Multi-center study on mortality in children, and adults with sickle cell anemia-risk factors and causes of death

Sickle cell disease (SCD) is a major public health burden worldwide with increasing morbidity and mortality. The study evaluates the risk factors associated with mortality in SCD patients, between the years 2006 and 2020 at three hospitals in Oman. The analysis includes clinical manifestations, haem...

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Bibliographic Details
Published in:Scientific reports 2024-04, Vol.14 (1), p.8584-8584, Article 8584
Main Authors: Alkindi, Salam, Al-Jadidi, Salma, Al-Adawi, Safa, Elsadek, Refaat Abdullah, Al Madhani, Ali, Al-Nabhani, Maryam, Pathare, Anil V.
Format: Article
Language:English
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Acute chest syndrome
Acute Chest Syndrome - etiology
Adult
Anemia, Sickle Cell - complications
Anti-Bacterial Agents
Antibiotics
Blood transfusion
Causality
Cause of Death
Cell death
Chest
Child
Computed tomography
Cough
Female
Ferritin
Hemoglobin
Humanities and Social Sciences
Humans
Male
Morbidity
Mortality
multidisciplinary
Patients
Platelets
Predictors of mortality
Public health
Risk Factors
SCD
Science
Science (multidisciplinary)
Sepsis
Sickle cell disease
Sudden death
VOCs
Volatile organic compounds
Young Adult
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Summary:Sickle cell disease (SCD) is a major public health burden worldwide with increasing morbidity and mortality. The study evaluates the risk factors associated with mortality in SCD patients, between the years 2006 and 2020 at three hospitals in Oman. The analysis includes clinical manifestations, haematological, biochemical, and radiological parameters, use of antibiotics, and blood and exchange transfusions. Our cohort included 123 patients (82 males, 41 females), with a median age of 27 (Interquartile Range 21–35 years). SCD related complications included acute chest syndrome (ACS) in 52.8%, splenic sequestration in 21.1%, right upper quadrant syndrome in 19.5%, more than > 6 VOC/year in 17.9%, and stroke in 13.8%. At the terminal admission, patients had cough, reduced O 2 saturation, crepitation and fever in 24.4%, 49.6%, 53.6% and 68.3% respectively. Abnormal chest X-ray and chest CT scan were seen in 57.7%, and 76.4% respectively. Laboratory parameters showed a significant drop in hemoglobin (Hb) and platelet counts from baseline, with a significant rise in WBC, LDH and CRP from baseline ( p  
ISSN:2045-2322
2045-2322
DOI:10.1038/s41598-024-58328-9