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This paper introduces a simple graph associated to a commutative ring. Nonzero ideals I and J of a commutative ring R are called Krull dimension-dependent whenever dim R / (I + J) = min { dim R / I , dim R / J } . Based on this, we introduce the dimension graph of R. We study and investigate this graph, and we determine all rings with disconnected dimension graph. Moreover, we introduce strongly Krull dimension-dependent ideals, and we examine these ideals using a subgraph of the dimension graph. [ABSTRACT FROM AUTHOR]

In this paper, we introduce the Krull dimension-dependent elements of a Noetherian commutative ring. Let x , y be non-unit elements of a commutative ring R. x , y are called Krull dimension-dependent elements, whenever dim R / (R x + R y) = min { dim R / R x , dim R / R y }. We investigate the elements of a ring according to this property. Among the many results, we characterize the rings that all elements of them are Krull dimension-dependent and we call them, closed under the Krull dimension. Moreover, we determine the structure of the rings with Krull dimension at most 1, that are closed under the Krull dimension. [ABSTRACT FROM AUTHOR]

In this paper, we associate a simple graph to a module over a commutative ring and call it extended graph, that adjacent condition depends on the properties of annihilator submodules. We determine the structure of modules, when the associated graph is star or complete. Moreover, we look for relationships between the extended graph and the zero-divisor graph of modules. [ABSTRACT FROM AUTHOR]

Let R be a commutative ring and M be an R -module. The compressed essential graph of M , denoted by E G (M) is a simple undirected graph associated to M whose vertices are classes of torsion elements of M and two distinct classes [ m ] and [ m ′ ] are adjacent if and only if Ann R (m) + Ann R (m ′) is an essential ideal of R. In this paper, we study diameter and girth of E G (M) and we characterize all modules for which the compressed essential graph is connected. Moreover, it is proved that ω (E G (M)) = | Ass R (M) | , whenever R is Noetherian and M is a finitely generated multiplication module with r ( Ann R (M)) = 0. [ABSTRACT FROM AUTHOR]

This consensus statement by a panel of neurology experts aimed to provide a practical and implementable guidance document to assist clinicians with the best clinical practice in terms of diagnosis, treatment, and monitoring of late-onset Pompe disease (LOPD). The participating experts consider the clinical suspicion of LOPD by the physician to be of utmost importance in the prevention of diagnostic and therapeutic delay in LOPD patients. A diagnostic algorithm is proposed to facilitate the diagnosis of LOPD in patients presenting with unexplained proximal/axial weakness (with or without respiratory symptoms) or restrictive respiratory insufficiency with hyperCKemia and/or exercise intolerance as the red flag symptoms/signs that raise the index of suspicion for LOPD diagnosis. The diagnosis is based on the subsequent use of dried blood spot (DBS) assay, and the DBS assay can be confirmed by acid alpha-glucosidase (GAA) tissue analysis in leukocytes, fibroblasts, or muscle fibers and/or genetic mutation analysis. Accordingly, experts consider increased awareness among physicians about potential presenting characteristics with a high index of suspicion for LOPD to be crucial to suspect and consider LOPD in the differential diagnosis, while strongly suggesting the use of a diagnostic algorithm combined with DBS assay and confirmatory tests in the timely diagnosis of LOPD and implementation of best practice patterns.

Objective: Balance problems and falls due to a progressive loss of muscle strength in neuromuscular diseases (NMD) negatively affect the activities of daily living and functional independence of patients. The timed up and go (TUG) test is an easy-to-apply and objective measure of balance. This study aimed to determine the cut-off value of the TUG test in adult individuals with NMD. Materials and Methods: This retrospective study included 102 patients. The receiver operating characteristic analysis was performed and cut-off, sensitivity, and specificity values were calculated in NMD to determine the diagnostic validity of the TUG test. Results: The mean ages of 55 patients with a fall history and 47 patients without a fall history were 34.40+-10.41 and 31.87+-10.11 years, respectively. The mean values of the TUG scores of these individuals were calculated as 11.79+-4.30 and 7.33+-1.51 s, respectively. The cut-off value of the TUG test in adult individuals with the NMD was determined as 8.9 s. Conclusion: The present study revealed that individuals with NMD who complete the TUG test over 8.9 s have a high risk of falling. Determining the risk of falling in NMD, which is one of the neurological patient groups where falling complaints are most common, is extremely important for patient safety and the effectiveness of rehabilitation.

Bombus terrestris dalmatinus is naturally common in many countries, including Turkey, and is also used commercially for the pollination of greenhouse plants. Intensive commercial production and international trade in many countries are considered as reasons for the disappearance of some natural populations. Hybridization of native bumble bees with those produced commercially, but having escaped from greenhouses and colonization of these commercial bees in natural habitats are cause for concern. In order to assess this concern, B. t. dalmatinus workers were collected from twelve different populations: five commercial producers, three surrounding greenhouse centers, three natural areas at least 30 km away from greenhouses, and one more recent greenhouse zone in Antalya, Turkey. The genetic variations and relationships among the twelve populations were estimated using SNP haplotypes determined in mitochondrial COI and CytB. Twenty and sixteen haplotypes were obtained for COI and CytB, respectively. A single haplotype, H1, was widespread with a high frequency in all individuals for both genes. Individuals collected from around greenhouse centers and commercial companies had more common haplotypes. The genetic variations of intra-populations were higher than the interpopulations in both COI (65.41%>34.59%) and CytB (72.47%>27.53%). The natural and commercial populations were genetically more distant from each other considering Fst values. However, samples from near the greenhouses had a higher similarity with the commercially produced samples, while the natural populations far away from greenhouses still retained their genetic distinctiveness.

