Investigation of endoplasmic reticulum stress-regulated chaperones as biomarkers in idiopathic nonobstructive azoospermia.

Azoospermia is a condition in which sperm cells are completely absent in a male's ejaculate. Typically, sperm production occurs in the testes and is regulated by a complex series of cellular and molecular interactions. Endoplasmic reticulum (ER) stress arises when there is a deviation from or damage to the normal functions of the ER within cells. In response to this stress, a cascade of response mechanisms is activated to regulate ER stress within cells. This study aims to investigate the role of ER stress-regulated chaperones as potential biomarkers in male infertility. ER stress associated with azoospermia can manifest in cells such as spermatogonia in the testes and can impact sperm production. As a result of ER stress, the expression and activity of a variety of proteins within cells can be altered. Among these proteins are chaperone proteins that regulate the ER stress response. The sample size was calculated to be a minimum of 36 patients in each group. In this preliminary study, we measured and compared serum levels of protein disulfide-isomerase A1, protein disulfide-isomerase A3 (PDIA3), mesencephalic astrocyte-derived neurotrophic factor (MANF), glucose regulatory protein 78 (GRP78), clusterin (CLU), calreticulin (CRT), and calnexin (CNX) between male subjects with idiopathic nonobstructive azoospermia and a control group of noninfertile males. Serum PDIA1 (P = 0.0004), MANF (P = 0.018), PDIA3 (P < 0.0001), GRP78 (P = 0.0027), and CRT (P = 0.0009) levels were higher in the infertile group compared to the control. In summary, this study presents novel findings in a cohort of male infertile patients, emphasizing the significance of incorporating diverse biomarkers. It underscores the promising role of ER stress-regulated proteins as potential serum indicators for male infertility. By elucidating the impact of ER stress on spermatogenic cells, the research illuminates the maintenance or disruption of cellular health. A deeper understanding of these results could open the door to novel treatment approaches for reproductive conditions, including azoospermia.

Effectiveness study of surface electromyography combined with spine 3D data system to evaluate scoliosis.

OBJECTIVE: To evaluate the clinical effectiveness of surface electromyography combined with a spine 3D data system. METHODS: 10 idiopathic scoliosis patients (age: 12.90±3.67 years, height: 155.90±20.07 cm, weight: 48.00±12.86 kg, 6 major thoracic lateral bends and 4 lumbar bends) who were selected to attend the outpatient clinic of Ruijin Rehabilitation Hospital, First diagnosed as scoliosis by imaging examination (EOS system), evaluation by using the surface electromyography monitoring system, the radiation-free 3D spine data acquisition and analysis system, the surface electromyography of the paravertebral muscles (root mean square value (RMS) of the resting standing and action position of the spine) and the Cobb angle of scoliosis were recorded. RESULTS: The RMS of the convex side was larger than that of the concave side in the two postures (P<0.05), and The RMS value of bilateral action position was significantly higher than that of rest position (P<0.05). There was no correlation between RMS ratio and Cobb angle in two positions of thoracic scoliosis (P>0.05). There was a significant correlation between the RMS ratio at rest position of lumbar scoliosis and Cobb angle. At rest, the RMS value of convex side was significantly correlated with the Cobb angle measured by the EOS system and the 3D data system (P<0.05, of which P<0.01). The Cobb angle measured by 3D data system and the EOS system was significantly correlated (P<0.01). CONCLUSION: Surface EMG can be used to evaluate the motor function of paravertebral muscles on both sides of scoliosis patients. The new 3D data system has high accuracy in evaluating scoliosis. The combination of the two can dynamically evaluate scoliosis, which is more clinically effective.

Anaphylaxis in a country where Asia and Europe meet: Evaluation according to World Allergy Organization (WAO) and European Academy of Allergy and Clinical Immunology (EAACI) diagnostic criteria.

