Assessment of hypoxemia among young adults with sickle cell anaemia in steady state in southwestern Nigeria: a crosssectional study.

OBJECTIVES: Hypoxia is a known feature of sickle cell anaemia (SCA) which results from chronic anaemia and recurrent vaso-occlusive crisis (VOC) which can cause tissue ischaemia that leads to an end organ damage. The hallmark of SCA is chronic anaemia and recurrent vaso-occlusive crisis. The aim of this study is to compare the oxygen saturation of sickle cell anaemic individuals with the normal haemoglobin type (Hb AA) control and also to determine the prevalence of hypoxemia among SCA. RESULTS: Two-hundred and twenty-two (136 Hb SS and 86 Hb AA) participated in the study. The mean ± SD of age (years), oxygen saturation (%) and pulse rate (bpm) of participants with sickle cell anaemia and Hb AA control were 21.85 ± 3.04 and 22.14 ± 3.18 (t = 0.701, p = 0.436), 95.21 ± 3.02 and 98.07 ± 0.81 (t=-8.598, p < 0.0001) and 77.10 ± 9.28 and 73.16 ± 8.52 (t = 3.173, p = 0.002) respectively. The prevalence of hypoxemia among SCA participants was 47.1%. Prevalence of hypoxemia in males with SCA was 60.9% while 39.1% of the females had hypoxemia.

Sickle Cell Disease in an Older Adult Population: A Retrospective Review of Health Care Resource Utilization.

Sickle cell disease (SCD) has a history of health inequity, as patients with SCD are primarily Black and often marginalized from the health care system. Although recent health care and treatment advancements have prolonged life expectancy, it may be insufficient to support the complex needs of the growing population of older adults with SCD. This retrospective study used a cohort (N = 812) of Medicare Advantage beneficiaries 45 years and older (ages: 45-54, 55-64, 65-74, 75-89) with SCD to identify associations of SCD-related complications and comorbidities with emergency department (ED) visits, potentially avoidable ED visits, all-cause hospitalization, and potentially avoidable hospitalizations, 2018-2020. The 75-89 age group had lower odds of an ED visit (OR 0.56; 95% CI 0.32-1.00), 65-74 age group had lower odds of an ED visit (OR 0.49; 95% CI 0.31-0.78) and hospitalization (OR 0.50; 95% CI 0.31-0.79), compared with the 45-54 age group. Acute chest syndrome was associated with increased odds of an ED visit (OR 2.02; 95% CI 1.10-3.71), avoidable ED visit (OR 1.87; 95% CI 1.14-3.06), and hospitalization (OR 3.61; 95% CI 2.06-6.31). Pain was associated with increased odds of an ED visit (OR 2.64; 95% CI 1.85-3.76), an avoidable ED visit (OR 3.08; 95% CI 1.90-4.98), hospitalization (OR 1.51; 95% CI 1.02-2.24), and avoidable hospitalization (OR 6.42; 95% CI 1.74-23.74). Older adults with SCD have been living with SCD for decades, often while managing pain crises and complications associated increased incidence of an ED visit and hospitalization. The characteristics and needs of this population must continue to be examined to increase preventative care and reduce costly emergent health care resource utilization.

Comparative pharmacovigilance assessment of adverse events associated with the use of hydroxyurea, L-glutamine, voxelotor, and crizanlizumab in sickle cell disease.

Using disproportionality analysis, this study compared the adverse events (AEs) associated with the use of the new agents (e.g., L-glutamine, voxelotor, and crizanlizumab) to the commonly used medication, hydroxyurea, in sickle cell disease. We found that the most frequent drug-related AEs observed in this real-world study were consistent with those in the HOPE (voxelotor) and SUSTAIN (crizanlizumab) trials, but the rates of AEs were lower. Our study demonstrates that the most common AEs and symptoms of an increased risk associated with the individual drugs varied by treatment. Disproportionate reporting signals of drug-related AEs may also capture information that is independent of subjective measures of patient-reported symptoms. Our study highlights the important need for facilitating patient-physician communication in routine clinical care to understand patient-reported symptoms.

Longitudinal Assessment of Macular Thickness and Microvascular Changes in Children with Sickle Cell Disease.