Trigeminal neuropathy is an infrequent condition, usually limited to the sensorial component of the nerve, and it is one of the most common neurologic manifestations of mixed connective tissue disease (MCTD). However, to the best of our knowledge, no cases have been reported with MCTD presenting as trigeminal sensory and motor neuropathy. Herein, we report a 28-year-old female with unilateral trigeminal sensory–motor neuropathy who presented with numbness over the distribution of the right trigeminal region and right masseter atrophy. We also reviewed the literature for trigeminal neuropathy associated with MCTD.

Sultan II. Abdülhamid kendi döneminde, ülke genelinde at ve beygir cinsinin ıslahı ve sayılarını artırmak, ordunun çeşitli sınıflarının ihtiyacını karşılamak, çiftlik sahiplerinin ve ziraat ile meşgul olanların kullanacağı hayvanları temin etmek ve bu konuda yabancı ülkelerden hayvan teminine ihtiyaç duymamak için “Hayvan Çiftlikleri” kurdurmuştur. Bu çiftliklerden bazıları özel olarak askeriyenin ihtiyacını karşılamaya yönelik olmuştur. Sultansuyu, Botan, Hamra, Veziriye, Harnini çiftlikleri örnek olarak verilebilir. Bu çiftlikler IV. V. ve VI. Ordunun Remont bölüklerinin at ve beygir ihtiyacını karşılamak amacıyla kurulmuştur.Bununla birlikte bu çiftliklerin bir özelliği de Dicle ve Fırat Nehri vadisinde yer almalarıdır. Sultan II. Abdülhamid Irak coğrafyasında devletin hâkimiyetini tesis etmek amacıyla buradaki arazileri bir yandan Hazine-i Hassa Nezareti çatısı altında toplarken diğer yanda da Dicle ve Fırat Nehri üzerindeki askeri kontrolü artırmaya çalışmıştır.Bu çalışmada Dicle Nehri vadisinde kurulan, Tikrit-Musul karayolu güzergahında ve aynı zamanda Şammar Aşiretinin temel yerleşim alanı içerisinde bulunan ve VI.Ordu Müşirliğine tahsis edilmiş olan Harnini/Kharnini çiftliği ele alınacaktır. Çalışma harita ve çizimlerle desteklenecektir.

Metachromatic leukodystrophy (MLD) is a glycosphingolipid storage disease caused by deficiency of the lysosomal enzyme arylsulfatase A (ASA) or its activator protein saposin B. MLD can affect all age groups in severity varying from a severe fatal form to milder adult onset forms. Diagnosis is usually made by measuring leukocyte ASA activity. However, this test can give false negative or false positive laboratory results due to pseudodeficiency of ASA and saposin B deficiency, respectively. Therefore, we aimed to evaluate patients with suspected MLD in a Turkish population by comprehensive clinical, biochemical, radiological, and genetic analyses for molecular and phenotypic characterization. We analyzed 28 suspected MLD patients and 41 relatives from 24 families. ASA activity was found to be decreased in 21 of 28 patients. Sixteen patients were diagnosed as MLD (11 late infantile, 2 juvenile and 3 adult types), 2 MSD, 2 pseudodeficiency (PD) and the remaining 8 patients were diagnosed as having other leukodystrophies. Enzyme analysis showed that the age of onset of MLD did not correlate with residual ASA activity. Sequence analysis showed 11 mutations in ARSA, of which 4 were novel (p.Trp195GlyfsTer5, p.Gly298Asp, p.Arg301Leu, and p.Gly311Asp), and 2 mutations in SUMF1 causing multiple sulfatase deficiency, and confirmed the diagnosis of MLD in 2 presymptomatic relatives. All individuals with confirmed mutations had low ASA activity and urinary sulfatide excretion. Intra- and inter-familial variability was high for the same ARSA missense genotypes, indicating the contribution of other factors to disease expression. Imaging findings were evaluated through a modified brain MRI scoring system which indicated patients with protein-truncating mutations had more severe MRI findings and late-infantile disease onset. MRI findings were not specific for the diagnosis. Anti-sulfatide IgM was similar to control subjects, and IgG, elevated in multiple sulfatase deficiency. In conclusion, the knowledge on the biochemical, clinical and genetic basis of MLD was expanded, a modified diagnostic laboratory algorithm for MLD based on integrated evaluation of ASA activity, urinary sulfatide excretion and genetic tests was devised.

Background: We carried out a study for the purpose of comparing the six sigma values of the C-reactive protein (CRP) assay measured by immunonephometry with references of different authorities’ quality criterias and evaluating the performance of the devices in our laboratory. Methods: In our study, internal quality control data of the CRP test performed on two different Siemens BN ProSpec (Dade Behring, Derfield, IL) devices at Bursa Yuksek Ihtisas Training and Research Hospital. Results: According to Ricos ‘desired Total allowable error (TEa) ratio’; all the sigma values of the CRP test in BN ProSpec-1 and BN ProSpec-2 nephelometry devices were calculated as> 6 σ in each of the three periods and the performance of the CRP test was notified as ‘world class’. Also, the sigma value determined according to Ricos 'optimal TEa' ratio was 5.8 σ (3.90 to 8.48 σ in each period) on both devices and CRP test performance was defined as ‘excellent’. Moreover, when we considered the ratio of Rilibak TEa, the mean sigma values were 3.9 σ (between 2.59 and 5.75 σ in all three periods) and the CRP test performance was identified ‘good’. Conclusion: As a consequence, the performance of the CRP test with nephelometry was evaluated as ‘good’ when the Rilibak criteria with the lowest TEa values is taken into consideration. Therefore, it is necessary for the correct quality control analysis to establish the reference TEa% values of our country for the CRP test.