Background: Anaphylaxis is an acute-onset, life-threatening clinical emergency involving more than one system. The World Allergy Organization (WAO) published anaphylaxis guidelines in 2020. The European Academy of Allergy and Clinical Immunology (EAACI) published anaphylaxis guidelines in 2021 and reviewed the diagnosis of anaphylaxis, risk factors, comorbid diseases, treatment management, and prevention studies.In this study, clinical features, demographic characteristics, risk factors, treatment management, and evaluations according to EAACI and WAO diagnostic criteria were analysed in patients diagnosed with anaphylaxis. In this way, it was aimed to provide a perspective on the diagnosis, etiology, and treatment management in patients with anaphylaxis and to open a window for new studies. Methods: We retrospectively analysed the data recording system of patients who were evaluated with a history of systemic allergic reaction in the Allergy and Immunology outpatient clinic of our tertiary referral hospital in a ninety-month period between January 2016 and June 2023. The total number of patients admitted to our Allergy and Immunology Clinic between January 2016 and June 2023 was 14,9425. Among these patients, 1032 patients were evaluated in the outpatient clinic according to the ICD-10 (International Statistical Classification of Diseases and Related Health Problems) coding system and diagnosed as T78.2 Anaphylaxis by Allergy and Immunology Specialist. Each patient file was re-evaluated by the authors of the study and 689 eligible patients were included. Results: Included in the study were 689 patients. The mean age of the patients was 46.2 ± 14.2 (35-57) years. 64.4% (n = 444) of the patients were female. Venom, drugs, and food were the 3 leading causes of anaphylaxis. In our study, younger age was determined as a statistically significant risk factor for food-induced anaphylaxis, female gender for drug-induced anaphylaxis and male gender for venom-induced anaphylaxis. (p < 0.001) If the cause of anaphylaxis can be identified, such as venom, drug or food, the frequency of anaphylaxis decreases statistically significantly compared to idiopathic anaphylaxis. (p < 0.001) The rate of acute hypotension, bronchospasm, or laryngeal involvement without skin involvement according to WAO and the rate of WAO severity classification grade 5 were significantly higher in patients who developed anaphylaxis due to venom compared to other patients (p < 0.001). The rate of Grade 3 in the EAACI classification was significantly higher in patients who developed anaphylaxis due to venom compared to other cases (p < 0.001). Conclusion: Our study was conducted in a city such as Istanbul, which connects both Asian and European continents. Moreover, it is important because it was conducted in a centre with the highest number of Allergy and Immunology outpatients per year in our country. It is important because it gives the prevalence of anaphylaxis and emphasises the risk factors for each allergen separately.

Idiopathic Gingival Fibromatosis: Report of a Rare Case.

The progressive overgrowth of the gingiva is the hallmark of idiopathic gingival fibromatosis (IGF). Excess gingival tissue can obscure the crown of a tooth, resulting in spaces between teeth, displacement, retention of primary or permanent teeth, and difficulties with feeding, speaking, and appearance. The diagnosis and management of inherited gingival fibromatosis are the focus of this case report. A 12-year-old girl was referred from the Department of Orthodontics to Oral Medicine as a result of progressive gingival enlargement, which impeded orthodontic treatment for misaligned lower front teeth. The patient underwent a conservative periodontal treatment regimen that encompassed gingivectomy and debridement. The excised gingival tissues were submitted for histopathological examination. Tissue sections stained with hematoxylin and eosin showed connective tissue with dense bundles of collagen fibers and little inflammation. The patient was reviewed after three months, and advised of orthodontic management for further aesthetic correction. The findings indicated that the oral symptoms of gingival fibromatosis are influenced by the severity of the condition and the age at which it begins. Early intervention helps mitigate potential difficulties for younger individuals.

Idiopathic Orbital Inflammatory Disease in a Pediatric Patient: A Case Report Highlighting the Diagnostic Reasoning and Treatment Strategy.

