Bone marrow edema syndrome (BMES), transient osteoporosis of the hip (TOH), and regional migratory osteoporosis (RMO), along with numerous variants of these terms, are used inconsistently to describe spontaneous pain, typically in the lower extremity, accompanied by bone marrow edema on MRI and/or bone demineralization. In the present review, we aimed to determine whether these designations represent distinct conditions or varying manifestations of a shared pathology. We employed a scoping review methodology, following a preregistered protocol, utilizing a comprehensive systematic search of electronic databases. Eligible publications reported on patients designated with BMES, TOH, RMO, or related terms. A total of 2924 patients, across 561 studies, were included. Data extraction focused on demographics, clinical features and imaging results. Descriptive statistics and meta-analytic methods were used to synthesize the data. Our results show that patients described by terms related to bone marrow edema syndrome, or transient or migratory osteoporosis, displayed similar demographic and clinical profiles. Our findings strongly suggest that these various designations refer to the same clinical entity. Bone marrow edema syndrome appears to be the most suitable term to describe this condition, facilitating a more standardized approach to future diagnosis, management and research.

This systematic review with meta-analysis explores the global demographic and etiological variations of retinal vasculitis (RV), focusing on differences in frequency, age, sex, and etiology across diverse geographic populations. RV is an inflammatory condition that can lead to visual impairment, making understanding its variations essential for targeted screening and management. Systematic searches were conducted in multiple databases up to February, 2023, following PRISMA guidelines. We included studies with at least 10 RV cases, such that a frequency measurement can be estimated, without restrictions on publication date or language. RV was categorized as Idiopathic RV in the absence of additional ocular or systemic disease, Syndromic RV for ocular involvement without systemic disease, and Secondary RV in those asssociated with systemic disease. The risk of bias was evaluated using standardized tools. A total of 95 studies, including 23,180 patients, were analyzed. The overall RV frequency among uveitis cohorts was 17 %, with European populations showing the highest frequency at 25 %. Idiopathic RV accounted for 1 % of uveitis cohorts and 38 % of RV cohorts, with significant differences across continents. Behçet disease had the highest RV frequency at 56 %. The median age of diagnosis was 33.5 years, and RV was more frequent in males (57 %). Our findings underscore the considerable geographic and demographic variability in RV, particularly in Idiopathic RV, tuberculosis-related RV, and Behçet disease, highlighting the need for tailored, region-specific, and gender-specific approaches to RV diagnosis and treatment.

The recent surge in serious adverse events (SAEs) and deaths associated with ferric carboxymaltose (FCM) underscores the importance of evaluating its safety profile.

We conducted a retrospective case/non-case study from Q4 of 2003 to Q4 of 2024 data on FCM in the FDA Adverse Event Reporting System (FAERS) and VigiBase databases. Signal detection was performed using proportional reporting ratio (PRR), reporting odds ratio (ROR), and information component (IC). The influence of concomitant medication on the identified signal was assessed and refined using Open Vigil 2.1. Additionally, to identify case reports on FCM-induced adverse events, a comprehensive search was performed in PubMed, Google Scholar, and Scopus databases from inception to April 12, 2025.

In the FAERS database, 46 deaths were reported in connection with FCM, though no significant death signal was observed (PRR = 0.3, LB (lower bound) ROR = 0.2, IC025 = - 2.3). Nonetheless, positive safety signals emerged for SAEs such as anaphylactic shock (PRR = 3.9, LB ROR = 2.3, IC025 = 1.0), circulatory collapse (PRR = 14.6, LB ROR = 10.5, IC025 = 3.1), respiratory distress (PRR = 9.6, LB ROR = 7.1, IC025 = 2.6), hypophosphatemia (PRR = 520.7, LB ROR = 530.1, IC025 = 8.0), and arrhythmia (PRR = 3.3, LB ROR = 2.2, IC025 = 1.0). After meticulously refining our analysis to account for the influence of concomitant medications, we observed that the strength of all signals remained unchanged, except for respiratory distress, bradycardia, hypotension, abdominal pain, and urticaria. Analysis of VigiBase data revealed 42 reported fatal cases and potential signals for hypersensitivity (PRR = 4.5, LB ROR = 4.4, IC025 = 2.1), anaphylactic shock (PRR = 2.3, LB ROR = 1.9, IC025 = 0.9), circulatory collapse (PRR = 7.2, LB ROR = 6.0, IC025 = 2.5), respiratory distress (PRR = 6.9, LB ROR = 5.7, IC025 = 2.5), and hypophosphatemia (PRR = 245.1, LBROR = 234.8, IC025 = 7.5) with Ferinject. The systematic review of 11 case reports emphasized SAEs linked to FCM, thereby strengthening this association.

This study reveals that FCM carries SAEs. Providers must weigh the benefits and risks on a case-by-case basis, considering patient-specific factors. Continuous monitoring and further research are crucial for the safe use of FCM in iron deficiency anemia.

Recent research underscores the significance of meniscal extrusion in knee osteoarthritis progression. Centralization of the meniscus has emerged as a potential intervention, yet comprehensive literature on this is limited. This systematic review aims to synthesize evidence on meniscus centralization's impact on extrusion and knee biomechanics. A search of PubMed Central and Scopus yielded 14 relevant articles until June 1, 2023. Included were 3 case series, 9 cadaveric studies, and 2 involving animals or patients. Biomechanical effects on both medial and lateral menisci were explored, alongside implications for anterior cruciate ligament reconstruction. Findings suggest centralization may mitigate extrusion and improve knee biomechanics, potentially benefiting clinical outcomes. However, further high-quality studies are warranted to validate these observations and ascertain optimal centralization techniques.

Despite their effectiveness in postoperative analgesia regimens for total knee arthroplasty (TKA) and total hip arthroplasty (THA), opioid medications are accompanied by well-known side effects and a risk of long-term dependence. These drawbacks have prompted the exploration of opioid-free analgesia protocols. The purpose of this study was to summarize the nature and extent of evidence available on opioid-free analgesia protocols in THA and TKA management.

A scoping review of all Medline, Embase, and CENTRAL-indexed studies published between March 2019 and May 2023 was conducted, focusing on opioid-free analgesia regimens following THA and TKA. All included studies were assessed for potential risk of bias. Meta-analyses of pooled opioid-free percentages and pain scores were conducted using odds ratio and standardized mean difference, respectively, in a random-effects model.

A total of 23 studies (15 TKAs and eight THAs) were included. Among both TKA and THA, rescue opioids were the most commonly reported postoperative intervention. The most commonly investigated nonopioid analgesic modality was local anesthetics/nerve blocks with 52.2% (12 of 23) of the studies, followed by multimodal combinations (21.7%) and intravenous corticosteroids (13.0%). Only two of the 10 included TKA randomized controlled studies demonstrated statistically significant increases in the postoperative opioid-free rates. Of the six included THA randomized controlled trials, four demonstrated statistically significant increases in patients completing the postoperative period opioid-free. Our meta-analysis demonstrated a statistically significant impact of nerve blocks following TKA on the opioid-free rate and postoperative pain scores. Among the included THA studies, all studies in which patients received postoperative intravenous corticosteroids demonstrated significant increases in opioid-free percentage.

Despite some nonopioid analgesics demonstrating promise, rescue opioids remained the most frequently employed postoperative pain medication. The optimized opioid-free analgesic regimen likely requires a multimodal approach, especially using both local anesthetics/nerve blocks and intravenous corticosteroids. Further investigation and reporting of opioid-free episodes of care are needed.

The construction industry, known for its high-risk environment, increasingly acknowledges the psychological risks to workers. Despite well-documented physical hazards, mental health challenges within this workforce have garnered attention. This systematic review provides a scholarly synthesis of literature on psychological risks and their causative factors affecting construction workers, adhering to PRISMA protocols.

An exhaustive literature search was conducted across PubMed, Scopus, PsycINFO, and Google Scholar using relevant keywords. Rigorous screening of selected studies focused on the psychological aspects of construction work, with systematic data extraction and analysis.