Abstract Background Perineuronal nets (PNNs), which are localized around neurons during development, are specialized forms of neural extracellular matrix with neuroprotective and plasticity-regulating roles. Hyaluronan and proteoglycan link protein 1 (HAPLN1), tenascin-R (TNR) and aggrecan (ACAN) are key elements of PNNs. In diseases characterized by neuritogenesis defects, the expression of these proteins is known to be downregulated, suggesting that PNNs may have a role in neural differentiation. Methods In this study, the mRNA and protein levels of HAPLN1, TNR and ACAN were determined and compared at specific time points of neural differentiation. We used PC12 cells as the in vitro model because they reflect this developmental process. Results On day 7, the HAPLN1 mRNA level showed a 2.9-fold increase compared to the non-differentiated state. However, the cellular HAPLN1 protein level showed a decrease, indicating that the protein may have roles in neural differentiation, and may be secreted during the early period of differentiation. By contrast, TNR mRNA and protein levels remained unchanged, and the amount of cellular ACAN protein showed a 3.7-fold increase at day 7. These results suggest that ACAN may be secreted after day 7, possibly due to its large amount of post-translational modifications. Conclusions Our results provide preliminary data on the expression of PNN elements during neural differentiation. Further investigations will be performed on the role of these elements in neurological disease models.

Objective: Hereditary neuropathy with liability to pressure palsy (HNPP) needs to be differentiated from entrapment neuropathies due to differences in the treatment management. Materials and Methods: Among 5075 patients with entrapment neuropathy, we retrospectively evaluated the neurophysiologic results of 20 patients with three or more entrapments. Results: Ten patients were diagnosed as having HNPP according to their genetic or nerve biopsy results; eight (80%) had bilateral Carpal tunnel syndrome, nine (90%) had bilateral ulnar entrapment neuropathy, eight (80%) had bilateral median and ulnar entrapment together; and three (30%) had one-sided peroneal neuropathy. Conclusion: Our data suggest that analyzing the neurophysiologic studies and keeping HNPP in mind are essential to characterize underdiagnosed patients with HNPP referred for entrapment neuropathy.

Dysferlinopathy is a rare autosomal recessive myopathy, resulting in the lack or absence of dysferlin production caused by mutations in the encoding gene. Dysferlin is a sarcolemmal membrane protein involved in the repair of membrane damage caused by calcium. There are four identified phenotypic dysferlinopathies, two of which are relatively frequently observed. Miyoshi myopathy and limb-girdle muscular dystrophy type 2B are frequently observed; the two very rare dysferlinopathies are distal anterior compartment myopathy and scapuloperoneal muscular dystrophy (observed in only one case). Serum CK levels are quite high, even in the pre-clinical period. A muscle biopsy typically shows dystrophic patterns, often accompanied with T-lymphocyte-based inflammatory changes. The clinical course of dysferlinopathy is usually much better than that of other recessive trait muscular dystrophies. Dysferlinopathies should be considered in the differential diagnosis of polymyositis to avoid unnecessary and potentially dangerous medications such as oral steroids or immunosuppressive therapies. Here we report the case a 21-year-old Syrian patient diagnosed with dysferlinopathy who has had serious CK elevations from the age of 1 and who had been diagnosed with polymyositis by a muscle biopsy 7 years ago and who therefore used steroids/azathioprine for the following 3 years.

OBJECTIVE: Polyneuropathy is the most frequent complication of diabetes mellitus (DM), which is a common disorder worldwide. Early diagnosis of this complication is critical for treatment planning. Recently, intra-epidermal nerve fiber (IENF) analysis has been used for the early diagnosis of diabetic neuropathy and was reported to be superior to both conventional fascicular nerve biopsy and other diagnostic tests. The present study aimed to determine the effect of early glucose metabolism disorders on the peripheral nerves using IENF analysis. METHODS: : This prospective study compared IENF nerve fiber density in 10 patients with early-stage DM and 12 patients with impaired glucose tolerance (IGT) to that of healthy controls in order to investigate fiber loss in the asymptomatic stage. RESULTS: Mean age of the DM and IGT groups was 53.4 ± 10.9 years and 50.8 ± 10.6 years, respectively. Neither the IGT nor DM patients had paresthesia. Neurological examination results of the patients were all in the normal range. Mean fiber density was 6.4 ± 2.7 mm-1 in the DM group, 7.5 ± 3.2 mm-1 in the IGT group, and 13.7 ± 2.7 mm-1 in the control group (p< 0.0001). Paired analysis of the groups showed a significant difference between the 2 patient groups and the control group. There was a statistically significant difference in mean nerve fiber density between the DM and control groups (p< 0.0001, Mann-Whitney U test), and between the IGT and control groups (p< 0.0001, Mann-Whitney U test); however, there wasn’t a significant difference between the DM and IGT groups (p= 0.5, Mann-Whitney U test). CONCLUSION: The results show that IENF density in the IGT and DM groups decreased in the asymptomatic stage of DM. This means that polyneuropathy can begin in the prediabetic period in patients with glucose metabolism disorders. This critical information highlighs the importance of the early diagnosis and treatment of glucose metabolism disorders in preventing neurological complications