PURPOSE: To longitudinally assess macular thickness and microvascular changes in children with sickle cell disease (SCD). DESIGN: A retrospective consecutive series. SUBJECTS: Children with SCD aged ≤ 18 years who had an ophthalmic examination at Boston Children's Hospital between January 1998 and August 2022. METHODS: Qualitative and quantitative analyses of both OCT and OCT angiography (OCTA) images were performed. MAIN OUTCOME MEASURES: Total retinal thickness measured on macular OCT, superficial capillary plexus and deep capillary plexus (DCP) vessel density (VD), and foveal avascular zone (FAZ) area measured on 6- × 6-mm OCTA scans. RESULTS: International Classification of Diseases, 10th Revision, code search identified 303 pediatric SCD patients who underwent ophthalmic examination during the study period. OCT and OCTA images were acquired on 104 (17.2%) and 60 (9.9%) eyes at presentation and on 159 (26.2%) and 100 (16.5%) eyes at final visit, respectively. Overall, temporal retinal thinning was noted qualitatively in 35.6% of SCD patients at presentation and 39.6% at final visit. Of those patients with macular thinning, 94.6% and 90.5% had peripheral sickle cell retinopathy (SCR) at presentation and final visit. On quantitative OCT analysis, HbSS eyes had a lower retinal thickness in the fovea and temporal parafovea compared with HbSC (P < 0.05). Eyes with peripheral SCR had a larger FAZ at presentation compared with eyes without peripheral SCR (P = 0.004), a lower DCP VD at final visit in the inferior temporal macula (P = 0.03), and a higher DCP VD at final visit in the superior nasal macula (P = 0.01). Eighty eyes of 40 patients had OCT, and 34 eyes of 20 patients had both OCT and OCTA images acquired at both initial and final visits. At final visit, retinal thickness decreased at the fovea, inferior perifovea, and temporal perifovea compared with presentation (P < 0.05). In parallel, VD DCP in the superonasal quadrant increased at final visit (P = 0.03). CONCLUSIONS: Macular retinal thinning was progressive and observed in eyes with and without peripheral SCR. Over time, there was a compensatory increase in DCP VD in the nasal macula on OCTA. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Assessment of the developmental defects of the enamel in children and adolescents with sickle cell disease: A comparative study.

BACKGROUND: Sickle cell disease (SCD) is the most important hemoglobinopathy in terms of frequency and social impact and can affect the stomatognathic system. AIM: To assess and compare the developmental defects of the enamel (DDE) in children and adolescents with and without SCD. DESIGN: This was a cross-sectional, analytical, and comparative study of 210 children and adolescents aged 5-18 years, who visited the Hematology and Hemotherapy Hospital of Pernambuco. RESULTS: Developmental defects of the enamel were observed in 55.2% of the SCD patients and 35.2% of the non-SCD patients (healthy group; p < .05). In the SCD group, DDE were more common in females than in males (69.1% vs. 40.0%; p < .05). The incidence of DDE in the permanent teeth was higher in the upper arch than in the lower arch (SCD group, 13.1% vs. 4.6%; healthy group, 8.9% vs. 3.6%; p < .05). CONCLUSION: Compared with the healthy group, the SCD patients were almost twice as likely to develop DDE, mostly affecting females and the permanent teeth. These findings suggest that individuals with SCD need early dental care to avoid future oral problems.

Two-Dimensional Ultrasound Assessment of Long-Term Intra-Abdominal Organ Changes in Children with Sickle Cell Anemia during Steady State: A Comparative Study.