Idiopathic orbital inflammatory disease (IOID) is a rare and poorly understood condition characterized by inflammation of the orbital tissues without an identifiable cause. This disorder can lead to symptoms such as proptosis, pain, and visual disturbances. We present the case of an eight-year-old female diagnosed with IOID who was admitted to the hospital with worsening right eye redness, proptosis, and pain. Her clinical course included significant right eye lagophthalmos, exposure keratopathy, and a corneal ulcer. Management involved a multidisciplinary approach with consultations from ophthalmology and rheumatology, treatment with corticosteroids, and supportive care. This case underscores the importance of early recognition and a comprehensive management strategy to improve outcomes for patients with IOID.

Xuanfei Baidu decoction ameliorates bleomycin-elicited idiopathic pulmonary fibrosis in mice by regulating the lung-gut crosstalk via IFNγ/STAT1/STAT3 axis.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a chronic progressive interstitial pneumonia, the available treatment option is limited because the etiology and pathological process are not well understood. Although gut-lung axis reported with an emerging area of host-associated microbiota exist in many chronic lung diseases, the connection between gut-lung microbiota composition with in-site inflammation in IPF development is not yet established. PURPOSE: We aimed to address the microbiota and immunity connection, and make it clear how a listed drug, Xuanfei Baidu Decoction (XFBD) affect the lung-gut crosstalk for IPF amelioration, which was previously reported for restoring disrupted lung in IPF and protecting intestinal injury. METHODS: Firstly, Micro-CT (µCT) and histopathology were used to check for pathological changes in the lungs and intestines of bleomycin (BLM)-induced IPF mice. Then, Reverse Transcription and Quantitative Real-time PCR (RT-qPCR) and Western blot (WB) assays were employed to detect the integrity of the barrier of lungs and intestines in IPF mice. Subsequently, flow cytometry and 16S rRNA sequencing were used to evaluate the immune and microbial microenvironment of the lungs and intestines. We analyzed the lung-gut microbiota crosstalk for further mechanism exploration. RESULTS: Firstly, we revealed that XFBD protected the integrity of the lung and intestinal barriers in the IPF mice, as evidenced by the up-regulation of ZO-1, Claudin-1, Occludin, and VE Cadherin protein expression. Then, we analyzed the changing microbiota and T cell in the gut-lung axis in IPF, and with XFBD, six highly relevant microenvironments were demonstrated that crossing damaged lung-gut barriers and XFBD could reverse these chaotic bacterial and immunity micro-environment, among them Akkermansia was an essential bacteria affecting the expression of systemic IFN-γ downstream STAT1/STAT3 axis was also studied. XFBD prominently up-regulated the production of IFN-γ and p-STAT1 and down-regulated p-STAT3, consequently exerting effects on the lung barrier and gut barrier. Taken together, XFBD ameliorated BLM-induced IPF mice by regulating IFNγ/STAT1/STAT3 axis. CONCLUSION: Altogether, our results revealed that XFBD improved the BLM-elicited IPF mice by regulating gut-lung crosstalk via IFN-γ/STAT1/STAT3 axis and provided a new insight of gut-lung crosstalk in IPF, especially the dynamic changes of microorganisms in the damaged lungs needed to pay more attention during IPF therapy.

Artificial Intelligence for Prediction of Shunt Response in Idiopathic Normal Pressure Hydrocephalus: A Systematic Review.