From 1992 to 2022, 68 studies met inclusion criteria, identifying key psychological harm factors: job insecurity, long working hours, high demands, poor work-life balance, and workplace bullying. A lack of mental health support and awareness in the sector was noted, with a significant correlation between these factors and increased stress, anxiety, and depression levels among workers.

The review highlights the urgent need for industry recognition of psychological hazards and the relationship between work conditions and mental health issues. It calls for supportive policies, awareness programs, and counseling services, with a suggestion for future longitudinal research on the long-term impacts and intervention efficacy.

This study provides an overview of psychological risks in the construction sector from 1994 to 2022, aiming to identify causative factors for mental health issues. It seeks to pave the way for targeted interventions and policy changes to improve mental health outcomes in the industry.

Selection of patients harboring mutations in homologous recombination repair (HRR) genes for treatment with a PARP inhibitor (PARPi) is challenging in metastatic castration-resistant prostate cancer (mCRPC). To gain further insight, we quantitatively assessed the differential efficacy of PARPi therapy among patients with mCRPC and different HRR gene mutations.

This living meta-analysis (LMA) was conducted using the Living Interactive Evidence synthesis framework. We included clinical trials assessing PARPi as monotherapy in pretreated mCRPC or in combination with an androgen receptor pathway inhibitor (ARPI) in treatment-naïve patients. Random-effects meta-analyses were performed for a priori subgroups stratified by HRR status, BRCA status, and each gene.

This first report for our LMA includes 13 trials (4278 patients). Among patients with pretreated mCRPC receiving PARPi monotherapy, the tumor response rate per 100 person-months was numerically higher for patients with BRCA2 (50% prostate-specific antigen response [PSA50%] 3.3; objective response rate [ORR] 3.3), BRCA1 (PSA50% 1.2; ORR 2.0), or PALB2 (PSA50% 3.3; ORR 1.4) alterations than for patients with ATM (PSA50% 0.4; ORR 0.3), CDK12 (PSA50% 0.2; ORR 0.2), or CHEK2 (PSA50% 1.0; ORR 0.7) alterations. Among patients receiving PARPi + ARPI, a significant radiographic progression-free survival benefit was observed in those with BRCA (hazard ratio [HR] 0.28, 95% confidence interval [CI] 0.13-0.62) or CDK12 (HR 0.58, 95% CI 0.35-0.95) alterations, but not in patients with PALB2 (HR 0.53, 95% CI 0.21-1.32), ATM (HR 0.93, 95% CI 0.57-1.53), or CHEK2 (HR 0.92, 95% CI 0.53-1.61) alterations. An overall survival benefit was observed for patients with BRCA alterations (HR 0.47, 95% CI 0.31-0.71) after adjustment for crossover and subsequent therapy, but not for patients with PALB2 (HR 0.33, 95% CI 0.10-1.16), ATM (HR 0.97, 95% CI 0.57-1.67), CDK12 (HR 0.80, 95% CI 0.36-1.78), or CHEK2 (HR 0.81, 95% CI 0.37-1.75) alterations.

Our LMA delivers information on the effect of PARPi therapy in relation to specific gene alterations in mCRPC via an interactive web platform. The evidence suggests the greatest PARPi benefit in patients with BRCA alterations, a strong signal of benefit in patients with PALB2 or CDK12 alterations, and no benefit in patients with ATM or CHEK2 alterations.

Background: Manganese (Mn) is an essential trace element for humans. It has been recognized as a potential occupational toxin, but its danger as a toxin in patients under parenteral nutrition is often forgotten. Case Presentation: A 73-year-old man was logged for 210 days in the intensive care unit (ICU), receiving parenteral nutrition (PN) for a month, and was then transferred, first, to the internal medicine ward and, then, to the rehabilitation hospital, and 223 days after discharge from the ICU, he had current disease, chorea-type movements in the head and neck, and left hemibody. Diagnostic tests: The magnetic resonance imaging findings suggested manganese deposits, with a total blood manganese concentration of 34 µg·L-1 (reference range: less than 13 µg·L-1). Discussion: Abnormal movements can be caused by manganese poisoning due to parenteral nutrition and are associated with liver failure in the ICU. Our patient showed toxic Mn concentrations in whole blood after 31 days of receiving 300 μg·d-1 of Mn in PN, a shorter duration than typically reported. Neurotoxicity was observed several months later (223 days). Factors such as liver dysfunction and iron deficiency can modulate neurotoxicity. Age may also be a susceptibility factor due to increased expression of Mn transport proteins. Magnetic resonance imaging (MRI) intensity in the globus pallidus is useful for detecting brain Mn accumulation, but it is not feasible for routine clinical practice. Conclusions: In this case, choreiform movements were attributed to manganese (Mn) accumulation in the basal ganglia. It is essential to monitor patients receiving parenteral nutrition (PN) solutions containing Mn, especially in those who have biomarkers of susceptibility, even if they have not yet shown neurological signs, and routinely measure whole-blood Mn concentrations, iron levels, age, and liver function. If Mn intoxication is suspected, a brain MRI examination should be conducted.

Vital signs monitoring (VSM) is used in clinical acuity scoring systems (APACHE [Acute Physiology and Chronic Health Evaluation], NEWS2 [National Early Warning Score 2], and SOFA [Sequential Organ Failure Assessment]) to predict patient outcomes for early intervention. Current technological advances enable convenient remote VSM. While the role of VSM for ill, hospital ward-treated patients is clear, its role in the community for acutely ill patients in the hospital at home (HAH) or postacute setting (patients who have just been discharged from an acute hospital stay and at increased risk of deterioration) is less well defined.

We assessed the efficacy of remote VSM for patients in the HAH or postacute setting.

This systematic review adhered to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) methodology. We searched studies in PubMed (MEDLINE), Embase, and Scopus. Studies focused on the postacute phase were included, as only 2 case series addressed the HAH setting. Risk of bias (ROB) was evaluated using the Cochrane Risk of Bias Tool for randomized controlled trials (RCTs), the Newcastle-Ottawa scale for observational studies, and the case methods outlined by Murad et al for case reports. The GRADE (Grading Recommendations Assessment, Development, and Evaluation) framework was used to assess the certainty of evidence. Outcomes of interest included hospital readmissions, mortality, patient satisfaction, and compliance. Risk ratios (RR) were used to measure effect sizes for readmission and mortality, with patient satisfaction and compliance reported descriptively.

The search yielded 5851 records, with 28 studies meeting eligibility criteria (8 RCTs, 7 cohort studies, and 13 case series). Two focused on HAH, while 26 studies addressed the postacute phase. Nineteen studies looked at heart failure, 3 studied respiratory conditions, and 6 studies studied other conditions. Meta-analysis was conducted with 6 studies looking at hospital readmission within 60 days and 4 studies at mortality within 30 days. Readmissions did not significantly decrease (RR 0.81, 95% CI 0.61-1.09; P=.16). Significant heterogeneity was observed for readmissions (I2=58%). Conversely, mortality reduced significantly (RR 0.65, 95% CI 0.42-0.99; P=.04). There was no significant heterogeneity in mortality (I2=0%). There was high heterogeneity in the study populations, interventions, and outcomes measured. Many studies were of poor quality, with 50% (4/8) of RCTs exhibiting a high ROB. The certainty of evidence for both readmission and mortality was very low.

Published data on the effects of remote VSM in acutely ill patients at home remains scarce. Future studies evaluating all common vital signs (heart rate, blood pressure, oxygen saturation, and temperature) with consistent monitoring frequencies and clear intervention protocols to better understand how to integrate remote VSM into HAH programs are needed.

PROSPERO CRD42023388827; https://www.crd.york.ac.uk/PROSPERO/view/CRD42023388827.