AIM: The aim of this study was to investigate the efficacy of sodium nitroprusside in the reduction of the intestinal ischemiareperfusion injury as a nitric oxide donor after intraperitoneal administration. METHODS: The histopathological examinations and tissue malonyldialdehyde levels of 35 Wistar albino rats that were subjected to ischemia-reperfusion, were performed in 5 groups. The groups include Control, Ischemia -reperfusion, Sodium nitroprusside, NG-Nitro-L-Arginine Methyl Ester (L-NAME) and Sodium nitroprusside+L-NAME. Each rat was subjected to ischemia for 40 minutes and reperfusion for 30 minutes, except the control group. The medications were done intraperitoneally as saline 4 ml/kg, Sodium nitroprusside 5 mg/kg, L-NAME 10 mg/kg just before reperfusions. RESULTS: Significant tissue injury in histological sections and an increase in tissue levels of Malonyldialdehyde was detected in the I/R group. The efficacy of intraperitoneal administration of Sodium nitroprusside in both Sodium nitroprusside alone and Sodium nitroprusside+L-NAME groups was found statistically significant for the reducing of injury scores (p

Purpose. We investigated the effect of eritoran, a Toll-like receptor 4 antagonist, on retinochoroidal inflammatory damage in an endotoxin-induced inflammatory rat model. Methods. Endotoxin-induced inflammatory model was obtained by intraperitoneal injection of 1.5 mg/kg lipopolysaccharide (LPS). Group 1 had control rats; in groups 2-3 LPS and 0.5 mg/kg sterile saline were injected; and in groups 4-5 LPS and 0.5 mg/kg eritoran were injected. Blood samples were taken and eyes were enucleated after 12 hours (h) (groups 2 and 4) or 24 hours (Groups 3 and 5). Tumor necrosis factor-α (TNF-α) and malondialdehyde (MDA) levels in the serum and retinochoroidal tissue and nuclear factor kappa-B (NFκB) levels in retinochoroidal tissue were determined. Histopathological examination was performed and retinochoroidal changes were scored. Results. Eritoran treatment resulted in lower levels of TNF-α, MDA, and NFκB after 12 h which became significant after 24 h. Serum TNF-α and retinochoroidal tissue NFκB levels were similar to control animals at the 24th h of the study. Eritoran significantly reversed histopathological damage after 24 h. Conclusions. Eritoran treatment resulted in less inflammatory damage in terms of serum and retinochoroidal tissue parameters.

Purpose: Bumblebees are an important group of insects in the pollination of various vegetables, fruits, oilseeds, legumes, and the fodder crops. Compared to honeybees, they have a wider choice of hosts and a longer flight period. These bees are used especially for the pollination of plants in greenhouses and are commercially produced for this purpose. Recently, serious decreases have been occurring in bumblebee populations due to various reasons such as pathogens, and some of species are even threatened with extinction. Due to the worldwide decline in pollinator insects, determining the distribution and prevalence of bumblebee pathogens is of great importance. Therefore, this study was conducted to determine the incidence and prevalence of pathogens in Turkish bumblebee populations and how much of each pathogen was in bumblebee samples., Methods: A total of 172 Bombus terrestris (Linnaeus,1758) samples (21 samples from commercial enterprises, 79 samples from greenhouses and 72 samples from nature) were randomly collected from 3 provinces (Antalya, Mersin and İzmir) where greenhouse cultivation is intensively carried out in Turkey. Eighty-nine of these samples were collected in the spring and eighty-three in the autumn. The presence of four pathogens (Nosema bombi, Crithidia bombi, Apicystis bombi, and Locustacarus buchneri) was investigated by PCR using universal primers., Results: The overall prevalence of Nosema bombi, Crithidia bombi, Apicystis bombi, and Locustacarus buchneri was determined as 7.55%, 9.3%, 11.62%, and 4.65%, respectively. Co-infections (5.81%) were only detected in wild-caught (nature) samples. C. bombi and A. bombi infections were detected at higher rates in the spring samples than in the autumn samples (p < 0.05). There was no significant difference between the spring and autumn samples with respect to the presence of N. bombi and L. buchneri (p > 0.05)., Conclusion: The results obtained could be important in determining the prevalence and spread rates of the bumblebee diseases in Turkey and to determine appropriate protection measures. The information gathered should increase our knowledge about the presence of these pathogens in Turkey and could contribute to improve apiarist's practice. More studies are needed to determine the transmission pathways of these pathogens between the populations. Also, complex pathogen interactions in bumblebee populations should be considered in the future to improve bumblebee health., (© 2023. The Author(s) under exclusive licence to Witold Stefański Institute of Parasitology, Polish Academy of Sciences.)

Introduction: Dermatomyositis (DM) is a rare inflammatory disease with diverse cutaneous and systemic manifestations, often associated with myositis-specific antibodies. Managing patients with refractory DM, or individuals presenting pecific complications, like calcinosis or rapidly progressive interstitial lung disease, presents unique challenges., Areas Covered: This review explores current and promising treatment options for DM, drawing from clinical studies, case series, and case reports that consider the underlying disease pathophysiology., Expert Opinion: Recent advancements have improved our understanding and management of DM. The discovery of distinct DM autoantibodies and their correlation with specific clinical phenotypes has transformed patient categorization and enhanced our knowledge of the pathogenesis of the disease. Intravenous immunoglobulin, a well-established treatment in dermatomyositis, has regained prominence and a large randomized clinical trial has reaffirmed its efficacy, confirming it as an effective therapeutic option in this group of patients. Identification of the type I interferon pathway as a key pathogenic mechanism in DM has opened up new avenues for more effective treatment strategies. Blocking the JAK/STAT pathway offers potential for improved management of refractory patients and prevention of highly morbid complications. These recent advancements have significantly impacted the management and care of dermatomyositis patients, enabling tailored approaches, targeted interventions, and improved outcomes for individuals affected by this complex condition.

