Rapid Fire

Porter, Michael E.

doi:10.1016/j.emc.2018.04.002

Cited by 9 publications

(5 citation statements)

References 18 publications

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“…Sickle cell anemia affects approximately one in 500 African Americans and one in 36,000 Hispanic Americans [ 3 ]. The problem with sickle cell anemia is that the hemoglobin polymerizes and sickles under conditions such as hypoxia, infection, dehydration, and cold temperatures [ 4 ]. This mechanism results in a VOC, whose complications are Acute Chest Syndromes (ACS), stroke, priapism, and splenic sequestration.…”

Section: Introductionmentioning

confidence: 99%

The Role of Non-genetic Therapies to Reduce the Incidence of Sickle Cell Crisis: A Systematic Review

Pingili,

Makkena,

Jaramillo

et al. 2023

Cureus

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Sickle cell anemia is a hemoglobinopathy that causes complications such as Vaso-Occlusive Crisis (VOC), stroke, priapism, Acute Chest Syndromes (ACS), and bone infarcts due to blood vessel occlusion, resulting in hypoxia, ischemia, and inflammation. Preventing these incidents improves the quality of life and lowers mortality rates in Sickle Cell Disease (SCD) patients. This systematic review aims to describe the drugs, their mechanisms of action, dosages, changes in hemoglobin parameters, decrease in VOCs, delay the time for the next VOC, decrease in the length of hospital stay, and side effects associated with these drugs. This review adheres to the Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) 2020 guidelines. For this review, we searched the PubMed, Google Scholar, and Cochrane databases and screened them for full free texts published in English and studied in humans in the last five years beginning in 2018. Randomized clinical trials (RCT), observational studies, meta-analyses, systemic reviews, and traditional reviews were all included in the search. According to the type of study, quality assessment tools are used, and eight papers are chosen. Full-text articles from these papers are studied, analyzed, and tabulated. We discussed seven interventions that are used to treat sickle cell disease. Voxelotor, crizanlizumab, L-glutamate, long-term blood transfusions, Zinc (Zn), Niprisan®, and Ciklavit* were found to reduce the number and severity of VOC. We discovered that VOCs containing L -glutamate reduced the length of hospitalization. Magnesium (Mg) did not affect the number and severity of VOCs. This review includes a few articles for the study. Future papers on this subject should include a large sample size and many papers. More clinical trials are required to evaluate the dosages and outcomes of using these drugs in combination to prevent VOCs.

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Section: Introductionmentioning

confidence: 99%

The Role of Non-genetic Therapies to Reduce the Incidence of Sickle Cell Crisis: A Systematic Review

Pingili,

Makkena,

Jaramillo

et al. 2023

Cureus

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“…VOC events are unpredictable in people with SCD, and there are no biomarkers that reliably predict VOC onset. While certain triggers (e.g., dehydration, inflammation, physical or emotional stress, cold temperature) had been shown to increase VOC risks, most episodes are not associated with an easily discernable cause 11 . As a result, it is difficult for a provider to effectively utilize and target measures to prevent VOCs.…”

Section: Introductionmentioning

confidence: 99%

“…While certain triggers (e.g., dehydration, inflammation, physical or emotional stress, cold temperature) had been shown to increase VOC risks, most episodes are not associated with an easily discernable cause. 11 As a result, it is difficult for a provider to effectively utilize and target measures to prevent VOCs. Thus, there is substantial interest in the development of prognostic biomarkers capable of stratifying individual patients at steady state based on their risk for developing VOCs.…”

Section: Introductionmentioning

confidence: 99%

Flow adhesion of whole blood to P‐selectin: a prognostic biomarker for vaso‐occlusive crisis in sickle cell disease