Background: Myopericytoma is a rare benign vascular tumour characterised by concentric spindle cell proliferation around blood vessels, often misdiagnosed due to its resemblance to other soft tissue masses. Dupuytren's disease (DD), a fibroproliferative disorder of the palmar fascia, causes progressive contractures, typically affecting the ring and little fingers. While these conditions are well-documented individually, their coexistence in the same region is rare and diagnostically challenging. Case Presentation: This report highlights a 67-year-old male with longstanding DD and a recurrent palmar mass initially attributed to fibrosis. Magnetic resonance imaging revealed hallmark vascular features suggestive of myopericytoma, confirmed by histopathological analysis showing spindle cell proliferation and immunohistochemical positivity for alpha-smooth muscle actin and h-caldesmon. Concurrent DD, characterised by fibrosis and activated myofibroblasts, further complicated the clinical picture. Methodology: PubMed, Scopus, Web of Science, and Embase databases were searched from January 1901 to December 2024, and 20 studies were found, reporting 41 cases of myopericytoma in hand and upper extremity. Histopathological analysis consistently showed spindle cell proliferation and smooth muscle actin positivity. Coexistence with DD was rare, highlighting the need for detailed imaging and histological evaluation for accurate diagnosis. Conclusions: This case emphasises the complexity of differentiating overlapping pathologies. Surgical excision of myopericytoma and tailored DD management yielded favourable outcomes. Further research into shared fibroinflammatory pathways, including tumour necrosis factor-alpha and interleukin-6, may enhance diagnostic accuracy and treatment strategies for overlapping conditions.

Androgenetic alopecia (AGA) is the most common form of hair loss, affecting a significant portion of both men and women. Despite the availability of various treatments, challenges such as limited efficacy, ongoing treatment requirements, side effects, and high costs persist. We sought to evaluate the efficacy and safety of erbium lasers, specifically erbium-doped yttrium-aluminum-garnet (Er: YAG) and erbium: glass lasers, in the management of AGA. A comprehensive search of PubMed/Medline, Web of Science, and Ovid-Embase databases was conducted until November 18th, 2023, adhering to PRISMA guidelines. Clinical studies involving Er: YAG or erbium: glass lasers for AGA treatment were included. A total of 12 studies recruiting 344 patients were included in this study; six of them investigated Er: YAG and six others evaluated erbium: glass laser. As a result, both erbium lasers were effective in treating AGA with respect to hair density, growth rate, and hair thickness. Erbium: glass lasers were particularly effective with combination therapies, such as those with minoxidil or Platelet-rich plasma. Er: YAG lasers also showed promising results, although some studies reported variability in patient satisfaction and aesthetic outcomes. The safety profile of erbium lasers was generally favorable, with minimal adverse effects. Erbium lasers, especially when used in combination with other therapies, show considerable potential in managing AGA. However, variability in treatment protocols and patient responses highlights the need for further research.

Bartter syndrome encompasses salt-losing tubulopathies characterized by hypokalaemia, hypochloremic metabolic alkalosis and hyperreninemic hyperaldosteronism with normal blood pressure and renal function. Acquired Bartter-like syndrome (ABLS) is commonly associated with diuretics and antibiotics such as aminoglycosides, colistin and amphotericin B. This review aims to understand the timing of Bartter syndrome onset after exposure to inciting drugs and explore its progression and management.

A systematic review was conducted following PRISMA guidelines. Databases searched include PubMed, Embase, medRxiv and bioRxiv. Case reports, case series and review articles from 1986 to March 2022 were screened. Forty-three cases were included, consisting of five case series and the rest as case reports.

The most common antimicrobial associated with ABLS was gentamicin (41.8%), followed by colistin (32.5%). The most frequent symptoms were paraesthesias (27.9%) and carpopedal spasms (27.9%), while 32.5% of patients were asymptomatic. Laboratory findings showed hypokalaemia (100%), metabolic alkalosis (97.2%), hypocalcaemia (92.5%) and hypomagnesaemia (100%), with renal wasting of these electrolytes but normal serum creatinine. The median time for Bartter-like syndrome to appear post-drug exposure was 10 days (IQR 7-18 days), with resolution occurring within 14 days (IQR 7-31 days) after drug discontinuation. Symptoms resolved completely upon cessation of the offending agent.

ABLS, though rare, should be suspected in patients on antimicrobials presenting with salt-losing tubulopathy. Aminoglycosides are the most frequently implicated drugs. Discontinuation of the offending drug, along with fluid and electrolyte management, leads to complete recovery.

Dissecting cellulitis of the scalp (DCS) is a type of neutrophilic scarring alopecia identified by the development of folliculitis with clusters of perifollicular pustules and then progresses to abscesses and intercommunicating sinus formation. In the absence of evidence-based guidelines, the treatment of DCS remains a therapeutic challenge. Our study aimed to assess the safety and efficacy of biologics, including tumor necrosis factor-α (TNF-α) blockers, anti-interleukins (ILs), and small molecule inhibitors, including Janus kinase (JAK) inhibitors and phosphodiesterase inhibitors in treating DCS.

PubMed/Medline, Scopus, and Ovid Embase databases were systematically searched until February 4th, 2024. Study selection was restricted to case reports, case series, cohort studies, and clinical trials published in English-language. NIH and Murad et al.'s quality assessment tools were utilized for critical appraisal.

A total of 34 articles involving 81 patients met the inclusion criteria. The immunomodulators studied for the treatment of DCS include adalimumab, infliximab, certolizumab pegol, ustekinumab, secukinumab, guselkumab, risankizumab, tildrakizumab, apremilast, upadacitinib, and baricitinib. Our findings implied that TNF-α blockers and IL inhibitors were associated with clinical improvement in most individuals with moderate-to-severe DCS, especially in those who had failed earlier treatments. Moreover, certolizumab pegol could be a safe option for DCS in pregnancy. In addition, the prescription of small molecule inhibitors, including JAK inhibitors and apremilast in DCS patients, demonstrated a significant amelioration in DCS symptoms with a desirable safety profile. Nevertheless, the available data was limited, warranting further investigation. Besides, all aforementioned immunomodulators are still debated for their effectiveness on hair regrowth and reversing the scarring process.

The application of immunomodulators in treating DCS was associated with satisfactory outcomes, although there is still a need to assess the long-term safety and effectiveness of these therapeutic agents in preventing disease progression and new flare-ups.

Hydroxyurea (HU) is an antimetabolite drug used to manage several hematological conditions, including chronic myeloid leukemia (CML) and sickle cell disease (SCD). Animal studies and limited human data have raised concern that HU exposures in pregnancy may increase the risk of congenital malformations or abnormal fetal growth. Although the quality of evidence is low, it has been recommended that HU is discontinued at least 3 months prior to conception.

We systematically reviewed all published studies up to July 2024 describing pregnancy and neonatal outcomes following HU exposure during pregnancy.

A total of 329 articles went through title and abstract screening, which resulted in 54 articles undergoing full-text review, and 15 articles were finally eligible for data extraction. These 15 studies included in the review, published between 1993-2023, comprised 7227 pregnancies, with 567 pregnancies (7.8%) exposed to HU. Patient ages ranged from 17 to 45 years. Most patients had SCD (n = 502), followed by CML (n = 26), essential thrombocythemia (n = 24), and chronic myeloid splenomegaly (n = 2). In 13 cases, the underlying disease was not specified. Timing of exposures to HU varied from conception till throughout pregnancy. Neither teratogenic nor hematologic effects on the fetus were observed in these cases.

Pregnancy risks associated to HU are lower than anticipated. Use of HU in pregnancy may be justified considering the significant risks associated to untreated conditions such as SCD.

Acute Mountain Sickness (AMS) is a prevalent and potentially debilitating condition affecting individuals who participate in high-altitude journeys, mostly above 2500 m. The main symptoms of AMS, listed in the Lake Louise Symptom score used to diagnose AMS, are headache, dizziness, nausea, and fatigue. However, mountaineering can also be associated with other neurological disturbances. Most records related to neurological disorders associated with high-altitude medicine focus on AMS and its typical neurological symptoms indicated in official criteria. Other conditions related to acute exposure to high altitudes are high-altitude headaches (HAH), which usually precede AMS and high-altitude cerebral oedema (HACE), which can be a complication of AMS or appear independently.

This review aimed to describe studies that included atypical neurological symptoms, which appear during acute exposure to high altitudes and are not mentioned in the criteria of AMS or HACE. Four databases, PubMed, Embase, Web of Science, and Medline Ultimate, were screened. PROSPERO registration ID for this review is CRD420250654251.

