Sickle cell crisis as a cause of death over the past 10 years in Vidarbha Region: A study of 18 cases

Abstract

Background: Sickle cell disease is a heritable disorder having an autosomal recessive inheritance. The disease arises as a consequence of a single base mutation in the sixth codon of the β-globin gene, which leads to an interchange of valine for glutamic acid. Aim: The aim of this study is to provide an insight to physicians about sickle cell disease being a cause of sudden demise, so that future unexpected deaths due to complications can be avoided. Materials and Methods: This study is a retrospective study conducted over a period of ten years from January 2010 to December 2019. The data was collected from the hospital information system of our institute. A total of 18 cases were collected over a period of ten years. The respective case related findings along with past history were reviewed. Results: Out of 18 cases, 11 cases were males and 7 were females. The youngest person was a nine-year old male and oldest was a 65-year-old male. Four cases were known patients of sickle cell disease, while in the remaining cases no history of sickle cell disease was available. Conclusions: Sickle cell crisis is an important reason of sudden unexplained deaths and this study highlights the role of autopsy in such cases.