Unique Manifestation Research Articles

Male patients with primary Sjögren's syndrome have unique clinical manifestations and circulating lymphocyte profiles.

We aimed to explore the relationship between clinical characteristics and circulating lymphocyte profiles in Chinese male patients with primary Sjögren's syndrome (pSS). Data from 397 patients with pSS were analyzed retrospectively. 37 were male, which is a prevalence of 9.3%. The clinical, laboratory, and immunophenotypic profiles of peripheral blood lymphocyte subsets were compared between male and female pSS patients. Male patients with primary Sjögren's syndrome have unique clinical manifestations and circulating lymphocyte profiles. Male patients complained more about xerophthalmia and presented with more extra-glandular manifestations as compared with female patients. The CD4+/CD8+ ratio (P = 0.030), the prevalence of CD4-CD8- T cells in lymphocytes (P = 0.020), the absolute number of CD4-CD8- T cells (P = 0.035), the prevalence of CD4+ T cells in lymphocytes (P < 0.001), and the absolute number of CD4+ T cells (P = 0.023) were significantly lower in male patients compared to female patients. On the other hand, the prevalence of CD8+CD28+ T cells (P = 0.030) and CD4+CD25high T cells (P = 0.040) in lymphocytes was significantly higher in male patients than in female patients. Moreover, compared to females with pSS, an elevated serum IgG level, low C3 and C4 levels, anti-SSB positivity, and ANA titers of ≥ 1:160 positivity were more frequent in male with pSS. Male patients with pSS have distinctive peripheral blood lymphocyte subpopulations, present with more severe clinical symptoms and immunological features, and have an unfavorable prognosis. Key Points • Male patients with pSS have more severe clinical symptoms and specific characteristics of peripheral blood lymphocyte subsets. • Male pSS patients exhibit a higher intensity of the disease (as evaluated by ESSDAI). • Male patients with pSS require individualized treatment regimens and closer follow-up.

Myospherulosis in the periapical region following root canal treatment

This report explores a unique manifestation of myospherulosis in the periapical region of lower anterior teeth following root canal treatment with calcium hydroxide Ca(OH)2. A 38-year-old woman complained of enduring pain in her lower front teeth for the past 15 days. During the clinical examination, it was observed that the labial vestibule in the lower anterior region was obliterated. The teeth exhibited tenderness upon percussion. Radiographic examination indicated an ill-defined radiolucency in the periapical region, prompting further investigation into the delayed healing. Histopathological examination of the periapical tissue unveiled cyst-like spaces containing brownish-black-stained spherules, indicative of myospherulosis. Curettage with apicectomy was performed to address the delayed healing. A 6-month follow-up revealed complete healing of the lesion, emphasizing the successful management of myospherulosis-induced delayed healing. The unintended consequence of myospherulosis development emphasizes the importance of preventing medicament overflow into the periapical region during root canal therapy to minimize the delayed healing process.

Subarachnoid Neurocysticercosis Case Series Reveals a Significant Delay in Diagnosis-Requiring a High Index of Suspicion Among Those at Risk.

Subarachnoid neurocysticercosis can be challenging to recognize, which often leads to a delay in diagnosis. We report 3 cases presenting as chronic headache disorders that highlight the unique manifestations seen with this form of neurocysticercosis and the role that the infectious diseases consultant can play in ensuring a timely diagnosis.

Cardiomyopathies in Women.

Heart failure affects over 2.6 million people in the United States. While women have better overall survival rates, they also suffer from higher morbidity as shown by higher rates of hospitalization and worse quality of life. Several anatomical differences in women's hearts affect both systolic and diastolic cardiac physiology. Despite these findings, women are significantly underrepresented in clinical trials, necessitating extrapolation of data from males. Because women have sex-specific etiologies of heart failure and unique manifestations in genetic-related cardiomyopathies, meaningful sex-related differences affect heart failure outcomes as well as access to and outcomes in advanced heart failure therapies in women. This review explores these gender-specific differences and potential solutions to balance care between women and men.

