Correction Of Defects Research Articles

Integrating Quality at Source into Supplier Management: A Pathway to Cost Efficiency and Regulatory Compliance

In an increasingly competitive global market, organizations are seeking to enhance both cost efficiency and regulatory compliance in their supply chains. This paper explores the integration of Quality at Source (Q@S) into supplier management as a strategic pathway to achieve these goals. Quality at Source is a proactive approach that emphasizes defect prevention and quality assurance at the earliest stages of production, specifically at the supplier level. By embedding Q@S principles within supplier management, companies can not only reduce the financial burden associated with defect detection and correction but also mitigate risks associated with non-compliance with industry regulations. The study identifies key benefits of Q@S, including cost savings through reduced waste, improved production efficiency, and minimized need for extensive downstream quality control. Additionally, it demonstrates how Q@S facilitates regulatory compliance by ensuring that quality standards are met consistently, thus simplifying audits and reducing the likelihood of penalties. The paper details a framework for Q@S implementation, covering essential steps such as supplier selection, establishing quality metrics, conducting regular audits, and fostering a culture of continuous improvement among suppliers. Through a combination of case studies and data-driven insights, the paper illustrates the tangible benefits of Q@S integration. Real-world examples showcase successful applications of Q@S across different industries, highlighting significant cost reductions and enhanced compliance. Furthermore, this research discusses the challenges companies may face, such as initial investment costs and resistance to change from suppliers, and provides strategies for overcoming these barriers. The paper concludes by examining the role of advanced digital technologies—such as artificial intelligence (AI), Internet of Things (IoT), and blockchain—in furthering Q@S initiatives. These technologies enable real-time monitoring and data transparency, enhancing the capacity of organizations to track supplier performance and maintain quality standards. The findings underscore the importance of Quality at Source as an essential component of modern supplier management, providing a roadmap for organizations aiming to achieve cost-effective, compliant, and sustainable supply chains.

VSD Surgical Closure in Colombia in Children with Secondary Pulmonary Hypertension. Does Altitude Influence Postoperative Pulmonary Pressure?

A retrospective, cross-sectional, descriptive, observational study was carried out to describe the history of pulmonary hypertension in pediatric patients living at different altitudes following surgical correction of ventricular septal defect. Data from 40 patients who underwent surgery in La Fundacion Cardioinfantil was collected and used for our analysis. Bivariate analysis showed no significant relationship between altitude and pulmonary hypertension after ventricular septal defect closure. Unrelated to the main objective of our study, our investigation revealed that our population underwent surgical correction of VSD at older ages than expected. While previous publications demonstrate the benefit of intervention at 4years of age or younger (19, 20), the average age in our studied population was found to be 7.8years old. These patients had unfavorable hemodynamic parameters for ventricular septal defect closure, but our study showed that our patients benefited from surgery with an immediate satisfactory postoperative result. Patients transitioned from parameters indicating severe PH to mild PH within the first 24-48h after surgery.

Relative Factor Analysis of Postoperative Complications in Infants Below 1 Year Undergoing Repair of Ventricular Septal Defect Combined with Other Procedures through a Right Axillary Incision

