCH) and mean corpuscular hemoglobin concentration (MCHC) with lower hemoglobin (Hb) were noted in the cases. The PCA revealed that the cases were more heterogeneous in terms of the variability of the iron status and hematological indices than the controls. Conclusion: The current study shows iron sufficiency in most cases of pregnancy with SCA and suggests that evaluation of iron status must be made before initiating iron prophylaxis in pregnant women with SCA, especially in regions having a high prevalence of sickle cell hemoglobinopathy.


Assuntos
Humanos , Gravidez , Gravidez , Anemia Falciforme , Sobrecarga de Ferro , Fármacos Hematológicos
2.
Hemoglobin ; 47(1): 11-18, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37122241

RESUMO

There is a paucity of literature on the association of α+-thalassemia, sickle-cell hemoglobin disorders, and malaria in India. This study aimed to understand the effect of α+-thalassemia on the severity of Plasmodium falciparum malaria in adults with respect to sickle-cell genotypes. The study subjects were categorized into 'severe-malaria' and 'uncomplicated-malaria' and age-gender matched 'control' groups. Sickle-cell and α+-thalassemia were investigated in all the recruited subjects. The effect of α+-thalassemia on the severity of malaria was analyzed in HbAA and sickle-cell genotypes (HbAS and HbSS) separately. The prevalence of α+-thalassemia in various groups ranged from 41.5% to 81.8%. The prevalence of α+-thalassemia was lower (OR = 1.64; p = 0.0013) in severe malaria (41.5%) as compared to healthy controls (53.8%) with HbAA genotype. In contrast, in HbAS genotype, the prevalence of α+-thalassemia was higher (OR = 4.11; p = 0.0002) in severe malaria (81.8%) compared to controls (52.2%). In severe malaria with HbAA genotype, there was a significantly higher hemoglobin level and low MCV and MCH level in patients with α+-thalassemia compared to the normal α-globin genotype. Further, the incidence of cerebral malaria, hepatopathy, and mortality was lower in patients (HbAA) with α+-thalassemia as compared to normal α-globin genotype (HbAA). In severe malaria with either HbAS or HbSS genotype, only a few parameters showed statistical differences with respect to α+-thalassemia. Low prevalence of α+-thalassemia in severe malaria with HbAA genotype compared to healthy controls with HbAA genotype indicates the protective effect of α+-thalassemia against severe malaria. However, the high prevalence of α+-thalassemia in patients with HbAS genotype depicts its interference in the protective effect of sickle-cell against severe malaria.


Assuntos
Anemia Falciforme , Malária Falciparum , Malária , Traço Falciforme , Talassemia alfa , Humanos , Adulto , Malária Falciparum/epidemiologia , Malária/epidemiologia , Malária/genética , Hemoglobinas/genética , Anemia Falciforme/epidemiologia , Anemia Falciforme/genética , Hemoglobina Falciforme/genética , Genótipo , Talassemia alfa/epidemiologia , Talassemia alfa/genética , Hospitais , Plasmodium falciparum/genética , Traço Falciforme/genética
3.
Hematol Transfus Cell Ther ; 45 Suppl 2: S11-S17, 2023 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-35216958

RESUMO

INTRODUCTION: During pregnancy, the iron requirement increases to meet the optimal growth of the fetus and prevent iron deficiency anemia-related complications in the mother. However, in sickle cell disease (SCD) primarily due to repeated blood transfusions and hemolysis-induced recycling of iron, its supplementation during pregnancy remains questionable and may be harmful. METHODS: Twenty-five pregnant women with homozygous SCD and 25 pregnant women with normal hemoglobin variants were included as cases and control, respectively. Pregnancy and sickle cell anemia (SCA) were diagnosed using standard protocols. The serum iron, serum ferritin, total iron-binding capacity (TIBC), percentage transferrin saturation and C-reactive protein were estimated, as per the manufacturer's protocol. The complete blood count was performed. The unpaired 't-test' was performed using the SPSS v23.0 and the principal component analysis (PCA) was performed using the online software MetaboAnalyst for statistical analysis. MAIN RESULTS: The studied cases had significantly lower mean hemoglobin and higher mean corpuscular volume (MCV), compared to controls. The mean serum-iron, serum-ferritin and percentage transferrin-saturation in the cases were significantly higher than that of the controls, while the TIBC was lower in the cases (p < 0.0001). The mean level of serum iron, ferritin, percentage transferrin saturation and TIBC were 309.44 ± 122.40mcg/dl, 860.36 ± 624.64ng/ml, 42.6 ± 17.30% and 241.32 ± 96.30 mcg/dl, respectively, in the cases and 95.36 ± 41.90mcg/dl, 122.28 ± 49.70ng/ml, 15.83 ± 3.10% and 492.6 ± 149.40mcg/dl in the controls, respectively. Higher MCV, mean corpuscular hemoglobin (MCH) and mean corpuscular hemoglobin concentration (MCHC) with lower hemoglobin (Hb) were noted in the cases. The PCA revealed that the cases were more heterogeneous in terms of the variability of the iron status and hematological indices than the controls. CONCLUSION: The current study shows iron sufficiency in most cases of pregnancy with SCA and suggests that evaluation of iron status must be made before initiating iron prophylaxis in pregnant women with SCA, especially in regions having a high prevalence of sickle cell hemoglobinopathy.