Studies that met our inclusion criteria presented symptoms related to well-known conditions, such as stroke, deep cerebral vein thrombosis, seizures, or transient neurological dysfunctions. Moreover, cranial nerve palsies, olfactory threshold impairment, multiple sclerosis worsening, or speech, memory, and sensation disturbances were described in patients at high altitudes.

This review shows that high altitude may be an inducing factor in other neurological disturbances besides AMS, HAH, and HACE symptoms. The growing popularity of high-altitude stays should be associated with increasing knowledge about the unusual neurological symptoms that may occur.

Switch access is a tool used by many individuals with physical disabilities. Switches used in assistive technology are typically electro-mechanical. Electromyography (EMG) has previously been suggested for assistive technology switch access but has been little explored.

Materials and Methods: An exploratory study aiming to investigate the feasibility, usability and performance of a dry sensor EMG switch when used by individuals with physical disabilities to control assistive technology was conducted.

Results: Twelve participants with a range of underlying conditions trialled a novel dry sensor EMG switch. Switch reaction performance data for both EMG and conventional switches and qualitative feedback from semi-structured interviews were collected and tabulated. Results showed that the EMG switch was feasible to use in a range of placements and with a range of individuals, that it was feasible in some situations where a conventional switch was not, and that it may be more appropriate for those with hypokinetic movement disorders. Some participants described use of the EMG switch as less effortful and fatiguing. Some participants had faster reaction times using the EMG switch compared to conventional switches, the fastest average reaction time reported in the study (483 ms) was using the EMG switch. More false positive activations occurred when using the EMG switch and participants described this as impacting on usability. Setup complexity was also noted as a key usability barrier.

Conclusions: This study highlights potential benefits of EMG switches but suggests further development is needed to improve ease of use and minimise false activations if EMG switches are to achieve broader adoption.

Although some reports indicate ocular involvement in Inborn Errors of Immunity (IEI) patients, the characteristics of this association remain unclear. Increased awareness can facilitate early diagnosis and prevention of visual complications.

To determine the prevalence and characterize ophthalmological manifestations in patients with IEI.

A systematic literature search was performed across Embase, PubMed, and Lilacs. Observational studies with at least 10 IEI patients exhibiting ophthalmological manifestations were reviewed. A meta-analysis using a random effects model, weighted proportion, and 95% confidence intervals were reported as appropriate.

Sixty-two articles out of the 6,884 studies were included. The pooled prevalence of ocular manifestations in IEI patients was 54% (95%CI = 39-69), with a mean age of 11.1 ± 7.8 years and male predominance. Regarding the type of IEI with ocular involvement, the most frequently affected group was the Combined immunodeficiencies with associated or syndromic features (82%, 95%CI = 66-91), followed by the diseases of immune dysregulation (73%, 95%CI = 27-95), auto-inflammatory disorders (48%, 95%CI = 10-88), and congenital defects of phagocytes (39%, 95%CI = 11-76). Europe had the highest prevalence of patients with ocular manifestations (68%, 95%CI = 32-90). The most common ocular manifestations observed in IEI patients were those affecting ocular mobility, followed by those that involved the anterior segment, posterior segment, eyelids, and adnexal structures.

These results highlight a significant burden of ocular involvement in IEI patients, mainly during childhood and associated with amblyogenic factors. Therefore, ophthalmologists, pediatricians, and immunologists must be involved in early detection to prevent ocular complications and overall well-being.

This fourth article in a seven part series presents the Core GRADE (Grading of Recommendations Assessment, Development and Evaluation) approach to addressing risk of bias, publication bias, and rating up certainty. In Core GRADE, randomised controlled trials begin as high certainty evidence and non-randomised studies of interventions (NRSI) as low certainty. To assess certainty of evidence for risk of bias, Core GRADE users first classify individual studies as low or high risk of bias. Decisions regarding rating down for risk of bias will depend on the weights of high and low risk of bias studies and similarities or differences between the results of high and low risk of bias studies. For publication bias, a body of evidence comprising small studies funded by industry should raise suspicion. Core GRADE users appraising results from well conducted NSRI can consider rating up certainty of evidence when risk ratios from pooled estimates suggest large or very large effects.

Ingested sharp objects pose increased risks of adverse events compared with other foreign bodies. We conducted the largest systematic review to date of sharp wooden object ingestions to elucidate patterns in clinical presentation and guide management practices.

Cochrane Library, Google Scholar, Ovid MEDLINE, Ovid Embase, PubMed, Scopus, and Web of Science Core Collection databases were searched for cases of adults who ingested sharp wooden objects. Descriptive statistics were reported for risk factors, clinical presentations, laboratory and imaging findings, adverse events, and treatments.

Analysis of 479 cases showed that most patients were unaware of ingestion (74.8%) and toothpicks were the most common ingested item (92.5%). Male sex (70.1%), substance use (9.4%), and edentulousness (6.1%) were risk factors. Common symptoms included abdominal pain (83.7%) and fever (36.7%). Imaging identified the object in 48.1% of cases, with computed tomography being the most sensitive (54.7%). Objects were commonly found in the gastrointestinal tract (79.3%). They were consistently found in the gastrointestinal tract when patients were aware of ingestion or imaging showed an intraluminal/transluminal location. Endoscopy visualized the objects 76.1% of the time, with successful removal in 88.8% of cases; 4.7% of patients required surgery following endoscopic removal. Adverse events included perforation (87.5%) and abscess (33.0%), with a mortality rate of 5.0%.

Ingestion of sharp wooden objects presents heterogeneously and can lead to serious complications. Endoscopic removal is safe and effective. We propose a clinical algorithm to guide physicians in diagnosing and managing suspected sharp wooden object ingestion.

Vogt-Koyanagi-Harada (VKH)-like uveitis is uniquely reported with immune checkpoint inhibitors (ICI) and BRAF/MEK inhibitors. This article aims to provide a comprehensive portrait of the comorbidities, ocular presentations, treatments, and visual outcomes of patients with VKH-like uveitis following ICI therapy.

ICIs are increasingly used in cancer therapy, but poorly understood ocular immune-related adverse events (irAEs) can lead to suspension of treatment and be vision-threatening.

We conducted a systematic review (PROSPERO #CRD42024558269) according to PRISMA guidelines. MEDLINE, Embase, CENTRAL, and Web of Science were searched for English articles published up to June 28, 2024. All study designs reporting on incident VKH-like uveitis following ICI were included. Risk of Bias was assessed using a tool modified from Murad et al. (2018).

Of 865 articles, we included 42 articles (4 observational studies, 28 case reports, 6 case series, 3 letters, and 1 editorial) from 12 countries, comprising 52 patients. The mean age was 60.0 ± 11.9 years, and 32 (61.5%) were females. Thirty-six (69.2%) had melanoma, and most were undergoing treatment with a PD-1 inhibitor alone (n = 33, 63.5%) or in combination with a CTLA-4 inhibitor (n = 10, 19.2%). The mean duration of ICI treatment before VKH-like uveitis symptoms was 22.2 ± 29.6 weeks, and the mean duration of ocular symptoms was 16.7 ± 18.6 weeks, with wide variation. Overall, 43 patients (73.1%) had imaging or exams suggesting bilateral involvement and 21 cases (40.4%) suggesting panuveitis. Only 31 cases (59.6%) met the acute initial-onset uveitis criteria, and 15 (28.8%) met the chronic phase criteria. Most (n = 47, 90.4%) required systemic or intravitreal steroids, termination of ICI (n = 31, 59.6%), and experienced full resolution or remission of visual symptoms (n = 43, 82.7%). Most articles (n = 40, 95.2%) were judged to be at medium risk of bias.

This descriptive systematic review consisted mostly of case reports, but it confirmed that a high proportion of VKH-like uveitis occur with PD-1 inhibitors and melanoma patients. VKH-like uveitis can lead to suspension of treatment. Further collaboration between oncologists and ophthalmologists is needed in the continuum of cancer care.