BACKGROUND: Sickle cell anemia (SCA) is a hereditary blood disorder with global prevalence, including in Nigeria. Despite advancements in SCA care management, understanding the long-term impact on organs during steady state has remained inconclusive. AIM: This study aimed to investigate the long-term changes in intra-abdominal organs of SCA children compared with non-SCA children during steady state using two-dimensional ultrasound assessment. MATERIALS AND METHODS: A total of 116 children (58 SCA and 58 controls) were enrolled between June 2021 and July 2022. Clinico-demographic data were collected through an interviewer-administered questionnaire. Two-dimensional ultrasound was used to measure the liver, spleen, kidneys, and inferior vena cava in all subjects. Age-matched controls had AA or AS genotypes. RESULTS: Of the 58 patients with SCA, 65.5% were males with an overall mean age of 8.1 ± 3.4 years, while among the non-SCA cohort (n = 58), 48.3% were males with an overall mean age of 8.7 ± 3.9 years. There was no statistically significant difference in the age and gender distribution between the SCA and non-SCA cohorts (P = 0.390 and P = 0.091, respectively). SCA subjects had a larger mean hepatic size than non-SCA subjects (12.09 cm ± 2.23 vs. 11.67 cm ± 1.96; P = 0.276) but smaller mean splenic size (8.01 cm ± 1.89 vs. 8.19 cm ± 1.61; P = 0.577) and inferior vena cava diameter (1.16 cm ± 0.29 vs. 1.25 cm ± 0.33; P = 0.100). Left kidney length and breadth were significantly greater in SCA patients (8.91 ± 1.16 vs. 8.27 ± 1.30; P = 0.006 and 4.15 ± 0.92 vs. 3.79 ± 0.48; P = 0.008, respectively). CONCLUSION: This study highlights the utility of two-dimensional ultrasound assessment in monitoring intra-abdominal organ changes in SCA children, suggesting its cost-effective benefits in monitoring health outcomes in SCA patients.

Stigma of sickle cell disease among Indian tribal population: A multi-centric qualitative study.

BACKGROUND: Sickle Cell Disease (SCD) is the most prevalent hemoglobinopathy, impacting around 5% of the global population. The Indian tribal population, which has been a key focus of the Indian SCD program, can experience health-related stigma due to the multidimensional impact of the disease. This preliminary qualitative inquiry delves into the lived experiences of individuals and synthesizes domains to identify the sources of stigma. METHODOLOGY: The study's framework for developing the stigma tool was rooted in Bronfenbrenner's Ecology of Human Development. The study was implemented in five tribal-dominated districts of India and involved in-depth interviews with sickle cell disease (SCD) patients and their caregivers to explore their stigmatizing experiences. RESULTS: The analysis revealed four overarching themes and several subthemes explaining the type of stigma, its source, and factors contributing to stigmatization. First, the study focused on elements associated with perceived stigma, such as disclosure, self-isolation/refusal to participate, and self-judgment. The second theme pertained to the internalization of stigma. The third theme addressed experienced stigma concerning the disease's impact on day-to-day events, and the fourth theme explored the support system patients needed. The framework highlighted the varying degrees of stigmatizing components within different aspects of patients' ecology. CONCLUSION: Our study highlights the importance of addressing stigma at various levels. Policies, programs, and healthcare interventions must target stigma across these levels. Culturally adaptive tools for identifying stigma, implementing appropriate interventions, and improving healthcare participation are essential for enhancing the quality of life and reducing the disease burden.

Prevalence and cost of sickle cell disease in France: real-world analysis using data from the Echantillon Généraliste des Bénéficiaires.

Sickle cell disease (SCD) is a genetic disorder of the hemoglobin resulting in chronic anemia, hemolysis, and vaso-occlusions. Its treatment mostly relies on hydroxycarbamide, transfusions, and stem cell transplantation. This study aimed at describing the epidemiology and management of SCD in adolescent and adult patients in France. This was a retrospective study performed among SCD patients aged ≥12 years between 2016 and 2018 and controls. SCD patients were matched on a 1:3 ratio with a group of individuals with no diagnosis of SCD, referred as control group. The matching of SCD patients and controls was a direct matching based on age, sex, CMU-c status (which corresponds to free-of-charge complementary coverage for people with low resources) and geographical region of residence. SCD patients and their matched controls were followed-up for the same amount of time by adjusting controls' follow-up period to that of the associated patients. This study used claims data from the French representative 1/97th sample of health data system. The main outcomes were the patients' characteristics and treatments received, healthcare consumptions and related costs among SCD cases and controls. Between 2016 and 2018, 151 patients with ≥6 months of follow-up were identified out of the total population of 732,164 individuals. SCD prevalence extrapolated to the entire population [95% CI] was 19,502 [19,230, 19,778] in 2018. The median (Q1-Q3) age at inclusion date was 37.0 (25.0-48.0) years, with 69.5% of patients being female. The mean (SD) reimbursed cost over follow-up was €24,310 (89,167), mostly represented by hospitalization costs accounting for €21,156 (86,402). A switch in SCD management was observed with age, as younger patients presented more frequent hospitalizations and acute procedures, while older ones had more frequent medical visits and paramedical care. Mean (SD) annual costs were €25,680 (91,843) and vs. €3,227 (23,372) for patients and controls, respectively (p < 0.001), representing an extra cost of almost €150 million over the entire SCD population. This study highlighted the important costs related to SCD and the related medical need with treatment alternatives, which could be filled by the emergence of new therapies.

