Impact of Race, Socioeconomic Status, and Geography on Healthcare Outcomes for Children With Sickle Cell Disease in the United States: A Scoping Review.

A large proportion of patients with sickle cell disease (SCD) identify as Black or African American (AA). Social bias and stigma in healthcare outcomes for children with SCD are impossible to explore without considering the impact of racial/cultural identity, socioeconomic status (SES), and geography. It is important to understand the current influences of social movements, expanded health insurance coverage, and telehealth on these variables when considering healthcare outcomes for patients with SCD. The objective of this study was to determine the roles of racial identity, SES, and geography in healthcare outcomes for the pediatric population of children with SCD in the United States (US). This study is a scoping review following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The databases utilized included Cochrane, CINHAL, Medline, and Nursing and Allied Health Collection, all accessed through the EBSCO Information Services. Studies met the following inclusion criteria: published in English, pediatric patients residing in the US, and published between 2017 and 2022. Search terms included "sickle cell" AND "pediatric", which were then combined with "minority" OR "racial" OR "rural" OR "urban" OR "poverty" OR "income" OR "socioeconomic status". The initial search yielded 635 unique articles, with 17 articles meeting full inclusion criteria. Overall, it was clear that there are examples of positive effects of race, low SES, and rural geographic location on positive health outcomes, though a large number of studies oscillated between showing negative associations or no association at all. Barriers to care for patients with SCD are multifaceted, making it difficult to isolate and analyze the impact of individual variables. Many studies demonstrated the significance of family, community, and institutional relationships as positive support for patients with SCD. This review highlights the need for additional research on the healthcare outcome benefits of patient/familial support groups aiming to bring together patients who share racial experience and SCD diagnosis regardless of SES and geography.

The efficacy of albumin-globulin ratio to predict prognosis in cancer patients.

The goal of this systematic review was to identify all of the research within the last 10 years that investigated both the Albumin-Globulin Ratio (AGR) and outcomes of solid tumor cancer patients via quantitative prognostic variables. Multiple scientific databases were researched for journal articles that included keywords relating AGR to prognosis. Once isolated from the databases, the articles were de-duplicated and manually screened based on standardized inclusion/exclusion criteria in a blind format via Rayyan. The collective data were sorted by cancer type, corrected for population size, and used to calculate the average cut-off values for the most popular prognostic variables. In total, 18 independent types of cancer have been evaluated to see if AGR is a prognostic indicator based on multivariate analyses. The average cut-off value for AGR in overall survival was 1.356, while the average cut-off value for AGR in progression free survival was 1.292. AGR was found to be significantly associated with at least one prognostic variable in every type of cancer evaluated based on multivariate analyses. The ease of access and affordability of AGR makes it an invaluable tool applicable to nearly all patients. Overall, AGR is a proven prognostic variable that should always be considered in the evaluation of a solid tumor cancer patient's prognosis. Further research needs to be conducted studying the potential prognostic effect in more types of solid tumors.

Enhancement of cyanobacterial PHB production using random chemical mutagenesis with detection through FACS.

Poly-hydroxy-butyrate (PHB) bioplastic resin can be made directly from atmospheric CO2 using cyanobacteria. However, higher PHB productivities are required before large-scale production is economically viable. Random mutagenesis offers a way to create new production strains with increased PHB yields and increased biomass densities without complex technical manipulation associated with genetically modified organisms. This study used staining with lipid fluorescent dye (BODIPY 493/593) and fluorescence-activated cell sorting (FACS) to select high lipid content mutants and followed this with a well plate growth screen. Thirteen mutants were selected for flask cultivation and two strains produced significantly higher PHB yields (29% and 26% higher than wild type), biomass accumulation (36% and 33% higher than wild type) and volumetric PHB density (75% and 67% higher than wild type). The maximum PHB yielding strain (% dcw) was 12.0%, which was 43% higher than the wild type (8.3% in this study). The highest volumetric PHB density was 18.8 mg PHB/L compared to 10.7 mg PHB/L by the wild type. To develop cyanobacterial strain with higher PHB productivities, the combination of random chemical mutagenesis and FACS holds great potential to promote cyanobacteria bioplastic production becoming economically viable.

Outcomes of Modified Harrington Reconstructions for Nonprimary Periacetabular Tumors: An Effective and Inexpensive Technique.

BACKGROUND: Metastatic disease to the acetabulum presents a difficult technical and philosophical challenge: complicated surgeries in patients with often short life expectancies force us to examine both the outcome and cost of these operations. Therefore, we studied the durability of a cement-screw rebar reconstruction technique and risk factors for failure, and we compare the results to other reconstruction options. METHODS: This is a retrospective review of 52 acetabular reconstructions in 50 patients for nonprimary disease using a retrograde screw-rebar-cement all-polyethylene technique. Mean age was 57 years (range 25-81 years). Twenty-four lesions were classified as Harrington class II; 28 were Harrington class III. Mean follow-up was 17.7 months (range 1-92 months). Outcomes included patient survival, prosthesis survival, and complications. RESULTS: Forty-eight of 50 (96 %) patients ambulated after surgery. Five of 52 (9.6 %) of prostheses failed, three from loosening due to tumor progression, one from aseptic loosening, and one from soft tissue instability (dislocation). The three cases of tumor progression failure occurred in patients with massive preoperative ischial tumor burden. Mean surgical time was 198 min, and hospital stay was 5.2 days. DISCUSSION: The screw-cement-rebar all-polyethylene cup reconstruction technique is a comparatively successful and inexpensive reconstruction option for treating nonprimary oncologic disease in the acetabulum. All cases of loosening occurred beyond the median patient survival. Surgeons should be wary of massive ischial tumor burden in patients with projected longevity, as it may be associated with implant failure. Surgical time and hospital stay are consistent with historical data for alternative implants, and implant cost is lower.

