Sickle cell disease in Madhya Pradesh, Central India: A comparison of clinical profile of sickle cell homozygote vs. sickle-beta thalassaemia individuals

Yadav, R. N.; Lazarus, Monica; Ghanghoria, Pawan; Singh, Mohinder; Gupta, Rajaneesh; Kumar, Santosh; Sharma, Rimple; Shanmugam, Rajasubramaniam

doi:10.1080/10245332.2016.1148893

Cited by 20 publications

(17 citation statements)

References 19 publications

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“…Requirement for regular blood transfusions was higher among Hb SS (33.33%) than severe Hb S/b+ (2.70%) and Hb S/b 0 (14.30%) patients in the present study. These ndings differ from a study reported from Madhya Pradesh (India) in which 16.1% of Hb SS and 17.4% of SBT patients were on regular blood transfusion (31).…”

Section: Genetic Ndingscontrasting

confidence: 99%

“…In the present study, in patients who were not on transfusion, mean Hb F concentrations were 24.4%, 23.7% and 21.2% in Hb SS, severe Hb S/b+ and Hb S/b 0 patients, respectively. These values are in accordance with the observations from the Maharashtra (India) but are higher than the values observed in Madhya Pradesh (India) (31,34). The Hb F levels are much higher than those found in SCD patients of Sub-Saharan African origin, and levels greater than 20% would typically be associated with less severe clinical picture.…”

Section: Genetic Ndingssupporting

confidence: 90%

“…Joint pains are not uncommon among sickle patients in the Indian subcontinent. A recent study from Madhya Pradesh, Central India reported that the incidence of joint pain is over 80% in both Hb SS and SBT groups (31). Bone pain reported at high frequencies in Indian SCD patients (32)(33)(34), was not present in any participants in our study.…”

Section: Genetic Ndingscontrasting

confidence: 46%

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Sickle cell disease in Sri Lanka: Clinical and molecular basis and the unanswered questions about disease severeity.

Darshana

Bandara²,

Nawarathne³

et al. 2020

Preprint

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Background Though case reports and limited case series of Sickle cell disease in Sri Lanka have been reported previously, no attempt has been made hitherto to undertake a comprehensive genotypic-phenotypic analysis of this “rare” group of patients. Results All accessible Sickle cell disease patients, totaling 60, including, 51 Sickle β-thalassaemia and 9 homozygous sickle patients were enrolled from seven thalassaemia treatment centres between December 2016 - March 2019. The majority of patients were of Sinhalese ethnicity (n=52, 86.67%). Geographically, two prominent clusters were identified and the distribution of Sickle haemoglobin in the island contrasted markedly with the other haemoglobinopathies. 3/ 9 homozygous sickle patients and 3/ 51 Sickle β-thalassaemia patients were receiving regular transfusion. Joint pain was the commonest clinical presentation among all sickle cell disease patients (n=39, 65.0%). Dactylitis was significantly more common in homozygous sickle patients compared with the Sickle β-thalassaemia groups (p 0.027). Two genetic backgrounds sickle mutation were identified namely, Arab Indian and Benin. Among the regulators of Foetal hemoglobin in Sickle patients of the present study rs1427407 G>T seemed to be the most prominent modifier, with a significant association with Foetal haemoglobin levels (p 0.04). Conclusions Overall, the clinical course of the Asian version of Sickle cell disease in Sri Lanka appears to be milder than that described in India.

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Section: Genetic Ndingscontrasting

confidence: 99%

Section: Genetic Ndingssupporting

confidence: 90%

Section: Genetic Ndingscontrasting

confidence: 46%

See 1 more Smart Citation

Sickle cell disease in Sri Lanka: Clinical and molecular basis and the unanswered questions about disease severeity.

Darshana

Bandara²,

Nawarathne³

et al. 2020

Preprint

View full text Add to dashboard Cite

show abstract

“…Requirement for regular blood transfusions was higher among Hb SS (33.33%) than SBT (5.88%) patients in the present study. These findings differ from a study reported from Madhya Pradesh (India) in which 16.1% of Hb SS and 17.4% of SBT patients were on regular blood transfusion [21]. Furthermore, twelve (23.53%) SBT patients had never received a transfusion in their lifetime.…”

Section: Discussioncontrasting

confidence: 89%

Sickle cell disease in Sri Lanka: Clinical and molecular basis and the unanswered questions about disease severeity.