Pregnancy-related thromboembolism in women with sickle cell disease: An analysis of National Medicaid Data.

Pregnancy and sickle cell disease (SCD) both individually carry a risk of thromboembolism (TE). Pregnancy in people with SCD may further enhance the prothrombotic effect of the underlying disease. The objectives of this study were to determine the rate and risk factors for arterial and venous thrombosis in pregnant people with SCD. Administrative claims data from the United States Centers for Medicare and Medicaid Service Analytic eXtract from 2006 to 2018 were used. The study population included people with SCD from the start of their first identified pregnancy until 1 year postpartum and a control cohort of pregnant people without SCD of similar age and race. Outcomes of interest were identified with ICD-9 or 10 codes. Logistic regression analyses were used to analyze risk factors. We identified infant deliveries in 6388 unique people with SCD and 17 110 controls. A total of 720 venous thromboembolism (11.3%) and 335 arterial TE (5.2%) were observed in people with SCD compared to 202 (1.2%) and 95 (0.6%) in controls. People with SCD had an 8-11 times higher odds of TE compared to controls (p < .001). Within the SCD cohort, age, hemoglobin SS (HbSS) genotype, hypertension, and history of thrombosis were identified as independent risk factors for pregnancy-related TE. Pregnancy-specific factors (pre-eclampsia, eclampsia, multigestational pregnancy) were not associated with TE. In conclusion, the risk of pregnancy-related TE is considerably higher in people with SCD compared with controls without SCD. Hence, people with SCD, particularly those with multiple risk factors may be candidates for thromboprophylaxis during pregnancy and the postpartum period.

Comparison of thromboembolism outcomes in patients with sickle cell disease prescribed hormonal contraception.

Patients with sickle cell disease (SCD) are at a risk of thromboembolism (TE), and use of hormonal contraception can further increase that risk. This study aims to assess patterns of hormonal contraceptive use and compare risk of contraception-related TE between combined hormonal contraceptives (CHCs) and progestin-only contraceptives (POCs). Patients with SCD aged between 12 and 44 years with a new prescription of a hormonal contraceptive in the Centers for Medicare and Medicaid Services Medicaid Analytic eXtract database (2006-2018) were followed up to 1 year. We identified 7173 new users: 44.6% initiated CHC and 55.4% initiated POC. Combined oral contraceptive pills (OCPs; 36.5%) and progestin-only depot medroxyprogesterone acetate (33.9%) were the most frequently prescribed agents. A total of 1.8% of contraception users had a new diagnosis of TE within 1 year of the first identified contraception prescription. There were no significant differences in TE event rates between CHC and POC users (17.2 and 24.7 events per 1000 person-years, respectively). In patients prescribed OCP, there were no differences in TE event rates based on estrogen dose or progestin generation. Transdermal patch had a 2.4-fold increased risk of TE as compared with that of OCP. Although limited by the retrospective study design and use of administrative claims data, this study found no significant differences in TE rates between new users of CHC and POC in patients with SCD. Careful evaluation of underlying TE risk factors should be considered for each patient with SCD before initiation of hormonal contraception.

Recent national trends in outcomes and economic disparities among adult sickle cell disease-related admissions.

