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Hurler–Scheie syndrome (MPS IH/S), also known as mucopolysaccharidosis type I-H/S (MPS IH/S), is a lysosomal storage disorder caused by deficiency of the enzyme alpha-L-iduronidase (IDUA) leading to the accumulation of glycosaminoglycans (GAGs) in various tissues, resulting in a wide range of symptoms affecting different organ systems. Postgenomic omics technologies offer the promise to understand the changes in proteome, phosphoproteome, and phosphorylation-based signaling in MPS IH/S. Accordingly, we report here a large dataset and the proteomic and phosphoproteomic analyses of fibroblasts derived from patients with MPS IH/S (n = 8) and healthy individuals (n = 8). We found that protein levels of key lysosomal enzymes such as cathepsin D, prosaposin, arylsulfatases (arylsulfatase A and arylsulfatase B), and IDUA were downregulated. We identified 16,693 unique phosphopeptides, corresponding to 4,605 proteins, in patients with MPS IH/S. We found that proteins related to the cell cycle, mitotic spindle assembly, apoptosis, and cytoskeletal organization were differentially phosphorylated in MPS IH/S. We identified 12 kinases that were differentially phosphorylated, including hyperphosphorylation of cyclin-dependent kinases 1 and 2, hypophosphorylation of myosin light chain kinase, and calcium/calmodulin-dependent protein kinases. Taken together, the findings of the present study indicate significant alterations in proteins involved in cytoskeletal changes, cellular dysfunction, and apoptosis. These new observations significantly contribute to the current understanding of the pathophysiology of MPS IH/S specifically, and the molecular mechanisms involved in the storage of GAGs in MPS more generally. Further translational clinical omics studies are called for to pave the way for diagnostics and therapeutics innovation for patients with MPS IH/S. [ABSTRACT FROM AUTHOR]

Introduction Military–Civilian Partnerships (MCPs) are vital for maintaining the deployment readiness of military health care physicians. However, tracking their clinical activity has proven to be challenging. In this study, we introduce a locally driven process aimed at the passive collection of external clinical workload data. This process is designed to facilitate an assessment of MCP physicians' deployment readiness and the effectiveness of individual MCPs. Materials and Methods From March 2020 to February 2023, we conducted a series of quality improvement projects at the Wright Patterson Medical Center (WPMC) to enhance our data collection efforts for MCP physicians. Our methodology encompassed several steps. First, we assessed our existing data collection processes and their outcomes to identify improvement areas. Next, we tested various data collection methods, including self-reporting, a web-based smart phone application, and an automated process based on billing or electronic health record data. Following this, we refined our data collection process, incorporating the identified improvements and systematically tracking outcomes. Finally, we evaluated the refined process in 2 different MCPs, with our primary outcome measure being the collection of monthly health care data. Results Our examination at the WPMC initially identified several weaknesses in our established data collection efforts. These included unclear responsibility for data collection within the Medical Group, an inadequate roster of participating MCP physicians, and underutilization of military and community resources for data collection. To address these issues, we implemented revisions to our data collection process. These revisions included establishing clear responsibility for data collection through the Office of Military–Civilian Partnerships, introducing a regular "roll call" to match physicians to MCP agreements, passively collecting data each month through civilian partner billing or information technology offices, and integrating Office of Military–Civilian Partnership efforts into regular executive committee meetings. As a result, we observed a 4-fold increase in monthly data capture at WPMC, with similar gains when the refined process was implemented at an Air Force Center for the Sustainment of Trauma and Readiness Skills site. Conclusions The Military–Civilian Partnership Quality Improvement Program concept is an effective, locally driven process for enhancing the capture of external clinical workload data for military providers engaged in MCPs. Further examination of the Military–Civilian Partnership Quality Improvement Program process is needed at other institutions to validate its effectiveness and build a community of MCP champions. [ABSTRACT FROM AUTHOR]

Background: Mucopolysaccharidosis (MPS) comprises a group of metabolic diseases with a disorder in the function of lysosomal enzymes that lead to the accumulation of glycosaminoglycans (mucopolysaccharides) (Kubaski et al. in Diagnostics 10:161, 2020; Hampe et al. in Cells 9:1838, 2020; Tomatsu et al. Mol Genet Metab 110(1–2):42–53, 2013). At least seven variants of this disorder have been identified to date (Muenzer et al. in Pediatrics 124(6):e1228–e1239, 2009; Muenzer et al. in Eur J Pediatr 171:181–8, 2012). this study aims to report a case of mucopolysaccharidosis in a 9-year-old child. Also, the treatments and dental observations made for the child have been described. Also, a review of past articles has been done to report the types of diseases, medical and dental considerations, etc. of this disease. Case description: the present case report describes the orofacial and systemic characteristics, diagnostic methods, and dental management of a 9-year-old boy with MPS with a one-year follow-up in association with a brief review of past articles. Conclusion: Since MPS patients have many changes in their oral and dental structures, they pose many challenges for dentists. Also, these patients need considerations in dentistry due to the involvement of different body organs. Knowing the oral-dental and systemic problems of these patients can help their dentists to provide effective and safe treatment for them. [ABSTRACT FROM AUTHOR]

