Multi-center study on mortality in children, and adults with sickle cell anemia-risk factors and causes of death
Abstract 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 manifestati...
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| Format: | Article |
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Nature Portfolio
2024-04-01
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| Online Access: | https://doi.org/10.1038/s41598-024-58328-9 |
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| author | Salam Alkindi Salma Al-Jadidi Safa Al-Adawi Refaat Abdullah Elsadek Ali Al Madhani Maryam Al-Nabhani Anil V. Pathare |
| author_facet | Salam Alkindi Salma Al-Jadidi Safa Al-Adawi Refaat Abdullah Elsadek Ali Al Madhani Maryam Al-Nabhani Anil V. Pathare |
| author_sort | Salam Alkindi |
| collection | DOAJ |
| description | Abstract 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 O2 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 < 0.05, Wilcoxon Signed Ranks test). All patients received antibiotics, whereas, 95.9% and 93.5% received simple blood transfusions, and exchange transfusions respectively, and 66.6% required non-invasive ventilation. Among the causes of death, ACS is seen in 32 (26%), sepsis in 49 (40%), and miscellaneous in 42 (34%). Sudden death was seen in 32 (26%) of patients. Male gender, with low HbF, rapid drop in Hb and platelet, and increased in WBC, LDH, ferritin, and CRP, correlated significantly with mortality in this cohort. |
| first_indexed | 2024-04-24T09:53:15Z |
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| institution | Directory Open Access Journal |
| issn | 2045-2322 |
| language | English |
| last_indexed | 2024-04-24T09:53:15Z |
| publishDate | 2024-04-01 |
| publisher | Nature Portfolio |
| record_format | Article |
| series | Scientific Reports |
| spelling | doaj.art-60ee325b232941d6a225a1372d4a724b2024-04-14T11:15:09ZengNature PortfolioScientific Reports2045-23222024-04-011411910.1038/s41598-024-58328-9Multi-center study on mortality in children, and adults with sickle cell anemia-risk factors and causes of deathSalam Alkindi0Salma Al-Jadidi1Safa Al-Adawi2Refaat Abdullah Elsadek3Ali Al Madhani4Maryam Al-Nabhani5Anil V. Pathare6Department of Haematology, College of Medicine and Health Sciences, Sultan Qaboos UniversityDepartment of Haematology, College of Medicine and Health Sciences, Sultan Qaboos UniversityDepartment of Haematology, College of Medicine and Health Sciences, Sultan Qaboos UniversityDepartment of Medicine, Nizwa HospitalDepartment of Medicine, Sohar HospitalEmergency Department, Royal HospitalDepartment of Haematology, College of Medicine and Health Sciences, Sultan Qaboos UniversityAbstract 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 O2 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 < 0.05, Wilcoxon Signed Ranks test). All patients received antibiotics, whereas, 95.9% and 93.5% received simple blood transfusions, and exchange transfusions respectively, and 66.6% required non-invasive ventilation. Among the causes of death, ACS is seen in 32 (26%), sepsis in 49 (40%), and miscellaneous in 42 (34%). Sudden death was seen in 32 (26%) of patients. Male gender, with low HbF, rapid drop in Hb and platelet, and increased in WBC, LDH, ferritin, and CRP, correlated significantly with mortality in this cohort.https://doi.org/10.1038/s41598-024-58328-9Sickle cell diseasePredictors of mortalityAcute chest syndromeSepsisSudden deathSCD |
| spellingShingle | Salam Alkindi Salma Al-Jadidi Safa Al-Adawi Refaat Abdullah Elsadek Ali Al Madhani Maryam Al-Nabhani Anil V. Pathare Multi-center study on mortality in children, and adults with sickle cell anemia-risk factors and causes of death Scientific Reports Sickle cell disease Predictors of mortality Acute chest syndrome Sepsis Sudden death SCD |
| title | Multi-center study on mortality in children, and adults with sickle cell anemia-risk factors and causes of death |
| title_full | Multi-center study on mortality in children, and adults with sickle cell anemia-risk factors and causes of death |
| title_fullStr | Multi-center study on mortality in children, and adults with sickle cell anemia-risk factors and causes of death |
| title_full_unstemmed | Multi-center study on mortality in children, and adults with sickle cell anemia-risk factors and causes of death |
| title_short | Multi-center study on mortality in children, and adults with sickle cell anemia-risk factors and causes of death |
| title_sort | multi center study on mortality in children and adults with sickle cell anemia risk factors and causes of death |
| topic | Sickle cell disease Predictors of mortality Acute chest syndrome Sepsis Sudden death SCD |
| url | https://doi.org/10.1038/s41598-024-58328-9 |
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