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Background: Hemoglobinopathies pose a significant health burden in India. Prevention programmes can significantly reduce this burden. Although sophisticated methods of screening for β thalassemia trait are available, a cheap and simple method is beneficial for population screening. Although the Naked Eye Single Tube Red Cell Osmotic Fragility Test (NESTROFT) has been evaluated in many studies, sample sizes were small in some and many earlier studies have not done complete blood count (CBC) and High-Performance Liquid Chromatography (HPLC) in all the cases. We evaluate the suitability of NESTROFT for detection of β-thalassemia trait in a high prevalence region in Saurashtra, Gujarat.Methods: Here, 1000 unrelated individuals were studied. NESTROFT, CBC and estimation of HbA2 and HbF or other hemoglobin variants were done by HPLC.Results: Prevalence of β thalassemia trait was 7.8% in this population. NESTROFT showed an overall sensitivity and specificity of 94.87 and 85.38 respectively for the detection of β thalassaemia trait. Using red cell indices (MCH <27 pg and MCV <80 fl), One β thalassemia trait with normal indices would have been missed. Among twelve individuals with other hemoglobinopathies (HbS, HbD, HbE, δβ thalassemia trait or HPFH), seven had a positive NESTROFT while three had normal MCV & MCH values.Conclusions: NESTROFT is a cost-effective sensitive test which does not require any equipment and can be done in remote areas. It remains a useful first line screening test when large populations have to be screened.
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Objective To verify result consistency of the improved examination method and the standard operation method with the erythrocyte osmotic fragility test (OFT).Methods The samples and reagents were reduced half volume and used different testing equipment to evaluate methods for OFT.Selected 100 samples that the brittleness were increased and de-creased (50 samples positive and negative respectively),used a kind of improved examination method and traditional test method to evaluate the consistency.Results The detection result of improved examination method and the detection results of the manufacturer standard method were consistent (t=1.660 8,P >0.05).So,there was no significant difference of con-trast between two groups of data.Conclusion Improved mothed OFT alternatives to traditional OFTscreening method could be faster,more objectively the results of clinical service,and could effectively reduce reagent and manpower cost,improve the efficiency of work.Therefore,this method could be used to groups detection and lack of equipment for the primary care of hospital screening thalassemia desease method is preferred.
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Background: Parts of baobab tree (Adansonia digitata) including especially the barks are commonly used for their medicinal properties. Aims: The aim of this work is to evaluate the antisickling activity of baobab tree barks, which are used in Congolese traditional medicine to manage Sickle Cell Disease. Study Design: Baobab tree barks was extracted with water by maceration. Phytochemical tests were conducted with standard procedures. Antisickling activity and the minimum concentration of extract required to normalize sickled cells was determined by Emmel test. Place and Duration of Study: This work was done at department of chemistry, Science Faculty, University of Kinshasa (DR Congo), between November 2012 and February 2013. Methodology: The barks collected from Adansonia digitata were dried and powdered. A chemical screening was perform and extraction of anthocyanins done. Antisickling activity was evaluated by Emmel test, membrane stability by osmotic fragility test and Fe3+ evolution by following solution absorbance at 630nm. The rate of sickle cell shape normalization was determined at different plant aqueous extract concentrations in order to determine the minimal concentration of extract required to normalize sickle cells. The shape modification was quantitatively evaluated from the values of parameters such as surface, radius and perimeters of sickle blood cells before or after treatment with plant extract using Motic software. Results: The aqueous extract of Adansonia digitata showed an antisickling activity with a maximal normalization rate of 65.7% and a minimal concentration required to normalize sickled cells of 5.0mg/mL. The cell surface, perimeter and radius were significantly different before and after treatment with plant extract. The chemical screening showed the presence of polyphenols among which anthocyanins. The biological activity of this plant would be due to these pigments. The anthocyanins extract have also shown a stabilization effect on sickle blood red cells membranes and a reduction of methemoglobin to hemoglobin effect Conclusion: The results obtained show significant antisickling activity of Adansonia digitata barks thus justifying the use of this plant by traditional healers in Congolese traditional medicine in the management of Sickle Cell Disease.
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Microspherocytosis is known as a hallmark of hereditary spherocytosis (HS) which is one of the most common hemolytic anemias with a prevalence of one per 5000, and is inherited as Mendelian dominant. In this disorder, the patient's red cells become spheroidal, osmotically less resistant in the peripheral circulation, and are selectively trapped in the spleen, but survive normally after splenectomy. The exact mechanism for the formation of microspherocytosis has not been elucidated, although extensive investigations demonstrate that HS red cells are intrinsiclly defective and the patient's spleen does "condition" the metabolically abnormal red cells. The authors report here, one case of severe microspherocytosis in which changes in facial bone structure and transfusion dependency are noted, and an early splenectomy is indicated.