Introduction While previous studies have analyzed military surgeon experience within military–civilian partnerships (MCPs), there has never been an assessment of how well military providers are integrated within an MCP. The Center for Sustainment of Trauma and Readiness Skills, Cincinnati supports the Critical Care Air Transport Advanced Course and maintains the clinical skills of its staff by embedding them within the University of Cincinnati Medical Center. We hypothesized that military trauma surgeons are well integrated within University of Cincinnati Medical Center and that they are exposed to a similar range of complex surgical pathophysiology as their civilian partners. Materials and Methods After Institutional Review Board approval, Current Procedural Terminology (CPT) codes were abstracted from billing data for trauma surgeons covering University of Cincinnati Hospitals in 2019. The number of trauma resuscitations and patient acuity metrics were abstracted from the Trauma Registry and surgeon Knowledge, Skills, and Abilities clinical activity (KSA-CA) scores were calculated using their CPT codes. Finally, surgeon case distributions were studied by sorting their CPT codes into 23 categories based on procedure type and anatomic location. Appropriate, chi-squared or Mann–Whitney U-tests were used to compare these metrics between the military and civilian surgeon groups and the metrics were normalized by the group's full-time equivalent (FTE) to adjust for varying weeks on service between groups. Results Data were available for two active duty military and nine civilian staff. The FTEs were significantly lower in the military group: military 0.583-0.583 (median 0.583) vs. civilian 0.625-1.165 (median 1.0), P  = 0.04. Per median FTE and surgeon number, both groups performed a similar number of trauma resuscitations (civilian 214 ± 54 vs. military 280 ± 13, P  = 0.146) and KSA-CA points (civilian 55,629 ± 25,104 vs. military 36,286 ± 11,267; P  = 0.582). Although the civilian surgeons had a higher proportion of hernia repairs (P  < 0.001) and laparoscopic procedures (P  = 0.006), the CPT code categories most relevant to combat surgery (those relating to solid organ, hollow viscus, cardiac, thoracic, abdominal, and tissue debridement procedures) were similar between the surgeon groups. Finally, patient acuity metrics were similar between groups. Conclusion This is the first assessment of U.S. Air Force trauma surgeon integration relative to their civilian partners within an MCP. Normalized by FTE, there was no difference between the two groups' trauma experience to include patient acuity metrics and KSA-CA scores. The proportion of CPT codes that was most relevant to expeditionary surgery was similar between the military and civilian partners, thus optimizing the surgical experience for the military trauma surgeons within University of Cincinnati Medical Center. The methods used within this pilot study can be generalized to any American College of Surgeons verified Trauma Center MCP, as standard databases were used. [ABSTRACT FROM AUTHOR]

Abstract Background Mucopolysaccharidosis (MPS) comprises a group of metabolic diseases with a disorder in the function of lysosomal enzymes that lead to the accumulation of glycosaminoglycans (mucopolysaccharides) (Kubaski et al. in Diagnostics 10:161, 2020; Hampe et al. in Cells 9:1838, 2020; Tomatsu et al. Mol Genet Metab 110(1–2):42–53, 2013). At least seven variants of this disorder have been identified to date (Muenzer et al. in Pediatrics 124(6):e1228–e1239, 2009; Muenzer et al. in Eur J Pediatr 171:181–8, 2012). this study aims to report a case of mucopolysaccharidosis in a 9-year-old child. Also, the treatments and dental observations made for the child have been described. Also, a review of past articles has been done to report the types of diseases, medical and dental considerations, etc. of this disease. Case description the present case report describes the orofacial and systemic characteristics, diagnostic methods, and dental management of a 9-year-old boy with MPS with a one-year follow-up in association with a brief review of past articles. Conclusion Since MPS patients have many changes in their oral and dental structures, they pose many challenges for dentists. Also, these patients need considerations in dentistry due to the involvement of different body organs. Knowing the oral-dental and systemic problems of these patients can help their dentists to provide effective and safe treatment for them.