scholarly journals Prevalence of Hemoglobinopathies in Tribal Region of India: A Retrospective Observational Study

2020 ◽  
Vol 8 (4) ◽  
pp. 3
Author(s):  
Naveen Dulhani ◽  
Pratima Koshewara ◽  
Rupendra K. Bharti ◽  
Sanat K Sharma
Keyword(s):  
Sickle Cell Disease ◽  
Observational Study ◽  
Sickle Cell ◽  
Retrospective Observational Study ◽  
Beta Thalassemia ◽  
Tribal Population ◽  
Tribal Community ◽  
Cell Disease ◽  
Hereditary Persistence ◽  
Tribal Region

Hemoglobinopathies are the common inherited diseases around the world. Thalassemia & sickle cell disease are the important challenges for tribal populations in India. Many study demonstrated the prevalence of haemoglobinopathies in India & among tribes of India but limited data available from Baster tribal region. This study will further lightens the haemoglobinopathies among Baster region of Chhattisgarh state of India. Methods: It was an retrospective observational study, carried out in Late. BRKM Government Medical College, Dimrapal, Jagdalpur which was located at baster region of Chhattisgarh state of India aims to determine the prevalence of various hemoglobinopathies in Baster. Out of 421 suspected patient’s screened for hemoglobinopathies by Capillary electrophoresis. Statistical Package for Social Sciences (SPSS) used for descriptive analysis. Results: Out of 421 cases, 276 were diagnosed with various type of hemoglobinopathies {49% has HbAS (sickle cell anaemia trait), 3% HbSS (sickle cell disease), 6% sickled beta-thalassemia & 8% HPFH (hereditary persistence of foetal hemoglobin)}. Non-tribal population has higher trends of sickled beta thalassemia 14 (8.28%), Hereditary persistence of foetal hemoglobin 26 (15.38%) and HbAS 122 (72.19%) as compare to tribal population but there was similar prevalence of HbSS among both of these groups. Conclusion: In India, hemoglobin disorders are the great threat for tribal population. As <10% of tribes residing in India and many were extinct. The non-tribal community has more prevent than tribal communities.

Distribution of Sickle Cell Disease Among Tribal Population of Nilgiri District of Tamil Nadu

2012 ◽  
Vol 2 (11) ◽  
pp. 243-247
Author(s):  
Brindha B Brindha B ◽  
◽  
Vidyalakshmi R Vidyalakshmi R ◽  
Prashanthi Devi M Prashanthi Devi M
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Tamil Nadu ◽  
Tribal Population ◽  
Cell Disease

Laparoscopic cholecystectomy in adult patients with beta-thalassemia or sickle cell disease

2005 ◽  
Vol 19 (12) ◽  
pp. 1668-1669 ◽  
Author(s):  
G. Marakis ◽  
T. E. Pavlidis ◽  
K. Ballas ◽  
S. Rafailidis ◽  
A. Sakantamis
Keyword(s):  
Laparoscopic Cholecystectomy ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Adult Patients ◽  
Beta Thalassemia ◽  
Cell Disease

Comprehensive integrated care for patients with sickle cell disease in a remote aboriginal tribal population in southern India

2013 ◽  
Vol 61 (4) ◽  
pp. 702-705 ◽  
Author(s):  
Vivek Nimgaonkar ◽  
Lakshmanan Krishnamurti ◽  
Hari Prabhakar ◽  
Nandakumar Menon
Keyword(s):  
Sickle Cell Disease ◽  
Integrated Care ◽  
Sickle Cell ◽  
Southern India ◽  
Tribal Population ◽  
Cell Disease

scholarly journals Impact of neuropathic pain on quality of life in adults with sickle cell disease: observational study

2020 ◽  
Author(s):  
Lismar Fernando Oliveira dos Santos ◽  
Milenna Wild Guimarães ◽  
Abrahão Fontes Baptista ◽  
Katia Nunes Sá
Keyword(s):  
Quality Of Life ◽  
Neuropathic Pain ◽  
Sickle Cell Disease ◽  
Observational Study ◽  
Sickle Cell ◽  
Cell Disease

scholarly journals Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease [see comments]

