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VEXAS syndrome is a rare auto-inflammatory disorder characterized by heterogeneous inflammatory and hematologic features. First identified in 2020, it predominantly affects men over the age of 50. Clinical manifestations commonly include recurrent fever, weight loss, skin lesions, and diverse inflammatory presentations across multiple organ systems. Diagnosing VEXAS syndrome can be challenging due to the overlap of symptoms with other autoimmune and inflammatory conditions, often requiring extensive medical evaluation before a definitive diagnosis is made. This case report highlights these diagnostic challenges and underscores the importance of early recognition to improve clinical management and outcomes. [ABSTRACT FROM AUTHOR]

ABSTRACT Topical steroid withdrawal syndrome (TSW) is a debated condition marked by burning erythema, severe itching, and dry skin following the discontinuation of topical corticosteroids (TCS). This study reviewed reported pediatric TSW cases. With a total of 21 cases reported (inconsistent data provided), 60% (6/10) used TCS on the face; 69% (9/13) were associated with an escalation in potency of TCS and 75% (3/4) were the erythematoedematous variant. Overall, data on TSW in children is lacking and standardized diagnostic criteria are needed. [ABSTRACT FROM AUTHOR]

This is the English version of the guidelines for the diagnosis and treatment of idiopathic portal hypertension, extrahepatic portal obstruction, and Budd–Chiari syndrome, which were established and revised in 2018 by the Aberrant Portal Hemodynamics Study Group under the jurisdiction of the Ministry of Health, Labor, and Welfare in Japan. These guidelines are excerpts, and the full version consists of 86 clinical questions and explanations, totaling 183 pages in Japanese. [ABSTRACT FROM AUTHOR]

We report a human case of severe fever with thrombocytopenia syndrome virus infection transmitted by a tick, confirmed by viral identification. Haemaphysalis aborensis, a tick species not native to Japan that has been observed to transmit the virus to humans, is now recognized as a potential vector of this virus in Japan. [ABSTRACT FROM AUTHOR]

Background: Isolated limb perfusion (ILP) is a regional cancer treatment in which high-dose chemotherapy is administered in an isolated extremity. The main side effect is regional toxicity, which occasionally leads to nerve damage. Measuring neuroaxonal biomarkers, might be a method predicting such complications. Therefore, the primary aim of the study is to investigate if neuronal biomarkers are measurable and alters in an isolated extremity during ILP. Secondly, if postoperative regional toxicity, alterations in sensitivity, and/or muscle strength are correlated to the biomarker levels. Methods: Eighteen scheduled ILP-patients were included in the study. Glial fibrillary acidic protein (GFAP), neurofilament light (NfL), and tau concentrations were measured in plasma sampled preoperatively, at the start and end of the ILP, on days 3 and 30, using ultrasensitive Single molecule array (Simoa) technology. The patients were assessed by a physiotherapist pre- and postoperatively. Results: At ILP end, significantly higher NfL and tau levels were measured in the extremity than in the corresponding systemic circulation (NfL; 17 vs 6 ng/L, p <.01, tau; 1.8 vs 0.6 ng/L, p <.01), and the extremity levels were significantly increased at ILP end (NfL; 66 ± 37%, p <.001, tau; 75 ± 45%, p =.001). On days 3 and 30, significantly increased NfL and GFAP levels were measured systemically (NfL day 3: 69 ± 30%, p <.001; day 30: 76 ± 26%, p <.001; GFAP day 3: 33 ± 22%, p <.002; day 30: 33 ± 23%, p ≤.004). Finally, no significant correlations were found between regional toxicity or between postoperative muscle or sensitivity decrease and biomarker release. Conclusion: During ILP, NfL and tau levels increased significantly. No obvious correlations were observed between biomarker release and regional toxicity or decreased muscle strength or sensitivity, although large-scale studies are warranted. [ABSTRACT FROM AUTHOR]

This paper investigates the impact of boundary-spanning search on innovation performance, with knowledge recombination as the underlying mechanism. It also explores the contingent effect of in house training of employees by considering not invented here syndrome derived from attitude theory, and examines communication investment as a second-order moderator. We test the hypotheses based on a sample of 184 Hong Kong manufacturing firms. The results show that boundary-spanning search is positively related to innovation performance, which is negatively moderated by in house training of employees. Moreover, communication investment has a suppressing effect on the moderating role of in house training of employees. This paper highlights the not invented here syndrome and its mechanism of attitude theory to examine the contingent effects, providing insights for managers to balance among in house training of employees, communication investment, and boundary-spanning search to enhance innovation performance. [ABSTRACT FROM AUTHOR]

How animals move and associate with conspecifics is rarely random, with a population's spatial structure forming the foundation on which the social behaviours of individuals form. Studies examining the spatial–social interface typically measure averaged behavioural differences between individuals; however, this neglects the inherent variation present within individuals and how it may impact the spatial–social interface. Here, we investigated differences in among-individual (co)variance in sociability, activity and site fidelity in a population of wild estuarine crocodiles, Crocodylus porosus, across a 10-year period. By monitoring 118 crocodiles using coded acoustic transmitters and an array of fixed underwater receivers, we discovered that not only did individual crocodiles repeatably differ (among-individual variation) in each behaviour measured but also in how consistently they expressed these behaviours through time (within-individual variation). As expected, crocodile activity and sociability formed a behavioural syndrome, with more active individuals being less sociable. Interestingly, we also found that individuals that were either more sociable or displayed greater site fidelity were also more specialized (lower within-individual variation) in these behaviours. Together, our results provide important empirical evidence for the interplay between spatial, temporal and social individual-level behavioural variation and how these contribute to forming behavioural niches. This article is part of the theme issue 'The spatial–social interface: a theoretical and empirical integration'. [ABSTRACT FROM AUTHOR]

