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  • Hull Downs posted an update 4 days, 12 hours ago

    Catastrophic antiphospholipid syndrome (CAPS) is an unusual complication of antiphospholipid syndrome (APS) occurring in about 1% of patients. Oxaliplatin If left untreated, mortality can be as high as 50%. Therapy of APS and its complication CAPS is hampered by the lack of validated prospective, controlled, intervention clinical trials, although there is consensus that treatment should include anticoagulation therapy. But there are issues that need to be addressed such as duration and intensity of therapy. The present report describes our experience in 7 patients with CAPS in whom anticoagulation was discontinued after 6 months of therapy. During an average follow-up of 5.5 years, only 2 patients exhibited one episode each of recurrent venous thrombosis, but none of the patients in whom anticoagulation was discontinued experienced recurrent CAPS.Key Points• Discontinuation of long-term anticoagulation therapy in CAPS patients was not followed by recurrence of CAPS.Gout is a metabolic disorder and one of the most common arthritic conditions. Hyperuricemia is the hallmark of developing gout and mostly caused by uric acid underexcretion. Gout disproportionately affects people of specific races and ethnicities. Filipinos are the second-largest Asian population in the USA and reported to have a higher prevalence of gout and hyperuricemia than non-Filipino counterparts and Filipinos residing in the Philippines. The genetic polymorphism rs2231142 G>T in the ABCG2 has been strongly associated with hyperuricemia and gout across multiple populations. However, the prevalence of this variant in Filipinos is unknown. Therefore, assessing the prevalence of this variant may provide insights on the high prevalence of hyperuricemia and gout in Filipinos. A total of 190 DNA samples from pregnant females who self-identified as a Filipino from the Hawaii Biorepository Bank were genotyped for rs2231142 G>T in the ABCG2. The prevalence of the gout risk allele (T) (46%) was significantly higher in Filipinos than in samples of Caucasians (12%, p  T in ABCG2 may predispose Filipinos to hyperuricemia and gout when acculturated to high-purine diet.OBJECTIVE To investigate possible association between sacroiliitis and HLA-B*35 positivity. METHOD After excluding patients with axial spondyloarthritis and HLA-B*27 positivity, psoriasis inflammatory bowel disease, preceding infections, or juvenile type of spondyloarthritis, 110 patients were recruited with a diagnosis of undifferentiated axial spondyloarthritis. All of them had inflammatory back pain of short duration (3 months to 2 years) and 72 were HLA-B*35 positive. In order to determine if there is a possible association of sacroiliitis and HLA-B*35 positivity, all patients underwent MRI of sacroiliac joints. RESULTS A statistically significant association between the detection of bone marrow edema at sacroiliac joints on MRI and HLA-B*35 positivity (χ2 = 6.25; p = 0.022) was found. A logistic regression analysis revealed that the presence of HLA-B*35 allele was associated with a 6 times greater chance of identifying bone marrow edema at sacroiliac joints on MRI (OR 6, 95% CI 1.3-27, p = 0.021). HLA-B*ated with a 4.7 times greater chance of finding elevated CRP and a 5 times greater chance of finding peripheral joint synovitis in un-axSpa patients.• HLA-B*35 allele could be a potential risk factor for developing sacroiliitis and axSpA.Splenic involvement is rarely reported in patients with ANCA-associated vasculitides (AAVs), particularly in those with granulomatosis with polyangiitis (GPA) and is in fact considered to be underestimated. We aimed to investigate the frequency of splenic lesions-mainly infarction-and related factors in patients with AAVs. Patients with AAV whose abdominal or thoracic computed tomographies (CTs) were performed at or after diagnosis were included in the study. CT images were examined for splenic lesions. Overall, 69 patients (median age at diagnosis 54 [IQR 24] years; 55% with GPA, 29% with microscopic polyangiitis, and 16% with renal-limited disease) were included in the analysis. Splenic pathologies were detected in 19 (28%) patients; 12/19 (63%) splenomegaly and 7/19 (37%) splenic infarction. All patients with splenic infarction exhibited GPA with PR3-ANCA-positive serology. Three of these seven patients had autosplenectomy. Patients with splenic infarction were younger at diagnosis (p = 0.018) with also significantly higher ear-nose-throat (ENT) (%100 vs 37; p = 0.002) and eye involvement (%50 vs %12; p = 0.044) than patients without splenic infarction. Splenic pathologies are not rare in AAVs. Furthermore, infarction can help separate GPA from MPA. In young patients with GPA, particularly those with ENT and eye involvements, physicians should consider splenic infarction.Key Points• Splenic infarction is more common than previously thought in ANCA-associated vasculitides, particularly in granulomatosis with polyangiitis.• Detecting splenic infarction can help differentiate granulomatosis with polyangiitis from other subgroups.Food insecurity (FI), or limited or uncertain access to food, is a significant public health issue, especially for individuals with serious mental illness. As such, mental health providers should regularly assess FI and link individuals to resources. The purpose of this project was to create and pilot test a provider training on FI assessment at a community mental health center. Fifteen providers participated in the training and completed surveys at post-training and one-month follow-up. Results suggested success in delivering an acceptable training that conveyed the importance of FI and inspired confidence in assessment. Yet, it was not sufficient to modify providers’ FI assessment behavior. A brief training can effectively teach providers about FI and promote confidence in assessment; however, it is not adequate to change behavior. Future work should examine the