Background: Compared to the traditional surgical repair of isolated ventricular septal defects, the proportion of complex congenital heart diseases has been increasing due to advancements in diagnostic capabilities and surgical techniques. Concomitant surgeries to correct multiple congenital heart diseases have become the preferred choice. Therefore, the evaluation of the safety of the combined correction of ventricular septal defects with other cardiac anomalies in young children through a right axillary incision and the analysis of related factors of perioperative complications needs to be investigated. Methods: A retrospective study was conducted on a single-center patient sample from January 2018 to December 2022 to compare outcomes between infants undergoing isolated ventricular septal defect (VSD) repair via a right axillary thoracotomy (control group) and those undergoing VSD repair combined with other cardiac procedures (experimental group). Results: A total of 397 eligible infants were included. After baseline data were matched using propensity score matching, the experimental group (n = 181) and the control group (n = 107) showed no significant differences in intraoperative blood loss, postoperative total drainage, length of hospital stay, duration of ICU monitoring, Vasoactive-Inotropic Score (VIS), pre-discharge left ventricular ejection fraction, fractional shortening (FS), postoperative severe pulmonary infection, reoperation, mortality, postoperative atrioventricular block, chylothorax, cardiac arrest, pericardial effusion, pneumothorax, pulmonary atelectasis, tricuspid regurgitation, myocardial injury, abdominal effusion, and metabolic acidosis. However, the control group had shorter surgical time [28.00 (90.00, 196.00) vs. 140.00 (95.00, 658.00) min, p < 0.05], shorter cardiopulmonary bypass time [58.00 (24.00, 114.00) vs. 66.00 (33.00, 422.00) min, p < 0.05], shorter aortic cross-clamp time [32.00 (17.00, 62.00) vs. 37.00 (15.00, 142.00) min, p < 0.05], and shorter postoperative mechanical ventilation time [22.00 (0, 99.00) vs. 23.00 (3.00, 193.00) h, p < 0.05]. The rate of abdominal effusion was lower in the experimental group than in the control group (p = 0.044). In the analysis of postoperative complication-related factors, the preoperative grade of tricuspid regurgitation, postoperative mechanical ventilation time, and VIS were positively correlated with abdominal effusion and satisfied the variable (p < 0.05) in each of three established models. In the single-factor analysis, both VIS and preoperative lymphocyte count were (p < 0.1) related to pericardial effusion; however, in the multi-factor analysis, only VIS met the threshold (p < 0.05). Gender, height, and VIS were each associated with pleural effusion (p < 0.05). Conclusion: The combination of ventricular septal defect repair with other cardiac procedures via a right axillary thoracotomy did not significantly differ from isolated VSD repair in terms of postoperative complications, monitoring, and length of hospital stay. However, increased surgical complexity was associated with longer surgical and mechanical ventilation times. Factors such as tricuspid regurgitation severity, duration of postoperative mechanical ventilation, and VIS were identified as relative factors for postoperative effusion. Additionally, the risk of postoperative pericardial effusion increased with higher VIS, while gender, height, and VIS were identified as relative factors for postoperative chylothorax.

Neuroimaging Applications for the Delivery and Monitoring of Gene Therapy for Central Nervous System Diseases.

Neurological disease due to single-gene defects represents a targetable entity for adeno-associated virus (AAV)-mediated gene therapy. The delivery of AAV-mediated gene therapy to the brain is challenging, owing to the presence of the blood-brain barrier. Techniques in gene transfer, such as convection-enhanced intraparenchymal delivery and image-guided delivery to the cerebrospinal fluid spaces of the brain, have led the field into highly accurate delivery techniques, which provide correction of genetic defects in specific brain regions or more broadly. These techniques commonly use magnetic resonance imaging (MRI), computed tomography, and fluoroscopic guidance. Even more, the neuroimaging changes evaluated by MRI, MR spectroscopy, diffusion tensor imaging, and functional MRI can serve as important biomarkers of therapy effect and overall disease progression. Here, we discuss the role of neuroimaging in delivering AAV vectors and monitoring the effect of gene therapy.

Delayed Onset Perinasal Mucocele Cyst in a Patient With Remote History of Orofacial Cleft Repair.

Nasoalveolar cysts are rare, nonodontogenic cysts with a subtle course that can lead to nasal deformity and, uncommonly, nasal airway obstruction. Herein the authors present the case of a 28-year-old male who presented with a recurrent left-sided nasoalveolar cyst. Upon presentation, this cyst had formed a significant alar defect and was obstructing the left nasal airway. Although nasoalveolar cysts have been well-described in literature, the patient represents an atypical case in that nasoalveolar cysts most commonly occur in females and in patients aged 40 to 60 years old. The patient's history of extensive facial reconstruction for cleft palate and lip is also anomalous and suggests an inclusion cyst rather than a cyst of typical epithelial origin. The complex course of this cyst and the history of this patient, which led to treatment with a subdermal dissection, septoplasty and turbinate reduction, and alar defect correction make this case of particular note.

Virtual Reality for Preprocedure Planning of Covered Stent Correction of Superior Sinus Venosus Atrial Septal Defects.