4.
Indian J Med Res ; 156(1): 122-129, 2022 07.
Artigo em Inglês | MEDLINE | ID: mdl-36510904

RESUMO

Background & objectives: Hydroxyurea (HU) has been useful in preventing sickle cell vaso-occlusive crises (VOC). A few studies also suggest utility of HU, during acute VOC. Sickle cell anaemia (SCA) is of high prevalence in western districts of Odisha State, India, and VOC is a common presentation, despite being mostly of Arab-Indian haplotype. This study was undertaken to evaluate the impact of HU on hospital stay and analgesic utilization in acute painful VOC of SCA. Methods: Homozygous sickle cell disease (HbSS) patients were categorized as cases who were receiving low-dose HU (10 mg/kg/day) and patients who were not on HU were considered as control. Days of hospital stay, analgesic utilization and visual analogue scale (VAS) score in patients were compared with that of control. Analgesics used to control pain were tramadol hydrochloride, ketorolac and diclofenac. Results: A total of 359 homozygous sickle cell disease (SCD) patients with VOC were studied (187 patients and 172 controls). The patient group had lesser mean days of hospital stay (1.4 days less than controls, P<0.001) and required lesser days of analgesic utilization than controls (1.18 days less than controls, P<0.001). Significant differences were observed between patients and controls concerning VAS score and amount of tramadol hydrochloride, ketorolac and diclofenac utilization (P<0.05). Interpretation & conclusions: In this study, HU was found to have beneficial effects in acute VOC of homozygous SCD, which includes shortening the duration of hospital stay and reducing the net amount of analgesic utilization during hospitalization.


Assuntos
Anemia Falciforme , Tramadol , Humanos , Tramadol/uso terapêutico , Tempo de Internação , Hidroxiureia/uso terapêutico , Anemia Falciforme/complicações , Anemia Falciforme/tratamento farmacológico , Anemia Falciforme/genética , Analgésicos/uso terapêutico
5.
Ann Hematol ; 99(12): 2737-2745, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-32918113

RESUMO

The vaso-occlusive crisis (VOCs) in sickle cell disease (SCD) is often associated with stress. Epinephrine released during stress acts via beta 2-adrenergic receptors (ß2-AR or ADRB2) to stimulate the synthesis of cyclic adenosine monophosphate (cAMP) in the red blood cells (RBCs). Higher cAMP levels promote adhesion of sickled RBCs to vascular endothelium, a major contributor for VOCs. Several single-nucleotide polymorphisms (SNPs) of the ß2-AR gene have been reported; two of them at codon 16 (rs1042713) and codon 27 (rs1042714) have been extensively studied for their clinical relevance. Therefore, we assessed the influence of polymorphism at these two sites of the ß2-AR gene on the RBC cAMP concentrations with and without epinephrine stimulation in SCD subjects. We determined the frequency distribution of different genotypes of codon 16 and codon 27 of the ß2-AR gene using the Sanger sequencing method in the SCD subjects. We measured the RBC-cAMP levels at baseline and after stimulation with epinephrine, to ascertain the influence of different genotypes in determining cAMP levels. There was no difference in the socio-demographic and hematological indicators in different genotypes of both codon 16 and 27. In the sham-treated erythrocytes, the cAMP levels were significantly different with three genotypes of codon 16 (F = 3.39, P = 0.036; one way ANOVA) but not with different genotypes of codon 27. A significant increase in cAMP levels was noticed with epinephrine treatment in all genotypes of codons 16 and 27 (P = 0.001; Wilcoxon signed-rank test). However, the extent of increase in the epinephrine-treated cAMP values from the sham-treated (baseline) cAMP values was significantly different between the three genotypes of codon 16 (H = 8.74; P = 0.012; Kruskal-Wallis test) but not in codon 27 genotypes. Polymorphism in codon 16 (rs1042713) of the ß2-AR gene influences cAMP concentrations in the RBC both before and after epinephrine treatment. Higher cAMP levels may lead to increased adhesion of sickle cell RBCs to vascular endothelium and may increase the frequency of VOCs.