Non-coronary artery systemic arterial aneurysms (SAAs) are rare and an under-reported sequelae of Kawasaki disease (KD). We hypothesize that practices regarding SAA screening and management vary widely among experts and published literature. A survey was sent to members of the International KD Registry regarding their experiences and practices with SAAs in KD patients. For comparison, a systematic scoping review was conducted using PRISMA methodology, from which 25 reports with 83 patients were included. Results from each were compared. Surveys were completed by 48 (56%) of 86 IKDR investigators; 35 (73%) respondents had > 10 years of experience caring for KD patients. However, 33% of respondents had not cared for a patient with SAA. Features prompting assessment for SAA included demographics, presence and degree of coronary artery (CA) involvement, and clinical features, including prolonged/persistent fever, progressing/persistent elevation of inflammatory markers, and resistance to standard treatment. Features prompting screening were somewhat concordant with the characteristics of patients with SAA identified in the scoping review. From the survey, the initial preferred assessment included computed tomographic angiography (48%), ultrasound (29%), and magnetic resonance imaging (24%). In contrast, assessment of patients with SAA from the scoping review commonly used multiple imaging modalities. SAA often regressed, but associated complications included thrombosis, calcification, stenosis, occlusion, and collateral formation. While SAA is a known but rare complication of acute KD, there remains a gap in evidence regarding which patients are at risk, best practices for screening and management, and outcomes. Prospective cohort studies are needed.

Custom acetabular components (CACs) have demonstrated promising results in revision total hip arthroplasties with substantial acetabular bone loss. However, previous systematic reviews are limited by short follow-up. The purpose of this systematic review was to report midterm outcomes of CACs in revision total hip arthroplasties.

The Preferred Reporting Items for Systematic reviews and Meta-Analyses guidelines were followed. We searched MEDLINE, EMBASE, Cochrane Databases, and Scopus databases for English publications with a minimum 2-year follow-up. There were 14 studies that met the inclusion criteria, with 592 CACs available for rerevision and implant removal analysis and 603 available for reoperation analysis. The mean age was 63 years, the mean body mass index was 29, and 68% were women. The mean follow-up was 7 years.

The CAC rerevision rate was 19 per 1,000 person-years of follow-up (n = 84), the all-cause rerevision rate was 21 per 1,000 person-years (n = 93), and the CAC removal rate was eight per 1,000 person-years (n = 36). The most common indications for CAC rerevision included dislocation (n = 31), periprosthetic joint infection (PJI, n = 30), and CAC aseptic loosening (n = 12). The indications for CAC removal included PJI (n = 22), aseptic loosening (n = 12), and dislocation (n = 2). In addition to the 93 rerevisions, there were 37 reoperations (28 per 1,000 person-years). The mean Harris Hip Score improved from 38 to 75 at final follow-up.

In this systematic review including over 600 CACs, the rerevision rate was 19 per 1,000 person-years, but 94% of CACs were retained at a mean of 7 years of follow-up. Considering the complexity of these cases, CACs have acceptable survivorship. Dislocation and PJI contributed to the high rerevision rate, while PJI and aseptic loosening were the most common reasons for implant removal.

Level IV, Systematic Review of case series.

The coexistence of parasellar meningiomas and anterior circulation aneurysms is rare, and their anatomical proximity complicates diagnosis and treatment, making management challenging and risky. This study aims to assess the literature on the treatment strategies, clinical-surgical outcomes, and complications in such complex cases. We searched Pubmed, Embase, Scopus, and Web of Science databases following PRISMA guidelines for studies reporting clinical and/or surgical outcomes related to concomitant parasellar meningiomas and anterior circulation aneurysms. Thirteen case reports and one case series were included, involving a total of 14 patients aged between 46 and 81 years, with 33% male. Only five studies reported tumor histopathology, with four cases of transitional meningioma. Most aneurysms were ipsilateral (8 cases), while 3 were both ipsilateral and contralateral, and 3 were bilateral. The most common presenting symptoms were visual disturbances in 50% (n = 7) of cases and headaches in 42.85% (n = 6). The sequence of interventions varied among patients, with 78,5% (n = 11) undergoing simultaneous treatment for both lesions and 21,5% (n = 3) undergoing interventions at different times. Only six studies reported the follow-up duration, which ranged from 3 months to 5 years. Postoperative outcomes varied across studies. It was reported that nine patients had no neurological deficits following treatment. In one case, postoperative complications were reported, including bilateral anosmia and normal-pressure hydrocephalus following a bilateral frontal craniotomy. This systematic review highlights the complexity and rarity of managing concomitant parasellar meningiomas and anterior circulation aneurysms. Overall, the treatment of this association was safe and effective in the fourteen patients found in the literature. However, the available data is limited by a lack of comprehensive reporting on crucial information such as resection and long-term outcomes and comprises only case reports. This review contributes to the literature by synthesizing the available evidence and highlighting the role of endovascular approaches in treating those complex cases.

Pemphigus is a severe autoimmune blistering disorder that significantly affects patients' quality of life. While corticosteroids and immunosuppressive agents are commonly used, they have substantial side effects, highlighting the need for safer alternatives. Apremilast, an oral phosphodiesterase 4 (PDE4) inhibitor, has shown efficacy in treating other autoimmune diseases and may offer promise for pemphigus. This systematic review evaluated the clinical outcomes, safety, and potential role of apremilast in pemphigus treatment by synthesizing available case reports and series. A literature search was conducted across multiple databases (PubMed, EMBASE, Cochrane, Web of Science, ScienceDirect, and Google Scholar) for case reports and series involving apremilast in pemphigus. Inclusion criteria were a confirmed pemphigus diagnosis and apremilast treatment. Five studies (four case reports and one case series) involving seven patients were included. Apremilast led to significant clinical improvement in four patients, with reductions in disease activity, lesion severity, and symptom scores (Pemphigus Disease Area Index, Autoimmune Bullous Skin Disorder Intensity Score, Visual Analog Scale, and Numerical Rating Score). Increases in regulatory T cells and decreases in anti-desmoglein antibodies were observed. No serious adverse events were reported, although one study noted treatment failure, possibly due to short follow-up or concurrent infections. Apremilast appears to be a promising treatment for therapy-resistant or corticosteroid-intolerant pemphigus patients. Although the evidence is limited, it supports apremilast's efficacy and favorable safety profile. Further research with larger sample sizes and randomized controlled trials is necessary to confirm these findings.

AbstractCOVID-19 may primarily cause respiratory symptoms but can lead to long-term effects known as long COVID. COVID-19-induced diabetes mellitus was reported in many patients which shares characteristics of types 1 and 2 (T1DM and T2DM). This study aims to identify and analyze the reported cases of new onset diabetes post-COVID-19 infection. Several databases were used to conduct a comprehensive literature search to target studies reporting cases of T1DM or T2DM post-COVID-19 infection. Screening, data extraction, and cross checking were performed by two independent reviewers. Only 43 studies met our inclusion criteria. Our results revealed that the overall prevalence of new onset diabetes post-COVID-19 was 1.37% with higher prevalence for T2DM (0.84%) as compared to T1DM (0.017%) while the type of diabetes was not reported in 0.51% of the cases. Several risk factors for developing diabetes post-COVID-19 infection were identified including the type of SARS-CoV-2 variant, age, comorbidities and the vaccination status. The direct viral attack of the pancreatic beta cells as well as inflammation and the anti-inflammatory corticosteroids were proposed as possible mechanisms of the COVID-19 induced diabetes. A multidisciplinary approach involving endocrinologists, primary care physicians, and infectious disease specialists should be implemented in the management of post-COVID patients to address both the acute and long-term complications, including metabolic changes and risk of diabetes.