Introduction Methods Results Conclusions Carotid sinus syndrome (CSS), characterized by exaggerated vagal responses leading to asystolic pauses with carotid sinus massage (CSM), often necessitates pacemaker implantation. This study investigates cardioneuroablation (CNA) as an alternative strategy for CSS.Prospective study of consecutive patients referred for CNA due to CSS. All patients underwent CSM, atropine test and 24‐h Holter monitoring before the procedure and at 6 months. The primary objective was the absence of any cardioinhibitory response to CSM following CNA. Secondary objectives included the combined endpoint of syncope and presyncope‐free survival, pacemaker‐free survival, differences in heart rate variation (HRV), as well as differences in the pre‐ and postprocedure atropine tests and in the SF‐36 quality‐of‐life questionnaire.A total of 13 consecutive patients (84.6% male, mean age 63.8 ± 12.3 years) were included. CSM revealed a symptomatic asystolic pause in all patients before CNA (7.3 [5.6–10.5] s). After the procedure, all the patients had a negative CSM, and only one patient (7.7%) had a positive CSM at 6 months. After a median follow‐up of 11.2 (10.6–16.3) months, syncope or presyncope‐free survival was 84.6%, and none required pacemaker implantation. There was an improvement in the energy and health change items in the SF‐36 questionnaire. There was a reduction in HR increase in the atropine test at 6 months (pre‐CNA: 66% [52–84] vs. post‐CNA 26.0% (19.8–29.3]; p = .008) and in HRV parameters.In this proof‐of‐efficacy study, performed in patients affected by asystolic CSS, CNA was effective in reducing the rate of cardioinhibitory responses, suggesting a potential efficacy in also reducing syncopal recurrences. Controlled trials are warranted to corroborate clinical findings. [ABSTRACT FROM AUTHOR]

Background Methods Results Conclusion The clinical relevance of cardiac troponin (cTn) elevation in takotsubo syndrome (TTS) remains uncertain. The present study sought to investigate the role of cardiac troponin (cTn) elevations in mortality prediction of patients with Takotsubo syndrome (TTS).Patients enrolled in the International Takotsubo (InterTAK) Registry from January 2011 to February 2020 with available data on peak cTn levels were included in the analysis. Peak cTn levels during the index hospitalization were used to define clinically relevant myocardial injury. The threshold at which clinically relevant myocardial injury drives mortality at 1 year was identified using restricted cubic spline analysis.Out of 2′938 patients, 222 (7.6%) patients died during 1‐year follow‐up. A more than 28.8‐fold increase of cTn above the upper reference limit was identified as threshold for clinically relevant myocardial injury. The presence of clinically relevant myocardial injury was significantly associated with an increased risk of mortality at 5 years (adjusted HR 1.58, 95% CI 1.18–2.12, p =.002). Clinically relevant myocardial injury was related to an increased 5‐year mortality risk in patients with apical TTS (adjusted HR 1.57, 95% CI 1.21–2.03, p =.001), in presence of physical stressors (adjusted HR 1.60, 95% CI 1.22–2.11, p =.001), and in absence of emotional stressors (adjusted HR 1.49, 95% CI, 1.17–1.89, p =.001).This study for the first time determined a troponin threshold for the identification of TTS patients at excess risk of mortality. These findings advance risk stratification in TTS and assist in identifying patients in need for close monitoring and follow‐up. [ABSTRACT FROM AUTHOR]

Background: We aimed to identify different Guillain–Barré syndrome (GBS) subtypes, demyelination, axonal degeneration, and reversible conduction failure (RCF) as early as possible by analyzing the initial clinical and electrophysiological examinations. Methods: This study retrospectively collected GBS patients between October 2018 and December 2022 at Beijing Tiantan Hospital. The diagnostic criteria for the initial electrophysiological study were based on Rajabally's criteria, and the criteria for the serial electrophysiological study were based on Uncini's criteria. All subjects underwent clinical and electrophysiological evaluations at least twice within 8 weeks. Results: A total of 47 eligible patients with GBS were included, comprising 19 acute inflammatory demyelinating polyradiculoneuropathy (AIDP), 18 axonal degenerations, and 10 RCFs. In the RCF group, 40%, 30%, and 30% patients were diagnosed as AIDP, axonal, and equivocal at the initial study, respectively. The AIDP group had significantly higher cerebrospinal fluid (CSF) protein than the RCF (123.8 [106.4, 215.1] mg/dL vs. 67.1 [36.8, 85.6] mg/dL, p = 0.002) and axonal degeneration (123.8 [106.4, 215.1] mg/dL vs. 60.8 [34.8, 113.0] mg/dL, p < 0.001) groups. The RCF group had significantly lower Hughes functional grades at admission (3 [2, 4] vs. 4 [4, 4], p = 0.012) and discharge (1.0 [1.0, 2.0] vs. 3.0 [2.0, 3.0], p < 0.001) than the axonal degeneration group and showed significantly shorter distal motor latency (DML), Fmin, Fmean, Fmax, and lower F% than the AIDP group (p < 0.05). Discussion: The early identification of RCF from AIDP had relatively obvious features, including slightly elevated CSF protein levels and normal or slightly prolonged DML and F‐wave latencies, contrasting with the apparent elevation and prolongation seen in AIDP. Differentiating RCF from axonal degeneration remains challenging. One potential distinguishing factor is that the motor function in RCF tends to be better than in the latter. [ABSTRACT FROM AUTHOR]

What is the definition of Syndrome? Since the beginning of studies in genetics, certain terminologies have been created and used to define groups of diseases or alterations. With the advancement of knowledge and the emergence of new technologies, the use of basic concepts is being done in a mistaken or often confusing way. Because of this, revisiting and readjusting the old terms becomes imminent. Here, we explore these concepts and their use, through a literature compilation of an already well‐defined genetic alteration (16q11.2 microduplication). We bring comparisons in clinical and molecular scope of the alteration itself and its diagnostic methods, to improve the report of cases, rescuing terminologies and their applicability nowadays. Significance Statement: In the genomic era, where genetic tests and diagnostic technologies are advancing rapidly, the correct classification of certain pathologies is essential. For this, revisiting and readjustment of terms becomes imminent. Here, we analyze 16p11.2 microduplication cases and use the data to support a discussion about the use of some classic clinical terms, proposing a new point of view in descriptive care, related to both technological interpretation and expression. [ABSTRACT FROM AUTHOR]

