This study describes the use of hydroxyurea therapy in patients with HbSD-Punjab. Low dose hydroxyurea (10 mg/kg/day) was found to be effective in reducing the clinical severity in patients with HbSD-Punjab without any short-term toxicity. In view of easy affordability amongst poor patients, widespread acceptability by patients and doctors, the need of infrequent monitoring and its potential effectiveness, low dose hydroxyurea is suitable for treatment of patients with HbSD-Punjab.
In view of easy affordability, better acceptability, minimal toxicity, the need of infrequent monitoring and its potential effectiveness, low and fixed dose of hydroxyurea is suitable for treatment of patients with HbSβ(+) -thalassemia in resource poor setting.
Inherited hemoglobin disorders like alpha thalassemia and sickle gene are common in the Indian subcontinent. These disorders in the heterozygous state act as malaria resistance genes and influence the susceptibility to Plasmodium falciparum malaria. There is inadequate knowledge about the epidemiology of these malaria resistance genes in the tribal dominated malaria endemic region of the state of Odisha in eastern India. A cross sectional prevalence study was undertaken in 594 subjects in five tribal populations in this region, namely, Sahara (42.4%), Kutia Kandha (30.0%), Kuda (15.8%), Gond (9.8%), and Oraon (2.0%). Sickling test, Hb electrophoresis, HPLC, and molecular studies were undertaken to diagnose the prevalence of sickle allele, β-thalassemia allele, and deletional alpha thalassemia. Sickle and β thalassemia alleles were found in 13.1% and 3.4% of subjects, respectively. Sickle allele was found both in heterozygous (10.1%) and homozygous state (3.03%). The prevalence of alpha thalassemia was 50.84% with an allelic frequency of 0.37. Both α
−3.7 and α
−4.2 alpha thalassemia were detected with an allele frequency of 0.33 and 0.04, respectively. The high prevalence of alpha thalassemia and sickle gene in this population is probably due to selection pressure of endemic malaria in this part of India.
HbF reduced the frequency of VOC but had no influence on the hemolytic markers in HbSD. HbSD with alpha thalassemia was associated with hypohromic and microcytic features of red blood cells.
HbS-Hofu has a variable clinical presentation. The retention time of Hb Hofu on HPLC is very close to that of HbA(0) and often elutes in the A0 window. Thus, there is every possibility of the HbS-Hofu chromatogram to be misinterpreted as that of a sickle cell trait/transfused sickle cell-beta-thalassemia case. This is the first time where Hb Hofu has been detected by HPLC, which is the widely accepted screening technique for hemoglobinopathies around the world.
The present investigation was carried out on the dead Asian elephant (Elephas maximus) presented to the Centre for Wildlife Health, OUAT, Bhubaneswar whose skeleton remains were attempted for fire burn by the land poachers. After ascertaining the sex, tentative age and height of the animal basing on the morphology of skull, number of lamellae on the table surface of molar tooth and shoulder height respectively, the morphological and biometrical studies were conducted on the skull, atlas and axis vertebra to develop a baseline data for the particular species, sex and age group of the Asiatic elephant. The present biometric study was conducted by using the instruments used for routine biometrical studies of the bones such as graduated tape, scale and digital Vernier's caliper. It was found that the skull contained air cavities that gave the inside of the skull a honeycomb-like appearance and back of the skull was flattened and spread out. The atlas had dorsal and ventral arches, united by the lateral masses of cartilage. The length and width of the body of atlas were comparatively smaller than that of axis. The length of cranial articular facet of left side of atlas was greater than that of the right side, where as a reverse relation was noted for their widths. The foramina transversarium were found to be circular in shape in atlas. Further, the axis was comprised of a body carrying the dens and the neural arch, united by a cartilage. Unlike atlas, the foramen transversarium of left side was larger than that of the right one in axis. It aimed to develop a baseline data for the particular species, sex and age group of the Asiatic elephant, which will help explore new avenues in the forensic wild life science in dealing with the vetero-legal cases particularly for the identification purposes. Further, the gross and biometric studies may be conducted on the whole axial skeleton of the female Asiatic elephant (Elephas maximus) along with their biomechanical role.
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