Clinical profile of sickle cell disease in Orissa

Indian J Pediatr. Jan-Feb 1997;64(1):73-7. doi: 10.1007/BF02795780.

Abstract

Children comprised 52% of patients with Sickle Cell Disease (SCD). Types of Sickle Cell Disease encountered were SS (92.7%). SB thalassaemia (6.7%) and SD disease (0.7%). The disease was widespread in almost all castes and communities in the society; largest number of patients (20%) belonging to scheduled castes and only 1.4% were from scheduled tribes. Maximum number of cases were in the age group 2-4 and 4-6 years, many of whom died around this age. Besides attacks of pain, jaundice and anemia, frequent attacks of fever with anemia or only anemia in childhood were a predominant presenting feature. Splenic sequestration was frequent (10.1%). The patients usually had a steady state hemoglobin level of 6-10 g/dl, with which they thrived well. Fetal hemoglobin was 5-30%. Blood transfusion was not a frequent requirement, but prophylactic long acting penicillin was helpful in preventing frequency of crisis.

MeSH terms

  • Adolescent
  • Antisickling Agents / administration & dosage
  • Child
  • Child, Preschool
  • Female
  • Health Surveys
  • Hemoglobin SC Disease / diagnosis*
  • Hemoglobin SC Disease / drug therapy
  • Hemoglobin SC Disease / epidemiology
  • Humans
  • Incidence
  • India / epidemiology
  • Male
  • Prognosis
  • Sickle Cell Trait / diagnosis*
  • Sickle Cell Trait / drug therapy
  • Sickle Cell Trait / epidemiology
  • Survival Rate
  • Thalassemia / diagnosis*
  • Thalassemia / drug therapy
  • Thalassemia / epidemiology

Substances

  • Antisickling Agents