Carotidynia is a neck pain syndrome characterized by tenderness at the carotid bifurcation secondary to various aetiologies. We describe two young females who presented with carotidynia and on evaluation were diagnosed with Takayasu arteritis. Carotidynia in a young female should alert the clinician to evaluate for Takayasu arteritis. [ABSTRACT FROM AUTHOR]

We report the clinical findings of mVEMP responses in conjunction with cVEMPs and oVEMPs in an individual with Wallenberg syndrome. The results indicated that the degree of abnormality in mVEMP was comparatively higher than in cVEMPs and oVEMPs. mVEMPs can offer valuable insights into the Wallenberg syndrome's brainstem pathophysiology. [ABSTRACT FROM AUTHOR]

BACKGROUND: Chemotherapy-induced peripheral neuropathy (CIPN) is a significant side effect of some chemotherapeutic agents. Effective treatment is limited. OBJECTIVES: This single patient case details gua sha as an intervention to reduce CIPN. METHODS: A 38-year-old female patient received weekly treatment of gua sha in one-hour sessions for 10 weeks. The patient completed the Functional Assessment of Cancer Therapy/Gynecologic Oncology Group-Neurotoxicity (FACT/GOG-NTX) subscale to describe her CIPN throughout and postintervention. A research assistant measured the extent of numbness or tingling along the limb from baseline to 18 months after gua sha. Descriptive data were used to summarize this case. FINDINGS: After gua sha, the total FACT/GOG-NTX subscale score increased from 13 to 36, indicating a sevenfold greater change than the minimum clinically important difference. The range of limb numbness and tingling decreased, and the symptoms remained stable during follow-up. Gua sha showed a positive clinical effect. [ABSTRACT FROM AUTHOR]

Objectives : The complex effects of multiple co-occurring symptoms are a major cause of reduced quality of life; thus, it is necessary to identify symptom clusters experienced by patients and explore interventions. This study aimed to provide an overview of non-pharmacological interventions based on symptom clusters in adults to identify effective intervention strategies and methods. Methods. This systematic review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) reporting guidelines. For the search databases, PubMed, Embase, Cochrane Library, CINAHL, RISS, KISS, and KISTI were used. It includes English and Korean experimental studies published up to May 2023. The literature quality was evaluated using the Joanna Briggs Institute Quality Assessment Tool. Results. Of the 18 studies selected, 15 were randomized controlled trials and three were quasi-experimental studies, all of which were conducted on patients with cancer. The symptom clusters-based interventions in the literature are classified as movement-based, relaxation-based, integrated, and miscellaneous interventions. Integrated interventions, which were effective in all studies, might prove to be very effective for managing symptom clusters. Movement-based intervention studies, which include dance, Qigong, and Tai Chi, might be very effective in improving health-related quality of life (HRQoL). Conclusions. This review demonstrates that non-pharmacological interventions reduce the occurrence and severity of symptom clusters, thereby improving HRQoL. Further studies are required to evaluate the effects of these interventions in patients with various chronic conditions other than cancer. [ABSTRACT FROM AUTHOR]

Lhermitte-Duclos Disease is a rare clinical entity involving a dysplastic lesion of the cerebellum. The dysplastic cerebellar ganglioblastoma is often seen in association with Cowden Syndrome, an autosomal dominant disorder consisting of a mutation in the phosphatase and homologous tensin (PTEN) gene. Characteristic findings on neuroimaging allow for a pre-operative diagnosis to be made, which guides further management of the condition. This report describes the diagnosis and management of Lhermitte-Duclos Disease in a 51-year-old lady, spanning a period of almost seven years. The characteristic radiological and histological findings are presented, along with the clinical features associated with Cowden Syndrome. This patient ultimately underwent surgical intervention for symptomatic relief, which is described here. [ABSTRACT FROM AUTHOR]

Aseptic abscess (AA) is a rare autoinflammatory disorder, characterized by the formation of sterile abscesses in various organs, and is accompanied by inflammatory bowel disease. Antibiotic treatment is ineffective, but steroid therapy shows a good response. AA can be difficult to differentiate from infection because abscesses appear similar both radiologically and histopathologically. Herein, we present the case of a 56-year-old woman with AA in the anterior chest wall and synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. [ABSTRACT FROM AUTHOR]

Horner syndrome, manifesting as ptosis and miosis, arises from disruptions within the oculosympathetic pathway. This syndrome is classified based on the lesion's location along the sympathetic nerve pathway into central, preganglionic, or postganglionic types. While endoscopic transthoracic sympathectomy, a surgical intervention for hyperhidrosis, is associated with several complications, including compensatory hyperhidrosis, Horner syndrome, and pneumothorax, these complications are notably rarer in sympathotomy procedures. Importantly, the incidence of Horner syndrome post-operatively is notably low, particularly in comparison to compensatory hyperhidrosis, with most cases being reversible and not necessitating further intervention. This report delineates a rare case of persistent Horner syndrome following a bilateral sympathotomy at the T3 and L3 levels, performed to alleviate symptoms of palmar and plantar hyperhidrosis. The discussion underscores the rarity of such a complication and explores the implications for surgical practice and patient counselling. [ABSTRACT FROM AUTHOR]

Background: Infants with Trisomy 21 are known to have increased incidence congenital anomalies including congenital heart diseases (CHD) and congenital gastrointestinal anomalies. It is not known if there are patterns of coexistence. Objectives: To examine the coexistence of CHD with various gastrointestinal anomalies in infants with Trisomy 21. Methods: We assessed a sample of infants with Trisomy 21 from the National Inpatient Sample (NIS), and its KID subversion, produced by the Healthcare Cost and Utilization Project for 2003–2015. We identified CHD using international classification of diseases version 9 (ICD9) and categorized them into four groups: left sided lesions, right sided lesions, conotruncal lesions, and shunt lesions. We identified small intestinal atresia and Hirschsprung disease with ICD9 codes. Results: The sample included 81,561 newborn infants diagnosed with Trisomy 21; 45% of them had CHD; 4.7% had small intestinal atresia, and 1.6% had Hirschsprung disease. All subcategories of CHD were associated with increased incidence of both small intestinal atresia and Hirschsprung disease, p value < 0.05 compared to infants with Trisomy 21 who did not have CHD. Conclusions: Among infants with Trisomy 21, the presence of CHD increased the odds of a concomitant congenital GI anomaly. [ABSTRACT FROM AUTHOR]