Covered stent correction (CSC) of a superior sinus venosus atrial septal defect is an alternative to surgery in selected patients, but anatomic variation means that assessment for CSC requires a 3-dimensional anatomic understanding. Heart VR is a virtual reality (VR) system that rapidly displays and renders multimodality imaging without prior image segmentation. The aim of this study was to evaluate the performance of the Heart VR system to assess patient suitability for CSC. In a blinded fashion, 2 interventionalists reviewed preprocedural computed tomography scans using Heart VR to assess the feasibility of CSC, including the potential need for pulmonary vein protection. The total review time using VR was recorded. Using conventional imaging, 15 patients were deemed suitable for CSC, but at catheterization, 3 cases were unsuitable. Using VR, when both interventionalists agreed that a case was suitable for CSC (n=12), all proved technically feasible. In the 3 cases that were unsuitable for CSC, the interventionalists using VR were either uncertain (n=1) or did not agree on suitability (n=2). The strategy for pulmonary vein protection was correctly identified by interventionalist 1 and 2 in 9/12 and 8/12 cases, respectively. In cases where pulmonary vein protection was required intraprocedurally (n=5), this was correctly identified using Heart VR. Using VR, in 3 cases it was determined that pulmonary vein protection would be required, but this was not the case on balloon interrogation. VR data loading and review times were 82 seconds and 7 minutes, respectively. Verbal feedback indicated that Heart VR assisted in the assessment of case suitability. Heart VR is a rapid and effective tool for predicting suitability for CSC in patients with a superior sinus venosus atrial septal defect and could be a feasible alternative to segmented virtual or physical 3-dimensional models.

Case Report: Correction of Extensive Osteochondral Defect with Structured Bone Graft Associated with Collagen Membrane Coating in Knee Due to Sequelae of Osteochondritis Dissecans

Objectives Case report of the treatment of a 35-year-old woman with sequelae of an extensive osteochondral lesion in the Medial Femoral Condyle of the Right Knee. This report presents the clinical and radiological results after using the structured cancellous bone grafting technique from the Iliac bone with the aid of instruments for mosaicplasty associated with coverage with Collagen Membrane (Chondro-Gide®). Methods We removed the entire remaining osteochondral fragment down to the healthy cancellous bone and made perforations with the Mosaicplasty instrument designed to remove the recipient area to receive the cancellous bone plugs. We inserted the cancellous bone plugs into the defect of the Medial Femoral Condyle so that they filled the entire defect, remaining stable through the press-fit. After fixing and modeling the cancellous bone graft, the bone was covered with a collagen membrane (Chondro-Gide®). Results 12 months after surgery, the patient performed weight training without complaints, returned to all previous usual activities and had a range of motion of 0-120˚. 18 months post-operatively, the IKDC score went from 5 pre-operatively to 52 out of a possible 87 (59.8%). Magnetic Resonance Imaging was performed 12 months after surgery for follow-up evaluation and the images showed the filling of the bone defect and the formation of fibrous scar tissue. Conclusion The presented technique proved to be a satisfactory option available in our country for the treatment of extensive osteochondral lesions with significant involvement of the subchondral bone when allografting is impossible. The presented patient is in good clinical function after an 18-month follow-up, with no recurrence of the preoperative clinical condition.

Corrigendum to “Improvements to a class of hybrid methods for radiation transport: Nyström reconstruction and defect correction methods”

Corrigendum to “Improvements to a class of hybrid methods for radiation transport: Nyström reconstruction and defect correction methods”

Инфузия субанестетических доз кетамина в послеоперационном периоде как средство церебропротекции у детей при хирургической коррекции врожденных пороков сердца: проспективное рандомизированное исследование