We investigate the clinical characteristics and complications of retinal vasculitis (RV), categorizing cases into Secondary RV (associated with systemic disease), Syndromic RV (linked to ocular syndromes without systemic disease), Idiopathic RV (without systemic disease or ocular syndrome diagnoses), and Multiple Etiology RV (cohorts of retinal vasculitis with more than one subcategories of the above). A systematic search was conducted on June 14, 2023, across PubMed, Embase, Cochrane (Ovid), VHL, and ProQuest databases, following PRISMA guidelines (PROSPERO registration: CRD42023489232). Out of 5533 screened articles, 97 studies involving 7619 patients with RV met the eligibility criteria. Bilateral involvement (64 %) and reduced vision (52 %) were common across all RV categories, with Idiopathic RV showing the highest rates of bilateral involvement (80 %) and vision loss (79 %). Syndromic RV was characterized by retinal ischemia (76 %) and vitreous hemorrhage (46 %), while Secondary RV exhibited higher incidences of cystoid macular edema (32 %) and neovascular glaucoma (24 %). Geographic variations were evident in Multiple Etiology RV, with inflammation in more than 1 intraocular structure more prevalent in Asia (64 %) than in Europe (29 %). These findings highlight the heterogeneity in RV presentation and complications, illustrating the need for standardized diagnostic criteria and improved clinical reporting to enable better classification, treatment strategies, and patient outcomes.

Andexanet alfa, a Food and Drug Administration (FDA)-approved antidote for apixaban and rivaroxaban, is used to manage life-threatening or uncontrolled bleeding. In patients undergoing cardiopulmonary bypass (CPB), preoperative exposure to andexanet can cause severe heparin resistance, necessitating effective mitigation strategies. A comprehensive review of such strategies remains lacking.

This study aimed to systematically review and characterize cases of andexanet-induced heparin resistance in patients undergoing CPB and to evaluate management strategies.

A systematic search was conducted across multiple databases via the Ovid interface, Cochrane Central Register of Controlled Trials, and the FDA Adverse Event Reporting System. Quality appraisal was performed using a validated instrument for case reports and series describing drug-induced adverse events.

Fourteen discrete patient cases met inclusion criteria. After andexanet administration, the mean initial activated clotting time (ACT) was 199.5 seconds, falling short of a target of ≥400 seconds despite additional heparin dosing (mean total, 1123 U/kg). Moreover, 35.7% of all cases involved thrombus formation in the reservoir, 2 of which required a circuit replacement. Antithrombin (AT) concentrate was administered to 75% of those received an adjunct therapy. A prophylactic AT use (mean, 49.9 IU/kg) resulted in an ACT over 400 seconds, while its effects in low dose after the occurrence of thrombosis varied on ACT values. Nafamostat mesylate was used in some cases reported from Japan.

Heparin resistance following andexanet exposure poses significant procoagulant risk during CPB. Pre-emptive high-dose AT therapy may improve ACT values. Further studies are needed to understand the mechanisms and optimize management of this condition.

Cerebellar ataxia, mental retardation, and disequilibrium syndrome type 4 (CAMRQ4) is a rare autosomal recessive neurological disorder caused by biallelic variants in the ATP8A2 gene. It is characterized by severe psychomotor impairment, hypotonia or spasticity, and intellectual disability. Despite increasing case reports, the full phenotypic spectrum remain incompletely defined.

We report the case of a 7-year-old girl born to consanguineous parents, presenting with severe psychomotor delay, quadriplegia, and craniofacial dysmorphisms. Whole exome sequencing identified a novel splicing variant in ATP8A2 (NM_016529.6:c.1580-3C > G). In silico tools predicted a disruption of the canonical splice acceptor site. To confirm the splicing effect, RNA was extracted from peripheral blood, followed by cDNA synthesis and PCR amplification of the region flanking the variant. Products were analyzed via gel electrophoresis.

Experimental validation revealed skipping of exon 18, confirming a significant impact on splicing and supporting the reclassification of the variant as "likely pathogenic" based on ACMG criteria (PM2, PP3, and now PS3). Additionally, a systematic literature review of published CAMRQ4 cases was conducted to delineate the clinical heterogeneity associated with ATP8A2 variants.

This case expands the mutational spectrum of ATP8A2 and provides strong evidence for the pathogenicity of a novel splicing variant. Our findings emphasize the clinical and genetic heterogeneity of CAMRQ4 and highlight the critical role of functional RNA studies in variant interpretation. Comprehensive genotype-phenotype correlation through systematic review enhances our understanding of ATP8A2-related disorders.

Aesthetic medicine has evolved towards minimally invasive procedures, with biostimulators like Poly-L-Lactic Acid (PLLA), Calcium Hydroxylapatite (CaHA), and Polycaprolactone (PCL) gaining attention for their role in collagen induction, improving skin texture, elasticity, and volume. Combining these agents with other treatments-such as botulinum toxin, dermal fillers, and energy-based devices (e.g. laser and radiofrequency therapies)-is hypothesised to provide enhanced aesthetic outcomes. However, studies on the efficacy and safety of these combinations remain sparse and methodologically varied, posing challenges in establishing definitive recommendations.

This systematic review adhered to PRISMA guidelines, involving a thorough literature search across PubMed, MEDLINE, Embase, and Cochrane databases. The search included terms related to biostimulators and combination treatments. Studies meeting inclusion criteria reported clinical outcomes of combined biostimulator treatments, including effectiveness, safety, patient satisfaction, and adverse effects. Key parameters extracted included treatment area, combination protocols, and outcomes. Data synthesis used a narrative approach due to variability in methodologies, treatment protocols, and outcome metrics.

Out of 1,237 studies initially identified, 29 met the inclusion criteria. These studies included various combinations of biostimulators with botulinum toxin, dermal fillers, and energy-based devices, with sample sizes ranging from 10 to 350 subjects. Treatments combining CaHA or PLLA with energy-based modalities like high-intensity focused ultrasound (HIFU), fractional lasers, and microneedling demonstrated notable improvements in skin texture, elasticity, and contouring, particularly in areas with ageing signs. Adverse events included erythema, bruising, and nodules in 15-30% of cases, with rare but severe complications such as granulomas and vascular occlusions. Management protocols for these events involved corticosteroids, hyaluronidase, or surgical intervention. The review also found a lack of molecular understanding of the synergistic mechanisms.

The review underscores the potential benefits of combined treatments in aesthetic outcomes, though limitations like heterogeneous methodologies, small sample sizes, and inconsistent protocols impact the reliability of findings. Current literature lacks a molecular understanding of the mechanisms underlying these combinations, limiting insights into the longevity and safety of results. Future studies with standardised protocols, objective outcome measures, and detailed molecular analyses are essential for developing evidence-based recommendations for combining biostimulators with other treatments in aesthetic practice.

This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors   www.springer.com/00266 .

To perform a systematic review describing the most common ultrasonographic findings of the hemostatic agent Surgicel® and to identify different ultrasonographic patterns depending on ultrasound timing and localization of Surgicel®.

A systematic literature search was performed to identify studies reporting ultrasonographic findings of Surgicel® according to the PRISMA guidelines. We queried PubMed, Web of Science, and Scopus using the terms "Surgicel®" OR "oxidized regenerated cellulose" AND "Ultrasound" from inception to march 2024. Patients with Surgicel® application during surgeries and posterior description of ultrasound features were included in the study. Risk of bias was evaluated with a modified standardized tool. (PROSPERO ID CRD42024542619).

We found 464 articles, from which 12 articles were included, with a pooled population of 226 patients. Most rating as a moderate risk of bias. The predominant anatomical sites investigated were the breast and thyroid gland (94.68 %). The most frequent ultrasound appearance of Surgicel® was a hypo/isoechoic lesion with well-defined margins and internal hyperechoic nodules (49.11 %), followed by a hypo/isoechoic lesion with well-defined margins without internal hyperechoic nodules (18.58 %) and completely anechoic lesions (15.04 %). Hypo/isoechoic lesions with well-defined margins and internal hyperechoic nodules were frequent in the breast (55.56 %) and thyroid (53.49 %). Hyperechoic lesions with posterior reverberation artifact were consistently found in the liver, spleen, coronary vessels, and cervix (100 % each).

The most frequent ultrasound finding of Surgicel® was hypo/isoechoic lesions with well-defined margins and internal hyperechoic nodules. There may be distinct ultrasound characteristics of Surgicel® based on location. The impact of the type of organ, and timing of ultrasound remains to be defined.

