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Serum 25(OH)D3 levels and sickle cell crisis related hospital visits in SCD patients. In an evaluation of ~1500 sickle cell disease (SCD) patient medical records over a 4-year period it was observed that SCD patients with severe vitamin D deficiency (<14.1 ng/mL) had on average more than 10 hospital visits/year. SCD patients with documented higher levels of circulating vitamin D (>34 ng/mL) had statistically significant less than two hospital visits/year. # p < 0.05 one-way ANOVA compared to the lowest circulating vitamin D group.
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Sickle cell disease (SCD) is a genetic disorder resulting from the presence of mutated hemoglobin S (HbS). Homozygous carriers will present with early manifestations of painful vaso-occlusive crises. SCD patients have been reported to be severely deficient in vitamin D (<20 ng/mL). Four years (2010–2014) of individual de-identified Sickle Cell Clin...
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Background
Sickle cell disease (SCD) is a genetic disorder of the red blood cells, resulting in multiple acute and chronic complications including pain episodes, stroke, and kidney disease. Patients with SCD develop chronic organ dysfunction, which may progress to organ failure during disease exacerbations. Early detection of acute physiological de...
Citations
... A literatura tem apontado que a mortalidade nessa população pode decorrer por diversos fatores, especialmente, as intensas e frequentes crises dolorosas na agudização da doença, que ocorrem devido a oclusão dos vasos sanguíneos, levando a dor intensa e comprometendo os órgãos vitais, responsáveis por inúmeras internações e óbitos (8,18) . (3,9,13) . ...
Introdução: A Doença Falciforme (DF) é uma condição hereditária causada por uma mutação na hemoglobina S, afetando principalmente os glóbulos vermelhos do sangue. Essa condição pode levar a crises agudas e diversas complicações, aumentando o risco de mortalidade em adultos com a doença, especialmente em situações de insegurança alimentar e nutricional. Objetivo: mapear as causas e manifestações da insegurança alimentar e nutricional entre adultos com doença falciforme. Método: O método seguirá as recomendações propostas pelo manual do Joanna Briggs Institute para revisão de escopo. Serão considerados estudos publicados a partir do ano 2000, sem restrição de idioma, que incluam adultos com idades entre 19 e 64 anos com DF e insegurança alimentar e nutricional (DECS). E excluídos estudos que envolvam pessoas com traço de anemia e talassemia, artigos de opinião, documentos técnicos, revisões integrativas, ensaios teóricos e literatura cinzenta. As fontes de busca abrangem bases de dados como SCIELO, LILACS, MEDLINE, COCHRANE LIBRARY, SCOPUS, ScienceDirect, EMBASE. Dois revisores independentes realizarão a seleção dos artigos, e eventuais discordâncias serão resolvidas por um terceiro revisor. As publicações serão selecionadas pelo Rayyan. Os dados serão extraídos usando um instrumento elaborado pelos revisores e os resultados serão apresentados por meio de tabelas, quadros, fluxogramas e síntese narrativa orientados pelo PRISMA-ScR flow diagram. Conclusão: Este estudo visa contribuir significativamente para o avanço do conhecimento científico na área de Alimentação e Nutrição, ao buscar sistematizar a produção científica mais atualizada sobre as causas e manifestações da insegurança alimentar em adultos com DF. Oferecendo subsídios para a prática clínica e para a formulação de políticas públicas voltadas para uma abordagem do cuidado integral e individualizada no manejo da doença. Ao compreender melhor essas inter-relações, será possível direcionar esforços de forma mais assertiva, visando não apenas a melhoria da qualidade de vida, mas também a prevenção das complicações inerentes à doença.
... En aquellos individuos con ACF y niveles bajos de vitamina D en suero (< 14.1 ng/ml) se ha demostrado que tienen más visitas al hospital relacionadas con las crisis vasooclusivas por año que aquellos con niveles séricos de 25(OH) D3 > 34 ng/ml (McCaskill et al., 2018;Soe et al., 2017). ...