INTRODUCTION: The world literature describes the cerebroprotective properties of ketamine when used in minimal doses, which is due to its effect on NMDA receptors and inhibition of damage to the neurovascular unit. However, there is a lack of research on the use of ketamine for cerebroprotection in pediatric populations and cardiac surgery. OBJECTIVES: To establish the safety and effectiveness of ketamine infusion for cerebroprotection in children in the postoperative period during cardiac surgery. MATERIAL AND METHODS: The study included 45 children aged from 1 to 60 months and weighing from 3.5 to 20 kg, who underwent surgical correction of atrial or ventricular septal defect of the heart under artificial circulation. Patients were randomized into a study group or a control group. In the study group, upon completion, patients received a ketamine infusion at a dose of 0.1 mg/kg/hour for 16 h. To establish the effectiveness of cerebroprotection, serum markers of damage to the neurovascular unit were used: protein S100-β, neuron-specific enolase (NSE), glial fibrillary acidic protein (GFAP), occludin, claudin-1. Blood sampling for marker analysis was carried out at three control points: before the start of the operation, immediately after completion of artificial circulation and 16 hours after completion of the operation. RESULTS: Al studied factors in the intra- and postoperative period did not differ significantly between groups. When analyzing markers of damage to a neurovascular unit, their maximum concentration was revealed at the second control point. For the NSE marker, a lower level was found in the group using ketamine — 16.52 [11.61–18.8] and 21.37 [17.78–28.74] ng/ml (p = 0.0019). CONCLUSIONS: The safety of using ketamine infusion in the postoperative period at a dose of 0.1 mg/kg/hour was revealed. Among all markers used, NSE serum concentrations were statistically significantly lower in the ketamine group.

Long-term lineage commitment in haematopoietic stem cell gene therapy

Haematopoietic stem cell (HSC) gene therapy (GT) may provide lifelong reconstitution of the haematopoietic system with gene-corrected cells1. However, the effects of underlying genetic diseases, replication stress and ageing on haematopoietic reconstitution and lineage specification remain unclear. In this study, we analysed haematopoietic reconstitution in 53 patients treated with lentiviral-HSC-GT for diverse conditions such as metachromatic leukodystrophy2,3 (MLD), Wiskott–Aldrich syndrome4,5 (WAS) and β-thalassaemia6 (β-Thal) over a follow-up period of up to 8 years, using vector integration sites as markers of clonal identity. We found that long-term haematopoietic reconstitution was supported by 770 to 35,000 active HSCs. Whereas 50% of transplanted clones demonstrated multi-lineage potential across all conditions, the remaining clones showed a disease-specific preferential lineage output and long-term commitment: myeloid for MLD, lymphoid for WAS and erythroid for β-Thal, particularly in adult patients. Our results indicate that HSC clonogenic activity, lineage output, long-term lineage commitment and rates of somatic mutations are influenced by the underlying disease, patient age at the time of therapy, the extent of genetic defect correction and the haematopoietic stress imposed by the inherited disease. This suggests that HSCs adapt to the pathological condition during haematopoietic reconstitution.

Molecular Phenotypes Segregate Missense Mutations in SLC13A5 Epilepsy

The sodium-coupled citrate transporter (NaCT, SLC13A5) mediates citrate uptake across the plasma membrane via an inward Na+ gradient. Mutations in SLC13A5 cause early infantile epileptic encephalopathy type-25 (EIEE25, SLC13A5 Epilepsy) due to impaired citrate uptake in neurons and astrocytes. Despite clinical identification of disease-causing mutations, underlying mechanisms and cures remain elusive. Here we mechanistically classify six frequent SLC13A5 mutations by phenotyping their protein cell surface expression and citrate transport functions. Mutants C50R, T142M, and T227M exhibit impaired citrate transport despite normal expression at the cell surface. In contrast, mutations G219R, S427L, and L488P show low total protein expression levels, absence of mature, glycosylated proteins at the cell surface, retention of the proteins in the endoplasmic reticulum, and diminished transport activity. This mechanistic classification divides SLC13A5 mutants into two groups, Class I (C50R, T142M, and T227M) and Class II (G219R, S427L, and L488P). Importantly, mutants’ mRNA levels resemble wildtype, suggesting post-translational defects. Class II mutations display immature core-glycosylation and shortened half-lives, indicating protein folding defects. Together, these experiments provide a comprehensive understanding of the disease-causing mutation’s defects in SLC13A5 Epilepsy at the biochemical and molecular level and shed light into the trafficking pathway(s) of NaCT. The two classes of mutations will require fundamentally different approaches for treatment to either restore transport function of the mutant protein that is capable of reaching the cell surface (Class I), or therapies that enable the correction of protein folding defects to enable escape to the cell surface where it may restore transport function (Class II).