BACKGROUND: Idiopathic normal pressure hydrocephalus (iNPH) is a reversible cause of dementia, typically treated with shunt surgery, although outcomes vary. Artificial intelligence (AI) advancements could improve predictions of shunt response (SR) by analyzing extensive data sets. METHODS: We conducted a systematic review to assess AI's effectiveness in predicting SR in iNPH. Studies using AI or machine learning (ML) algorithms for SR prediction were identified through searches in MEDLINE, EMBASE, and Web of Science up to September 2023, adhering to Synthesis Without Meta-Analysis reporting guidelines. RESULTS: Out of 3541 studies identified, 33 were assessed for eligibility, and 8 involving 479 patients were included. Study sample sizes varied from 28 to 132 patients. Common data inputs included imaging/radiomics (62.5%) and demographics (37.5%), with Support Vector Machine being the most frequently used ML algorithm (87.5%). Two studies compared multiple algorithms. Only four studies reported the Area Under the Curve (AUC) values, which ranged between 0.80 and 0.94. The results highlighted inconsistency in outcome measures, data heterogeneity, and potential biases in the models used. CONCLUSIONS: While AI shows promise for improving iNPH management, there is a need for standardized data and extensive validation of AI models to enhance their clinical utility. Future research should aim to develop robust and generalizable AI models for more effective diagnosis and management of iNPH.

Steroid pulse therapy in idiopathic nephrotic syndrome in the era of modern immunosuppressive treatment-still up to date?

BACKGROUND: Intravenous steroid pulses (SP) are successfully used for the treatment of patients with idiopathic nephrotic syndrome (INS) resistant to oral prednisone. METHODS: We performed a retrospective analysis of all patients in the three pediatric nephrology centers of the Paris region from 2002 to 2022 who were resistant to a 30-day course of oral prednisone and who received SP for their first INS flare and analyzed their disease course over 4 years. RESULTS: Forty-seven patients (17 girls), median age 3.4 years, were analyzed. Of them, 68% reached remission within 7 days of SP. No significant short-term side effects were noted. Half of the patients started immunosuppressive treatment immediately after their first remission and 62% of them relapsed at least once, whereas all the patients who did not receive immunosuppressive treatment since their first remission relapsed. Among the SP-sensitive patients, 75% needed calcineurin inhibitor (CNI) or B-cell depletion during their disease course to achieve stable remission. Forty-two percent of the whole cohort received B-cell-depleting agents. Among the 15 SP-resistant patients, all received CNI. Twelve/fifteen patients reached remission. After 4 years, 68% among the SP-sensitive patients and 87% of SP-resistant patients still had an active disease. CONCLUSIONS: SP are helpful to obtain rapid remission in pediatric INS patients resistant to oral steroids. However, as most SP-sensitive patients need immunosuppressive drugs, mainly CNI and B-cell-depleting agents it could be interesting to discuss the possibility to start CNI directly after the 30-day course of prednisone instead of SP.

Multiple Idiopathic External Apical Root Resorption: A Case Report of a Rare Entity.

Idiopathic root resorption is characterized by the gradual destruction of tooth roots without a clear cause; the possible underlying factors include genetic predispositions, immune system abnormalities, or environmental influences. This case report highlights an unusual instance of a 27-year-old young female patient who presented with multiple decayed teeth; the orthopantomographic examination incidentally revealed extensive root resorptions. Thorough biochemical investigations such as acid phosphatase, alkaline phosphatase, thyroid-stimulating hormone (TSH), thyroxine (T4), and triiodothyronine (T3), as well as calcium, were within normal limits, with no identifiable local or systemic factors, leading to a diagnosis of idiopathic root resorption. Diagnosing multiple idiopathic external apical root resorption (MIEARR) is particularly challenging, underscoring the importance of regular monitoring and comprehensive dental care to prevent further deterioration. The uniqueness of our case report lies in its investigative approach, employing advanced diagnostic techniques such as nano CT and scanning electron microscopy (SEM). It also includes a comparative analysis of cases previously reported in the literature.

Case report: Multi-antibody-positive myasthenia gravis concomitant myositis associated with thymoma.