Background: This study aimed to evaluate antibiotic resistance genes and virulence genes and the clonal relationship of the carbapenem-nonsusceptible Klebsiella pneumoniae strains by molecular methods which are isolated from various clinical specimens from patients treated in tertiary care hospital in Turkey., Methods: Identification of 32 carbapenem non-susceptible K. pneumoniae were determined by VITEK-2 (BioMérieux, France) automated system. Thirteen colistin-resistant strains were tested with the broth microdilution method. Various antibiotic resistance genes and virulence genes frequently seen in carbapenem-resistant strains were screened by PCR. Immunochromatographic tests used in the rapid diagnosis of carbapenemases were compared with PCR results. In addition, PFGE, MLST and MALDI-TOF MS methods were used to determine the clonal relationship among these strains., Results: PCR demonstrated that 31 of the strains carried at least one of the carbapenemase genes. In one strain, the coexistence of bla OXA-48+NDM was shown. The most common resistance genes were determined as bla SHV (84.3%), bla CTX-M-1 (46.8%), bla OXA-48 (40.6%), bla KPC (40.6%), bla TEM (31.2%), bla NDM (18.8%) respectively. Among the virulence genes; magA (68.7%) was the most common, followed by kpn (59.3%) and K2 (9.3%). Immunochromatographic tests were found to be 100% compatible with PCR results. All colistin-resistant isolates were also found to be resistant by colistin broth microdilution. In PFGE analysis, 25 different genotypes were determined and clustering isolates were collected in 5 different clusters and the clustering rate was 35.4%. In MLST analysis, ST101 type was determined as the most common ST type with a rate of 29%. ST101 is followed by ST16, ST307, ST14, ST147, ST309, ST377, ST395 and ST2096, respectively. The compatibility rate between MALDI-TOF MS and VITEK-2 was found 94.3%, in bacterial identification. In MALDI-TOF MS typing, the maximum similarity between the strains was less than 70% and clustering not shown., Conclusion: In addition to OXA-48, which is endemic in our country, it has been determined that KPC, which is more common in the world, is becoming increasingly common in our region. ST101 type was determined as the most common type between the strains. To the best of our knowledge, this is the first study that compares these three methods in our country. There may be differences between bacterial identifications made with VITEK-2 and MALDI-TOF MS. In this study, it was observed that MALDI-TOF MS analyses were not compatible with the typing of strains according to PFGE and MLST analysis results., (© 2023. The Author(s).)

Acarapis woodi (Rennie 1921) (Acari: Tarsonemidae) is one of the mites that settles in the respiratory system of honeybees (Apis mellifera L. (Hymenoptera, Apidae)) and distributed throughout the world. It causes significant economic losses on honey production. In Türkiye, studies on the existence of A. woodi are very limited and so far, no studies on the molecular diagnosis and phylogenetic of it have been reported in Türkiye. This study was conducted to investigate the prevalence of A. woodi in Türkiye, especially in areas where beekeeping is intense. Diagnosis of A. woodi was performed using both microscopic and molecular methods using specific PCR primers. Adult honeybee samples were collected from 1.193 hives in 40 provinces of Türkiye between 2018 and 2019. Based on identification studies, the presence of A. woodi was detected in a total of 3 hives (0.5%) in 2018 and 4 hives (0.7%) in 2019. This is the first report for determination of A. woodi in Türkiye., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Relapsing polychondritis (RP) is an uncommon inflammatory disorder that predominantly targets cartilaginous structures. The disease frequently affects the nose, ears, airways, and joints, but it can also impact organs that aren't primarily cartilage-based, such as blood vessels, skin, inner ear, and eyes. Given its infrequent occurrence and recurrent symptoms, patients often experience delays in proper diagnosis. Lately, based on the organs involved, the disease's diverse manifestations have been categorized into specific clinical groups, based on the most likely organ involvement including auricular, nasal, pulmonary, and musculoskeletal. More recently the discovery of a new disease, called (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) VEXAS syndrome, due to mutations in UBA1 gene, identified the cause of 8 % of the patients with a clinical diagnosis of RP. VEXAS is likely the cause of a previously described "hematologic subgroup" in RP. This discovery is proof of concept that RP is likely more than one disease (Beck et al., Dec 31 2020; Ferrada et al., 2021). People diagnosed with RP face numerous hurdles, with the quality of their lives and overall prognosis being affected. Diagnosing the condition is particularly challenging due to its fluctuating symptoms, the absence of specific markers, and the lack of universally recognized classification criteria. For a correct diagnosis, it's imperative for healthcare professionals to identify its unique clinical patterns. Moreover, there are no approved metrics to gauge the disease's severity, complicating patient management. This review seeks to equip clinicians with pertinent insights to better diagnose and attend to these complex patients., (Copyright © 2023. Published by Elsevier Ltd.)

