• Users Online: 710
  • Home
  • Print this page
  • Email this page
Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 
ORIGINAL ARTICLE
Year : 2019  |  Volume : 10  |  Issue : 4  |  Page : 134-140

Double heterozygosity for hemoglobin S and D Punjab in Basra, Iraq: A Clinical and hematological study of 42 patients


1 Department of Laboratory, Basra Children Specialty Hospital, Al-Nahrain Medical College, Baghdad, Iraq
2 Department of Pathology and Forensic Medicine, Al-Nahrain Medical College, Baghdad, Iraq
3 Department of Pediatrics, College of Medicine, University of Basra; Center for Hereditary Blood Diseases, Basra Health Directorate, Basra, Iraq
4 Center for Hereditary Blood Diseases, Basra Health Directorate, Basra, Iraq

Correspondence Address:
Dr. Meaad Kadhum Hassan
Department of Pediatrics, College of Medicine, University of Basra, Basra
Iraq
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/joah.joah_65_19

Rights and Permissions

BACKGROUND: Patients with compound heterozygosity for sickle hemoglobin (HbS) and hemoglobin DPunjab (Hb SDPunjab) may present with a variable clinical course and may be indistinguishable from those with homozygous HbS. OBJECTIVES: The objective was to identify Hb SDPunjab phenotypes and the association of fetal Hb with disease severity. PATIENTS AND METHODS: This descriptive, cross-sectional study included 42 (17 males and 25 females) patients with double heterozygosity for HbS and D. In addition to full clinical data, the complete blood count and Hb quantitation were assessed. Statistical analyses were performed using SPSS software version 20.0. RESULTS: The mean age at diagnosis was 4.6 ± 0.7 years. Pallor and acute painful episodes (>90%) were the most common presenting symptoms, followed by jaundice (76.2%). Six patients (14.3%) had acute splenic sequestration crises and five (11.9%) had cardiac complications. Of the 42 studied patients, 17 (40.5%) had nonsevere disease and 25 (59.5%) had severe disease. Patients with severe disease had statistically significantly more blood transfusions (47.4 ± 10.27) than those with nonsevere disease (17.14 ± 1.82, P < 0.001). No significant association was reported between HbF level and either the number of blood transfusions or acute painful episodes requiring hospitalization/year. Conclusion: Double heterozygosity for Hb S and DPunjab in Basra results in SCD with a severe phenotype, and the Hb F level did not modulate the disease severity.


[FULL TEXT] [PDF]*
Print this article     Email this article
 Next article
 Previous article
 Table of Contents

 Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
 Citation Manager
 Access Statistics
 Reader Comments
 Email Alert *
 Add to My List *
 * Requires registration (Free)
 

 Article Access Statistics
    Viewed79    
    Printed3    
    Emailed0    
    PDF Downloaded23    
    Comments [Add]    

Recommend this journal