Assuntos
Anemia Falciforme/genética , AMP Cíclico/genética , Eritrócitos/fisiologia , Polimorfismo de Nucleotídeo Único/genética , Receptores Adrenérgicos beta 2/genética , Adolescente , Anemia Falciforme/sangue , Anemia Falciforme/epidemiologia , Criança , AMP Cíclico/sangue , Feminino , Humanos , Índia/epidemiologia , Masculino , Receptores Adrenérgicos beta 2/sangue , Adulto Jovem
6.
Ann Hematol ; 98(10): 2257-2265, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31440871

RESUMO

Vascular complications of sickle cell anemia (SCA) are influenced by many factors. Elevated plasma homocysteine (Hcy) is supposed to be an independent risk factor and is either genetic or nutritional origin. The present study evaluated the plasma Hcy level, MTHFR C677T gene polymorphism, effect of folic acid (FA) supplementation' and hemato-biochemical parameters in SCA and their effect on the vaso-occlusive crisis (VOC) in SCA patients of an Asian-Indian haplotype population. One hundred twenty cases of SCA (HbSS) and 50 controls with normal hemoglobin(HbAA) were studied. It was found that the plasma Hcy level is significantly higher (p < 0.0001) in patients with SCA (22.41 ± 7.8 µmol/L) compared to controls (13.2 ± 4.4 µmol/L). Moreover, patients without FA supplementation had a significantly (p < 0.001) higher Hcy level (27 ± 7 µmol/L) compared to those with supplementation (17.75 ± 5.7 µmol/L). Turkey-Kramer multiple comparison tests show that there is a significant difference (p < 0.05) in HbF percent, hemoglobin (Hb), platelet count, serum bilirubin (direct:Bil-D and total:Bil-T), aspartate transaminase (AST), lactate dehydrogenase (LDH), and plasma Hcy levels between mild and severe VOC. Between moderate VOC and severe VOC, there was a significant difference (p < 0.05) in HbF%, Bil-D, AST, Hcy. Pearson correlation revealed that plasma Hcy had a significantly (p < 0.05) positive correlation with AST, serum bilirubin (indirect and total), LDH, jaundice, stroke, VOC per year, and hospitalization per year whereas it was inversely correlated with HbF percentage, Hb level, and FA treatment. In the study population, increased plasma Hcy level, hemolysis, and platelet activation were found to influence VOC in SCA.


Assuntos
Anemia Falciforme , Homocisteína/sangue , Metilenotetra-Hidrofolato Redutase (NADPH2)/genética , Polimorfismo Genético , Doenças Vasculares , Adolescente , Adulto , Anemia Falciforme/sangue , Anemia Falciforme/complicações , Anemia Falciforme/genética , Anemia Falciforme/fisiopatologia , Aspartato Aminotransferases/sangue , Bilirrubina/sangue , Plaquetas/metabolismo , Feminino , Hemoglobina Fetal/genética , Hemoglobina Fetal/metabolismo , Hemólise , Homocisteína/genética , Humanos , L-Lactato Desidrogenase/sangue , Masculino , Metilenotetra-Hidrofolato Redutase (NADPH2)/sangue , Pessoa de Meia-Idade , Ativação Plaquetária , Contagem de Plaquetas , Doenças Vasculares/sangue , Doenças Vasculares/etiologia , Doenças Vasculares/genética , Doenças Vasculares/fisiopatologia
7.
Blood Cells Mol Dis ; 75: 30-34, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30597429

