Prevalence, clinical, and hematological profile of sickle cell disease in South Rajasthan

Mandot, Sanjay; Ameta, Gaurav

doi:10.32677/ijch.2016.v03.i03.017

Cited by 6 publications

(3 citation statements)

References 5 publications

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“…In homozygous cases, Mandot et al . [ 9 ] showed that 64% of people had musculoskeletal pain, 35% had abdominal discomfort, 7% had chest pain, and 71% had splenomegaly. Their hospital-based investigation could be the cause of the elevated occurrence.…”

Section: Discussionmentioning

confidence: 99%

Morbidity pattern and impact of hydroxyurea therapy among sickle cell patients in Raipur district of Chhattisgarh

Vaibhav,

Panda,

Debata

et al. 2024

Journal of Family Medicine and Primary Care

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Background: Sickle cell disease (SCD) is a disorder marked by a single-point mutation in the beta-globin gene. Hydroxyurea is a globally accepted disease-modifying agent that sounds to be effective in managing clinically and probably preventing complications of SCD. The current study aims to document the morbidity pattern and impact of Hydroxyurea therapy in the Outpatient Department of Sickle Cell Institute, Raipur. Materials and Methods: This cross-sectional study was conducted among randomly selected sixty-five patients (adults and children above six years). After obtaining informed consent, relevant data were collected in a predesigned pretested questionnaire. The appropriate statistical exercise was applied for the interpretation of results and inferences. Results: Acute febrile illness 54 (83%) and 53 (81.5%) reported pain crisis observed to have the most common morbidity among the study subjects, followed by 55.4% (36), 33 (50.8%) jaundice and difficulty breathing, respectively. Joint pain was the most commonly observed complaint, particularly at the knee joint (76.9%). Other complaints such as hand-foot syndrome (24.6%), epistaxis (27.7%), and acute chest syndrome (21.5%). Vaso-occlusive crisis (72.4%), difficulty in walking (60.0%) and eyesight (35.4%), leg ulcers (9.2%), and dactylitis (3.1%) were also documented as clinical manifestations among study participants. Less than half (44.46%) had an awareness about SCD. Hydroxyurea therapy was highly significant in improving the patient’s clinical picture (P < 0.01), especially following the frequency of hospitalization and the requirement for blood transfusion. Conclusion: Pain crisis is the most common morbidity among study participants with a low level of knowledge about SCD with febrile illness. Hydroxyurea therapy was found to be quite effective as a disease-modifying therapy, especially for reducing the frequency of blood transfusion and lowering hospitalization rates among SCD patients.

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Section: Discussionmentioning

confidence: 99%

Morbidity pattern and impact of hydroxyurea therapy among sickle cell patients in Raipur district of Chhattisgarh

Vaibhav,

Panda,

Debata

et al. 2024

Journal of Family Medicine and Primary Care

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“…In the present study, 36,752 students were screened and the prevalence of SCD/SCT was reported at the village level in the tribal sub-plan region of Rajasthan. Mandot and Ameta 22 screened a total of 1090 children belonging to Garasia tribe in Sirohi district of Rajasthan and the prevalence of sickle cell disorder reported by them was 8.53 per cent. Wherein 0.77 per cent were homozygous (HbSS) and 7.7 per cent were heterozygous (HbAS) which was comparatively lower than that in the present study.…”

Section: Discussionmentioning

confidence: 99%

Burden of sickle cell disease in tribal students in Maa-Baadi institutions in southern Rajasthan - A pilot study

2022

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Background & objectives: Sickle cell disease (SCD), an inherited disorder of erythrocytes, is highly prevalent in the tribal population of India. The tribal population of India is approximately 100 million and it is necessary to identify the magnitude of this problem. Furthermore, the prevalence of the disease is unknown among the five million tribal people of southern provinces of Rajasthan. In this study, we intended to determine the prevalence and characteristics of sickle cell disorder among the tribal inhabitants of southern Rajasthan. Methods: This cross-sectional study was conducted among the tribal students of the Maa-Baadis and hostels situated in the five tribal sub-plan districts of Rajasthan. Maa-Baadi centres are located in every village, whereas for every four to five villages, one hostel is allocated to accommodate the tribal students. The screening for SCD was done by solubility test and electrophoresis was used for confirmation. Results: A total of 36,752 tribal students were screened from 1,006 Maa-Baadi centres and 243 hostels. The prevalence of SCD among the tribal students was 5.8 per cent. The prevalence of heterozygous and homozygous conditions was 5.61 and 0.17 per cent, respectively. Among the five sub-plan districts, the highest prevalence was observed in Sirohi district (10.5%) followed by Banswara (7.42%), Udaipur (6.53%), Pratapgarh (5.51%) and Dungarpur (1.89%). Among the four major tribes belonging to these districts, the highest prevalence was recorded in Garasia tribes (13.81%). The history of leg ulcers and the mean pulse rate were significantly high in SCD individuals. Interpretation & conclusions: SCD is a significant problem among the tribes of southern Rajasthan, with the highest prevalence among the Garasia tribe. The present study recommends that a structured screening programme targeting the entire tribal population with appropriate counselling as well as providing treatment through the existing health system is the need of the hour.