Myasthenia gravis (MG) and idiopathic inflammatory myopathy (IIM) are autoimmune diseases of the nervous system, and their main clinical manifestation is muscle weakness. The concurrent presence of both conditions in the same patient is clinically rare and easily missed. Here, we report the case of a 74-year-old woman who went to the doctor with fluctuating weakness of the limbs and muscle pain. By analyzing the patient's history and the results of repeated frequency electrical stimulation, chest computed tomography, thigh muscle magnetic resonance imaging, serum antibody detection, lymph node biopsy, etc., she was finally diagnosed with MG-concomitant IIM with squamous cell carcinoma of the thymus. Acetylcholine receptor antibody, titin antibody, ryanodine receptor antibody, anti-JO-1 antibody, and Ro-52 antibody tests were positive. MG-concomitant IIM is often associated with thymoma. The immunopathology mechanism may be different from that of pure MG or IIM, which needs further research.

Implications of the 2022 lung function update and GLI global reference equations among patients with interstitial lung disease.

BACKGROUND: Lung function testing remains a cornerstone in the assessment and management of interstitial lung disease (ILD) patients. The clinical implications of the Global Lung function Initiative (GLI) reference equations and the updated interpretation strategies remain uncertain. METHODS: Adult patients with ILD with baseline forced vital capacity (FVC) were included from the Australasian ILD registry and the National Healthcare Group ILD registry, Singapore.The European Coal and Steel Community and Miller reference equations were compared with the GLI reference equations to assess (a) differences in lung function percent predicted values; (b) ILD risk prediction models and (c) eligibility for ILD clinical trial enrolment. RESULTS: Among 2219 patients with ILD, 1712 (77.2%) were white individuals. Idiopathic pulmonary fibrosis (IPF), connective tissue disease-associated ILD and unclassifiable ILD predominated.Median FVC was 2.60 (2.01-3.36) L, forced expiratory volume in 1 s was 2.09 (1.67-2.66) L and diffusing capacity of the lungs for carbon monoxide (DLCO) was 13.60 (10.16-17.60) mL/min/mm Hg. When applying the GLI reference equations, the mean FVC percentage predicted was 8.8% lower (87.7% vs 78.9%, p<0.01) while the mean DLCO percentage predicted was 4.9% higher (58.5% vs 63.4%, p<0.01). There was a decrease in 19 IPF and 119 non-IPF patients who qualified for the nintedanib clinical trials when the GLI reference equations were applied. Risk prediction models performed similarly in predicting mortality using both reference equations. CONCLUSION: Applying the GLI reference equations in patients with ILD leads to higher DLCO percentage predicted values and smaller lung volume percentage predicted values. While applying the GLI reference equations did not impact on prognostication, fewer patients met the clinical trial criteria for antifibrotic agents.

Efficacy of botulinum neurotoxin A in persistent idiopathic dentoalveolar pain: a case series.

OBJECTIVES: Persistent idiopathic dentoalveolar pain (PIDP) is a challenging clinical entity associated with both physical and emotional consequences. Currently, the management is symptom-based and includes both topical and/or systemic treatments. More recently, botulinum neurotoxin-A (BONT-A) has been suggested as a treatment option. MATERIALS AND METHODS: We present a case series of 9 patients (5 female) with mean age 56 ± 15 diagnosed with PIDP. All patients reported prior experience with systemic drugs without a sufficient pain-relieving effect. BONT-A (BOTOX, Allergan) 100 U diluted with saline solution was used and the dose ranged from 20U to 50U distributed in 3 sites (intraoral and/or extraoral) per session. Patients underwent further injections (50U) monthly if pain severity measured using a Numerical Rating Scale (NRS 0-10) was still > 3 for 3 months. Pain severity and characteristics were recorded at baseline (T0), after 1 month (T1), 2 months (T2) and 3 months (T3). RESULTS: Mean pain intensity at baseline was NRS 6 (4-10). Latency before analgesic effect was at least 5-10 days after injection. Minor adverse effects were sickness and muscular hypotonia. Pain significantly reduced to NRS 4 (0-8) at T1, to NRS 2 (0-8) at T2 and to NRS 2 (NRS 0-8) at T3. Patients' functional variables (discomfort while chewing, talking, swallowing) were also recorded. CONCLUSIONS: BONT-A is widely used and although the exact mechanism of action remains unclear, it can be used effectively in reducing pain for a variety of conditions including PIDP. CLINICAL RELEVANCE: Our results suggest that BONT-A seems to be an alternative therapeutic approach for patients with PIDP.

