Sickle cell disease in Western Nepal

Abstract

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.

Glucose 6 phosphate dehydrogenase deficiency and hemoglobinopathy in South Western Region Nepal: a boon or burden

“…However, these tests do not accurately define G6PD activity in females, potentially exposing women with intermediate G6PD activity to the risk of severe anemia, hemolysis and other health impacts [13]. The present study highlighted the highest prevalence of G6PD deficiency in Dang and Bheri [11], [14]. The studies conducted in Afghanistan, Bangladesh, Bhutan, India, Nepal, and Pakistan found that the G6PD deficiency prevalence ranges from 3.8 to 15%, with endemic areas of Nepal [15].…”

Spontaneous extradural hematoma in a Sickle cell Beta Thalassemia patient—A rare complication

“… 2 Although stroke is commonly encountered in the African and African American population, in our Nepalese population stroke is rarely documented. 4 , 5 , 6 …”

“…2 Although stroke is commonly encountered in the African and African American population, in our Nepalese population stroke is rarely documented. [4][5][6] Spontaneous extradural hematoma is a rare complication of SCD with few cases reported in the literature. 7,8 Majority of the cases are from the African and Middle Eastern population, with no any case reported in the English literature affecting the Tharu population of Nepal.…”

“…The various manifestations include chronic headache, epilepsy, ischemic or hemorrhagic stroke, cognitive impairment due to chronic anemia, Moyamoya disease, hypoxia, and silent infarcts 2 . Although stroke is commonly encountered in the African and African American population, in our Nepalese population stroke is rarely documented 4–6 …”

“…Sickle cell disease (SCD) and its variants encompass a group of autosomal recessive disorders resulting from a single nucleotide polymorphism in the beta globin gene. Although considered rare in Nepal, recent studies have demonstrated increased prevalence of SCD as well as SCD along with other genetic hemoglobinopathies such as beta Thalassemia 4 . Although there has been a tremendous advance in the understanding of inherited disorders of hemoglobin at molecular level, they are still under recognized resulting in significant morbidity as well as mortality 1…”