Sickle Cell Disease - Case Reports

Adhikari, Ram Chandra; Shrestha, Tumla; Shrestha, Rupesh; Subedi, Roshan; Parajuli, Keshab; Dali, Sanu Maiya

doi:10.31729/jnma.715

Cited by 3 publications

(1 citation statement)

References 3 publications

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“…[4][5] Although studies have previously reported blood disorders such as glucose-6-phosphate dehydrogenase deficiency, alpha thalassemia, and SCD in the Tharu community, only few cases of SCD have officially been reported in Nepali medical journals since 2003. [5][6][7][8] Here, we present the first largest study that provides data on demographic status and types of SCD reported in Western region of Nepal.…”

Section: Introductionmentioning

confidence: 99%

Sickle cell disease in Western Nepal

Pande¹,

Ghimire

Chand³

et al. 2019

Nep J Med Sci

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Introduction: Since 2003, only few cases of sickle cell disease have officially been reported in Nepalese medical journals, and all reported patients belong to the Tharu ethnic group of Nepal. This is the first study that details sickle cell disease and carrier in 1250 individuals in western Nepal. Methods: This is a retrospective review of the patients and carriers of sickle cell disease diagnosed by either a positive haemoglobin electrophoresis report or a positive high performance liquid chromatography (HPLC) report. Analysis was done using SPSS 20. Results: Out of the 1250 individuals, 51.4% were females. 601 (48.08%) were patients with a form of sickle cell disease, the mean age was 24.5  12 yrs years. Most patients came from Bardiya district. Most common symptoms were related to joint pain. The patients and carriers of sickle cell disease were mostly from Tharu ethnic group (97.7%). Conclusion: These data suggest that sickle cell disease and other haemoglobinopathies are more prevalent than previously reported among members of the Tharu ethnic group and other residents of western Nepal. More research is imperative to assess the burden of the sickle cell disease and other haemoglobinopathies in Nepal.

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Section: Introductionmentioning

confidence: 99%

Sickle cell disease in Western Nepal

Pande¹,

Ghimire

Chand³

et al. 2019

Nep J Med Sci

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Exploring barriers to sickle cell disease care in a lower-middle income country – A qualitative exploration of the Tharu perspective in rural Nepal

Liu,

Benson,

Liang

et al. 2023

Clinical Epidemiology and Global Health

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The Assessment and Sustainable Management of Sickle Cell Disease in the Indigenous Tharu Population of Nepal

et al. 2017

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Sickle cell disease is an inherited hemoglobinopathy associated with significant morbidity and mortality. Reports suggest a high sickle cell disease burden among the indigenous Tharu population of Nepal, who for centuries have inhabited regions where malaria is endemic. Unfortunately, health care resources are limited and often inaccessible for Tharu individuals suffering from sickle cell disease. We conducted a large-scale screening effort to estimate the prevalence of Hb S (HBB: c.20A>T) among the Tharu population and delivered community-based education sessions to increase sickle cell disease awareness. A total of 2899 Tharu individuals aged 6 months to 40 years in the rural district of Dang in Western Nepal were screened using a sickling test, of whom, 271 [9.3%; 95% confidence interval (95% CI): 8.3-10.4%] screened positive for Hb S. Those who screened positive were offered diagnostic gel electrophoresis testing. Of the 133 individuals who underwent diagnostic testing, 75.9% (n = 101) were confirmed to be Hb AS heterozygotes, 4.5% (n = 6) were confirmed to be Hb SS homozygotes and 19.5% (n = 26) were false positives. These findings support a large burden of sickle cell disease among the Tharu population and highlight the importance of appropriate resource allocation and management. With a positive predictive value of 80.0% (95% CI: 73.0-87.0%), the sickling test in conjunction with raising local sickle cell disease awareness may be a simple and sustainable way to promote access to health resources.

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Sickle Cell Disease - Case Reports

Cited by 3 publications

References 3 publications

Sickle cell disease in Western Nepal

Sickle cell disease in Western Nepal

Exploring barriers to sickle cell disease care in a lower-middle income country – A qualitative exploration of the Tharu perspective in rural Nepal

The Assessment and Sustainable Management of Sickle Cell Disease in the Indigenous Tharu Population of Nepal

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