RESUMO

BACKGROUND: Sickle cell disease (SCD) is a Mendelian single gene disorder with highly variable phenotypic expression. In the present study, we analyzed the influence of HbF, alpha thalassemia and other hematological indices to determine their association with acute pain episodes. METHOD: This case control study consisted of SCD subjects with HbS phenotype experiencing three or more acute pain episodes in last twelve months (cases) and without any episode of acute pain during last twelve months (controls). Hematological parameters, HbF, and presence of alpha thalassemia were assessed in all subjects. RESULTS: A statistically significant difference between HbF levels (P < 0.025, χ2 test) and alpha thalassemia (P < 0.008, χ2 test) was observed between controls and cases group. Univariate analysis indicated that increased HbF levels > 25% (OR: 0.37, 95% CI: 0.18-0.77, P < 0.008) and presence of alpha thalassemia (OR: 0.53, 95% CI: 0.33-0.85, P < 0.009) provided protection, while multivariate analysis revealed significant protection was attributable only by higher HbF levels (OR: 0.39, 95% CI: 0.17-0.88, P < 0.025). Significantly higher HbF levels were observed only in the 11-20 age group of cases in comparison to controls (Student's t-test, P < 0.001). CONCLUSION: Higher concentrations of HbF are associated with protection against frequent episodes of acute pain crisis in SCD patients.


Assuntos
Dor Aguda/etiologia , Anemia Falciforme/sangue , Hemoglobina Fetal/análise , Adolescente , Anemia Falciforme/complicações , Estudos de Casos e Controles , Criança , Feminino , Humanos , Masculino , Adulto Jovem , Talassemia alfa
8.
Ars pharm ; 59(3): 173-183, jul.-sept. 2018. graf, tab
Artigo em Inglês | IBECS | ID: ibc-177733

RESUMO

Introduction: Main objective of any pharmaceutical scientist is to develop drug delivery system that is safe, effective, stable, having good patient compliance and fulfill the requirements of customers. Lead to a great interest of research to develop the drug delivery system that will enable to supply drug «on-demand» basis. These "stimuli-responsive and intelligent" systems have been designed to deliver the drug on various times or at various sites in the body, according to a stimulus that is either endogenous or externally applied. Objectives: This paper aim to review various researches in the field of self regulatory drug delivery systems in tabular form so one can utilize these finding for further development of in intelligent drug delivery systems. Method: Various physicochemical principles and chemical schemes have been applied by researchers to get release pattern of drug as per requirement of body. Such devices can be used for intelligent drug delivery needed for the treatment of many diseases like diabetes. Results & Discussion: This type of intelligent system firstly sense the signals caused by disease, judge the magnitude of signals, and then release the drug in direct response. Conclusion: In this article we have discuss various innovations in the field of self regulatory drug delivery Systems and suggest that here is a lot of research scope in this field


Introducción: El objetivo principal de cualquier científico farmacéutico es desarrollar un sistema de administración de fármacos que sea seguro, efectivo, estable, que cumpla con los requisitos del paciente y cumpla con los requisitos de los clientes. Llevar a un gran interés de investigación para desarrollar el sistema de entrega de medicamentos que permitirá suministrar medicamentos "a demanda". Estos sistemas "sensibles a estímulos e inteligentes" han sido diseñados para administrar el farmaco en varios momentos o en varios sitios en el cuerpo, de acuerdo con un estímulo endógeno o aplicado externamente. Objetivos: Este artículo tiene como objetivo revisar diversas investigaciones en el campo de los sistemas autorreguladores de administración de fármacos en forma tabular para que uno pueda utilizar estos hallazgos para un mayor desarrollo de sistemas inteligentes de administración de fármacos. Método: Los investigadores han aplicado varios principios fisicoquímicos y esquemas químicos para obtener el patrón de liberación del fármaco según las necesidades del cuerpo. Dichos dispositivos se pueden usar para la administración inteligente de medicamentos necesarios para el tratamiento de muchas enfermedades, como la diabetes. Resultados y discusión: Este tipo de sistema inteligente primero detecta las señales causadas por la enfermedad, juzga la magnitud de las señales y luego libera la droga en respuesta directa. Conclusión: En este artículo, hemos discutido varias innovaciones en el campo de los sistemas autorreguladores de administración de fármacos y sugerimos que aquí hay mucho campo de investigación en este campo