Introduction: Propylene glycol (PG) is frequently used as a solvent for various medications. However, there is substantial evidence of Propylene glycol toxicity, such as depression, agitation, and seizures, particularly when used in combination with other drugs. Here, we aimed to study the effect of Propylene glycol administration in combination with amyloid β1-40 injection on hippocampal neurons. Materials & Methods: Thirty-six male Wistar rats were randomly divided into four groups: sham, amyloid β1-40 injection group, Propylene glycol group, and amyloid β1-40+ Propylene glycol group. Alternation behavior, number of neurons in the hippocampus, lipid peroxidation markers, and superoxide dismutase levels were analyzed in all rats. Results: When Propylene glycol was co-administered with amyloid β1-40, a notable reduction in the mean neuronal count was observed in the CA1, CA3, and DG regions compared with the amyloid β1-40 only injected animals (P < 0.05). Furthermore, Propylene glycol induced an increase in lipid peroxidation markers (10.78±0.4) and a decrease in antioxidant content (2.8 ± 0.17) when administered with amyloid β1-40s compared to the animals that received only amyloid β1-40 (P<0.05). A similar pattern was found in alternation behavior compared with the group with amyloid β1-40 injection (P<0.001). Conclusion: Propylene glycol could produce excessive neurotoxicity in regions of the hippocampus when co-administered with amyloid β1-40. It likely increases lipid peroxidation and reduces superoxide dismutase in the rat brain. The use of different agents as a vehicle should be considered, especially in the elderly. [ABSTRACT FROM AUTHOR]

Background: The mechanisms at the basis of depression are still matter of debate, but several studies in the literature suggest common pathways with dementia (genetic predispositions, metabolic and inflammatory mechanisms, neuropathological changes) and other geriatric syndromes. Aims: To evaluate the role of cortisol (as marker of the HPA, hypothalamus–pituitary–adrenal axis hyperactivity) in elderly subjects with depressive symptoms (by the means of the AGICO, AGIng and COrtisol, study), in relationship to the presence of the major geriatric syndromes. Methods: The AGICO study enrolled patients from ten Geriatric Units in Italy. Every subject received a comprehensive geriatric assessment or CGA (including the Mini Mental State Examination or MMSE, Geriatric Depression Scale or GDS and Cornell Scale for Depression in Dementia or CSDD), the neurological examination (with a computed tomography scan or magnetic resonance imaging of the brain), the assessment of the metabolic syndrome (MetS), the evaluation of the cortisol activity by two consecutive urine collections (diurnal and nocturnal), a CGA-derived frailty index (FI) and a modified measure of allostatic load (AL). Results: The MMSE scores were significantly and inversely related to the values of GDS (p < 0.001) and CSDD (p < 0.05), respectively. The patients with depressive symptoms (GDS/CSDD > 8) showed significantly increased disability, MetS, inflammation, FI and AL and significantly reduced MMSE and renal function. The diurnal and nocturnal urinary cortisol levels in the patients with depressive symptoms (GDS/CSDD > 8) were higher with respects to controls (p < 0.05 for nocturnal difference). Discussion: The AGICO study showed that the stress response is activated in the patients with depression. Conclusion: The depression in elderly patient should be reconsidered as a systemic disease, with coexisting major geriatric syndromes (disability, dementia, frailty) and combined pathogenetic mechanisms (metabolic syndrome, impaired renal function, low-grade inflammation, and allostatic load). Cortisol confirmed its role as principal mediator of the aging process in both dementia and metabolic syndrome. [ABSTRACT FROM AUTHOR]

The syndrome of anterior fibroneural stalk, vertebral anomaly, enteric duplication cyst, and diaphragmatic hernia is a manifestation of abnormal notochordal development due to persistence of the neurenteric canal beyond early fetal gestational age. Our description of the third such published case to date supports this novel tetralogy and further illustrates the role of both pre- and postnatal imaging in achieving the diagnosis. [ABSTRACT FROM AUTHOR]

The COVID-19 pandemic has contributed to significant societal challenges, including increased substance misuse. The COVID stress syndrome is a constellation of interrelated processes that occur in response to pandemics, including danger/contamination fears, fears concerning economic consequences, xenophobia, compulsive checking/reassurance-seeking, and pandemic-related traumatic stress symptoms. In the present study, using a sample of 812 adults collected during the early stages of the COVID-19 pandemic in May 2020, we examined the relations between identified profiles of the COVID Stress Scales (CSS) and behavioral and cognitive aspects of substance misuse. Using profile analysis via multidimensional scaling (PAMS), we identified two core profiles of the CSS, which explained 60 % of the variance in participant responding: 1) High compulsive checking & Low xenophobia and 2) High xenophobia & Low danger/contamination. The first profile is consistent with the COVID stress syndrome, while the second profile aligns with the COVID disregard syndrome, which is a constellation of interrelated processes distinguished by a denial or downplaying of the seriousness of the COVID-19 pandemic and lack of perceived vulnerability to disease. Both profiles demonstrated significant positive correlations with drug and alcohol misuse, respectively. However, only the High xenophobia & Low danger/contamination profile demonstrated relations with cognitive aspects of substance misuse via positive and negative correlations with positive and negative expectancies of alcohol use, respectively. These findings provide further support for the relationship between the COVID stress syndrome and substance misuse and offer insight into how unique profiles of this syndrome may impact pandemic-related mental and public health interventions. • COVID stress syndrome is a set of processes occurring in response to pandemics. • The COVID stress scales (CSS) was developed to measure the COVID stress syndrome. • Two distinct profiles were identified characterizing participant responding on CSS. • Both profiles were related to substance abuse-related outcomes. • Relationship between COVID stress syndrome and substance use warrants more research. [ABSTRACT FROM AUTHOR]