Objective: In antiphospholipid antibody (aPL) nephropathy, activation of the mammalian target of rapamycin (mTOR) contributes to endothelial cell proliferation, a key finding of aPL microvascular disease. Here, we examined mTOR activation in the skin of aPL-positive patients with livedo., Methods: Three patient groups with livedo were studied: (1) persistently aPL-positive with systemic lupus erythematosus (SLE); (2) persistently aPL-positive without SLE; and (3) aPL-negative SLE (control). After collecting aPL-related medical history, two 5-mm skin biopsies of livedo were performed on each patient: (1) peripheral (erythematous-violaceous lesion); and (2) central (nonviolaceous area). We stained specimens for phosphorylated protein kinase B (p-AKT) and phosphorylated S6 ribosomal protein (p-S6RP) as mTOR activity markers, CD31 to identify endothelial cells, and Ki-67 to show cellular proliferation. We counted cells in the epidermis and compared mTOR-positive cell counts between peripheral and central samples, and between patient groups, using Freidman test and Wilcoxon signed-rank test., Results: Ten patients with livedo reticularis were enrolled: 4 aPL-positive without SLE (antiphospholipid syndrome [APS] classification met, n = 3), 4 aPL-positive SLE (APS classification met, n = 3), and 2 aPL-negative SLE (control). In all aPL-positive patients, epidermal p-AKT and p-S6RP staining were significantly increased in both peripheral and central skin samples when compared to aPL-negative SLE controls; both were more pronounced in the lower basal layers of epidermis., Conclusion: Our study demonstrates increased mTOR activity in livedoid lesions of aPL-positive patients with or without SLE compared to aPL-negative patients with SLE, with more prominent activity in the lower basal layers of the epidermis. These findings may serve as a basis for further investigating the mTOR pathway in aPL-positive patients., (Copyright © 2022 by the Journal of Rheumatology.)

Objectives: To describe the outcomes of pregnancies in antiphospholipid antibody (aPL)-positive patients since the inception of the AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking Registry., Methods: We identified persistently aPL-positive patients recorded as 'pregnant' during prospective follow-up, and defined 'aPL-related outcome' as a composite of: (1) Preterm live delivery (PTLD) at or before 37th week due to pre-eclampsia (PEC), eclampsia, small-for-gestational age (SGA) and/or placental insufficiency (PI); or (2) Otherwise unexplained fetal death after the 10th week of gestation. The primary objective was to describe the characteristics of patients with and without aPL-related composite outcomes based on their first observed pregnancies following registry recruitment., Results: Of the 55 first pregnancies observed after registry recruitment among nulliparous and multiparous participants, 15 (27%) resulted in early pregnancy loss <10 weeks gestation. Of the remaining 40 pregnancies: (1) 26 (65%) resulted in term live delivery (TLD), 4 (10%) in PTLD between 34.0 weeks and 36.6 weeks, 5 (12.5%) in PTLD before 34th week, and 5 (12.5%) in fetal death (two associated with genetic anomalies); and (2) The aPL-related composite outcome occurred in 9 (23%). One of 26 (4%) pregnancies with TLD, 3/4 (75%) with PTLD between 34.0 weeks and 36.6 weeks, and 3/5 (60%) with PTLD before 34th week were complicated with PEC, SGA and/or PI. Fifty of 55 (91%) pregnancies were in lupus anticoagulant positive subjects, as well as all pregnancies with aPL-related composite outcome., Conclusion: In our multicentre, international, aPL-positive cohort, of 55 first pregnancies observed prospectively, 15 (27%) were complicated by early pregnancy loss. Of the remaining 40 pregnancies, composite pregnancy morbidity was observed in 9 (23%) pregnancies., Competing Interests: Competing interests: MAP is the chair for the Belimumab Pregnancy Registry, supported by GSK. DWB is the co-principal investigator of The TNF-alpha Blockade with Certolizumab to Prevent Pregnancy Complications in High-Risk Patients with APS, supported by a grant from the NIH/NIAMS R21AR21069189-03S1. DWB is the principal investigator of The Certolizumab to Prevent Pregnancy Complications in High-Risk Patients with APS or SLE, supported by UCB Pharma Inc., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Methods: We identified 20 adult patients with UCTD enrolled in the UCTD and Overlap Registry at our tertiary care level hospital. A licensed clinical social worker administered a 30-minute semistructured interview by telephone. The standardized questionnaire consisted of 14 open-ended questions on UCTD. A team of physicians, research coordinators, and a social worker used grounded theory to analyze the qualitative data and identify themes., Results: Among 14/20 study participants (100% female; mean age, 53.6 ± 13.2 years [range, 27-74 years]), all had at least an associate's/bachelor's degree; 9 (64%) were White. The mean disease duration was 14.5 ± 13.5 years (range, 0.5-44 years). Nine study participants (64%) were engaged in counseling or mindfulness training. Ten specific psychosocial themes and categories emerged, including the need for professional guidance and peer and family support to increase awareness, reduce isolation, and promote self-efficacy., Conclusions: Emerging themes from semistructured interviews of women with UCTD at a major academic center suggest the need for psychosocial interventions (e.g., patient support groups, educational materials, peer counselors) to help UCTD patients manage and cope with their illness. Future studies evaluating the psychosocial impact of UCTD diagnosis on diverse cohorts are needed., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: To describe the baseline characteristics of patients with positivity for antiphospholipid antibodies (aPLs) who were enrolled in an international registry, the Antiphospholipid Syndrome (APS) Alliance for Clinical Trials and International Networking (APS ACTION) clinical database and repository, overall and by clinical and laboratory subtypes., Methods: The APS ACTION registry includes adults who persistently had positivity for aPLs. We evaluated baseline sociodemographic and aPL-related (APS classification criteria and "non-criteria") characteristics of patients overall and in subgroups (aPL-positive without APS, APS overall, thrombotic APS only, obstetric APS only, and both thrombotic APS/obstetric APS). We assessed baseline characteristics of patients tested for the presence of three aPLs (lupus anticoagulant [LAC] test, anticardiolipin antibody [aCL], and anti-β 2 -glycoprotein I [anti-β 2 GPI]) antibodies by aPL profiles (LAC only, single, double, and triple aPL positivity)., Results: The 804 aPL-positive patients assessed in the present study had a mean age of 45 ± 13 years, were 74% female, and 68% White; additionally, 36% had other systemic autoimmune diseases. Of these 804 aPL-positive patients, 80% were classified as having APS (with 55% having thrombotic APS, 9% obstetric APS, and 15% thrombotic APS/obstetric APS). In the overall cohort, 71% had vascular thrombosis, 50% with a history of pregnancy had obstetric morbidity, and 56% had experienced at least one non-criteria manifestation. Among those with three aPLs tested (n = 660), 42% were triple aPL-positive. While single-, double-, and triple aPL-positive subgroups had similar frequencies of vascular, obstetric, and non-criteria events, these events were lowest in the single aPL subgroup, which consisted of aCLs or anti-β 2 GPI only., Conclusion: Our study demonstrates the heterogeneity of aPL-related clinical manifestations and laboratory profiles in a multicenter international cohort. Within single aPL positivity, LAC may be a major contributor to clinical events. Future prospective analyses, using standardized core laboratory aPL tests, will help clarify aPL risk profiles and improve risk stratification., (© 2020, American College of Rheumatology.)