Assessment of magnetic resonance imaging and histopathological changes in chordoid glioma: A retrospective analysis of 13 cases

AbstractChordoid glioma (CG) is a rare tumor, and its predominantly anterior location is in the third ventricle. However, the clinical characteristics of CG were not obvious, and there was less knowledge of CG‐related imaging. This study aimed at exploring the MRI imaging features of CG. We retrospectively analyzed the MRI data of 13 patients diagnosed with CG from January 2015 to May 2021. The pathological change of tumor tissue was observed using H&amp;E staining and immunohistochemical. The MRI manifestations of CG often include phyllode masses in the anterior part of the third ventricle. T2WI is dominated by iso‐ and hypointensity, with internal patchy hyperintensity, marked enhancement, and peritumoral edema. The “eight‐character edema sign” runs along the optic beam and may be useful for distinguishing CG from other tumors. CG has unique MRI manifestations that can serve as a diagnostic basis. Our study may help to enhance the understanding of imaging about CG and improve the diagnosis of patients with CG.

Heterozygous MAP3K20 variants cause ectodermal dysplasia, craniosynostosis, sensorineural hearing loss, and limb anomalies.

Biallelic pathogenic variants in MAP3K20, which encodes a mitogen-activated protein kinase, are a rare cause of split-hand foot malformation (SHFM), hearing loss, and nail abnormalities or congenital myopathy. However, heterozygous variants in this gene have not been definitively associated with a phenotype. Here, we describe the phenotypic spectrum associated with heterozygous de novo variants in the linker region between the kinase domain and leucine zipper domain of MAP3K20. We report five individuals with diverse clinical features, including craniosynostosis, limb anomalies, sensorineural hearing loss, and ectodermal dysplasia-like phenotypes who have heterozygous de novo variants in this specific region of the gene. These individuals exhibit both shared and unique clinical manifestations, highlighting the complexity and variability of the disorder. We propose that the involvement of MAP3K20 in endothelial-mesenchymal transition provides a plausible etiology of these features. Together, these findings characterize a disorder that both expands the phenotypic spectrum associated with MAP3K20 and highlights the need for further studies on its role in early human development.

Pilgrimage and State-Security: Visiting the Tombs of Tzadikim in the Socialist Hungary—Before 1989

Visiting the graves of the (Hasidic) Rebbes of Bodrogkeresztúr, Nagykálló, Olaszliszka, Sátoraljaújhely and other (Hasidic) places of worship are unique manifestations of Jewish popular religiosity in Hungary. These visits are mainly made on the anniversaries of the deaths of the great Rabbis (“Yahrzeit”). The literature does not pay much attention to the fact that these customs were still alive during the decades of Socialism, and even after 1957, although to a limited extent, foreign citizens also took part in these pilgrimages. The pilgrims were monitored by State security. The increase in the intensity of state security surveillance was not related to religiosity, but to the anti-Zionist state policy that emerged in Eastern Europe after the Six-Day-War, which saw all Jewish organizations as Zionism. The study gives an account of the Rabbis behind the custom, the religious significance of the visit and its role in local society. At the same time, it also shows how the memory of the Hasidic “wonder rabbis” was passed on during the decades of the Hungarian Socialist Kádár regime. Moreover it presents how (from the point of view of the Socialist regime) the pilgrimage (peregrinatio religiosa) and participating in it became elements of the power-relationship system and what it meant for the church politics of the period.

A review of type 3 Gaucher disease: unique neurological manifestations and advances in treatment.

Gaucher disease (GD) is a rare lysosomal storage disease that is caused by mutations in the GBA gene. It is classified into three main phenotypes according to the patient's clinical presentation. Of these, chronic neuronopathic GD (GD3) is characterized by progressive neurological damage. Understanding the unique neurological manifestations of GD3 has important diagnostic and therapeutic implications. Our article summarizes the neurological symptoms specific to GD3 and related therapeutic advances, and it highlights the relevance of the gene to clinical symptoms, so as to provide a reference for the diagnosis and treatment of GD3.