Introduction and purpose Munchausen syndrome by proxy is a factitious disorder characterized by the search for and induction of somatic symptoms, the falsification of laboratory results, and even the mutilation of a loved one, usually a child, by a parent or caregiver. Paediatricians often only have information about the child's condition from an interview with the child's guardian, so it is very important to differentiate feigned disorders from real, sometimes difficult to diagnose, cases of illness. We may suspect them if the parent reports unusual symptoms in the child, insinuates further treatments and hospital stays, has above-average knowledge of medical terminology, and is aggressive towards the staff. Prominent among the causes of Munchausen syndrome by proxy are first and foremost the desire to focus the parent/carer's attention on themselves and the expectation of recognition for their care and concern for the child. Methods and materials The following review was based on articles from the PubMed and Google Scholar databases. Key search terms included munchausen syndrome, munchausen syndrome by proxy, treatment, diagnosis, paediatric condition falsification, factitious disorder. Conclusions Due to the nature of this disorder, physicians of different specialities may come across it during their practice. Increasing their knowledge on this topic and the effects of Munchausen syndrome by proxy will allow the correct diagnosis to be established and appropriate psychiatric therapy with psychological care to be introduced, as well as protecting children who are victims of the actions of their caregivers. [ABSTRACT FROM AUTHOR]

Weight-adjusted-waist index (WWI) is an emerging parameter for evaluating obesity. We sought to ascertain the link between WWI and circadian syndrome (CircS). The study population consisted of 8275 eligible subjects who were included in the ultimate analysis from the NHANES 2011–2018. By using multivariable regression models, the association of WWI and CircS was analyzed. In subgroup analysis, we explored the relationship in different groups and tested the stability of the intergroup connection using interaction testing. To investigate whether WWI and CircS had a potential non-linear relationship, smooth curve fittings, and threshold effects tests were also constructed. In a multivariate linear regression model, WWI is significantly positively related to CircS (OR = 1.77, 95% CI 1.50–2.08). Through subgroup analysis and interaction testing, the stability of this positive association was also validated. It was further found that there was an inverted U-shaped association, with a turning point of 11.84, between WWI and CircS. Our findings supported a strong association between WWI values and CircS. Central obesity management is pivotal for preventing or alleviating CircS. [ABSTRACT FROM AUTHOR]

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The emergence of gene therapy offers opportunities for treating a myriad of genetic disorders and complex diseases that previously had limited or no treatment options. The key basic strategies for gene therapy involve either the addition, inhibition, or introduction of a new gene, with a crucial component being the use of a delivery vector to effectively target cells. Particularly promising is the application of gene therapy for the treatment of inherited arrhythmia syndromes, conditions associated with significant mortality and morbidity that have limited treatment options, and a paucity of disease modifying therapy. This review aims to summarize the utility of gene therapy for the treatment of inherited arrhythmia syndromes by exploring the current state of knowledge, limitations, and future directions. [ABSTRACT FROM AUTHOR]

SLC29A3 spectrum disorder, also known as histiocytosis‐lymphadenopathy plus syndrome (HLPS), presents a wide variety of multi‐systemic manifestations that can be mistaken for other conditions. Herein, we report a 9‐year‐old girl who presented with a complex clinical presentation since birth, including chronic generalized lymphadenopathy in association with hepatosplenomegaly, short stature, flexion contractures, hearing loss, hyperpigmentation, and heart anomalies. She was ultimately diagnosed with the SLC29A3 spectrum disorder. [ABSTRACT FROM AUTHOR]

Introduction: Hindfoot varus deformity is common in people with unilateral upper motor neuron syndrome (UMNS) and can be dynamic or persistent. The aims of this study were (1) to gain insight into plantar pressure characteristics of people with chronic UMNS in relation to hindfoot varus and (2) to propose a quantitative outcome measure, based on plantar pressure, for the scientific evaluation of surgical interventions. Methods: In this retrospective study, a cohort comprising plantar pressure data of 49 people with UMNS (22 "no hindfoot varus", 18 "dynamic hindfoot varus", and 9 "persistent hindfoot varus"), and 586 healthy controls was analyzed. As an indication of plantigrade foot contact, the ratio between the plantar contact area of the affected and the non‐affected foot was calculated. To investigate spatial and temporal aspects of plantar pressure, normalized plantar pressure patterns and center of pressure trajectories were computed. Results: People with UMNS had lower plantar pressure area ratios compared to healthy controls. Additionally, increased plantar pressure underneath the lateral foot was found in people with a persistent hindfoot varus. Center of pressure trajectories were more lateral during the first 26% of the stance phase in people with a dynamic hindfoot varus and during the first 82% of the stance phase in people with a persistent hindfoot varus compared to healthy controls. Conclusion: Spatial and temporal differences in plantar pressure were found in people with dynamic or persistent hindfoot varus deformity. We propose to primarily use the medio‐lateral center of pressure trajectory as outcome measure for the scientific evaluation of surgical interventions targeting hindfoot varus. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Destroyed lung syndrome is a recognized devastating complication of pulmonary tuberculosis (TB) that affects the quality of life of patients. Clinicians in TB‐endemic countries should be aware of this complication aiming at prevention. TB control programs should intensify efforts at early detection and treatment and also provide interventions for post‐TB complications. [ABSTRACT FROM AUTHOR]

Background: In 2022 the total world migrant population was 281 million (an increase of approximately 62% compared to year 2000), of whom 169 million were migrant workers. The number of refugees, asylum seekers and others in need of international protection increased by 22% compared to 2021. Research has shown that the forcibly displaced have high rates of mental disorders (including depression, post-traumatic stress disorder (PTSD) and anxiety), with an estimated prevalence almost 6 to 7 times higher than the general population. Given the increase of the overall migration phenomenon and the challenge raised by the cultural features concerning mental health, we consider that this is an area that requires close attention to ensure that culturally sensitive health services be available to the migrant and displaced population. Aims: The aim of this narrative review is to provide a background to the issue and take stock of what is currently available in the literature regarding culture-bound illnesses and the relevant diagnostic tools. Methods: A comprehensive search was performed in PubMed, Psychinfo, Embase, Google Scholar, organized in stages to assure inclusion of all the relevant studies. Of the 703 papers initially identified, only 30 papers finally satisfied the inclusion criteria. Results: Eleven diagnostic scales were found, only two of which are being used for displaced people. Conclusions: Further work is required in this field, including a debate as to whether scales are indeed an appropriate tool for use with this population. [ABSTRACT FROM AUTHOR]