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“…Tribal groups with high prevalence of Hb-S (20-35%) include Bhils, Madiyas, Pawaras, Pardhans, and Otkars [42,43]. In India, apart from tribal individuals [44][45][46][47][48][49][50][51][52][53][54], red cell genetic disorders, such as abnormal Hbs, thalassaemia syndrome, and Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency, have also been reported in various ethnic groups belonging to Scheduled Castes (SCs), Other Backward Castes (OBCs) and minorities including Muslim, Bohra-Muslim and Jain communities have also been reported in Rajasthan state [55][56][57][58][59][60][61][62][63][64][65][66][67]. In fact, this state has two distinct ecosystems arid or desert and humid where malaria is found to be hypo and hyperendemic, respectively.…”

Section: Sickle Cell Genes Among Tribal Peoplementioning

confidence: 99%

How Do Sickle Cell Genes Protect Tribal People from Deadly Malaria? Is This a Type of Natural Selection?

Choubisa

2023

annhematoloncol

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In humans, genetic mutations have led to the formation of several haemoglobin (Hb) variants during human evolution. These mutations usually occur in the genes for a and Β- polypeptide or globin chains of haemoglobin located on chromosomes 11 and 16, respectively. Among these haemoglobin variants, sickle cell haemoglobin (Hb-S) is highly significant in the humans. In fact, this abnormal or mutant haemoglobin is the resultant of a point mutation in the gene for Β-globin, located on chromosome 11. No doubt, this sickle cell gene is relatively more prevalent among tribal people and widely distributed in those geographic regions where the dreaded Plasmodium falciparum-malaria is hyperendemic. In the human population, this mutant gene exists in two forms, the heterozygous (Hb-AS) and homozygous (Hb-SS) states. But this sickle cell gene is also found in association with other mutant genes of erythrocyte or red cell genetic disorders, such as abnormal Hb-C, Β-thalassemia, G-6-PD enzyme deficiency, etc. It is well known that tribal people generally live in forest ecosystem where dreaded malaria is also found to be endemic. How have these tribal people been able to protect themselves from this dreaded malaria for thousands of years in such a hostile environment, when they were most likely to be infected with the malaria parasite? Actually, its secret is hidden in these sickle cell genes only. Tribal individuals who have these genes in the heterozygous state (Hb-AS) do not get malaria or are protected from malaria and lead a healthy life. Several studies have confirmed that AS heterozygote people are protected against malaria. In contrast, those who have these genes in the homozygous (Hb-SS) state (homozygotes) develop sickle cell anaemia, which is fatal. Such patients die early due to severe malaria i.e AS heterozygotee individuals have a double advantages. In fact, it is a type of natural selection and also shows a balanced polymorphism in tribal people which is based on Darwin’s theory. This current communication provides factual and scientific information about the origin of sickle cell genes in the context of natural selection and how these genes protect tribal individuals from malaria. This communication is useful and important for zoologists, scientists working on human evolution, and medical scientists.

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Prevalence, clinical, and hematological profile of sickle cell disease in South Rajasthan

Cited by 6 publications

References 5 publications

Morbidity pattern and impact of hydroxyurea therapy among sickle cell patients in Raipur district of Chhattisgarh

Morbidity pattern and impact of hydroxyurea therapy among sickle cell patients in Raipur district of Chhattisgarh

Burden of sickle cell disease in tribal students in Maa-Baadi institutions in southern Rajasthan - A pilot study

How Do Sickle Cell Genes Protect Tribal People from Deadly Malaria? Is This a Type of Natural Selection?

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