Rheumatoid Arthritis-Associated Interstitial Lung Disease (RA-ILD): Update on Prevalence, Risk Factors, Pathogenesis, and Therapy.

PURPOSE OF REVIEW: Rheumatoid arthritis is frequently complicated by interstitial lung disease (RA-ILD), an underappreciated contributor to excess morbidity and mortality. The true prevalence of RA-ILD is difficult to define given the variability in diagnostic criteria used. The lack of standardized screening methods, an incomplete understanding of disease pathogenesis, and dearth of validated biomarkers have limited the development of controlled clinical trials for this disease. RECENT FINDINGS: Numerous studies have focused on clinical, radiographic, genetic, molecular, and/or serologic markers of disease severity as well as risk of disease progression. In addition to defining valuable clinical biomarkers, these studies have provided insights regarding the pathogenesis of RA-ILD and potential therapeutic targets. Additional studies involving immunomodulatory and/or anti-fibrotic agents have assessed new therapeutic options for different stages of RA-ILD. RA-ILD continues to be a major contributor to the increased morbidity and mortality associated with RA. Advancements in our understanding of disease pathogenesis at a molecular level are necessary to drive the development of more targeted therapy.

Successful management of idiopathic early-onset scoliosis: effect of curve correction and bracing after Mehta casting.

STUDY DESIGN: Patients with idiopathic EOS treated by Mehta casting followed by bracing or observation from a single institution. OBJECTIVES: To determine casting protocol parameters leading to successful management; to determine efficacy of bracing vs. observation after cast discontinuance. BACKGROUND: Previous studies have not precisely defined parameters for cast discontinuance (amount of correction, number of casts), nor have documented the efficacy of brace treatment. METHODS: 73 patients undergoing Mehta casting were braced (n = 56) or observed (n = 17) after casting with follow-up for a mean of 51-58 months. 57 patients had ≥ 4 casts applied; 39 had ≥ 5 casts. Success was defined as no further treatment required. Curve magnitude was measured at time points pre-casting, at cast discontinuance, and last follow up. RESULTS: There was no difference in success rate between braced patients (79% success) and observed (71%). Curve correction to < 30° at cast discontinuance was crucial parameter for success, as 95% (45/47) of patients with this correction achieved success, braced or not, while only 42% (11/26) with residual curves ≥ 30° achieved success (p < .001) and 14 of these required surgery compared to 0/47 successful patients. The number of casts (over/under 4 or 5) made no difference in achieving success. 10/26 patients who had residual curves ≥ 30° and were braced achieved success due to further curve diminution during bracing. CONCLUSIONS: Cast correction to < 30° followed by bracing achieved 100% success in 34 patients compared to 85% (13 patients) who were observed (p = .07). For residual curves ≥ 30° bracing can produce some correction and succeed in delaying further treatment.

Magnetic resonance imaging evaluation of the cerebellar tonsil position before and after posterior spinal fusion in adolescent idiopathic scoliosis.