Superior mesenteric artery (SMA) syndrome or Wilkie's syndrome is a vascular compression disorder that causes the abnormal compression of the third portion of the duodenum by the SMA. It has a low incidence rate, which is higher in young women, and is rarely associated with the Nutcracker phenomenon: a condition of the compression of the left renal vein between the SMA and the aorta, which manifests as pain in the left flank and pelvis. Here, we report on the case of a 54-year-old woman with a history of repeated episodes of abdominal pain caused by the Nutcracker syndrome and Wilkie's syndrome. [ABSTRACT FROM AUTHOR]

Lipid Transfer Protein (LTP) syndrome refers to a complex clinical pattern in which affected individuals have allergies to multiple plant foods due to cross-reactions between botanically unrelated pollens and plant foods that contain LTPs. Pollens and plants containing LTPs include mugwort, plane and olive pollens, and peach, apricot and walnut respectively. Published reports of LTP syndrome are few in the paediatric population in non-Mediterranean regions. [ABSTRACT FROM AUTHOR]

Purpose: Age‐related nonparalytic hypertropia (ARNH) is reported to be involved in sagging eye syndrome, where excyclotorsion is large and the lower eye is more extorted. The primary aim of this study was to describe the clinical profiles of patients with ARNH. The secondary aim was to compare cyclotorsion in patients with ARNH with that in normal individuals. Methods: Inclusion criteria for ARNH were insidious onset of diplopia at distance after 50 years of age, nonparalytic hyperdeviation and follow‐up >6 months. Objective cyclotorsion was measured as the disc‐to‐fovea angle (DFA) on fundus photographs obtained from two groups: 75 patients with ARNH (age, 74.1 ± 7.9 years) and 75 sex‐ and age‐matched normal controls (73.9 ± 8.1 years). Results: The hypertropia angle was 4.6 ± 2.8 prism dioptres. One patient also experienced diplopia at near‐gaze. DFA in ARNH (right/left eye; 11.0 ± 4.8°/11.6 ± 3.9°) was larger than that of the control (6.6 ± 3.7°/9.2 ± 3.5°) (p < 0.0001). The DFA in the non‐dominant eye (12.5 ± 4.1°) was larger than that in the dominant eye (10.2 ± 4.3°) (p = 0.0003). The lower eye did not have the larger DFA in 29 patients. Conclusion: The angle of hyperdeviation in patients with ARNH was small. The DFA in the ARNH group was larger than that in the normal group. The lower eye was not the eye with a larger DFA in 39% of ARNH, inconsistent with sagging eye syndrome. Decreased phoria adaptation (fusional ability) may trigger diplopia in patients with ARNH. [ABSTRACT FROM AUTHOR]

This research aimed to investigate the relationship between childhood traumatic experiences and bodily distress syndrome, and the mediating role of somatoform dissociation. A total of 241 individuals living in Iran aged 20–40 years (M = 26.41 years, SD = 6.30; 74.7% females) were selected by convenience sampling to participate online in the research in March 2023. They answered the Childhood Trauma Questionnaire (CTQ-SF), the Bodily Distress Syndrome Checklist (BDS-25), and the Somatoform Dissociation Questionnaire (SDQ-20). The results of the structural equation modeling showed that the model had a good fit, and significant relationships were observed between childhood traumatic experiences and bodily distress syndrome, between childhood traumatic experiences and somatoform dissociation, and also between somatoform dissociation and bodily distress syndrome. The results indicated that somatoform dissociation partially mediates the relationship between childhood traumatic experiences and bodily distress syndrome. Furthermore, the prevalence of bodily distress syndrome was higher in the female than the male participants. The results thus highlight the role of childhood traumatic experiences and somatoform dissociation in creating bodily distress syndrome. [ABSTRACT FROM AUTHOR]

The article describes cases abnormalities seen on electroencephalography (EEG) from patients experiencing neurotoxicity induced by cefepime, a cephalosporin antibiotic. A 65-year old male with a history of chronic hepatitis C and chronic right middle cerebral artery infarcts from a gliosis demonstrated right frontal sharps. A 64-year old male demonstrated myoclonic status epilepticus. An 87-year old male showed generalized sharp discharges considered as an ictal-interical continuum pattern.

Emotion-focused Group Therapy has demonstrated preliminary efficacy for multiple syndromes. The groups involve individual chair-work in the context of the group, followed by interpersonal processing of the aroused emotions. So far there has been limited investigation of the mechanisms of change, including participants' experiences of the change process. This study aimed to qualitatively examine participants' experiences of the change process during Emotion-focused Group Therapy. Sixteen participants were interviewed about their experiences of the change process during the therapy group. The interview was structured to move from overarching reflections on the process of change, to specific experiences of their own chair-work and watching others' chair-work. During the interview, participants were shown salient video segments of the group to prime their recall of moments of high arousal that were deemed likely to contain emotional change processes. Thematic Analysis extracted five main themes: Value of Vulnerability; Connectedness; Vicarious Emotional Processing; Understanding My Critic; and Barriers to Emotional Processing. These findings suggest that there may be both common and unique change processes that occur during Emotion-focused Group Therapy, compared with the individual Emotion-focused Therapy. [ABSTRACT FROM AUTHOR]