provider training in a larger sample and wider variety of CMHC providers and explore ways to integrate FI assessment into existing practices to address implementation barriers.Individuals who carry pathogenic mutations in DNA mismatch repair (MMR) genes have high risks of cancer, and small studies have suggested that these risks depend on the sex of the parent from whom the mutation was inherited. We have conducted the first large study of such a parent-of-origin effect (POE). Our study was based on all MMR gene mutation carriers and their relatives in the Colon Cancer Family Registry, comprising 18,226 people. The POE was estimated as a hazard ratio (HR) using a segregation analysis approach that adjusted for ascertainment. HR = 1 corresponds to no POE and HR > 1 corresponds to higher risks for maternal mutations. For all MMR genes combined, the estimated POE HRs were 1.02 (95% confidence interval (CI) 0.75-1.39, p = 0.9) for male colorectal cancer, 1.12 (95% CI 0.81-1.54, p = 0.5) for female colorectal cancer and 0.84 (95% CI 0.52-1.36, p = 0.5) for endometrial cancer. Separate results for each MMR gene were similar. Therefore, despite being well-powered, our study did not find any evidence that cancer risks for MMR gene mutation carriers depend on the parent-of-origin of the mutation. Based on current evidence, we do not recommend that POEs be incorporated into the clinical guidelines or advice for such carriers.PURPOSE High tibial osteotomy (HTO) is an effective treatment option in early osteoarthritis. However, preoperative planning and surgical execution can be challenging. Computer assisted three-dimensional (3D) planning and patient-specific instruments (PSI) might be helpful tools in achieving successful outcomes. Goal of this study was to assess the accuracy of HTO using PSI. METHODS All medial open wedge PSI-HTO between 2014 and 2016 were reviewed. Using pre- and postoperative radiographs, hip-knee-ankle angle (HKA) and posterior tibial slope (PTS) were determined two-dimensionally (2D) to calculate 2D accuracy. Using postoperative CT-data, 3D surface models of the tibias were reconstructed and superimposed with the planning to calculate 3D accuracy. RESULTS Twenty-three patients could be included. A mean correction of HKA of 9.7° ± 2.6° was planned. Postoperative assessment of HKA correction showed a mean correction of 8.9° ± 3.2°, resulting in a 2D accuracy for HKA correction of 0.8° ± 1.5°. The postoperative PTS changed by 1.7° ± 2.2°. 3D accuracy showed average 3D rotational differences of - 0.1° ± 2.3° in coronal plane, - 0.2° ± 2.3° in transversal plane, and 1.3° ± 2.1° in sagittal plane, whereby 3D translational differences were calculated as 0.1 mm ± 1.3 mm in coronal plane, - 0.1 ± 0.6 mm in transversal plane, and - 0.1 ± 0.6 mm in sagittal plane. CONCLUSION The use of PSI in HTO results in accurate correction of mechanical leg axis. In contrast to the known problem of unintended PTS changes in conventional HTO, just slight changes of PTS could be observed using PSI. The use of PSI in HTO might be preferable to obtain desired correction of HKA and to maintain PTS.Smaller owls and hawks are high-threat predators to small songbirds, like chickadees, in comparison to larger avian predators due to smaller raptors’ agility (Templeton et al. in Proc Natl Acad Sci 1045479-5482, 2005). The current literature focuses only on high- and low-threat predators. We propose that there may be a continuum in threat perception. In the current study, we conducted an operant go/no-go experiment investigating black-capped chickadees’ acoustic discrimination of predator threat. After obtaining eight hawk and eight owl species’ calls, we assigned each species as (1) large, low-threat, (2) mid-sized, unknown-threat and (3) small-, high-threat predators, according to wingspan and body size. Black-capped chickadees were either trained to respond (‘go’) to high-threat predator calls or respond to low-threat predator calls. When either low-threat predator calls were not reinforced or high-threat predator calls were not reinforced the birds were to withhold responding (‘no-go’) to those stimuli. We then tested transfer of training with additional small and large predator calls, as well as with the calls of several mid-sized predators. We confirmed that chickadees can discriminate between high- and low-threat predator calls. We further investigated how chickadees categorize mid-sized species’ calls by assessing transfer of training to previously non-differentially reinforced (i.e., pretraining) calls. Specifically, transfer test results suggest that mid-sized broad-winged hawks were perceived to be of high threat whereas mid-sized short-eared owls were perceived to be of low threat. However, mid-sized Cooper’s hawks and northern hawk owls were not significantly differentially responded to, suggesting that they are of medium threat which supports the notion that perception of threat is along a continuum rather than distinct categories of high or low threat.Marmoset monkeys show high levels of proactive prosociality, a trait shared with humans, presumably because both species rely on allomaternal care. However, it is not clear whether the proximate regulation of this convergent trait is also similar, in particular with regard to intentionality, which is a defining characteristic of prosocial behavior in the human literature. The aim of this paper was to investigate whether marmoset monkeys’ prosociality fulfils the criteria of intentionality developed in primate communication research. The results show that marmoset prosocial behavior (i) has some degree of flexibility, since individuals can use multiple means to reach their goal and adjust them to specific conditions, (ii) depends on the presence of an audience, i.e. potential recipients (social use), and (iii) is goal-directed, because (a) it continues exactly until the putative goal is reached, and (b) individuals check back and look at/for their partner when their prosocial actions do not achieve the putative goal (i.