Objectives: Osteoarthritis is a common disease affecting the quality of life in the elderly population. Osteoarthritis is a painful condition commonly encountered in patients aged 65 years and older and it may cause muscle weakness. Sarcopenia is a condition that has an increasing prevalence in the elderly population. The present study evaluated the relationship between sarcopenia and osteoarthritis., Methods: The study included 100 patients aged 65 years and older who were diagnosed with diabetes mellitus. The patients were divided into two groups as Group 1 and Group 2. Group 1 was composed of 50 patients with diabetes and osteoarthritis and Group 2 was composed of 50 patients with diabetes but without osteoarthritis. A detailed medical history was obtained from all patients and all patients underwent physical examination. The get-up and go test was performed, handgrip strength was measured with a hand dynamometer, bioimpedance analysis was performed, and mid-upper arm circumference, calf circumference and waist circumference were measured, and laboratory tests including complete blood count, biochemical nutritional parameters, liver and kidney function tests, and erythrocyte sedimentation rate were ordered. The Kellgren and Lawrence grading system was used to evaluate the severity of osteoarthritis and the skeletal muscle mass index was used to evaluate the muscle mass. These parameters were compared between the two groups., Results: Of the study participants, 1% had severe sarcopenia, 22% had moderate sarcopenia, and 77% did not have sarcopenia. Albumin (p=0.013), magnesium (p=0.038), total protein (0.004), erythrocyte sedimentation rate (p=0.047), hemoglobin level (p=0.018), muscle strength (p=0.046), height (p=0.033), and muscle mass (p<0.05) were significantly different in patients with osteoarthritis compared to patients without osteoarthritis. Patients with osteoarthritis achieved poorer results on the get-up and go test (p=0.014), and mid-upper arm circumference (p=0.028), and calf circumference (p=0.016) were lower in this group. There was a negative moderate correlation between the muscle mass and the Kellgren and Lawrence grade (p<0.05, r: -0.405), whereas there was a positive moderate correlation between sarcopenia index and the Kellgren and Lawrence grade (p<0.05, r: 0.320) in patients with osteoarthritis., Conclusion: The present study is the first to evaluate the relationship between sarcopenia and osteoarthritis in geriatric diabetic patients. The present study found a significant relationship between osteoarthritis and sarcopenia in geriatric patients with type II diabetes mellitus. The authors suggest that pain associated with osteoarthritis results in immobility, decrease in functional performance, and thus development of sarcopenia., (Copyright © by The Medical Bulletin of Sisli Etfal Hospital.)

Background: DMSO is a cryoprotective agent (CPA) that is widely used in the cryopreservation of cells. However, evidence suggests that it is more effective when combined with other CPAs., Objective: To investigated the effect of combining serum and balanced solutions with DMSO for cryopreservation., Materials and Methods: Cartilage cells cultured with Dulbecco's Modified Eagle Medium (DMEM) medium were taken from the ears of adult cattle. Then, these cells were frozen by supplementation with different concentrations of fetal bovine serum (FBS) and different balanced solutions with DMSO., Results: The highest cell viability was obtained using freezing solutions containing 10% DMSO and 40% serum, in dextran 40 or dextrose solution or DMEM., Conclusion: Our results indicated that serum concentration is important for cell viability and that supplementing DMSO with dextran or dextrose or DMEM benefits the cryopreservation of bovine cartilage cells.