Background: Early surgical management of syndromic craniosynostosis varies widely between centers, with patients typically undergoing multiple intracranial procedures through childhood. This study evaluates the long-term impact of early posterior vault distraction osteogenesis (PVDO) versus conventional treatment paradigms on the number and timing of subsequent craniofacial procedures. Methods: We retrospectively analyzed the longitudinal operative patterns of patients with syndromic craniosynostosis treated from 2000 to 2023 with greater than five years of follow-up. Outcomes of patients who underwent early PVDO and conventional vault reconstruction were compared. Results: Fifty-five patients met the inclusion criteria (30 PVDO and 25 conventional). Age at initial vault surgery was similar between the PVDO and conventional cohorts (7.6 vs. 8.8 months), as were baseline craniometrics (p > 0.05). Multiple fronto-orbital advancement (FOA) procedures were performed in only 1/30 (3%) PVDO-treated patient versus 12/25 (48%) conventionally-treated patients (p < 0.001). Twelve (40%) PVDO-treated patients did not undergo FOA at all. Among patients with Apert and Crouzon syndromes, fewer PVDO-treated patients required FOA prior to midface surgery (33% vs. 92%, p = 0.004) or repeat FOA (6% vs. 50%, p = 0.005) compared to conventionally-treated patients. Among patients with Muenke and Saethre–Chotzen syndromes, a similar proportion of patients required FOA in the PVDO and conventional cohorts (91% vs. 100%, p = 0.353), though no PVDO-treated patients required repeat FOA (0% vs. 44%, p = 0.018). Conclusions: Early PVDO is associated with a reduction in the high burden of both FOA and, critically, revisionary frontal procedures which are commonly seen in conventionally-treated patients with syndromic craniosynostosis. [ABSTRACT FROM AUTHOR]

Objective: The aim of the study is to analyze microstate patterns in GLUT1-DS, both before and after the ketogenic diet (KD). Methods: We conducted microstate analysis of a patient with GLUT-1 DS and 27 healthy controls. A systematic literature review and meta-analysis was done. We compared the parameters of the patients with those of healthy controls and the incorporating findings in literature. Results: The durations of the patient were notably shorter, and the occurrence rates were longer than those of healthy controls and incorporating findings from the review. After 10 months of KD, the patient's microstate durations exhibited an increase from 53.05 ms, 57.17 ms, 61.80 ms, and 49.49 ms to 60.53 ms, 63.27 ms, 71.11 ms, and 66.55 ms. The occurrence rates changed from 4.0774 Hz, 4.9462 Hz, 4.8006 Hz, and 4.0579 Hz to 3.3354 Hz, 3.7893 Hz, 3.5956 Hz, and 4.1672 Hz. In healthy controls, the durations of microstate class A, B, C, and D were 61.86 ms, 63.58 ms, 70.57 ms, and 72.00 ms, respectively. Conclusions: Our findings suggest EEG microstates may be a promising biomarker for monitoring the effect of KD. Administration of KD may normalize the dysfunctional patterns of temporal parameters. [ABSTRACT FROM AUTHOR]

Olfactory neuroblastomas rarely secrete adrenocorticotropic hormone, leading to ectopic adrenocorticotropic hormone syndrome. However, the prevalence, timing, and triggers of ectopic adrenocorticotropic hormone syndrome in patients with olfactory neuroblastomas remain unclear. This study aimed to investigate these factors and conduct a literature review. Fifteen patients with olfactory neuroblastomas who underwent surgery at our institution were included. The prevalence of ectopic adrenocorticotropic hormone syndrome development was assessed by evaluating adrenocorticotropic hormone expression using immunohistochemistry. Furthermore, 26 patients with olfactory neuroblastomas who developed ectopic adrenocorticotropic hormone syndrome from previous reports were reviewed. Among the 15 patients, three (20%) showed adrenocorticotropic hormone-positive tumor cells at the time of initial surgery, and two (13%) developed ectopic adrenocorticotropic hormone syndrome. The timing of developing ectopic adrenocorticotropic hormone syndrome was 2.5 and 10 years following the initial treatment of olfactory neuroblastoma. Based on the literature review, nine patients with recurrent and metastatic olfactory neuroblastoma developed ectopic adrenocorticotropic hormone syndrome after the initial surgery, of whom, three had confirmed disease after developing ectopic adrenocorticotropic hormone syndrome, three developed during disease progression, two developed after receiving chemotherapy, and one developed after undergoing a biopsy. The timing of ectopic adrenocorticotropic hormone syndrome was 2.5–15 years after initial treatment. Our study revealed that acknowledging olfactory neuroblastomas can manifest as ectopic adrenocorticotropic hormone syndrome with a certain low prevalence is crucial. Moreover, our study speculated that tumor stimulation, such as biopsy or chemotherapy, as well as disease progression, could trigger ectopic adrenocorticotropic hormone syndrome onset. Thus, olfactory neuroblastomas can develop into ectopic adrenocorticotropic hormone syndrome, even long after the initial treatment. [ABSTRACT FROM AUTHOR]

Introduction: Postintensive care syndrome (PICS) is a combination of short‐, medium‐ and long‐term morbidities that occur in patients discharged from the Intensive care unit (ICU). ICU professionals have a crucial role in managing and understanding the PICS. This study aimed to develop the PICS Knowledge Test (PICS‐KT), which measures ICU professionals' awareness and knowledge levels regarding PICS, and to determine its validity and reliability. Methods: The databases were searched in detail, scientific research related to PICS was analyzed, and the draft scale was created accordingly. A total of 117 doctors and nurses who had been working in the ICU for at least 6 months were included in the study. For the validity and reliability analysis of the test, content validity ratio, item difficulty index, item discrimination index values and Cronbach α were examined. Results: The Cronbach's α reliability coefficient for the 46‐item PICS‐KT is 0.93, indicating high reliability. Scores range from 0 to 46, with 32 or higher considered successful, suggesting adequate knowledge of PICS among ICU professionals. Scores of 14 or less indicate minimal knowledge. Those with scores between 14 and 32 possess some knowledge but need improvement. PICS‐KT assesses knowledge in four main areas: general information, risk factors/causes, symptoms and findings and interventions. ICU professionals show high awareness of interventions for preventing and treating PICS, as indicated by a high mean score in the interventions subdimension. Conclusion: The PICS‐KT is crucial in assessing healthcare professionals' understanding of the various short‐, medium‐ and long‐term morbidities associated with PICS. The study ensures that the test is a robust and dependable instrument for evaluating ICU professionals' knowledge about PICS. [ABSTRACT FROM AUTHOR]

Tjalma's syndrome is a benign combination of ascites, pleural effusion, and elevated CA-125 occurring in patients with systemic lupus erythematosus. Reports of Tjalma's syndrome are scarce. An elevated CA-125 level often suggests the possibility of the presence of a malignant tumor. We report a case of generalised erythema and blisters with pruritus, massive unilateral pleural effusion and elevated CA-125. This patient was finally diagnosed with bullous systemic lupus erythematosus after exclusion of tumour and other maculopapular disorders. We hope that this particular case may provide a more comprehensive and novel diagnostic idea of systemic lupus erythematosus and pleural effusion, avoiding unnecessary anxiety, laboratory tests and surgical interventions. [ABSTRACT FROM AUTHOR]