La anemia de células falciformes (ACF) es la forma más frecuente de hemoglobinopatía estructural, de carácter autosómica recesiva, originada por una mutación en el gen Hemoglobina Subunidad Beta (HBB) que codifica para la cadena beta globina (?-globina), como producto de una sustitución en la cadena de un ácido glutámico por valina en la sexta posición, denominada hemoglobina-S (HbS). Tiende a presentarse con mayor frecuencia entre personas cuyo linaje puede vincularse con el África subsahariana, Medio Oriente, el Mediterráneo, la India, el Caribe, América Central y del Sur. Como parte de su fisiopatología, los eritrocitos se observan en forma de hoz, generando una polimerización de las moléculas de hemoglobina, conduciendo a la rigidez e inestabilidad de los hematíes, produciendo manifestaciones multisistémicas tanto agudas como crónicas; consecuentemente se aumenta la susceptibilidad a infecciones, a la aparición de anemia crónica, hemólisis, episodios de oclusión vascular y crisis dolorosas. En la actualidad, no existe cura disponible para la patología. Sin embargo, se ha demostrado que un manejo multidisciplinar adecuado puede contribuir a coadyuvar los síntomas, disminuir la exacerbación de la enfermedad y mejorar la calidad de vida de estos pacientes. Esta revisión tiene como finalidad enfatizar la importancia que posee el soporte nutricional en el tratamiento de la anemia de células falciformes, destacando el papel de la alimentación y suplementación dietética simultáneo a la práctica estándar.
... Total number of hospitalizations was inversely proportional to the vitamin D level (p=0.018). McCaskill et al. (27) also found similar results that vitamin D serum levels are inversely associated with medical record reported hospitalizations visits (p=0.04). ...
Aim:Children with sickle cell disease (SCD) are at high risk of vitamin D deficiency (VDD). The prevalence of VDD in different countries is between 65-100% in these patients. The present study was undertaken to find the prevalence of VDD among sickle cell children and to assess the association of co-morbidities with VDD.Materials and Methods:Total 89 children who were attending a sickle cell clinic/admitted to paediatric wards of a Tertiary Care Centre were enrolled in this study. After their history, clinical examination and anthropometry were investigated, samples were taken for serum 25-hydroxy vitamin D ng/mL and calcium level assessment. The outcome was morbidity in sickle cell children in terms of frequency of pain episodes, number of febrile episodes, number of blood transfusions and total number of admissions.Results:Out of 89 cases, 58 (65.17%) cases were deficient in vitamin D (30 ng/dL). The mean vitamin D level was 19.42ng/dL. Morbidity in SCD was more in VDD children compared to vitamin D sufficient children with significance in the number of pain episodes and the total number of hospital admissions but not in the number of admissions for acute febrile illness or the total number of blood transfusions.Conclusion:VDD was prevalent in 65.17% of children with SCD. Children between 4-12 years were more affected with a male predominance. As this study involved children with SCD alone, future studies need to be carried out involving children without SCD to establish a better possible link between vitamin-D and SCD morbidity.
... [8] Furthermore, pediatric patients with sickle cell anemia are five times at higher risk of vitamin D deficiency compared to the rest of the population. [9] This significant deficiency of vitamin D puts them at high risk of sickle cell anemia, particularly pain. In this case, pain can be attributed to multiple factors, such as vaso-occlusion, and tissue injury. ...
Background: Sickle cell anaemia is a common condition among
... The first study compared a group of patients with SCD who received vitamin D3 supplementation with a group that received placebo for 6 weeks. At the end of the study, the group that received vitamin D supplementation had significantly higher serum vitamin D levels and fewer days during which they experienced pain compared with the placebo group [11,14]. A similar study was conducted in patients with SCD to investigate the effects of vitamin D supplementation in the treatment and placebo groups. ...
Patient: Female, 1-year-old
Final Diagnosis: Sickle cell anaemia
Symptoms: Fracture
Medication: —
Clinical Procedure: —
Specialty: Hematology • Pediatrics and Neonatology
Objective
Rare co-existance of disease or pathology
Background
Bone health is influenced by multiple factors, including genetic disorders such as osteogenesis imperfecta (OI) and sickle cell disease (SCD). OI is a genetic disorder caused by mutations in genes that encode type 1 collagen. Type 1 collagen synthesizes bones, skin, and other connective tissues. Defective synthesis can lead to brittle bones and other abnormalities. Patients with OI present with spontaneous fractures.
SCD is an autosomal-recessive disorder resulting in a major hemolytic anemia. The formation of sickle hemoglobin results in increased blood viscosity and sickling of red blood cells, which causes painful vaso-occlusive crisis in bones and joints, acute chest syndrome, and stroke.