et al. 2021

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Blood cell adhesion to P-selectin and vascular cell adhesion molecule-1 (VCAM-1) contributes to the pathophysiology of vaso-occlusion crisis (VOC) events in individuals with sickle cell disease (SCD). We evaluated the use of standardized flow adhesion biomarkers in a six-month, 35-subjects longitudinal study (ELIPSIS). Flow adhesion of whole blood on P-selectin (FA-WB-Psel) and VCAM1 (FA-WB-VCAM), and of isolated white blood cells on P-selectin (FA-WBC-Psel) and VCAM-1 (FA-WBC-VCAM) were elevated on VOC days compared with non-VOC days, but only FA-WB-Psel reached statistical significance (P = 0Á015). Optimal cut-off values were established with Cox regression models for FA-WB-Psel [46 cells/mm²; hazard ratio (HR): 2Á3; 95% confidence interval (CI):1Á4-4Á0; P = 0Á01] and FA-WB-VCAM (408 cells/mm², HR:1Á8; 95% CI: 0Á9-3Á45; P = 0Á01). A combined (FA-WB-Psel and FA-WB-VCAM) multimarker risk score was also significantly (P = 0Á0006) correlated with VOC risk that was two-fold higher for intermediate and 5Á64-fold higher for high score. The concordance (C)index for the multimarker score was 0Á63 in the six-month period (95% CI: 0Á56-0Á70), indicating a better ability to distinguish patient risk of VOC, compared to individual biomarkers FA-WB-VCAM (C-index: 0Á57; 95% CI: 0Á49-0Á65) or FA-WB-Psel (C-index: 0Á58; 95% CI: 0Á53-0Á62). The presented multimarker score can be used to risk-stratify individuals with SCD during their steady state into low, intermediate, and high-risk strata for self-reported VOCs. Such risk stratification could help focus healthcare resources more efficiently to maintiain health, personalize treatment selection to each patient's individual needs, and potentially reduce healthcare costs.

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“…Acute chest syndrome (ACS) is a major cause of pulmonary disease and early mortality in sickle cell disease (SCD) patients, occurring in 15%–43% of all cases. [1234] In Saudi Arabia, the prevalence of ACS in SCD patients varies across regions. [5] Although ACS can occur at any age, children in general are most likely to be affected; worldwide, the annual incidence of ACS among children with SCD is 24.5 cases per 100 patients compared with an annual incidence of 8.8 cases per 100 adult SCD patients.…”

Section: Introductionmentioning

confidence: 99%

“…ACS is characterized by chest pain, shortness of breath, tachypnea, cough, hypoxia and low-grade fever, and has distinctive radiological changes, defined as new pulmonary lobar or multilobar opacities. [1234] The presence of new pulmonary airspace densities in chest X-rays is an essential criterion for the diagnosis of ACS. However, the diagnosis can be missed for several days because of the delayed appearance of some infiltrate(s) on chest X-rays.…”

Section: Introductionmentioning

confidence: 99%

Radiological patterns in sickle cell disease patients with acute chest syndrome: Are there age-related differences?

Al-Sharydah

Alshahrani

Al-Dhaferi

et al. 2019

Saudi J Med Med Sci

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Background: Acute chest syndrome is a major cause of pulmonary disease and mortality in sickle cell disease patients. Its diagnosis can be delayed due to differing imaging patterns between children and adults. Objective: The purpose of this study was to describe the pulmonary and extrapulmonary imaging findings in sickle cell disease patients with acute chest syndrome and determine differences in findings between adult and pediatric patients. Patients and Methods: This retrospective study analyzed the data of all sickle cell disease patients who were admitted with a diagnosis of acute chest syndrome to King Fahd Hospital of the University, Al Khobar, Saudi Arabia, between January and June 2015 ( n = 150). After grouping the patients into adults and pediatrics, the pulmonary and extrapulmonary characteristics were identified and the digital radiography, computed tomography and laboratory findings were compared. Results: A total of 116 patients with 163 acute chest syndrome episodes met the inclusion criteria, of which 69 (60%) were adults. In both adult and pediatric patients, the most frequent pulmonary finding was consolidation of the lung parenchyma. The right lung was most frequently involved: the lower lobe in adult patients and the middle lobe in pediatric patients. In addition, pleural effusion was observed in both age groups. Extrapulmonary radiological findings, such as avascular necrosis and cardiomegaly, were significantly more common in adult patients than in pediatric patients ( P < 0.05). Compared with adults, pediatric patients had significantly lower hemoglobin levels ( P = 0.001) and oxygen tension fraction in arterial blood ( P = 0.007). Conclusions: Pediatric and adult sickle cell disease patients with acute chest syndrome typically exhibited similar pulmonary characteristics, whereas extrapulmonary findings were more prominent in adult patients. Furthermore, low levels of hemoglobin and oxygen tension fraction were dependent predictors of acute chest syndrome.

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Rapid Fire

Cited by 9 publications

References 18 publications

The Role of Non-genetic Therapies to Reduce the Incidence of Sickle Cell Crisis: A Systematic Review

The Role of Non-genetic Therapies to Reduce the Incidence of Sickle Cell Crisis: A Systematic Review

Flow adhesion of whole blood to P‐selectin: a prognostic biomarker for vaso‐occlusive crisis in sickle cell disease

Radiological patterns in sickle cell disease patients with acute chest syndrome: Are there age-related differences?

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