Digital transformation in business development: A comparative review of USA and Africa

This comparative review explores the dynamics of digital transformation in business development, drawing parallels between the United States (USA) and various countries in Africa. Digital transformation, characterized by the integration of digital technologies into all aspects of business operations, is a global phenomenon with unique manifestations in diverse economic landscapes. The study delves into the distinct approaches and challenges faced by businesses in the USA and Africa as they navigate the complex terrain of digital transformation. In the USA, a mature and technologically advanced market, businesses have embraced digital transformation as a strategic imperative. The review analyzes the adoption of cutting-edge technologies, such as artificial intelligence, data analytics, and cloud computing, and their impact on enhancing operational efficiency, customer experiences, and overall competitiveness. Case studies and success stories from American businesses provide insights into best practices and lessons learned in the realm of digital business development. Contrastingly, the review examines the digital transformation landscape in various African countries, acknowledging the diversity of economic contexts and technological infrastructures. It explores the challenges faced by African businesses, including limited access to digital infrastructure, the digital skills gap, and regulatory complexities. Case studies from African businesses showcase innovative strategies employed to overcome these challenges, highlighting the resilience and adaptability of entrepreneurs on the continent. The comparative analysis sheds light on the similarities and differences in the pace and nature of digital transformation between the USA and Africa. By understanding the unique challenges and opportunities in each context, businesses, policymakers, and researchers can derive valuable insights to inform strategies for fostering digital business development. Ultimately, the review advocates for a nuanced and context-specific approach to digital transformation, recognizing that successful strategies must be tailored to the distinctive characteristics of each economic landscape.

Unilateral interstitial lung disease: A unique manifestation of asymptomatic pulmonary artery atresia

Interstitial pulmonary fibrosis is idiopathic, bilateral, or secondary to exposure to organic antigens, or collagen disorders. Unilateral pulmonary fibrosis is uncommon and intriguing as it has different pathogenetic mechanisms. We present a case of a patient with a short 5-day history of dyspnea with no ailment in childhood. His radiograph revealed increased reticular markings in the right lung with a small right hilum. Contrast-enhanced computed tomography showed unilateral pulmonary fibrosis and confirmed the presence of an atretic right pulmonary artery contributing to the small hilum, with collateral supply from the right internal mammary artery. This asymptomatic patient had unilateral pulmonary fibrosis which had developed as a consequence of impaired blood supply to the right lung due to atresia of the right pulmonary artery with collateral from systemic circulation unable to keep abreast with normal pulmonary development.

The Assessment and Treatment of Post-traumatic Stress Disorder in Autistic People: A Systematic Review

AbstractAdverse life events and mental health conditions are unfortunately common amongst autistic adults and children; this may present a vulnerability to developing post-traumatic stress disorder (PTSD). This systematic review provides an update of Rumball’s (Review Journal of Autism and Developmental Disorders, 6, 294–324, 2019) systematic review of PTSD in autistic individuals and identifies 18 new studies published from 2017 to 2022, reflecting increased research interest in PTSD in autistic populations. Included literature suggests that autistic adults and children experience more severe PTSD symptoms compared to their non-autistic peers, with at least comparable rates of occurrence. We provide a comprehensive overview of this emerging field and identify the need for future research to validate PTSD symptom assessment tools and treatment strategies and investigate unique manifestations of trauma-related symptoms in autistic individuals.

Paediatric syndromic scoliosis: proceedings of the half-day course at the 57th annual meeting of the Scoliosis Research Society.

There are some syndromes that present with unique manifestations pertaining to the spinal column. A good working understanding of these common syndromes is useful for the spinal deformity surgeons and related healthcare providers. This review attempts to encompass these unique features and discuss them in three broad groups: hypermobility syndromes, muscle pathology-related syndromes, and syndromes related to poor bone quality. This review explores the features of these syndromes underpinning the aspects of surgical and medical management. This review represents the proceedings of the Paediatric Half-Day Course at the 57th Annual Meeting of the Scoliosis Research Society.

Radiation of a short linear antenna above a topologically insulating half-space

The topological magnetoelectric effect is a unique macroscopic manifestation of quantum states of matter possessing topological order and it is described by axion electrodynamics. In three-dimensional topological insulators, for instance, the axion coupling is of the order of the fine structure constant, and hence, a perturbative analysis of the field equations is plenty justified. In this paper, we use Green’s function techniques to obtain time-dependent solutions to the axion field equations in the presence of a planar domain wall separating two media with different topological order. We apply our results to investigate the radiation of a short linear antenna near the domain wall.