Anesthetic management of laparoscopically assisted fetoscopic surgery to correct spina bifida

Introduction. The growing incidence of intrauterine pathology in the fetus at the present stage dictates the need to develop and improve intrauterine surgical correction of defects in the fetus. Myelomeningocele (SPINA BIFIDA) is the most common and severe neural tube defect in the fetus, the severity of the neurological deficit is determined by the level of location and extent of the anomaly. By uniting doctors of various specialties (obstetricians-gynecologists, neonatologists, surgeons, anesthesiologists-resuscitators), the Clinic of the St. Petersburg State Pediatric Medical University effectively introduces methods of prenatal surgery.The objective was to demonstrate by clinical example the experience of successful fetoscopic correction of myeloneningocele to reduce maternal risks and injuries, to reduce risks aimed at protecting the tissues of the spinal cord of the fetus, as well as to reduce the need for postpartum correction of the defect in the fetus, to improve prognosis and risks of subsequent disability.Materials and methods. The article presents the first successful experience of minimally invasive laparoscopic correction of myelomeningocele in the fetus with the participation of the multidisciplinary team and the features of anesthesiological support of the method.Conclusion. Laparoscopic access, together with the optimal choice of anesthetic management in fetal surgery, allows not only to successfully correct the defect in myelomeningocele in the fetus, but also to significantly minimize and reduce the risks of injury to the mother, and also reduces the need for postpartum surgical treatment and reduce disability. Accumulation and improvement of experience, a multidisciplinary approach is the key to successful correction of intrauterine fetal pathology.

Cerebroprotective properties of nitric oxide in children in cardiac surgery (literature review)

Background. Cardiac surgery in conditions of cardiopulmonary bypass (CPB) in children with congenital heart defects is characterized by a complex of damaging factors (initial immaturity of organs and systems, the fact of non-physiology of cardiopulmonary bypass, frequent use of perfusion and non-perfusion hypothermia, comorbid infection), which dictates the validity of using a number of strategies aimed at protecting vital organs and the brain above all.The objective was to study the effectiveness of the use of nitric oxide to protect the brain in children during surgical correction of congenital heart defects in cardiopulmonary bypass conditions.Materials and methods. A literary search was performed in domestic and international bibliographic databases for keywords: nitric oxide, cerebroprotection, cardiopulmonary bypass, organoprotection, inhalation of nitric oxide, children.Results. The review provides up-to-date information on the effect of nitric oxide on the components of the neurovascular unit: angiogenesis, proliferation and myelination of nerve cells, the role in neuroinflammation and deep hypothermic circulatory arrest.Conclusion. The results of many studies confirm the effectiveness of nitric oxide for neuroprotection. However, there is a deficit of clinical researches in general and in the pediatric patient population, which does not yet allow to definitely state its effectiveness.

The accordion technique did not improve bone healing in a mouse model of distraction osteogenesis.

Distraction osteogenesis (DO) is a valuable surgical method for limb lengthening and bone defect correction, but its lengthy consolidation phase presents challenges. The accordion technique (AT), involving compression and distraction of bone segments, has shown potential for enhancing healing. This study aimed to investigate the effectiveness of the AT conducted at three different time points (distraction phase, early consolidation phase, or late consolidation phase) compared to conventional DO in a mouse osteotomy model. Healing was evaluated using in vivo microCT, histology, and computational modeling. Results showed that bridging frequency, BV, and callus tissue composition were similar between conventional DO and late consolidation AT. In contrast, distraction phase AT led to delayed healing at day 15 with a 72% reduction in BV compared to DO, but no significant differences by the endpoint. Early consolidation AT showed significantly impaired healing compared to DO, with only 29% of mice achieving bony bridging, and significantly reduced bone marrow area of the endpoint callus. In silico modeling was generally predictive of in vivo findings and suggested that application of the AT during early consolidation results in destruction of newly-formed vascular tissue. Overall, no benefit was observed for the AT compared to conventional DO with the parameters employed in this study.