Case Report
We present the case of an infant with a dual diagnosis of OI and SCD. The patient was born at 26 6/7 weeks gestational age to a mother who had sickle trait. The infant was admitted to the Neonatal Intensive Care Unit for prematurity and respiratory distress with a clinical course that was complicated by other comorbidities. Newborn screening revealed a diagnosis of SCD-SS type. At 83 days of life, the infant presented with swelling and tenderness of the left leg. Imaging revealed a non-displaced fracture of the femoral shaft. The patient was evaluated for OI and genetic testing confirmed the diagnosis of OI type 1.
Conclusions
An association between SCD and OI is rare. The impact of these 2 major diagnoses on clinical features and outcome as well as challenges to care remains to be seen.
... The results showed that diets high in fish, milk, cheese, and eggs were associated with reduced hospital/ED visits in individuals with SCD. 71 The study also showed statistically fewer crisis-related hospitalizations for individuals with vitamin D levels >24 ng/ml; among 102 individuals with SCD, mean hospitalizations over 12 months were higher for those with severe vitamin D deficiency (0-19 ng/ml, 9 days) and mild to moderate deficiency (20-29 ng/ml, 5 days) compared with those who were not deficient (>30 ng/ml, <3 days; p < 0.05 versus severe group). 71 Another study found that high-dose vitamin D therapy in children and adolescents with SCD (n = 20) reduced the number of pain days and increased physical activity quality of life compared with patients randomized to receive placebo (n = 19). ...
... 71 The study also showed statistically fewer crisis-related hospitalizations for individuals with vitamin D levels >24 ng/ml; among 102 individuals with SCD, mean hospitalizations over 12 months were higher for those with severe vitamin D deficiency (0-19 ng/ml, 9 days) and mild to moderate deficiency (20-29 ng/ml, 5 days) compared with those who were not deficient (>30 ng/ml, <3 days; p < 0.05 versus severe group). 71 Another study found that high-dose vitamin D therapy in children and adolescents with SCD (n = 20) reduced the number of pain days and increased physical activity quality of life compared with patients randomized to receive placebo (n = 19). 72 Although further study is needed, the results suggest an impact of dietary factors and maintaining adequate vitamin D levels to potentially reduce the occurrence of VOCs in individuals with SCD. ...
Individuals with sickle cell disease (SCD) are living further into adulthood in high-resource countries. However, despite increased quantity of life, recurrent, acute painful episodes cause significant morbidity for affected individuals. These SCD-related painful episodes, also referred to as vaso-occlusive crises (VOCs), have multifactorial causes, and they often occur as a result of multicellular aggregation and vascular adherence of red blood cells, neutrophils, and platelets, leading to recurrent and unpredictable occlusion of the microcirculation. In addition to severe pain, long-term complications of vaso-occlusion may include damage to muscle and/or bone, in addition to vital organs such as the liver, spleen, kidneys, and brain. Severe pain associated with VOCs also has a substantial detrimental impact on quality of life for individuals with SCD, and is associated with increased health care utilization, financial hardship, and impairments in education and vocation attainment. Previous treatments have targeted primarily SCD symptom management, or were broad nontargeted therapies, and include oral or parenteral hydration, analgesics (including opioids), nonsteroidal anti-inflammatory agents, and various other types of nonpharmacologic pain management strategies to treat the pain associated with VOC. With increased understanding of the pathophysiology of VOCs, there are several new potential therapies that specifically target the pathologic process of vaso-occlusion. These new therapies may reduce cell adhesion and inflammation, leading to decreased incidence of VOCs and prevention of end-organ damage. In this review, we consider the benefits and limitations of current treatments to reduce the occurrence of VOCs in individuals with SCD and the potential impact of emerging treatments on future disease management.
... Since vitamin D is involved in the regulation of calcium level and bone health, its deficiency could further impair bone comorbidity and contribute to the musculoskeletal disorders already present in patients with SCD [11,12,16]. A recent study has investigated the association of vitamin D status with inflammatory parameters and anti-inflammatory cytokines in patients with SCD, and showed the contribution of VDD to the uncontrolled, chronic inflammation and concurrent vaso-occlusion seen in the course of SCD [17]. ...