Complications of cemented long-stem hip arthroplasty in metastatic bone disease revisited.

BACKGROUND: The literature suggests that a cemented long-stem femoral arthroplasty is associated with increased intraoperative and perioperative risks. Embolic events may precipitate cardiopulmonary complications and even death; by contrast, others have reported that the use of a cemented long-stem femoral arthroplasty in patients with metastatic bone disease is a safe procedure. QUESTIONS/PURPOSES: Specifically, in this study, we sought to identify (1) intraoperative complications potentially attributable to the use of cemented long-stem femoral components, and (2) early postoperative complications potentially attributable to the use of cemented long-stem femoral components in patients having an arthroplasty for metastatic bone disease. METHODS: In this study, we performed a retrospective chart review of 42 patients (44 arthroplasties), in which the same surgical technique was used. The primary outcome measure was perioperative complications, including intraoperative cement-associated desaturation, cement-associated hypotension, sympathomimetic administration, postoperative hypotension/desaturation, and death. RESULTS: In this series, 19% of the patients had cement-associated hypotension and sympathomimetics were administered to 48%. Two patients required prolonged intubation. One death occurred during hospitalization but there were no cardiopulmonary events. CONCLUSIONS: This study showed that some patients experienced postoperative desaturation, prolonged intubation, and increased use of sympathomimetics, however, these events were short-lived and did not result in patient mortality. Although there are significant risks to cemented long-stem femoral arthroplasty, it can be performed with a low risk of fatal cardiopulmonary complications and remains a surgical option when treating patients with metastatic bone disease. LEVEL OF EVIDENCE: Level IV, therapeutic study. See Instructions for Authors for a complete description of levels of evidence.

The clone wars - revenge of the metastatic rogue state: the sarcoma paradigm.

Ewing sarcoma (ES) is the second most common bone tumor affecting primarily adolescents and young adults. Despite recent advances in biological understanding, intensification of chemotherapeutic treatments, and progress in local control with surgery and/or radiation therapy, patients with metastatic or recurrent ES continue to have a dismal prognosis with less than 20% overall survival. All ES is likely metastatic at diagnosis although our methods of detection and classification may not account for this. Progressive disease may arise via a combination of: (1) selection of chemotherapy-resistant clones in primary tumor, (2) signaling from bone or lung microenvironments that may attract tumor cells to distant locations, and/or (3) genetic changes within the ES cells themselves due to DNA-damaging chemotherapeutic agents or other "hits." These possibilities and the evidence base to support them are explored.

Fine-needle aspiration diagnosis of an intraosseous amyloidoma.

Fine-needle aspiration (FNA) is frequently used as the initial diagnostic procedure for the investigation of bone and soft tissue masses. The majority of the lesions detected will represent metastatic carcinoma. Amyloid is a rare cause of a bone mass, with less than 15 published reports describing amyloid deposition within bone. The majority of reported cases involve the vertebral column. We report the finding of a massive amyloidoma of the iliac wing in a 46-year-old man. FNA smears and cell block preparations demonstrated fragments of waxy acellular material misinterpreted as necrotic debris. Subsequent open biopsy established the diagnosis of amyloid with congo red staining demonstrating apple green birefringence. Subsequent workup disclosed the patient to have anemia, hypogammaglobulinemia and trace monoclonal light chain gammopathy. Bone marrow examination revealed CD138a positive lambda restricted plasma cells consistent with plasma cell dyscrasia. Careful attention to the staining characteristics of amyloid in FNA derived material should allow the diagnosis of amyloidoma.

Osteosarcoma in a Marfan patient with a novel premature termination codon in the FBN1 gene.

Osteosarcoma is a malignant neoplasm of mesenchymal origin that is presumed to arise from osteoblasts. Considered a rare tumor, approximately 1000 cases of osteosarcoma are diagnosed in the United States each year, and osteosarcoma of the foot is rarer still. Marfan syndrome (MFS) is a rare genetic disorder that affects 1 in 5000 individuals and is caused by mutations in the fibrillin 1 (FBN1) gene. MFS phenotype affects several body systems, including soft connective tissue and bone. Here we report, for the first time, an individual with MFS that was treated for osteosarcoma. Surgically resected tissue was used to initiate an osteosarcoma cell line (PSU-OS-M) that exhibits attachment-independent growth and loss of contact inhibition in vitro. Genomic DNA was isolated from the tumor cells, and primers that anneal to intronic regions were used to amplify and sequence all 65 coding exons of the FBN1 gene. A two base pair insertion that results in a novel premature termination codon (PTC) was found in exon 52. Protein from the normal allele is detectable in PSU-OS-M cell-conditioned medium, but protein from the mutant allele was not detectable. Immunofluorescent microscopy demonstrates that PSU-OS-M cells can assemble fibrillin 1 microfibrils in culture, and fibronectin assembly is normal. As such, the PSU-OS-M cell line is to our knowledge the first oncogenically transformed cell line with a mutant fibrillin gene and may serve as a useful tool for studying molecular mechanisms of MFS and nonsense-mediated decay.