Assuntos
Humanos , Controle de Medicamentos e Entorpecentes/organização & administração , Tratamento Farmacológico/métodos , Preparações Farmacêuticas/normas , Controle de Medicamentos e Entorpecentes/métodos , Controle de Medicamentos e Entorpecentes/tendências
11.
J Vector Borne Dis ; 54(1): 74-79, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28352049

RESUMO

BACKGROUND & OBJECTIVES: Many host genetic factors are associated with the disease severity and fatal outcome of falciparum malaria. CD40L gene has been found to be one of the most important factors associated with malaria in African countries. This study was aimed to investigate the possible association of CD40L gene polymorphism in severe falciparum malaria in Indian adults. METHODS: One hundred fifteen adult cases with severe falciparum malaria were included in the study. Two single- nucleotide polymorphisms (SNPs) of CD40L gene, CD40L-726(C/T) and CD40L+220(C/T) were investigated, and the possible association with different clinical sub-phenotypes of severe falciparum malaria were analyzed. RESULTS: Statistically no significant difference was observed in the incidence of CD40L-726C between the patients and control group. The incidence of CD40L+220C allele was found to be significantly higher (OR, 2.25; p = 0.03) in male patients compared to controls but no significant difference was observed in females. Haplotype data showed the susceptibility of -726T/+220C haplotype to severe malaria whereas -726C/+220T was associated with protection against severe malaria. CD40L+220C allele was associated with severe malarial anaemia in males (χ2 = 6.60; p = 0.01). INTERPRETATION & CONCLUSION: CD40L gene polymorphism was found to be associated with severe falciparum malaria in Indian population especially in severe malarial anaemia. CD40L may be considered as a factor of immunity in understanding the pathophysiology of falciparum malaria.


Assuntos
Ligante de CD40/genética , Predisposição Genética para Doença , Malária Falciparum/genética , Malária Falciparum/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Casos e Controles , Feminino , Humanos , Índia , Malária Falciparum/complicações , Masculino , Pessoa de Meia-Idade , Polimorfismo de Nucleotídeo Único , Índice de Gravidade de Doença , Adulto Jovem
13.
Hemoglobin ; 40(4): 270-2, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27254408

RESUMO

We describe here a rare ß-globin gene variant, Hb Tianshui [ß39(C5)Glu→Arg; HBB: c.119A > G], detected during routine screening in Odisha, India. This is the second report of Hb Tianshui and the first to describe the cation exchange high performance liquid chromatography (HPLC) and DNA studies of two cases of this variant. Both cases had coinherited Hb S (HBB: c.20A > T) but none presented with typical symptoms of sickle cell disease. One of the cases was heterozygous for a common α-thalassemia (α-thal) allele (-α(3.7)) (rightward) (NG_000006.1: g.34164_37967del3804) and marginally raised Hb F percentage, while the other Hb S/Hb Tianshui case was completely benign and healthy. An atypical Asian Indian haplotype [+ - + - +] could be assigned to the Hb Tianshui variant. Hb Tianshui seems to mimic a few other Hb variants in cation exchange HPLC. However, we report two specific patterns in the chromatograms that are characteristic to Hb Tianshui. Combining an alkaline electrophoresis result with cation exchange HPLC at screening would be preferred to detect this rare variant, especially in regions with considerable frequency of Hb E [ß26(B8)Glu→Lys; HBB: c.79G > A] or Hb S.


Assuntos
Hemoglobina Falciforme/genética , Hemoglobinopatias/diagnóstico , Hemoglobinas Anormais/genética , Cromatografia Líquida de Alta Pressão , Cromatografia por Troca Iônica , Eletroforese , Heterozigoto , Humanos , Índia , Talassemia alfa
14.
Hemoglobin ; 40(6): 422-424, 2016 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-28361588

RESUMO

In this short report, we describe the clinical presentation of a rare hemoglobin (Hb) variant, Hb Limassol [ß8(A5)Lys→Asn; HBB: c.27G>C] with a faster electrophoretic mobility than Hb A and that elutes in the P3 window on cation exchange high performance liquid chromatography (HPLC). This sequence variation at codon 8 (AAG>AAC) of the HBB gene was found in the four heterozygous cases, all of whom were clinically asymptomatic.


Assuntos
Variação Genética , Hemoglobinas Anormais/genética , Globinas beta/genética , Substituição de Aminoácidos , Cromatografia Líquida de Alta Pressão , Códon , Eletroforese , Feminino , Heterozigoto , Humanos , Índia , Masculino , Linhagem
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