Vitamin D Deficiency (VDD) is reported to be more frequent with serious clinical outcomes in patients with Sickle Cell Disease (SCD). There is a wide disparity in data in the existing literature regarding the prevalence and risk of VDD in patients with SCD. These data require further summary and analyses for better accuracy. This review aimed to assess the association between VDD and SCD, and was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Medline/PubMed, World Health Organization Virtual Health Library, ScienceDirect, and Google Scholar were used for the systematic search. A random effects model was used to estimate the pooled prevalence, Risk Ratio (RR), and Standardized Mean Difference (SMD) estimates with the corresponding 95% Confidence Interval (CI) using OpenMeta Analyst software version 10.10 (Tufts Medical Center, Boston, MA, USA). Twenty-five studies fulfilled the eligibility criteria. The prevalence of VDD among patients with SCD was 63.8% (95% CI, 52.5–75.1). The risk of VDD among patients with SCD was more than two times that of the general population (RR = 2.129; 95% CI, 1.024–4.423; p < 0.001). Serum vitamin D levels were significantly lower in SCD patients than in their controls (SMD = −1.883: 95% CI, −3.006 to −0.760; p < 0.001). This review provides a comprehensive view of the association between vitamin D status and SCD.
... The number of VOC per sickle days and duration of hospitalization per sickle days were significantly decreased for 12 weeks. These results were in accordance with the study done in 2018 by McCaskill et al. who found that SCD patients receiving Vitamin D supplementation had less SCD-related hospitalizations when compared to patients who did not receive the supplementation [17]. In the present study, there was a significant decrease in pain hours among the Vitamin D supplemented group as compared to non-deficient group. ...
Background: Vitamin D status in sickle cell children (SC) has been widely discussed and its effect on clinical profile among SC is currently highly debatable. Objective: The objective of this study was to determine the clinical outcomes in terms of morbidities for Vitamin D supplementation among Vitamin D deficiency (VDD) SC. Materials and Methods: A total of 428 children as per predefined inclusion and exclusion criteria were enrolled in this present prospective study, after receiving written informed parental consent. Patients were supplemented with Vitamin D according to their serum status and followed up at 3 subsequent visits (0, 6, and 12 weeks). All the relevant statistics were done by SPSS v 25.0 (IBM, New York). Results: A total of 428 diagnosed cases of sickle cell anemia were enrolled, from which 272 (63.6%) were male and 156 (36.4%) were female, and 393 cases were found to be VDD (92%). The mean age of the study population was 88.39 (46.27) months. Vitamin D supplementation was significantly associated with decrease in number of vaso-occlusive crisis, duration of hospitalization, and duration of pain hours (p<0.05). Conclusion: Vitamin D supplementation among sickled children with Vitamin D deficiency has a positive effect on its clinical parameters.
... The research team found that SCD patients with severe vitamin D deficiency (<14.1 ng/mL) had on average more than 10 hospital and ED visits per year compared to fewer than 2 visits per year from patients with higher serum 25-OHD levels (>34 ng/mL). 28 Given the findings in adult SCD patients, the goal of the present study is to determine whether a similar relationship between ED visits and vitamin D deficiency were present in a pediatric SCD population. ...
Objectives:
Sickle cell disease (SCD) is the most prevalent inherited hematological disorder and affects 100,000 individuals in the United States. Pain is the most common cause of emergency department (ED) visits in the SCD population, which profoundly affects quality of life. Vitamin D supplementation is a potential target for reducing pain. Thus, the goal of the present study was to identify the prevalence of vitamin D deficiency and explore the relationship between vitamin D supplementation and ED visits in pediatric patients with SCD.
Design:
We conducted a retrospective chart review of 110 patients with SCD aged 8-16 years who had at least one ED visit for SCD pain during the 6-year study period. Patients were categorized into three vitamin D supplementation groups: patients who did not receive supplementation, patients supplemented with 25-hydroxyvitamin D levels (< 30 ng/mL), and patients supplemented with at least one sufficient 25-hydroxyvitamin D level (≥ 30 ng/mL).
Results:
Overall, 45 % of patients were vitamin D deficient. Only 20 % of patients had sufficient vitamin D levels. This number increased to 55 % when examining only patients who did not receive vitamin D supplementation. For patients supplemented with vitamin D, the number of ED visits was significantly lower after they reached the sufficient range (≥ 30 ng/mL), p = 0.03.
Conclusions:
Our findings indicate that reductions in the number of pain-related ED visits may be achieved by normalizing 25-hydroxyvitamin D levels with supplementation. In addition, findings highlight the need for screening and vitamin D supplementation being incorporated into routine care for pediatric patients with SCD.
... Patients with low serum Vitamin D (<14.1 ng/ml) have more crisis-related hospital visits per year than those with 25 (OH)D 3 serum levels >34 ng/ml. Dietary intake of fish correlated with these findings.30Vitamin D also functions to regulate immune ...