BACKGROUND: Chiari malformation type I(CM-I) is one of the most common neurological malformations observed in patients with adolescent idiopathic scoliosis (AIS). It remains uncertain whether AIS patients without neurological symptoms require CM-I decompression before undergoing spinal corrective surgery to prevent cerebellar tonsil herniation and potential neurological complications. This study aims to assess variations in the position of the cerebellar tonsil after posterior spinal fusion (PSF) in neurologically intact patients with AIS. METHODS: We retrospectively evaluated 40 patients with AIS who underwent posterior spinal fusion (PSF) without neurological symptoms. Sagittal hindbrain MRIs were performed before and after spinal surgery. The cerebellar tonsil's position relative to the foramen magnum was measured using the method described by Aboulezz (J Comput Assist Tomogr 1985). We assessed variations in the cerebellar tonsil position concerning spinal correction and elongation after PSF. RESULTS: The average preoperative magnitude of the scoliotic curve was 53.15 degrees (SD 10.46 degrees), and that of thoracic kyphosis was 35.42 degrees (SD 12.38 degrees). In the immediate postoperative period, the average magnitudes were 7.45 degrees (SD 7.33 degrees) and 27.87 degrees (SD 9.03 degrees), respectively. The average correction achieved with PSF was 86% in the coronal plane (p < 0.00001). The average length of the spine in the coronal plane was 44.5 cm (SD 5.25 cm) preoperatively and 48.27 cm (SD 4.40 cm) postoperatively (p < 0.00001). The average length of the vertebral column in the sagittal plane was 50.87 cm (SD 4.47 cm) preoperatively and 55.13 cm (SD 3.27 cm) postoperatively (p < 0.00001). No significant difference was observed in the position of the cerebellar tonsil in the measurements taken before and after spinal deformity correction (p = 0.6042). In 10 out of 40 patients, caudal displacement of the cerebellar tonsil was observed after PSF. In 28 patients, no variation in the position of the cerebellar tonsil was observed, and in 2, cephalic displacement was noted. CONCLUSION: In AIS patients with a normal position of the cerebellar tonsil undergoing posterior spinal fusion, there is no significant change in their position after surgery. Our analysis did not reveal any correlation between curve correction, spine elongation, and variations in the cerebellar tonsil position. SIGNIFICANCE: The position of the cerebellar tonsil remained largely unchanged after PSF in AIS patients without neurological symptoms. Further studies would be necessary to determine whether surgical decompression is required in asymptomatic patients with CM-I before undergoing surgery to correct spinal deformity.

Do teams of strangers create health care dangers? The effect of OR team consistency on operative times in adolescent idiopathic scoliosis.

PURPOSE: Teamwork and communication are important components of any surgical team. This study uses a simple, reproducible, and quantitative "team consistency score" and a nodal-based model for examining prior interactions amongst team members to represent and quantify the regularity of an OR team for a specific surgical case. METHODS: The electronic medical record (EMR) at our institution was queried for pediatric patients undergoing spinal surgery from January 2021 through December 2023. The number of prior interactions between individuals filling distinct roles in the OR for each case was recorded. A metric coined the consistency score was developed representing the sum total of these prior interactions standardized to a reference case. Spearman's Correlation as well as the Mann-Whitney comparison test were used to analyze the associations between case team consistency score and efficiency measures. RESULTS: 154 cases were included for analysis. There was a statistically significant negative correlation between case consistency score and both anesthesia time (rho = -0.159; p < 0.05) and patient preparation time (rho = -0.218; p < 0.01). When looking at the consistent (above median consistency score of 0.46) vs. inconsistent cohorts, the inconsistent cohort had a higher mean patient preparation time (53.3 ± 14.0 min vs. 49.0 ± 9.3 min; p < 0.05), as well as a higher overall mean case length (336.6 ± 47.4 min vs. 321.9 ± 42.4 min; p < 0.05). CONCLUSION: The findings suggest that increased team consistency, as measured by a "team consistency score" metric, is related to heightened efficiency and reduced intraoperative times.

Advances in Juvenile Dermatomyositis: Pathophysiology, Diagnosis, Treatment and Interstitial Lung Diseases-A Narrative Review.