Research progress in clinical diagnosis and treatment of osteosarcoma of the jaw

Osteosarcoma of the jaw (JOS), is a relatively rare type of osteosarcoma, with a unique pathogenesis and pathological manifestations. The clinical manifestation of JOS is not characteristic, and it often needs to be diagnosed by combining radiological and pathological examination. At present, the conventional treatment of JOS is a comprehensive treatment based on surgery and supplemented by radiotherapy and chemotherapy. Recently, the emergence of new therapies such as immunotherapy, gene therapy, phototherapy and traditional Chinese medicine has provided more choices for treatment and brought new hope to patients with JOS. Therefore, this article summarized the current understanding of diagnosis and the latest treatment development of JOS.

Cute sarcoidosis Löfgren Syndrome treated by Chinese medicinal formula based on Syndrome Differentiation: A case-based review

Löfgren syndrome (LS) is a unique acute manifestation of sarcoidosis and characterized by erythema nodosum, bilateral hilar lymphadenectasis, and/or bilateral anklearthritis or periarthritis. A 37-year-old female patient with LS presented with fever accompanied by multiple joint swelling and pain, nodular skin erythema, and bilateral hilar lymphadenectasis. The patient had received treatment involving non-steroidal anti-inflammatory drugs and glucocorticoids in other hospitals, but the effects were poor, and the conditions reemerged. The LS duration has lasted for more than 3 months. Following traditional Chinese medicine (TCM) treatment, syndrome differentiation as well as giving patients oral Chinese medicine decoction, the symptoms of the patient were rapidly relieved within one week and did not recur during a six-month follow-up period. This case is the first clinical report of acute sarcoidosis LS treated using TCM and reflects the significant advantages of this form of therapy in emergency treatment

Isolated massive pleural effusion as a manifestation of chronic graft versus host disease successfully treated with corticosteroid

Isolated pleural effusion is a rare manifestation of chronic graft versus host disease (cGVHD) after hematopoietic stem cell transplantation (HSCT). We herein report a 58-year-old woman presenting with massive pleural effusion approximately 1 year after allogeneic HSCT, who was successfully treated with corticosteroid. She had discontinued tacrolimus approximately 1 month before she presented with pleural effusion, which was attributed to cGVHD after a thorough exclusion process. This case illustrates a unique manifestation of atypical cGVHD and highlights the need for prompt therapy initiation.

Spontaneous Coronary Artery Dissection Presenting as Electrical Storm: A Case Report.

Spontaneous coronary artery dissection (SCAD) is an important cause of myocardial infarction in patients of younger age without typical atherosclerotic risk factors and can present with ventricular arrhythmia such as ventricular tachycardia (VT) or ventricular fibrillation (VF). Electrical storm (ES) is defined as greater than or equal to 3 episodes of VT or VF occurring within 24 hours. A healthy 38-year-old-male developed chest pain while exercising at the gym and presented to the emergency department unresponsive in a ventricular arrhythmia defined as ES. The patient's cardiac arrest was initially refractory to standard defibrillation and Advanced Cardiac Life Support medications. He was ultimately diagnosed with SCAD of the left anterior descending artery, which was stented. The patient survived neurologically intact after a 13-day hospital stay. Spontaneous coronary artery dissection is a significant cause of acute coronary syndrome and occurs in healthier patients without cardiac risk factors. Electrical storm represents a unique manifestation of SCAD. Emergency physicians should maintain a heightened suspicion for SCAD for accurate diagnosis and treatment.

Aging or chronic stress impairs working memory and modulates GABA and glutamate gene expression in prelimbic cortex.

