Clinical and hematological effects of hydroxyurea therapy in sickle cell patients: a single-center experience in Brazil / Efeitos clinicos e hematologicos do tratamento com hidroxiureia em pacientes falciformes: experiencia de um centro no Brasil

CONTEXT AND OBJECTIVES Sickle cell disease (SCD) is the most common genetic disorder among people of African descent, affecting approximately 3,500 newborns each year in Brazil. Hydroxyurea (HU) is the only effective drug to treating patients with SCD, thereby reducing morbidity and mortality. The objective was to analyze the effects of HU on SCD patients at our institution. DESIGN AND SETTING Retrospective study conducted at a sickle cell centre in Ribeirão Preto, São Paulo, Brazil. METHODS We analyzed clinical and laboratory data on 37 patients. The hematological parameters and clinical events that occurred during the year before and the first year of treatment with HU were analyzed. The mean dose of HU was 24.5 ± 5.5 mg/kg/day. RESULTS There were rises in three parameters: hemoglobin (8.3 g/dl to 9.0 g/dl, P = 0.0003), fetal hemoglobin (HbF) (2.6% to 19.8%, P < 0.0001) and mean cell volume MCV (89 to 105 fl, P = 0.001); and reductions in the numbers of leukocytes (10,050/µl to 5,700/µl, P < 0.0001), neutrophils (6,200/µl to 3,400/µl, P = 0.001), platelets (459,000/µl to 373,000/µl, P = 0.0002), painful crises (1.86 to 0.81, P = 0.0014), acute chest syndromes (0.35 to 0.08, P = 0.0045), infections (1.03 to 0.5, P = 0.047), hospitalizations (1.63 to 0.53, P = 0.0013) and transfusions (1.23 to 0.1, P = 0.0051). CONCLUSION The patients presented clinical and hematological improvements, with an increase in HbF and a reduction in the infection rate, which had not been addressed in most previous studies. .

CONTEXTO E OBJETIVO A doença falciforme (SCD) é o distúrbio genético mais comum entre afrodes-cendentes, afetando aproximadamente 3.500 recém-nascidos a cada ano no Brasil. A hidroxiureia (HU) é a única droga efetiva para o tratamento dos pacientes com SCD, reduzindo a morbidade e a mortalidade da doença. O objetivo do estudo foi analisar os efeitos da HU em pacientes com SCD em nossa instituição. TIPO DE ESTUDO E LOCAL Estudo retrospectivo realizado em um centro de anemia falciforme em Ribeirão Preto, São Paulo, Brasil. MÉTODOS Nós analisamos os dados clínicos e laboratoriais de 37 pacientes. Os parâmetros hematológicos e eventos clínicos que ocorreram no ano anterior e durante o primeiro ano de tratamento com HU foram analisados. A dose média de HU foi 24.5 ± 5.5 mg/kg/dia. RESULTADOS Houve aumento em três parâmetros estudados: hemoglobina (8,3 g/dl para 9,0 g/dl, P = 0,0003), hemoglobina fetal (HbF) (2,6% para 19,8%, P < 0,0001) e volume corpuscular médio (VCM) (89 para 105 fl, P = 0,001); e redução do número de leucócitos (10.050/µl para 5.700/µl, P < 0,0001), neutrófilos (6.200/µl para 3.400/µl, P = 0,001), plaquetas (459.000/µl para 373.000/µl, P = 0,0002), crises dolorosas (1,86 para 0,81, P = 0,0014), síndrome torácica aguda (0,35 para 0,08, P = 0,0045), infecções (1,03 para 0,5, P = 0,047), hospitalizações (1,63 para 0,53, P = 0,0013) e número de transfusões (1,23 para 0,1, P = 0,0051). CONCLUSÃO Os pacientes apresentaram melhora clínica e hematológica, com aumento da HbF e redução da taxa de infecção, dado este não explorado na maioria dos estudos clínicos já publicados. .

Changes in haemoglobin, red cell count, red cell indices and reticulocyte count in response to daily versus intermittent oral iron supplementation during pregnancy in local population

To assess and compare the relative effectiveness of different oral iron supplementation regimens on hematological parameters in pregnant anemic women. This study was carried out in the Department of Physiology, Basic Medical Sciences Institute with collaboration of Department of Obstetrics and Gynecology, Jinnah Postgraduate Medical Centre Karachi. Subjects were pregnant women [n=90] showing clinical anemia [Hb<11g /dl] in their 20-24 weeks of gestation. All selected women were randomly assigned to one of the three treatment groups at the time of registration [30 in daily, 30 in TW, 30 in WS] 75 out of 90 enrolled women successfully completed 12 weeks trial. Group-I received 60 mg oral iron daily, Group-2 received 60 mg TW and Group-3 received 120mg once weekly. Haemoglobin, red cell count, red cell indices and reticulocyte count were evaluated by routine methods initially at baseline before iron therapy then at four weeks interval for 12 weeks following iron therapy. There was no significant difference existed in the mean haemoglobin level, RBC count, Red cell indices and Reticulocyte count between three treatment groups at the end of 12 weeks therapy. The increase in reticulocyte count showed effective erythropoiesis following iron therapy. The results within the group were compared from baseline to twelve weeks and all the groups were compared with each other. Intermittent iron supplementation is an equally effective mode of treatment of anemia as it has similar effect on hematological status to those of standard daily supplements

Utility of gamma-glutamyl transpeptidase and mean corpuscular volume in alcoholic liver disease.