Juvenile idiopathic inflammatory myopathy (JIIM) is a rare systemic autoimmune disease characterized by skeletal muscle weakness with or without a skin rash. Juvenile dermatomyositis (JDM) is the most common subtype of JIIM, accounting for 80% of JIIM. Recent studies identified several myositis-specific autoantibodies (MSAs) and myositis-associated autoantibodies (MAAs). Each MSA or MAA is associated with distinct clinical features and outcomes, although there are several differences in the prevalence of MSA/MAA and autoantibody-phenotype relationships between age and ethnic groups. Histopathological studies have revealed critical roles of type I interferons and vasculopathy in the development of JDM. Serological classification mostly corresponds to clinicopathological classification. Novel therapeutic agents, such as biologics and Janus kinase inhibitors (JAKi), have been developed; however, to date, there is a lack of high-level evidence. As advances in treatment have reduced the mortality rate of JIIM, recent studies have focused on medium- and long-term outcomes. However, rapidly progressive interstitial lung disease (RP-ILD) remains a major cause of death in anti-melanoma differentiation gene 5 autoantibody-positive JDM. Early diagnosis and intervention using a multi-drug regimen is critical for the treatment of RP-ILD. Rituximab and JAKi may reduce mortality in patients with JDM-associated RP-ILD refractory to conventional therapy.

Real-World Impact of Deep Targeted Sequencing on Erythrocytosis and Thrombocytosis Diagnosis: A Reference Centre Experience.

Despite advances in diagnosis of erythrocytosis and thrombocytosis due to driver mutation testing, many cases remain classified as "idiopathic". This can be explained by the absence of an evident secondary cause, inconclusive bone marrow biopsy or neglection of family history. Analysis of a broad panel of genes through next-generation sequencing (NGS) could improve diagnostic work-up identifying underlying genetic causes. We reviewed the results of NGS performed in our laboratory and its diagnostic impact on 117 patients with unexplained erythrocytosis and 58 with unexplained thrombocytosis; six patients (5.1%) were diagnosed with polycythaemia vera (PV) and 8 (6.8%) with familial erythrocytosis after NGS testing. Low EPO and a family history seemed to predict a positive result, respectively. However, a greater percentage of patients were ultimately diagnosed with secondary erythrocytosis (36%), remained as idiopathic (28.2%) or were self-limited (15%). The yield of NGS was shown to be slightly higher in patients with thrombocytosis, as 15 (25.9%) were diagnosed with essential thrombocythemia (ET) or familial thrombocytosis after variant detection; previous research has shown similar results, but most of them carried out NGS retrospectively, while the present study exhibits the performance of this test in a real-world setting. Overall, the low rate of variant detection and its poor impact on diagnostic work-up highlights the need for a thorough screening prior to NGS, in order to improve its yield.

Therapeutic Cerebral Fluid Puncture in Patients with Idiopathic Intracranial Hypertension: No Short-Term Effect on Neurocognitive Function.

BACKGROUND: Idiopathic intracranial hypertension (IIH) is typically characterized by headaches and vision loss. However, neurocognitive deficits are also described. Our study aimed to test the influence of therapeutic lumbar puncture on the latter. METHODS: A total of 15 patients with IIH were tested with a battery of neurocognitive tests at baseline and after therapeutic lumbar drainage. Hereby, Logical Memory of the Wechsler Memory Scale-Revised Edition (WMS-R), the California Verbal Learning Test Short Version (CVLT), alertness, selective attention, and word fluency were used. Changes in cognitive functioning in the course of CSF pressure lowering were analysed and compared with age, sex, and education-matched healthy controls. RESULTS: Before intervention, scores of Logical Memory, the RWT, and the HADS-D were significantly lower in IIH patients compared to matched controls. After short-term normalization of CSF pressure, the RWT improved significantly. Additionally, significant positive correlations were found between headache intensity and subjective impairment, as well as between BMI and CSF opening pressure. CONCLUSIONS: Our findings confirm lower performance in terms of long-term verbal memory and word fluency compared to controls, as well as depressive symptoms in IIH patients. Significant improvement after short-term normalization of intracranial pressure by means of CSF drainage was seen only for word fluency. This indicates that short-term normalization of CSF pressure is not sufficient to normalize observed neurocognitive deficits.