Objective: The APS ACTION Registry studies long-term outcomes in persistently antiphospholipid antibody (aPL)-positive patients. Our primary objective was to determine whether clinically meaningful aPL profiles at baseline remain stable over time. Our secondary objectives were to determine (1) whether baseline characteristics differ between patients with stable and unstable aPL profiles, and (2) predictors of unstable aPL profiles over time., Methods: A clinically meaningful aPL profile was defined as positive lupus anticoagulant (LAC) test and/or anticardiolipin (aCL)/anti-β 2 glycoprotein-I (anti-β 2 -GPI) IgG/M ≥ 40 U. Stable aPL profile was defined as a clinically meaningful aPL profile in at least two-thirds of follow-up measurements. Generalized linear mixed models with logit link were used for primary objective analysis., Results: Of 472 patients with clinically meaningful aPL profile at baseline (median follow-up 5.1 yrs), 366/472 (78%) patients had stable aPL profiles over time, 54 (11%) unstable, and 52 (11%) inconclusive. Time did not significantly affect odds of maintaining a clinically meaningful aPL profile at follow-up in univariate ( P = 0.906) and multivariable analysis ( P = 0.790). Baseline triple aPL positivity decreased (OR 0.25, 95% CI 0.10-0.64, P = 0.004) and isolated LAC test positivity increased (OR 3.3, 95% CI 1.53-7.13, P = 0.002) the odds of an unstable aPL profile over time., Conclusion: Approximately 80% of our international cohort patients with clinically meaningful aPL profiles at baseline remain stable at a median follow-up of 5 years; triple aPL-positivity increase the odds of a stable aPL profile. These results will guide future validation studies of stored blood samples through APS ACTION Core Laboratories., (Copyright © 2021 by the Journal of Rheumatology.)

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by thrombosis, pregnancy morbidity, or nonthrombotic manifestations in patients with persistently positive antiphospholipid antibodies (aPL). Conventional APS treatment focuses on antithrombotic strategies, which are usually ineffective for the microvascular and nonthrombotic manifestations of aPL. Using a case-based presentation, this review focuses on the role of immunosuppression in nonobstetric APS, including B-cell inhibition (rituximab, belimumab, and bortezomib), complement inhibition (eculizumab), mechanistic target of rapamycin inhibition (sirolimus), vascular endothelial cell modulation (defibrotide), statins, and traditional rheumatologic disease-modifying agents (hydroxychloroquine, mycophenolate mofetil, azathioprine, and cyclophosphamide)., Competing Interests: Conflict-of-interest disclosure: D.E. received research grants from American College of Rheumatology (ACR), European League Against Rheumatism (EULAR), Lupus Clinical Trials Consortium, and National Institutes of Health; is a clinical research investigator for GSK-sponsored studies; and is a consultant for GSK, UCB, and Exagen. E.S. and R.W. declare no competing financial interests., (© 2019 by The American Society of Hematology. All rights reserved.)

Background: Variability remains a challenge in lupus anticoagulant (LA) testing., Objective: To validate LA test performance between Antiphospholipid Syndrome Alliance for Clinical Trials and International Networking (APS ACTION) Core laboratories and examine agreement in LA status between Core and local/hospital laboratories contributing patients to this prospective registry., Methods: Five Core laboratories used the same reagents, analyzer type, protocols, and characterized samples for LA validation. Non-anticoagulated registry samples were retested at the corresponding regional Core laboratories and anticoagulated samples at a single Core laboratory. Categorical agreement and discrepancies in LA status between Core and local/hospital laboratories were analyzed., Results: Clotting times for the reference/characterized plasmas used for normalized ratios were similar between Core laboratories (CV <4%); precision and agreement for LA positive/negative plasma were similar (all CV ≤5%) in the four laboratories that completed both parts of the validation exercise; 418 registry samples underwent LA testing. Agreement for LA positive/negative status between Core and local/hospital laboratories was observed in 87% (115/132) non-anticoagulated and 77% (183/237) anticoagulated samples. However, 28.7% (120/418) of samples showed discordance between the Core and local/hospital laboratories or equivocal LA results. Some of the results of the local/hospital laboratories might have been unreliable in 24.7% (41/166) and 23% (58/252) of the total non-anticoagulated and anticoagulated samples, respectively. Equivocal results by the Core laboratory might have also contributed to discordance., Conclusions: Laboratories can achieve good agreement in LA performance by use of the same reagents, analyzer type, and protocols. The standardized Core laboratory results underpin accurate interpretation of APS ACTION clinical data., (© 2019 International Society on Thrombosis and Haemostasis.)

Objectives: To assess whether patients with antiphospholipid syndrome (APS) and history of recurrent thrombosis have higher levels of adjusted Global AntiphosPholipid Syndrome Score (aGAPSS) when compared to patients without recurrent thrombosis., Methods: In this cross-sectional study of antiphospholipid antibody (aPL)-positive patients, we identified APS patients with a history of documented thrombosis from the AntiPhospholipid Syndrome Alliance For Clinical Trials and InternatiOnal Networking (APS ACTION) Clinical Database and Repository ("Registry"). Data on aPL-related medical history and cardiovascular risk factors were retrospectively collected. The aGAPSS was calculated at Registry entry by adding the points corresponding to the risk factors: three for hyperlipidemia, one for arterial hypertension, five for positive anticardiolipin antibodies, four for positive anti-β 2 glycoprotein-I antibodies and four for positive lupus anticoagulant test., Results: The analysis included 379 APS patients who presented with arterial and/or venous thrombosis. Overall, significantly higher aGAPSS were seen in patients with recurrent thrombosis (arterial or venous) compared to those without recurrence (7.8 ± 3.3 vs. 6 ± 3.9, p<0.05). When analyzed based on the site of the recurrence, patients with recurrent arterial, but not venous, thrombosis had higher aGAPSS (8.1 ± SD 2.9 vs. 6 ± 3.9; p<0.05)., Conclusions: Based on analysis of our international large-scale Registry of aPL-positive patients, the aGAPSS might help risk stratifying patients based on the likelihood of developing recurrent thrombosis in APS., (Copyright © 2019 Elsevier Inc. All rights reserved.)