Darshana

Bandara²,

Nawarathne³

et al. 2020

Preprint

View full text Add to dashboard Cite

Background Though case reports and limited case series of Sickle cell disease in Sri Lanka have been reported previously, no attempt has been made hitherto to undertake a comprehensive genotypic-phenotypic analysis of this “rare” group of patients.Results All accessible Sickle cell disease patients, totaling 60, including, 51 Sickle β-thalassaemia and 9 homozygous sickle patients were enrolled from seven thalassaemia treatment centres between December 2016 - March 2019. The majority of patients were of Sinhalese ethnicity (n=52, 86.67%). Geographically, two prominent clusters were identified and the distribution of Sickle haemoglobin in the island contrasted markedly with the other haemoglobinopathies. 3/ 9 homozygous sickle patients and 3/ 51 Sickle β-thalassaemia patients were receiving regular transfusion. Joint pain was the commonest clinical presentation among all sickle cell disease patients (n=39, 65.0%). Dactylitis was significantly more common in homozygous sickle patients compared with the Sickle β-thalassaemia group ( p 0.034). Two genetic backgrounds sickle mutation were identified namely, Arab Indian and Benin. Among the regulators of Foetal hemoglobin in Sickle patients of the present study rs1427407 G>T seemed to be the most prominent modifier, with a significant association with Foetal haemoglobin levels ( p 0.04).Conclusions Overall, the clinical course of the Asian version of Sickle cell disease in Sri Lanka appears to be milder than that described in India.

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“…Another study done by Yadav et al, found higher incidence of splenomegaly in patients with Sickle cell anaemia and sickle β thalassemia. 10 In their study 68.8% children with homozygous SCA and 82.6% children with Sickle β thalassemia had splenomegaly. However, occurrence of recurrent bacteremia in spite of penicillin prophylaxis does indicate the non-functioning nature of spleen in central India population.…”

Section: Discussionmentioning

confidence: 99%

Clinical and haematological profile of children with sickle cell anaemia admitted to a rural medical college of Chhattisgarh, India

Nayak¹,

Motwani²,

Dewangan³

et al. 2017

Int J Contemp Pediatr

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Background: Sickle cell disease (SCD) is the most common single gene disorder resulting in hemolytic anemia. Aim of the study was to describe the clinico-haematological profile of children with sickle cell anaemia admitted to Paediatric ward/PICU with any acute clinical event and to find out the association between high HbF level, frequency of crises episodes and requirement of blood transfusion in sickle cell anemia. Methods: Hospital based descriptive study. Retrospective data analysis was done from medical records of patients between 0-15 years age group admitted to hospital from March 2014 to August 2017. Results: Total 68 clinical events were recorded in 60 patients during the study period. More than half of the children were in 0-5 years age group. Mean age of diagnosis was 2.79 years. Severe anemia requiring blood transfusion was the most common cause of hospitalization followed by painful crises. Mean Hb level in the children was 6.65(±2.38). More than one third of children had associated nutritional anemia. Children with high HbF level were found to have less number of painful crises episodes. Conclusions: Severe anemia followed by painful crises were the commonest presentations requiring admission in our hospital. Chronic anemia with microcytosis and high HbF level is the hematological profile of the present study group. Children with high HbF level suffered from less number of painful crises episodes when compared to children with low HbF. But the requirement of blood transfusion was similar in both groups.

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Sickle cell disease in Madhya Pradesh, Central India: A comparison of clinical profile of sickle cell homozygote vs. sickle-beta thalassaemia individuals

Cited by 20 publications

References 19 publications

Sickle cell disease in Sri Lanka: Clinical and molecular basis and the unanswered questions about disease severeity.

Sickle cell disease in Sri Lanka: Clinical and molecular basis and the unanswered questions about disease severeity.

Sickle cell disease in Sri Lanka: Clinical and molecular basis and the unanswered questions about disease severeity.

Clinical and haematological profile of children with sickle cell anaemia admitted to a rural medical college of Chhattisgarh, India

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