Navicular Drop Height Asymmetry as an Intrinsic Risk Factor for Lower Limb Injury in Male Recreational Athletes

Morphological and functional asymmetry of the lower limbs is a well-recognized factor contributing to musculoskeletal injuries among athletes across different levels. However, limited research exists on evaluating foot mobility asymmetry as a potential predictor of such injuries. This study aimed to (1) assess the frequency of foot mobility asymmetries among amateur athletes, (2) investigate the predictive value of foot mobility asymmetry (measured via navicular height drop) for injury risk, and (3) explore the relationship between foot type and injury occurrence. A cross-sectional sampling method was employed to select 45 physically active male amateur athletes (runners and team sports practitioners) from a university. Injury history was retrospectively recorded, and a modified navicular drop test was conducted to classify foot arch height. The predictive power of navicular height drop asymmetry was analyzed using ROC curves, and the relationship between foot type (neutral and defective combinations—pronated or supinated) and injury occurrence was examined using chi-square tests for independence. Multiple logistic regression was applied to calculate injury risk odds ratios across different foot type subgroups. The results revealed a significant frequency (51.1%) of participants with at least one defective foot, including 31.1% with one neutral and one defective foot and 20% with both feet defective. Navicular height drop asymmetry emerged as a valuable predictor of injuries, with a 36% asymmetry identified as the cut-off for increased injury risk (AUC = 0.832, 95% CI: 0.691–0.973, p < 0.001). A significant relationship was found between foot type and injury occurrence. Only one out of 22 participants with neutral feet (4.55%) experienced an injury, compared to 9 out of 14 (64.29%) with one neutral and one defective foot and 5 out of 9 (55.56%) with both feet defective. These differences were statistically significant (χ2 = 16.24, p < 0.001, Cramer’s V = 0.60). The odds ratio for injury risk was 37.8 (p = 0.016) for those with asymmetry (one neutral and one defective foot) and 26.3 (p = 0.102) for those with both feet defective, compared to participants with both feet neutral. In clinical practice, these findings suggest that routine screenings for physically active individuals should incorporate foot mobility asymmetry assessment. However, it is essential to integrate this factor with other risk indicators. For individuals exhibiting high asymmetry, general foot defect correction programs may be beneficial, but injury prevention strategies should adopt a more comprehensive approach, focusing on overall fitness and tailored interventions for high-risk individuals.

Minimally Modified HIV-1 Infection of Macaques: Development, Utility, and Limitations of Current Models.

Nonhuman primate (NHP) studies that utilize simian immunodeficiency virus (SIV) to model human immunodeficiency virus (HIV-1) infection have proven to be powerful, highly informative research tools. However, there are substantial differences between SIV and HIV-1. Accordingly, there are numerous research questions for which SIV-based models are not well suited, including studies of certain aspects of basic HIV-1 biology, and pre-clinical evaluations of many proposed HIV-1 treatment, prevention, and vaccination strategies. To overcome these limitations of NHP models of HIV-1 infection, several groups have pursued the derivation of a minimally modified HIV-1 (mmHIV-1) capable of establishing pathogenic infection in macaques that authentically recapitulates key features of HIV-1 in humans. These efforts have focused on three complementary objectives: (1) engineering HIV-1 to circumvent species-specific cellular restriction factors that otherwise potently inhibit HIV-1 in macaques, (2) introduction of a C chemokine receptor type 5 (CCR5)-tropic envelope, ideally that can efficiently engage macaque CD4, and (3) correction of gene expression defects inadvertently introduced during viral genome manipulations. While some progress has been made toward development of mmHIV-1 variants for use in each of the three macaque species (pigtail, cynomolgus, and rhesus), model development progress has been most promising in pigtail macaques (PTMs), which do not express an HIV-1-restricting tripartite motif-containing protein 5 α (TRIM5α). In our work, we have derived a CCR5-tropic mmHIV-1 clone designated stHIV-A19 that comprises 94% HIV-1 genome sequence and replicates to high acute-phase titers in PTMs. In animals treated with a cell-depleting CD8α antibody at the time of infection, stHIV-A19 maintains chronically elevated plasma viral loads with progressive CD4+ T-cell loss and the development of acquired immune-deficiency syndrome (AIDS)-defining clinical endpoints. However, in the absence of CD8α+ cell depletion, no mmHIV-1 model has yet displayed high levels of chronic viremia or AIDS-like pathogenesis. Here, we review mmHIV-1 development approaches, the phenotypes, features, limitations, and potential utility of currently available mmHIV-1s, and propose future directions to further advance these models.

