دورية أكاديمية

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

التفاصيل البيبلوغرافية
العنوان: Multi-center study on mortality in children, and adults with sickle cell anemia-risk factors and causes of death.
المؤلفون: Alkindi S; Department of Haematology, College of Medicine and Health Sciences, Sultan Qaboos University, P. O. Box 35, 123, Muscat, Oman. sskindi@yahoo.com., Al-Jadidi S; Department of Haematology, College of Medicine and Health Sciences, Sultan Qaboos University, P. O. Box 35, 123, Muscat, Oman., Al-Adawi S; Department of Haematology, College of Medicine and Health Sciences, Sultan Qaboos University, P. O. Box 35, 123, Muscat, Oman., Elsadek RA; Department of Medicine, Nizwa Hospital, Nizwa, Oman., Al Madhani A; Department of Medicine, Sohar Hospital, Sohar, Oman., Al-Nabhani M; Emergency Department, Royal Hospital, Muscat, Oman., Pathare AV; Department of Haematology, College of Medicine and Health Sciences, Sultan Qaboos University, P. O. Box 35, 123, Muscat, Oman.
المصدر: Scientific reports [Sci Rep] 2024 Apr 13; Vol. 14 (1), pp. 8584. Date of Electronic Publication: 2024 Apr 13.
نوع المنشور: Multicenter Study; Journal Article
اللغة: English
بيانات الدورية: Publisher: Nature Publishing Group Country of Publication: England NLM ID: 101563288 Publication Model: Electronic Cited Medium: Internet ISSN: 2045-2322 (Electronic) Linking ISSN: 20452322 NLM ISO Abbreviation: Sci Rep Subsets: MEDLINE
أسماء مطبوعة: Original Publication: London : Nature Publishing Group, copyright 2011-
مواضيع طبية MeSH: Acute Chest Syndrome*/etiology , Anemia, Sickle Cell*/complications, Adult ; Female ; Humans ; Male ; Young Adult ; Anti-Bacterial Agents ; Causality ; Cause of Death ; Risk Factors
مستخلص: 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 < 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.
(© 2024. The Author(s).)
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فهرسة مساهمة: Keywords: Acute chest syndrome; Predictors of mortality; SCD; Sepsis; Sickle cell disease; Sudden death
المشرفين على المادة: 0 (Anti-Bacterial Agents)
تواريخ الأحداث: Date Created: 20240413 Date Completed: 20240415 Latest Revision: 20240430
رمز التحديث: 20240501
مُعرف محوري في PubMed: PMC11016086
DOI: 10.1038/s41598-024-58328-9
PMID: 38615021
قاعدة البيانات: MEDLINE
الوصف
تدمد:2045-2322
DOI:10.1038/s41598-024-58328-9