Objective: To analyze the spatial autocorrelation and spatiotemporal clustering characteristics of severe fever with thrombocytopenia syndrome(SFTS) in Anhui Province from 2011 to 2023. Methods: Data of SFTS in Anhui Province from 2011 to 2023 were collected. Spatial autocorrelation analysis was conducted using GeoDa software, while spatiotemporal scanning was performed using SaTScan 10.0.1 software to identify significant spatiotemporal clusters of SFTS. Results: From 2011 to 2023, 5720 SFTS cases were reported in Anhui Province, with an average annual incidence rate of 0.7131/100,000. The incidence of SFTS in Anhui Province reached its peak mainly from April to May, with a small peak in October. The spatial autocorrelation results showed that from 2011 to 2023, there was a spatial positive correlation(P < 0.05) in the incidence of SFTS in all counties and districts of Anhui Province. Local autocorrelation high-high clustering areas are mainly located in the south of the Huaihe River. The spatiotemporal scanning results show three main clusters of SFTS in recent years: the first cluster located in the lower reaches of the Yangtze River, the eastern region of Anhui Province; the second cluster primarily focused on the region of the Dabie Mountain range, while the third cluster primarily focused on the region of the Huang Mountain range. Conclusions: The incidence of SFTS in Anhui Province in 2011–2023 was spatially clustered. [ABSTRACT FROM AUTHOR]

Post-decompressive craniectomy syndrome (PDCS) is a complication following decompressive craniectomy (DC). PDCS or also known as trephine or sunken skin flap syndrome has an indirect relationship with traumatic brain injury (TBI). The mechanism of PDCS is not yet fully understood and the clinical manifestations are diverse, causing PDCS to often be underdiagnosed. In this study, the authors aim to create a systematic review of PDCS following TBI including a discussion of incidence, clinical and radiological manifestations, management and outcome. This systematic review is conducted based on the Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) guideline. The literature search included electronic databases PubMed, Cochrane, JNS and EMBASE. All studies included were available in English and full-text format. In this research, 42 case reports were obtained. The population was predominantly male (72%) with a mean population age of 44.7 ± 17.3 years. The mean interval for onset and cranioplasty procedure was 80.17 ± 77.34 days and 92.05 ± 77.06, respectively. The most common clinical manifestations were sunken skin flap in the defect area (74%) and decreased consciousness (64%). Paradoxical herniation (74%) was the most common radiological manifestation. There was no connection between the occurrence of PDCS and the size of the defect. Cranioplasty remains the mainstay of management with clinical improvement in 96% of cases. PDCS should be suspected in every patient with symptoms of new neurological deficits after DC. Early management must be carried out to prevent further deterioration. [ABSTRACT FROM AUTHOR]

The authors report the surgical management and outcomes of neurotrophic keratopathy in two patients with Stüve-Weidemann syndrome who underwent single-stage bilateral corneal neurotization. Both patients experienced improvement in corneal sensation based on Cochet-Bonnet aesthesiometry measurements or cotton tip testing in addition to clinical improvement in ocular surface health. [J Pediatr Ophthalmol Strabismus. 2024;61(5):e54–e58.] [ABSTRACT FROM AUTHOR]

Background: There have been only few reports on Rhupus syndrome with severe visceral involvement. Moreover, there was little consensus regarding its treatment. Belimumab is one of the options for treating this disease. For patients with clinical symptoms and elevated levels of anti CCP antibodies and anti-double stranded DNA antibodies, and it suggests Rhupus syndrome. After effective treatment, the decrease in levels of anti CCP antibodies and anti-double stranded DNA (ds-DNA) antibodies can effectively delay the progression of the disease and protect target organs. Methods: We used a chemiluminescence instrument, (Yahuilong; Shenzhen, China), to measure the changes in CCP and dsDNA before and after treatment. Results: Prior to treatment, the patient presented with symptoms of rheumatoid arthritis and systemic lupus erythematosus. Her laboratory tests showed dsDNA (214 IU/mL) and CCP level of ˃ 3,000 U/mL. After treatment with belimumab, the clinical symptoms were significantly relieved, and the patient's CCP IgG level decreased to 263.5 U/mL. A blood test found that her anti-dsDNA was negative. Conclusions: CCP and dsDNA can serve as indicators for the diagnosis and treatment of Rhupus syndrome. [ABSTRACT FROM AUTHOR]

Introduction: Chinese gay men face substantial stress, such as internalized homonegativity, and may have high levels of demoralization syndrome. However, few studies have examined the relationship between the two. This study aimed to investigate the relationship and the mediating mechanism between internalized homonegativity and demoralization syndrome in Chinese gay men. Methods: From March to May 2022, 319 Chinese gay men were recruited nationwide through the Internet (mean age = 23.99, SD = 4.23). The researchers used the internalized homonegativity scale, the sexual orientation concealment scale, the sense of self scale, and the short version of the demoralization scale for the measurements. Results: Sense of self plays a mediating role between internalized homonegativity and demoralization (standardized estimate = 0.104, 95% CI [0.004, 0.205]). Sexual orientation concealment and sense of self play a sequential mediating role between internalized homonegativity and demoralization (standardized estimate = 0.178, 95% CI [0.113, 0.258]). Internalized homonegativity contributes to increased demoralization among gay men through the increment of sexual orientation concealment and the reduction of sense of self. Conclusions: Internalized homonegativity may be a risk factor for demoralization syndrome among Chinese gay men. Internalized homonegativity is associated with increased demoralization syndrome through increased sexual orientation concealment and decreased sense of self. Policy Implications: Policymakers can formulate some corresponding laws, such as ensuring that sexual minorities are treated equally with heterosexuals in terms of studying and finding jobs in order to reduce the sources of internalized homonegativity formation and the symptoms of demoralization syndrome. [ABSTRACT FROM AUTHOR]