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1.
Sci Rep ; 14(1): 8584, 2024 04 13.
Artigo em Inglês | MEDLINE | ID: mdl-38615021

RESUMO

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.


Assuntos
Síndrome Torácica Aguda , Anemia Falciforme , Adulto , Criança , Feminino , Humanos , Masculino , Adulto Jovem , Causas de Morte , Causalidade , Fatores de Risco , Anemia Falciforme/complicações , Síndrome Torácica Aguda/etiologia , Antibacterianos
2.
Oman Med J ; 34(4): 327-335, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31360322

RESUMO

OBJECTIVES: Many barriers contribute to the underutilization of hydroxyurea (HU) in the treatment of sickle cell disease (SCD), and adherence to its use is often reported to be suboptimal. It is important to have information on the safety of HU in patients with SCD. Our study assessed the pattern of use, patients' adherence to medication, discontinuation of use, and safety of HU in patients with SCD. METHODS: This cross-sectional study was conducted in the department of medicine of a referral hospital in Oman over five months and included a review of patient files and patient interview. Approval was obtained from the Regional Research and Ethics Committee of the A'Dakhiliyah Governorate and the hospital administration. The parameters were compared between groups using the chi-square test. RESULTS: Of 298 patients studied, 128 (43.0%) had used HU at some points. The difference in the prevalence of HU use was statistically significant based only on age (p = 0.014), with younger patients more likely to be currently using HU or used HU in the past. The majority of patients were adherent (82.5%) based on self-reported adherence. The prevalence of discontinuation (temporary or permanent) of HU use was high (57.0%), and suspected adverse drug reaction (ADR) was the most common reason. Among those who had never used HU, 33.7% of patients had an indication for the initiation of HU. A quarter of patients who used HU developed a suspected ADR, with blood abnormalities being the commonest. The duration of HU use influenced ADR prevalence (p = 0.015). CONCLUSIONS: Among the current users of HU, the majority of the patients were adherent based on self-reported medication adherence. The prevalence of discontinuation of HU use and instances of non-initiation of HU among those indicated were high. A larger study, ideally of a prospective nature, in various governorates of Oman, would provide a wider picture at the national level.

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