Extended C2 laminectomy may expand the formation of the neocisterna magna in Chiari malformation (CM) surgery to improve cerebrospinal fluid (CSF) flow in the craniovertebral junction (CVJ). We aimed to clarify indications, outcomes, and complications of C2 laminectomy in CM. A systematic review (PROSPERO CRD42022340553) following the PRISMA protocol was performed in Pubmed, Web of Science, Cochrane, Embase, and Scopus databases with the Medical Subject Headings-based search strategy "("Arnold-Chiari Malformation") AND ("Decompression, Surgical" OR "Axis, Cervical") AND (Headache OR Vertigo OR "Deglutition Disorders")". The endpoints were symptom improvement and complication rates. Thirteen original articles included were case reports or series. The extent of laminectomy was directly related to the degree of tonsillar herniation. Six studies presented the perception of improvement (Gestalt) range from 83% to 100%. Three articles applied objective scales to report improvement in headache (VAS) and strength (MRC or ASIA). Nine studies reported complication rates. Fistula occurred in a range of 2% to 9.5% of cases. Three articles presented data on multilevel laminectomy for CM, and five studies reported on pediatric cases. Spine deformity was observed in children after wide multilevel laminectomy. This review showed a few concerns about C2 laminectomy. The descent of tonsilar herniation was the parameter for removing C2 lamina with good global results and complication rates. C2 laminectomy can be performed in young patients without previous instability. However, the lack of stratification of cases and systematization of the evaluation of results compromised the comparative analysis of the usefulness of C2 laminectomy in treating CM.

Syndromic craniosynostosis is a complex condition involving the craniofacial skeleton, often requiring surgical midface advancement (SMA) to address functional and aesthetic concerns. A systematic review of literature was conducted on the impact of Lefort III/monobloc advancement on midface growth of children with syndromic craniosynostosis. Comprehensive search of multiple databases yielded 21 studies that met the inclusion criteria. Data extraction and risk-of-bias assessment were performed using standardized tools. The review included 309 children who underwent SMA at a median age of 7-8 years. While some studies reported positive post-operative growth, others found no evidence of growth. The type of surgical intervention (osteotomy vs. distraction) and age at surgery did not consistently influence growth outcomes. Vertical growth of the midface was uninterrupted post-operatively, whereas sagittal growth was minimal. This systematic review highlights the variability in midface growth outcomes following LeFort III/monobloc advancement in children with syndromic craniosynostosis. Further research is needed to elucidate the factors influencing growth and to optimize surgical techniques for improved outcomes.

The outbreak of COVID-19 pandemic has raised urgent vaccine development to prevent viral transmission. Cutaneous adverse events such as erythema multiforme (EM), Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN) have been observed following COVID-19 vaccination. In this systematic review, we aimed to investigate the clinical features and outcomes of EM/SJS/TEN following COVID-19 vaccination. A comprehensive literature search was conducted in PubMed, Embase, Web of Science, and Cochrane databases up to July 3, 2022. We included studies reporting patients who developed EM, SJS, or TEN following COVID-19 vaccination. A total of 47 studies involving 90 patients with EM and 16 patients with SJS/TEN were reviewed and outlined. EM predominantly occurred after the messenger ribonucleic acid vaccines (70.4%), mostly after the first (47.5%) and second doses (42.4%), with delayed onsets ranging from 1 day to 30 days. SJS/TEN were observed following either the first (55.6%)- or second-dose (33.3%) vaccination, with onset times ranging from 6 hours to 14 weeks. Three EM cases and 1 SJS case showed recurrence upon reexposure to the same vaccines. No mortality was reported. Most patients exhibited improvement or resolution after treatment, with resolution times ranging from 6 days to 8 weeks. In conclusion, EM and epidermal necrolysis, including SJS and TEN, have emerged as potential cutaneous adverse events following COVID-19 vaccine administration. Further research is warranted to elucidate the pathogenesis and casual relationship between COVID-19 vaccines and EM/SJS/TEN.

This systematic review and meta-analysis evaluates the current evidence on the management of intermittent claudication (IC), a prevalent manifestation of peripheral arterial disease (PAD).

We conducted comprehensive searches of MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews, and Scopus. We addressed six questions developed by a guideline committee from the Society for Vascular Surgery, addressing pharmacological treatments, exercise regimens, endovascular interventions, and predictors of major adverse cardiovascular, limb-related events, and mortality.

The search resulted in 5333 citations, from which we included 73 studies (46 randomized trials). In patients with PAD and IC who had one or more high-risk comorbidities, low-dose rivaroxaban and aspirin were associated with lower risk of major adverse limb events and major adverse cardiovascular events than aspirin alone. In patients who have undergone surgical or endovascular interventions for PAD, the addition of low-dose rivaroxaban to aspirin may improve limb outcomes. Of note, rivaroxaban trials excluded patients at high risk of bleeding. Single antiplatelet agents showed no significant efficacy differences head-to-head in ambulatory patients with IC and had a lower bleeding risk compared with combination therapy or anticoagulation. Home exercise programs were feasible and may be an alternative to supervised exercise in ambulatory patients with IC and in those who had revascularization. Several comorbidities increased the risk of adverse outcomes after revascularization for IC, such as advanced age, diabetes, coronary artery disease, chronic obstructive pulmonary disease, previous interventions, congestive heart failure, infrapopliteal artery involvement, and longer lesion lengths. In patients with IC undergoing endovascular intervention for superficial femoral artery disease, plain balloon angioplasty was associated with worse outcomes than drug elution or stent implantation for intermediate or longer lesions (ie, >5 cm).

This systematic review summarizes the current evidence base for the management of IC, offering insights into the relative benefits and risks of various therapeutic strategies. The findings underscore the need for individualized patient care, considering both the potential benefits and risks associated with different interventions.

ObjectiveMirrored self-misidentification syndrome (MSMS) is a rare form of delusional misidentification syndrome characterized by the inability to recognize one's own reflection. We conducted a systematic review aiming to describe the epidemiology, clinical presentation, and management of individuals with MSMS.MethodsA comprehensive literature search was performed using original case reports/series on patients with MSMS. Univariate analyses were performed to assess patient demographics, clinical, paraclinical, and treatment-related characteristics. The methodological quality of included articles was evaluated using a standardized tool.ResultsOf 76 articles screened, 28 were included, with 36 patients analyzed. Median age was 77.0 (interquartile range: 72.0, 80.0) years; most patients were female (60.7%). Over half of the cases had a diagnosis of dementia, mostly Alzheimer's disease (50.0%), Lewy Body Disease (20.0%), and vascular dementia (10.0%), while the other diagnosis included stroke (3.3%), schizophrenia (3.3%), schizoaffective disorder (3.3%), and rabies (3.3%). Initial clinical manifestations included psychiatric symptoms (66.7%) and cognitive decline (70.0%). Brain magnetic resonance (MRI) was reported in 31 cases, with 14 cases (45.1%) showing right hemisphere dysfunction. Pharmacological interventions were effective for twelve cases (48.0%), and non-pharmacological interventions such as covering mirror were effective for 8 cases (32.0%). Most included articles (64.3%) were evaluated to be at low risk of bias.ConclusionsMSMS are rare conditions that mostly present in patients with dementia. Despite the varied clinical presentations, frontal and right hemisphere dysfunctions appear to play a role in the pathophysiology of MSMS, adding to the evidence supporting "a neuroanatomy of the self" in the non-dominant hemisphere.

BackgroundHepatic portal venous gas (HPVG) is an uncommon radiological finding in acute pancreatitis. This systematic review aims to consolidate existing literature on HPVG in acute pancreatitis and assess its clinical significance, particularly regarding surgical intervention. We also report a clinical case from our center.MethodsA systematic search was conducted across Medline, Scopus, Cochrane, and Google Scholar databases to identify studies reporting concurrent HPVG and pancreatitis. Data included demographics, clinical presentation, management, and outcomes. Descriptive statistics were employed for analysis, and methodological quality was assessed using established criteria.ResultsOut of 259 articles screened, 13 met inclusion criteria, yielding data on 17 patients (76.5% male; median age 62 years). Nonoperative management (NOM) was utilized in 75% of cases and was associated with a 54% mortality rate. In contrast, the surgical cohort (n = 4) experienced a similar overall mortality rate of 50%. Importantly, necrotic bowel was implicated in 44% of evaluable cases and was uniformly fatal. However, 2 patients who underwent early surgical resection of nonviable bowel survived the initial phase of their illness, suggesting that prompt operative intervention may confer an early survival advantage in selected patients. Fluid collections and pneumatosis intestinalis were significantly more prevalent in deceased patients (100% vs 17% in survivors; P < 0.0034). All deceased patients had either severe or necrotizing pancreatitis.ConclusionHPVG associated with severe acute pancreatitis appears to have a higher mortality than HPVG alone. It can be managed nonoperatively but when indicative of bowel necrosis, necessitates prompt surgical intervention.