The glucocorticoid (GC) hypothesis posits that effects of stress and dysregulated hypothalamic-pituitary-adrenal axis activity accumulate over the lifespan and contribute to impairment of neural function and cognition in advanced aging. The validity of the GC hypothesis is bolstered by a wealth of studies that investigate aging of the hippocampus and decline of associated mnemonic functions. The prefrontal cortex (PFC) mediates working memory which also decreases with age. While the PFC is susceptible to stress and GCs, few studies have formally assessed the application of the GC hypothesis to PFC aging and working memory. Using parallel behavioral and molecular approaches, we compared the effects of normal aging versus chronic variable stress (CVS) on working memory and expression of genes that encode for effectors of glutamate and GABA signaling in male F344 rats. Using an operant delayed match-to-sample test of PFC-dependent working memory, we determined that normal aging and CVS each significantly impaired mnemonic accuracy and reduced the total number of completed trials. We then determined that normal aging increased expression of Slc6a11, which encodes for GAT-3 GABA transporter expressed by astrocytes, in the prelimbic (PrL) subregion of the PFC. CVS increased PrL expression of genes associated with glutamatergic synapses: Grin2b that encodes the GluN2B subunit of NMDA receptor, Grm4 that encodes for metabotropic glutamate receptor 4 (mGluR4), and Plcb1 that encodes for phospholipase C beta 1, an intracellular signaling enzyme that transduces signaling of Group I mGluRs. Beyond the identification of specific genes that were differentially expressed between the PrL in normal aging or CVS, examination of Log2 fold-changes for all expressed glutamate and GABA genes revealed a positive association between molecular phenotypes of aging and CVS in the PrL but no association in the infralimbic subregion. Consistent with predictions of the GC hypothesis, PFC-dependent working memory and PrL glutamate/GABA gene expression demonstrate comparable sensitivity to aging and chronic stress. However, changes in expression of specific genes affiliated with regulation of extracellular GABA in normal aging vs. genes encoding for effectors of glutamatergic signaling during CVS suggest the presence of unique manifestations of imbalanced inhibitory and excitatory signaling in the PFC.

Oral Chagas Disease in Colombia-Confirmed and Suspected Routes of Transmission.

Chagas disease (CD) remains endemic throughout many regions of Colombia despite implementing decades of vector control strategies in several departments. Some regions have had a significant decrease in vectorial transmission, but the oral ingestion of Trypanosoma cruzi through consumption of contaminated food and drink products is increasingly described. This form of transmission has important public health relevance in Colombia due to an increase in reported acute CD cases and clinical manifestations that often lead to significant morbidity and mortality. Oral CD in Colombia has been associated with the consumption of contaminated fruit juices, such as palm wine, sugar cane, or tangerine juice and water for consumption, or contaminated surfaces where food has been prepared. Another interesting route of oral transmission includes ingestion of unbeknownst infected armadillos' blood, which is related to a traditional medicine practice in Colombia. Some earlier reports have also implemented consumption of infected bush meat as a source, but this is still being debated. Within the Amazon Basin, oral transmission is now considered the principal cause of acute CD in these regions. Furthermore, new cases of acute CD are now being seen in departments where CD has not been documented, and triatomine vectors are not naturally found, thus raising suspicion for oral transmission. The oral CD could also be considered a food-borne zoonosis, and odoriferous didelphid secretions have been implemented in contaminating the human dwelling environment, increasing the risk of consumption of infectious metacyclic trypomastigotes. In this article, we will discuss the complex transmission dynamics of oral CD in Colombia and further examine the unique clinical manifestations of this route of infection. New insights into the oral transmission of Trypanosoma cruzi are being discovered in Colombia, which can help bring increased awareness and a better understanding of this neglected tropical disease to reduce the burden of CD throughout Latin America.

О пище, напитках и их приготовлении в XX веке на юге Воронежской области (этнолингвистический взгляд)

This article reflects the traditional village cuisine of one settlement in the Central Black Earth Region. The purpose of the study is to determine the nutritional content of the residents of the village of Sknarovka, Kantemirovsky District, Voronezh Region, in the early and mid-20th century as an element of folk culture and to represent the corresponding vocabulary. The work uses the following methods: survey, observation, analysis and description. The scientific essay displays dishes and drinks, dishes and utensils of the past. It has been established that food was not only a vital necessity, but also represented a kind of value for villagers. Food and drinks had a certain cultural meaning. They were produced in the household, recipes were passed down from generation to generation and formed part of the picture of the human world. In the process of research activities, it was shown that in the 21st century the table of villagers and their preferences became more diverse, so the process of cooking in the oven and the utensils used in it previously lost their former relevance, since the tradition has changed. The Sknarovsky Russian-Ukrainian dialect contains commonly used, dialectal, colloquial and vernacular lexical units. The novelty of the study is that it presents for the first time the food culture of the inhabitants of the past century of a gradually dying rural settlement in the Voronezh region. The practical significance of the study is that it introduces interesting dialectal materials into circulation, illustrating the tradition in its unique manifestation in the outback in the south of Central Russia.