Outcomes of Hydroxyapatite Bone Cement for Craniofacial Reconstruction in 1983 Patients.

Hydroxyapatite bone cement (HABC) has evolved to have diverse applications in craniofacial reconstruction. This ranges from filling cranial defects to secondary contouring of residual defects after primary surgeries. This study aims to determine patient outcomes after reconstruction with HABCs. A systematic review and meta-analysis were conducted according to Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) guidelines. PubMed, Embase, Web of Science, and Cochrane Library databases were queried. The results were limited to English-language literature with extractable data on HABC for craniofacial reconstruction. A total of 1983 patients were included in the final analysis from 35 studies. HABCs were mostly used for large defect cranioplasty (21.5%), retrosigmoid (44.6%) and translabyrinthine cranioplasty (8.9%), and correction of residual craniofacial defects after congenital craniofacial surgeries (25%). The rates of cerebrospinal fluid (CSF) leak and infection/explantation were 0% and 7.5% [95% CI: 2.4-11.8] for large defect cranioplasty and 0.8% [0.03-2.07] and 1.5% [95% CI: 0.92-3.3] for retrosigmoid cranioplasty, respectively. The infection/explantation rate was 6.2% [95% CI: 2.6-18.7] for HABCs in the correction of residual craniofacial defects after congenital craniofacial surgeries. The total reoperation rates were 20.4% % [95% CI: 4.8-11.8] for large defect cranioplasty and 12% [95% CI: 3.3-15.3] for correction of residual defects after congenital craniofacial surgeries. Aesthetic satisfaction rates were 93.1% [95% CI: 90.3-98.8] for large defect cranioplasty, 99.4% [95% CI 97.2-99.9] for retrosigmoid cranioplasty, and 92.6% [95% CI: 83.3-95.8] for HABC use in the correction of residual craniofacial defects after congenital craniofacial surgeries. HABC is versatile and associated with a high level of patient reported aesthetic satisfaction after cranioplasty and may have a comparably lower complication profile than those of most other alloplastic materials.

TREATMENT OF PULMONARY ARTERIAL HYPERTENSION ASSOCIATED WITH CONGENITAL HEART DEFECTS

Patients with pulmonary arterial hypertension, associated with congenital heart disease (PAH-CHD) are a heterogeneous population with a varied course of PH. Improvements in pediatric cardiac surgery have changed the epidemiology and survival rate of patients with CHD, of which 90% reach adulthood. Progress in terms of prognosis has also been observed among patients with PAH-CHD. Better survival was observed in ES compared with PAH after defect correction. Advances in surgical treatment of CHD and an increase in life expectancy have led to the study of PAH-CHD and the need to create recommendations for drug treatment of this category of patients. In most of studies, the evaluation of drug treatment of group 1 PAH was carried out without identifying its subgroups. And thus, according to existing recommendations, treatment algorhithms for patients with PAH-CHD are similar to approaches to other forms of PAH. However, various clinical, functional, physical and hemodynamic characteristics of patients with PAH-CHD call into question of correct risk stratification approaches development. Multicenter randomized clinical trials included predominantly a small number of patients with corrected defects, which does not allow the results to be interpreted for the entire population of patients with PAH-CHD. Data from single-center observational studies, expert opinion, and several randomized trials primarily involving patients with Eisenmenger syndrome (ES) indicate the effectiveness and safety of PAH-specific therapy in patients with PAH-CHD. In this literature review, we examined and showed the results of studies involving patients with PAH-CHD and their response to specific therapy. The results obtained significantly expanded the possibilities of using bosentan, sildenafil, epoprostenol, riociguat, ralinepag, sotatercept as they lead to improvement of functional capacity and hemodynamic parameters in patients with PAH-CHD, and only epoprostenol demonstrated an effect on prognosis. Combination PAH-specific therapy, initial or sequential administration of two or more drugs with different mechanisms of action, is an important treatment strategy for patients with PAH. The role of such therapy has increased in recent years. Based on the results of the AMBITION, SERAPHIN, GRIPHON, COMPASS-2 studies, initial or sequential oral combination PAH-specific therapy is recommended for patients with WHO FC II or III. At the same time, there is little evidence to support the effectiveness of this approach in ES patients. The use of anticoagulants in PAH-CHD remains controversial. Low-flow oxygen therapy should be considered individually and continued when there is a significant predominance of subjective or objective benefit. Iron deficiency is associated with poor survival in ES. It is important to note that microcytosis is rare in patients with iron deficiency cyanosis and a normal mean red cell volume does not indicate the absence of anemia. In cases of intolerance to oral iron, intravenous drugs should be used. Currently, based on existing guidelines, most centers follow a consistent symptom-based approach in the treatment of patients with PAH-CHD. Therapy begins with oral ERAs or PDE-5 inhibitors and is escalated if symptoms persist or clinical worsening occurs. If there is no effect of oral PAH-specific therapy, it is recommended to consider parenteral drugs.