PharyngoCutaneous Fistulae (PCF) represent a significant clinical challenge in otorhinolaryngology practice. The use of biomaterials could improve outcomes or even replace the use of a flap in pharyngolaryngeal surgery.

This systematic review included all studies evaluating the application of biomaterials for the prevention of PCF after pharyngeal surgery.

A total of 19 articles (12 clinical and 7 animal studies) were included. They assessed the use of a biomaterial to cover mucosal sutures (n = 15) or to replace a mucosal defect and guide secondary healing (n = 4). Most studies evaluated commercialized biomaterials (n = 11) rather than experimental devices (n = 5) or platelet-derived autologous medical devices (n = 4).

There are already strong arguments to support the use of several biomaterials to cover pharyngeal sutures in order to decrease the rate of PCF after TL or TPL. Replacing a pharyngeal mucosal defect by a biomaterial is currently a more elusive goal.

In patients with Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS; population), is the presence of functional endometrium (FE) in uterine remnants (URs) (exposure), compared with the absence of FE in UR/absence of UR (comparison), associated with a higher prevalence of endometriosis (outcome)?

The aggregate prevalence of endometriosis was considerably higher in MRKHS patients with FE (MRKHSFE+) than in those without FE (MRKHSFE-).

The pathogenesis of endometriosis is not fully understood. The finding of pelvic endometriosis in patients with MRKHS is one of the main objections to the retrograde menstruation (RM) hypothesis. The recent advent of high-resolution ultrasonography and magnetic resonance imaging (MRI) allowed the reliable preoperative identification of FE concealed within UR, and histopathological examination after UR removal is no longer the only means of verifying the presence of a mucosal component. A similar prevalence of endometriosis in MRKHSFE+ and MRKHSFE- patients, as assessed by preoperative ultrasound (US) and/or MRI, would essentially rule out the RM/implantation theory, whereas a substantially higher prevalence of endometriosis in MRKHSFE+ than in MRKHSFE- patients would challenge the embryonic remnants/coelomic metaplasia hypothesis.

This systematic review was restricted to full-length, English-language articles published in peer-reviewed journals between 1 January 1980 and 1 June 2024. The electronic PubMed and Embase databases were searched in June 2024. The keyword 'endometriosis' was used in combination with 'Mayer-Rokitansky-Küster-Hauser syndrome', 'Müllerian agenesis', 'uterine agenesis', 'vaginal agenesis', 'Müllerian anomalies', and 'female genital malformations'. References from relevant publications were screened, and PubMed's 'similar articles' and 'cited by' functions were used.

Studies were selected if they reported the presence or absence of FE within UR investigated by preoperative US or MRI or histology after surgical removal, and the presence or absence of surgically confirmed endometriosis. Case series and case reports were deemed eligible for inclusion. Studies not specifically stating the intent to search for the presence of endometrium within UR, or not reporting the results of ultrasonography or MRI, or histological examinations were excluded. Two reviewers independently abstracted data. The risk of bias was assessed using a tool specifically devised to ascertain the methodological quality of case series and case reports.

A total of 102 studies (29 case series and 73 case reports), comprising 666 MRKHS patients in whom the presence or absence of FE was verified were included. Endometriosis was detected in 71 participants (10.7%; 95% CI, 8.5-13.2%), and its prevalence was 8.6% (51/593; 95% CI, 6.6-11.2%) in case series and 27.4% (20/73; 95% CI, 18.4-38.6%) in case reports (P<0.0001). When considering only the 19 case series with ≥10 participants, the proportion of MRKHS patients with endometriosis was 3.4% (41/1219; 95% CI, 2.5-4.5%). Among the 71 MRKHS patients with endometriosis, 64 had coexisting FE, and only seven had no evidence of FE within UR or did not have UR. The proportion of patients with endometriosis was 32.0% in the subgroup with FE (64/200; 95% CI, 25.9-38.8%) and 1.5% (7/466; 95% CI, 0.7-3.1%) in the subgroup without FE within UR/without UR. At meta-analysis considering case series, the overall prevalence estimates of endometriosis in patients with and without FE were, respectively, 16.8% (95% CI, 1.8-38.5%) and 0% (95% CI, 0-0%). In order to evaluate the association between FE and endometriosis we also conducted a meta-analysis that included case series reporting both FE+ and FE- patients. A significantly increased risk of endometriosis was observed in MRKHSFE+ patients compared with MRKHSFE- patients (overall odds ratio estimate was 12.0; 95% CI, 5.1-28.3%). The quality of the evidence score was higher in the case series subgroup (median score, 4 points; interquartile range, 3-5 points) than in the case reports subgroup (median score, 3 points; interquartile range, 2-4 points).

Due to the uncontrolled and non-experimental study design, case series and case reports are associated with an increased risk of selection, performance, detection, attrition, and reporting bias. In the seven cases of endometriosis in patients purportedly without detected FE, a preoperative MRI was not systematically performed or erroneous findings were reported, the anatomical description at surgery was incomplete or inconsistent, the histopathological diagnosis of endometriosis was missing or questionable, and precise microscopic features were not always described. Whether FE (exposure) was truly absent in all these cases and/or whether all lesions diagnosed as endometriosis (outcome) were indeed true disease, seems uncertain.

Our findings should raise awareness of the importance of accurately assessing and reporting the presence or absence of FE within UR, and of systematically performing biopsies of visually diagnosed endometriosis in MRKHS patients. Considering the high risk of bias, the detection of endometriosis in MRKHS patients allegedly without FE in the few relevant case reports published in the last four decades should no longer be interpreted tout court as proof for the coelomic metaplasia/embryonic remnants theory.

No funding was received for this review. P.Ve. is a member of the Editorial Board of Human Reproduction Open, the Journal of Obstetrics and Gynaecology Canada, and the International Editorial Board of Acta Obstetricia et Gynecologica Scandinavica; has received royalties from Wolters Kluwer for chapters on endometriosis management in the clinical decision support resource UpToDate; and maintains both a public and private gynaecological practice. E.S. is Editor-in-Chief of Human Reproduction Open; discloses payments from Ferring for research grants and honoraria from Merck-Serono for lectures; and maintains both a public and private gynaecological practice. P.Vi. is Co-Editor-in-Chief of Journal of Endometriosis and Uterine Disorders. All other authors declare they have no conflict of interest.

The study protocol was registered on PROSPERO (registration number, CRD42024512351).

The strength of evidence supporting a 2-step wide local excision (WLE) with 5 mm safety margins for melanoma in situ non-lentigo maligna (non-LM) type, compared with narrower margins, is unclear. This review aims to compare the frequency of local recurrence (LR) with different safety margins, after the complete surgical excision (R0) of non-LM MIS. We performed a systematic literature search in PubMed, Scopus, and the Cochrane Library up to March 17, 2024. The PRISMA checklist was used. Of 3,047 articles retrieved, seven retrospective studies were included, enrolling a total of 1,526 non-LM MIS cases excised with clear safety margins, after WLE (6 studies) or Mohs surgery (1 study). Most non-LM MIS were located on the trunk/extremities (68%-100%). Narrower margins were used in four studies, ranging from no WLE to 4 mm, and there was only one LR. Standard or wider margins were used in the remaining three studies reporting only two LR. The median follow-up ranged from 48 months to 6.6 years. The overall certainty and quality of evidence were very low. These findings of our systematic review highlight that current guidelines recommending the re-excision for non-LM MIS with clear initial margins lack strong evidence in support of this practice.