The present study was designed to establish the role of Gamma-glutamyl transpeptidase (gamma-GT) and mean corpuscular volume (MCV) in alcoholic liver disease (ALD). Serum gamma-GT, total and direct bilirubin, albumin, total protein, AST, ALT and ALP were assayed by standard methods in a clinical chemistry autoanalyser. MCV, Hb, PCV and RBC were measured by an automated cell counter. Activity of gamma-GT and MCV levels were significantly higher in the patients with ALD compared to controls. A gamma-GT level of > or = 25 U/l was found to be significantly associated with ALD. MCV level > or = 100 fl/l showed a significant association with ALD. An AST to ALT ratio > 1 was found in 92% of the patients. None of the patients showed an ALT level > or = 300 IU/l. The degree of AST elevation in the patients with ALD was higher (3.7 times) then ALT (3.2 times). A gamma-GT level > or = 25 IU/L and an MCV level > or = 100 fl/l stand as markers of heavy alcohol consumption in this study. An AST to ALT ratio > 1 was present in most of the patients with ALD. The degree of elevation of AST was higher than ALT in the patients with ALD.

Haematological response of fish Channa punctatus (Bloch) to chronic zinc exposure.

Sub-lethal concentrations of zinc administered to freshwater teleost, Channa punctatus for a period of 135 days brought about significant haematological alterations. The RBC count, haemoglobin and haematocrit content progressively decreased while WBC count, MCV, MCH and MCHC increased. Alterations in the haematological parameters were dose and duration dependent and can thus serve as a useful physiological index.

Toxic effects of copper and zinc mixtures on some haematological and biochemical parameters in common carp, Cyprinus carpio (Linn).

The effect of heavy metals and mixture were studied on some haematological and biochemical parameters in fish, Cyprinus carpio at sublethal level over the period of 30 days. Heavy metal significantly decreased total RBC count, haemogoblin, heamatocrit (Hct) (except copper after 10 days exposure). The WBC count was increased significantly in all the treated fish. The MCH, MCHC, MVC were increased depending upon the exposure period; declined PVC was noticed at 1% level of significance after 30 days on all the treated fish. Serum level of chloride and calcium content were increased where as serum glucose, cholesterol and total serum protein were decreased significantly. The alteration in haematological and biochemical parameters shows more toxic in mixture after 30 days, showed more than 'addition action' of the individual toxicant.

Effect of vegetable oil intake on erythrocyte physical properties and hemorheology.

Effects of vegetable oil intake on red cell physical properties and blood flow condition were evaluated. The study was done on two groups of volunteers taking either soybean oil (6 volunteers) or palm oil (another 6 subjects) for a period of eight weeks. The continuous effects were followed up after continuing the vegetable oil intake for another four weeks. Significant reduction in red cell deformability was demonstrated by laser diffractometry in both groups. The palm oil had greater extent in causing reduced red cell deformability as early as four weeks after the oil intake, whereas, such effect was shown in eight weeks after the soybean oil intake. The reducing effect of red cell deformability by palm oil intake was more obvious than that by the soybean oil intake as indicated by the fall of deformability index from the control by 47.6 per cent in palm oil and 21.9 per cent in the soybean oil group. The decrease still persisted and could be investigated on the four weeks' followup. The hematologic parameters measured by the laser-based instrument, the H*1 analyser were all within the normal ranges in both groups. However, the group taking soybean oil showed decreasing hemoglobin levels, which may be a physiological response to facilitate blood flow condition. The platelet volume was increased in both groups, indicating the possibility of adjustment of thrombopoiesis in the subjects although no clinical indication related to this finding was shown. In conclusion, intake of vegetable oil had an effect on red cell deformability. Soybean oil had an advantage over palm oil in having less effect on reducing the red cell deformability.

Alteraçöes no eritrograma produzidas pelo sulfato de gentamicina em ratos / Erithrocyte changes produced by gentamicin sulfate in rats

O sulfato de gentamicina é um dos antibióticos aminoglicosídeos mais utilizados em antibioticoterapia. Ele produz efeitos colaterais como nefrotoxicidade, que requer muito cuidado no seu uso, e achamos relevante analisar as possíveis alteraçöes na série vermelha em doses terapêuticas e doses elevadas, estas compatíveis com lesäo renal. Para isto utilizou-se 173 ratos divididos em grupos, que receberam água destilada, e 5 mg/kg, 10 mg/kg, 20 mg/kg e 40 mg/kg de Gentamicina durante 5, 10, 15 e 20 dias respectivamente. Foram retiradas amostras sanguíneas, de todos os animais efetuado eritrograma. Observou-se que ocorreu diminuiçäo dos eritrócitos com doses de 20 mg/kg e 40 mg/kg aos 5 e 10 dias, e que estas foram reversíveis