Assessing the Feasibility of a Cancer Biomarker Testing Navigator in the Laboratory

Abstract Introduction/Objective Cancer biomarker testing is a crucial component of precision oncology, as it can guide medical treatment decisions with targeted therapies and immune checkpoint inhibitors. However, complex, fragmented, and inefficient operational processes involved in biomarker testing lead to delays, errors, and variability in testing. One potential solution is to introduce a novel role in the pathology laboratory: the cancer biomarker testing navigator (BTN). Methods/Case Report The American Society for Clinical Pathology (ASCP) conducted a mixed-methods project to assess the current state of biomarker testing operations and explore how a BTN may improve biomarker testing processes. ASCP conducted an online survey, focus groups, and worked with two hospital-based cancer centers on a feasibility pilot. Results (if a Case Study enter NA) Survey and focus group insights reveal several operational challenges around the biomarker testing process. 47 completed the survey and 16 participated in the focus groups. Participants report that biomarker test orders may be incorrect or incomplete. 37% of survey respondents experience delays preparing tissue samples for send-out cancer biomarker testing. 42% of survey respondents are unable to track the processing status of send-out tests. Excessive time is required to coordinate and facilitate send-out tests, especially when multiple reference laboratories are used since each has its own portal, requisition form, specimen requirements, etc. 87% agree they would benefit significantly from having a dedicated person to coordinate and manage cancer biomarker tests. The cancer centers that piloted this role found that they could catch and correct defects such as incorrect test ordered, incorrect specimen types marked on the test order, outdated requisition used, etc. The BTN also found instances where the test report was incomplete or had not been uploaded into the EHR. The BTN role could be justified by evaluating metrics such as task efficiency, workplace satisfaction in the lab, and levels of service satisfaction from oncologists. Patient care can be improved by reducing delays in testing and by reviewing other metrics of quality care delivery. Conclusion The BTN role in the laboratory is a novel solution for ensuring the proper collection and triage of pathology samples for biomarker testing. This role could serve as a liaison between the laboratory and ordering clinicians, provide collection and testing guidance, and help with benchmarking and quality measurement initiatives.

Post-Treating Grain Boundaries and Surface Defects by Long-Chain Polymer for Highly Efficient and Stable Perovskite Solar Cells.

Grain boundaries (GBs) and surface defects within perovskite films are inherent challenges that affect the photovoltaic performance of perovskite solar cells (PSCs. In this work, Nylon 11 (N11) is utilized, a long-chain polymer, for post-treating the GBs and surface defects within FAPbI3 films. The multifunctional groups of N11 exhibit unique passivation abilities, enabling self-regulation and selective correction of reverse-charged defects. Post-treating with N11 results in high-quality FAPbI3 films characterized by tight GBs and low surface defect density. Despite fabrication under full open-air conditions, the N11 post-treatment significantly enhances the power conversion efficiency (PCE) value of FAPbI3 PSCs, increasing it from the reference value of 21.89% to 23.54%. Importantly, the long alkyl chain present in N11 significantly enhances the humidity stability of the PSCs. Unencapsulated PSCs treated with N11 maintain 89% of their initial PCE after exposure to air with 30% relative humidity (RH) for 1000h, demonstrating resilience to elevated humidity levels. This work highlights the substantial improvement in the photovoltaic performance of PSCs achieved through the post-treatment with N11.