Sickle cell disease (SCD) is an inherited disorder caused secondary to a mutation in the hemoglobin beta subunit. There is sparse information regarding the trends in outcomes of SCD admissions in the past decade where rapid advances have been made in treatment. In this study, we wanted to analyze the trends and outcomes of SCD admissions in the United States from 2011 to 2019 and the influence of socio-economic status. Data were obtained from the National Inpatient Sample (NIS) database using the International Classification of Disease (ICD-9) and ICD-10 codes. Trends for primary in-hospital outcomes including mortality, length of stay (LOS), and total hospitalization charges (THC) were assessed. The impact of economic status on these outcomes was also studied. There was an annual percent change (APC) in the number of admissions for SCD of + 2.5% from 2010 to 2015 (95% CI: 1.3-3.8%, p = 0.003). However, there was no significant change in the number of admissions between 2015 and 2019 (95% CI - 1.8-0.7%, p = 0.323). The overall mortality across the years has decreased by about 4% yearly at the population level (p = 0.008, 95% CI 2-8%). However, the inpatient mortality for the high-income group had decreased significantly from 2010 to 2019, whereas there was no difference in the mortality rate for the low-income group across the decade. Despite the advances in the understanding of SCD and its treatment, its benefits have not reached all the people affected. Meaningful progress in healthcare is not achievable unless these economic disparities are addressed. Economic policies to address these inequities are the need of the hour.

Association of Sickle Cell Disease With Racial Disparities and Severe Maternal Morbidities in Black Individuals.

Importance: Little is known about the association between sickle cell disease (SCD) and severe maternal morbidity (SMM). Objective: To examine the association of SCD with racial disparities in SMM and with SMM among Black individuals. Design, Setting, and Participants: This cohort study was a retrospective population-based investigation of individuals with and without SCD in 5 states (California [2008-2018], Michigan [2008-2020], Missouri [2008-2014], Pennsylvania [2008-2014], and South Carolina [2008-2020]) delivering a fetal death or live birth. Data were analyzed between July and December 2022. Exposure: Sickle cell disease identified during the delivery admission by using International Classification of Diseases, Ninth Revision and Tenth Revision codes. Main Outcomes and Measures: The primary outcomes were SMM including and excluding blood transfusions during the delivery hospitalization. Modified Poisson regression was used to estimate risk ratios (RRs) adjusted for birth year, state, insurance type, education, maternal age, Adequacy of Prenatal Care Utilization Index, and obstetric comorbidity index. Results: From a sample of 8 693 616 patients (mean [SD] age, 28.5 [6.1] years), 956 951 were Black individuals (11.0%), of whom 3586 (0.37%) had SCD. Black individuals with SCD vs Black individuals without SCD were more likely to have Medicaid insurance (70.2% vs 64.6%), to have a cesarean delivery (44.6% vs 34.0%), and to reside in South Carolina (25.2% vs 21.5%). Sickle cell disease accounted for 8.9% and for 14.3% of the Black-White disparity in SMM and nontransfusion SMM, respectively. Among Black individuals, SCD complicated 0.37% of the pregnancies but contributed to 4.3% of the SMM cases and to 6.9% of the nontransfusion SMM cases. Among Black individuals with SCD compared with those without, the crude RRs of SMM and nontransfusion SMM during the delivery hospitalization were 11.9 (95% CI, 11.3-12.5) and 19.8 (95% CI, 18.5-21.2), respectively, while the adjusted RRs were 3.8 (95% CI, 3.3-4.5) and 6.5 (95% CI, 5.3-8.0), respectively. The SMM indicators that incurred the highest adjusted RRs included air and thrombotic embolism (4.8; 95% CI, 2.9-7.8), puerperal cerebrovascular disorders (4.7; 95% CI, 3.0-7.4), and blood transfusion (3.7; 95% CI, 3.2-4.3). Conclusions and Relevance: In this retrospective cohort study, SCD was found to be an important contributor to racial disparities in SMM and was associated with an elevated risk of SMM among Black individuals. Efforts from the research community, policy makers, and funding agencies are needed to advance care among individuals with SCD.

Economic and Clinical Burden of Managing Sickle Cell Disease with Recurrent Vaso-Occlusive Crises in the United States.

