Abstract

Background: Sickle cell disease (SCD) is an inherited blood disorder caused by abnormal haemoglobin. Present study was done to study the relationship of clinical presentation & haematological findings in case of symptomatic & asymptomatic sickle cell disorder patients . Methods: In the present study, the RBC parameters like Hb, MCV, MCH, MCHC, RDW, RBC COUNT & PCV were recorded & results of sickling test & Hb electrophoresis were used to confirm a case of sickle cell disorder. Result: Categorical Variables (age &sex ) were expressed in actual number & percentages. Continuous variables ( Hb, MCV, MCH, MCHC, RDW, RBC COUNT & PCV ) were presented as Mean. Continuous variables were compared between Sickle cell disease subjects, sickle cell trait subjects & normal subjects by one way Analysis of Variance (ANOVA) test.p value < 0.05 was considered to be statistically significant Conclusion: The most number of symptomatic patients of sickle cell disorder were found in the 2 nd decade of age, followed by 3rd decade. There was a greater percentage of female subjects detected as sickle cell Disease (52.7%) & Sickle cell trait (62.8%) which points towards female preponderance of sickle cell disorder. Weakness & fatigue were the predominant symptoms of presentations among both sickle cell disease & sickle cell trait subjects. The peripheral smear examination in sickle cell disorder predominantly shows normocytic to microcytic with hypochromic picture. DOI: 10.21276/APALM.1247

Highlights

  • Sickle cell disease (SCD) is an inherited blood disorder caused by abnormal haemoglobin

  • There was a greater percentage of female subjects detected as sickle cell Disease (52.7%) & Sickle cell trait (62.8%) which points towards female preponderance of sickle cell disorder

  • Weakness & fatigue were the predominant symptoms of presentations among both sickle cell disease & sickle cell trait subjects

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Summary

Introduction

Sickle cell disease (SCD) is an inherited blood disorder caused by abnormal haemoglobin It limits the oxygenating role of haemoglobin, resulting in the damaging or the “sickling” of the red blood cells .There is high prevalence of sickle cell disease in socioeconomically backward groups in India. Sickle cell disease refers to a group of genetic disorders, characterised by presence of sickled haemoglobin (HBS), anemia, acute & chronic tissue injury secondary to blockage of blood flow by abnormally shaped Red cells. It is one of the variants of disorders of haemoglobin inherited from both the parents in an autosomal recessive pattern. Present study was done to study the relationship of clinical presentation & haematological findings in case of symptomatic & asymptomatic sickle cell disorder patients

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