Blood ◽  
1995 ◽  
Vol 86 (2) ◽  
pp. 776-783 ◽  
Author(s):  
FM Gill ◽  
LA Sleeper ◽  
SJ Weiner ◽  
AK Brown ◽  
R Bellevue ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Clinical Course ◽  
The United States ◽  
Beta Thalassemia ◽  
Acute Chest Syndrome ◽  
Cooperative Study ◽  
Cell Disease ◽  
Splenic Sequestration ◽  
Clinical Events

Within the Cooperative Study of Sickle Cell Disease, 694 infants with confirmed sickle cell disease were enrolled at less than 6 months of age. Information about the nature and frequency of complications was collected prospectively over a 10-year period. Painful crises and acute chest syndrome were the most common sickle cell-related events in homozygous sickle cell anemia (SS), hemoglobin SC disease (SC), and S beta thalassemia patients (overall incidence in SS patients of 32.4 and 24.5 cases per 100 person-years, respectively). Bacteremia occurred most frequently in SS children under 4 years of age and in SC patients less than 2 years of age. The mortality rate was low in this cohort compared with that found in previous reports. Twenty children, all with Hb SS, died (1.1 deaths per 100 person-years among SS patients). Infection, most commonly with Streptococcus pneumoniae and Hemophilus influenzae, caused 11 deaths. Two children died of splenic sequestration, 1 of cerebrovascular accident, and 6 of unclear causes. Two patients underwent cholecystectomies, and 17 underwent splenectomies after one or more splenic sequestration crises. The experience of this cohort should reflect closely the true clinical course of those children with Hb SS and Hb SC disease who are observed in sickle cell centers in the United States.

scholarly journals Hydroxyurea increases hemoglobin F levels and improves the effectiveness of erythropoiesis in beta-thalassemia/hemoglobin E disease

Blood ◽  
1996 ◽  
Vol 87 (3) ◽  
pp. 887-892 ◽  
Author(s):  
S Fucharoen ◽  
N Siritanaratkul ◽  
P Winichagoon ◽  
J Chowthaworn ◽  
W Siriboon ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Beta Thalassemia ◽  
Limited Information ◽  
Cell Disease ◽  
Hemoglobin F ◽  
Hemoglobin E ◽  
Globin Chains ◽  
Hb E ◽  
Alpha Globin

Hydroxyurea (HU) is one of several agents that have been shown to enhance hemoglobin (Hb) F levels in patients with sickle cell disease and may be useful as a therapy for beta-globinopathies. However, limited information exists on the effects of HU in patients with thalassemia. Accordingly, we examined the hematologic effects of orally administered HU in 13 patients with beta-thalassemia/Hb E, including four patients who had been splenectomized. These patients were treated with escalating doses (final range, 10 to 20 mg/kg/d) for 5 months and were observed in the outpatient hematology clinic every 2 to 4 weeks. Complete blood counts including reticulocyte counts, amounts of Hb E and Hb F, G gamma:A gamma and alpha:non-alpha globin biosynthetic ratios were evaluated before and during treatment. Almost all patients responded with an average increase of 33% in Hb F levels, from a mean (+/- SD) of 42% +/- 11% to 56% +/- 8% (P < .0001), and a reciprocal decline in the percentage of Hb E from 59% +/- 9% to 49% +/- 8% (P < .001). Reticulocytosis was decreased from a mean (+/- SD) of 18.0% +/- 15.6% to 11.7% +/- 9.1% (P < .05); there was also a slight (10%) but statistically significant increase in hemoglobin levels and an improved balance in alpha:non-alpha globin chains ratios. The side effects were minimal in most patients, although these patients tended to tolerate a lower dose of HU before significant myelosuppression than has been our previous experience in sickle cell disease. One splenectomized patient died of sepsis during the trial. We conclude that increased Hb F production in beta-thalassemia/Hb E patients, with an improvement in the alpha:non-alpha globin ratios and, probably, the effectiveness of erythropoiesis, can be achieved using HU. Longer trials of HU in this population, including at other doses and in combination with other agents, appear warranted.

Maternal and Perinatal Outcome of Women With Sickle Cell Disease of a Tribal Population in Central India

Hemoglobin ◽  
2014 ◽  
Vol 38 (2) ◽  
pp. 91-94 ◽  
Author(s):  
Neeta Natu ◽  
Seema Khandelwal ◽  
Ravindra Kumar ◽  
Anupama Dave
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Perinatal Outcome ◽  
Central India ◽  
Tribal Population ◽  
Cell Disease
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