INTRODUCTION: The aim of this study was to describe the clinical complications, treatment use, healthcare resource utilization (HCRU), and costs among patients with sickle cell disease (SCD) with recurrent vaso-occlusive crises (VOCs) in the US. METHODS: Merative MarketScan Databases were used to identify patients with SCD with recurrent VOCs from March 1, 2010, to March 1, 2019. Inclusion criteria were ≥ 1 inpatient or ≥ 2 outpatient claims for SCD and ≥ 2 VOCs per year in any 2 consecutive years after the first qualifying SCD diagnosis. Individuals without SCD in these databases were used as matched controls. Patients were followed for ≥ 12 months, from their second VOC in the 2nd year (index date) to the earliest of inpatient death, end of continuous enrollment in medical/pharmacy benefits, or March 1, 2020. Outcomes were assessed during follow-up. RESULTS: In total, 3420 patients with SCD with recurrent VOCs and 16,722 matched controls were identified. Patients with SCD with recurrent VOCs had a mean of 5.0 VOCs (standard deviation [SD] = 6.0), 2.7 inpatient admissions (SD 2.9), and 5.0 emergency department visits (SD 8.0) per patient per year during follow-up. Compared to matched controls, patients with SCD with recurrent VOCs incurred higher annual ($67,282 vs. $4134) and lifetime ($3.8 million vs. $229,000 over 50 years) healthcare costs. CONCLUSION: Patients with SCD with recurrent VOCs experience substantial clinical and economic burden driven by inpatient costs and frequent VOCs. There is a major unmet need for treatments that alleviate or eliminate clinical complications, including VOCs, and reduce healthcare costs in this patient population.

Analysis of the 2007-2018 National Health Interview Survey (NHIS): Examining Neurological Complications among Children with Sickle Cell Disease in the United States.

This study compared neurological complications among a national sample of United States children with or without sickle cell disease (SCD) and evaluated health status, healthcare and special education utilization patterns, barriers to care, and association of SCD status and demographics/socioeconomic status (SES) on comorbidities and healthcare utilization. Data was acquired from the National Health Interview Survey (NHIS) Sample Child Core questionnaire 2007-2018 dataset that included 133,542 children. An affirmation from the guardian of the child determined the presence of SCD. Regression analysis was used to compare the associations between SCD and demographics/SES on neurological conditions at p < 0.05. Furthermore, adjusted odds ratios (AORs) were estimated for having various neurological conditions. Of the 133,481 children included in the NHIS, the mean age was 8.5 years (SD: 0.02) and 215 had SCD. Of the children with SCD, the sample composition included male (n = 110), and Black (n = 82%). The SCD sample had higher odds of having neuro-developmental conditions (p < 0.1). Families of Black children (55% weighted) reported household incomes < 100% of federal poverty level. Black children were more likely to experience longer wait times to see the doctor (AOR, 0.3; CI 0.1-1.1). Compared to children without SCD, those with SCD had a greater chance of seeing a medical specialist within 12 months (AOR 2.3; CI 1.5-3.7). This representative sample of US children with SCD shows higher odds of developing neurological complications, increased healthcare and special education services utilization, with Black children experiencing a disproportionate burden. This creates the urgency to address the health burden for children with SCD by implementing interventions in healthcare and increasing education assistance programs to combat neurocognitive impairments, especially among Black children.

Assessment of orthodontic treatment needs in patients with ß-thalassemia major and sickle cell disease.

Patients suffering from hemoglobinopathies may suffer from pathogic dental and orofacial features. This study aimed to assess the prevalence of malocclusion and the need for orthodontic treatment in patients with ß-thalassemia major (BTM) and sickle cell disease (SCD). The study was conducted on 311 blood transfusion-dependent patients with BTM or SCD and 400 healthy individuals aged 10 to 16. The types of malocclusion were evaluated based on Angle's classification and Dewey's modification, and their oral habits were recorded using a questionnaire. The need for orthodontic treatment was assessed through the Dental Health Component of the Index of Orthodontic Treatment Need (IOTN), and the data were compared with normal participants. The Index of Orthodontic Treatment Need-Dental Health Component (IOTN-DHC) assessment showed that patients had a higher prevalence of objective need for treatment (IOTN grades 4 and 5) compared to healthy children. The prevalence of class II malocclusion was significantly higher in patients. Patients showed significantly less Angle's Class I malocclusion compared to normal participants. Oral habits were presented in 61%, 64.15% and 62.4% of normal participants, BTM and SCD patients, respectively. The higher prevalence of Angle's class II malocclusion and higher percentage of IOTN grade 4 and 5 among BTM and SCD patients reveal the importance of early orthodontic assessment and intervention in children with BMT and SDC.

Urinary Biomarkers for the Assessment of Acute Kidney Injury of Pediatric Sickle Cell Anemia Patients Admitted for Severe Vaso-occlusive Crises.

Sickle cell nephropathy is a progressive morbidity, beginning in childhood, which is incompletely understood partially due to insensitive measures. We performed a prospective pilot study of pediatric and young adult patients with sickle cell anemia (SCA) to assess urinary biomarkers during acute pain crises. Four biomarkers were analyzed with elevations potentially suggesting acute kidney injury: (1) neutrophil gelatinase-associated lipocalin (NGAL), (2) kidney injury molecule-1, (3) albumin, and (4) nephrin. Fourteen unique patients were admitted for severe pain crises and were found to be representative of a larger SCA population. Urine samples were collected at the time of admission, during admission, and at follow-up after discharge. Exploratory analyses compared cohort values to the best available population values; individuals were also compared against themselves at various time points. Albumin was found to be moderately elevated for an individual during admission compared with follow-up ( P = 0.006, Hedge g : 0.67). Albumin was not found to be elevated compared with population values. Neutrophil gelatinase-associated lipocalin, kidney injury molecule-1, and nephrin were not found to be significantly elevated compared with population values or comparing admission to follow-up. Though albumin was found to be minimally elevated, further research should focus on alternative markers in efforts to further understand kidney disease in patients with SCA.

Positive rate and quality assessment of CT pulmonary angiography in sickle cell disease: a case‒control study.

BACKGROUND: Pulmonary complications are common in sickle cell disease (SCD) and can mimic pulmonary embolisms (PEs), leading to potential overuse of computed tomography pulmonary angiography (CTPA). Maximizing the quality of CTPA is essential for its diagnostic accuracy. However, little is known about the positive rate and quality of CTPA in SCD. METHODS: This retrospective case‒control study aimed to determine the positive rate and quality of CTPA studies performed to rule out PE in SCD (HbSS genotype) patients compared to a control group. Logistic regression analysis was used to identify independent factors associated with suboptimal CTPA studies, defined as a mean enhancement of < 210 HU in the pulmonary artery. RESULTS: The study included 480 patients, consisting of 240 SCD patients and 240 controls. The positive rate of PE was 4.0%, with a similar rate in both SCD patients and the control group (4.2% vs. 3.8%, p = 0.08). However, SCD patients had significantly lower contrast enhancement of the pulmonary artery than the control group (266.1 ± 90.5 HU vs. 342.2 ± 116.1 HU, p < 0.01). Notably, 25.4% of SCD patients had suboptimal scans. The logistic regression model demonstrated that SCD was significantly associated with suboptimal pulmonary arterial contrast enhancement compared to the control group (OR = 4.4; 95% CI: 2.4-8.3). CONCLUSIONS: This study revealed a relatively low positive rate of CTPA in both SCD patients and the control group. However, SCD was significantly associated with suboptimal image quality due to inadequate contrast enhancement of the pulmonary artery. Further research is needed to identify measures that can enhance the quality of CTPA studies in SCD patients and to establish a specific imaging protocol for this patient population.

A Qualitative Systematic Review of Pediatric Patient and Caregiver Perspectives on Pain Management for Vaso-Occlusive Episodes in the Emergency Department.

OBJECTIVES: The primary objective of this study is to describe the experiences of pediatric patients with sickle cell disease (SCD) and their caregivers who have presented to the emergency department (ED) for management of vaso-occlusive pain events. METHODS: We conducted a qualitative systematic review. The search protocol was developed to identify both published and unpublished literature that met inclusion/exclusion criteria. Included articles were primary hospital-based research with study populations that included (but were not limited to) pediatric patients aged 21 years or younger and qualitative or mixed-method analysis. RESULTS: Four themes were identified: (1) patients and caregivers perceive the ED as the last resort; (2) health care professionals in the ED lacked knowledge about SCD but rejected patients' and caregiver's attempts to share experience or advocate for their needs; (3) patients' accounts of pain are doubted because they do not always have "typical" signs of pain; and (4) caregivers identify racism as a reason for suboptimal care in the ED. CONCLUSIONS: There are multiple opportunities to improve management for vaso-occlusive pain events in the ED, including education of health care providers about SCD and complications, partnership between patients/caregivers and providers, and efforts to reduce the impact of systemic racism on health care delivery.