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Meningiomas are the most common primary intracranial tumors and can be associated with significant morbidity and mortality. Radiologists, neurosurgeons, neuro-oncologists, and radiation oncologists rely on brain MRI for diagnosis, treatment planning, and longitudinal treatment monitoring. However, automated, objective, and quantitative tools for non-invasive assessment of meningiomas on multi-sequence MR images are not available. Here we present the BraTS Pre-operative Meningioma Dataset, as the largest multi-institutional expert annotated multilabel meningioma multi-sequence MR image dataset to date. This dataset includes 1,141 multi-sequence MR images from six sites, each with four structural MRI sequences (T2-, T2/FLAIR-, pre-contrast T1-, and post-contrast T1-weighted) accompanied by expert manually refined segmentations of three distinct meningioma sub-compartments: enhancing tumor, non-enhancing tumor, and surrounding non-enhancing T2/FLAIR hyperintensity. Basic demographic data are provided including age at time of initial imaging, sex, and CNS WHO grade. The goal of releasing this dataset is to facilitate the development of automated computational methods for meningioma segmentation and expedite their incorporation into clinical practice, ultimately targeting improvement in the care of meningioma patients.
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Imagen por Resonancia Magnética , Neoplasias Meníngeas , Meningioma , Meningioma/diagnóstico por imagen , Humanos , Neoplasias Meníngeas/diagnóstico por imagen , Masculino , Femenino , Procesamiento de Imagen Asistido por Computador/métodos , Persona de Mediana Edad , AncianoRESUMEN
INTRODUCTION: Obesity is a serious situation that leads to non-communicable diseases like diabetes, hypertension, and others. The prevalence of obesity is growing very fast worldwide, so follow the results bariatric surgery, the most effective treatment of obesity, is increasing. Portomesentric vein thrombosis (PMVT) is one of the rare, fatal post-bariatric complications seen most commonly in sleeve gastrectomy and Roux-en-Y gastric bypass. PRESENTATION OF CASE: A 50-year-old menopausal female with a body mass index (BMI) of 38 was admitted with acute abdominal pain 10 days after one-anastomosis gastric bypass (OAGB). Her lab tests were normal, but in her abdominal CT scan with IV contrast, subacute complete intraluminal thrombosis with luminal expansion at the left branch of the portal vein and its segmental branches was seen. Her diagnostic laparoscopy was normal, and she was discharged with no symptoms and a prescription for rivaroxaban. DISCUSSION: PMVT is one of the complications after bariatric surgery that is very uncommon and fetal. It has been seen more in laparoscopic sleeve gastrectomy and Roux-en-Y gastric bypass, and early diagnosis of PMVT is essential due to its high mortality rate and cause of gastrointestinal ischemia. CONCLUSION: In this case report, we saw that PMVT could happen after OAGB, and it is important to consider PMVT as one of the complications after OAGB to not miss the cases.
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Background and purpose: Excitotoxicity in nerve cells is a type of neurotoxicity in which excessive stimulation of receptors (such as N-methyl-d-aspartate glutamate receptors (NMDAR)) leads to the influx of high-level calcium ions into cells and finally cell damage or death. This complication can occur after taking some of the plasminogen activators like tissue plasminogen activator and reteplase. The interaction of the kringle2 domain in such plasminogen activator with the amino-terminal domain (ATD) of the NR1 subunit of NMDAR finally leads to excitotoxicity. In this study, we assessed the interaction of two new chimeric reteplase, mutated in the kringle2 domain, with ATD and compared the interaction of wild-type reteplase with ATD, computationally. Experimental approach: Homology modeling, protein docking, molecular dynamic simulation, and molecular dynamics trajectory analysis were used for the assessment of this interaction. Findings/Results: The results of the free energy analysis between reteplase and ATD (wild reteplase: -2127.516 ± 0.0, M1-chr: -1761.510 ± 0.0, M2-chr: -521.908 ± 0.0) showed lower interaction of this chimeric reteplase with ATD compared to the wild type. Conclusion and implications: The decreased interaction between two chimeric reteplase and ATD of NR1 subunit in NMDAR which leads to lower neurotoxicity related to these drugs, can be the start of a way to conduct more tests and if the results confirm this feature, they can be considered potential drugs in acute ischemic stroke treatment.
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Introduction: Chronic progressive neuroinflammation is a hallmark of neurological lysosomal storage diseases, including mucopolysaccharidosis III (MPS III or Sanfilippo disease). Since neuroinflammation is linked to white matter tract pathology, we analyzed axonal myelination and white matter density in the mouse model of MPS IIIC HgsnatP304L and post-mortem brain samples of MPS III patients. Methods: Brain and spinal cord tissues of human MPS III patients, 6-month-old HgsnatP304L mice and age- and sex-matching wild type mice were analyzed by immunofluorescence to assess levels of myelin-associated proteins, primary and secondary storage materials, and levels of microgliosis. Corpus callosum (CC) region was studied by transmission electron microscopy to analyze axon myelination and morphology of oligodendrocytes and microglia. Mouse brains were analyzed ex vivo by high-filed MRI using Diffusion Basis Spectrum Imaging in Python-Diffusion tensor imaging algorithms. Results: Analyses of CC and spinal cord tissues by immunohistochemistry revealed substantially reduced levels of myelin-associated proteins including Myelin Basic Protein, Myelin Associated Glycoprotein, and Myelin Oligodendrocyte Glycoprotein. Furthermore, ultrastructural analyses revealed disruption of myelin sheath organization and reduced myelin thickness in the brains of MPS IIIC mice and human MPS IIIC patients compared to healthy controls. Oligodendrocytes (OLs) in the CC of MPS IIIC mice were scarce, while examination of the remaining cells revealed numerous enlarged lysosomes containing heparan sulfate, GM3 ganglioside or "zebra bodies" consistent with accumulation of lipids and myelin fragments. In addition, OLs contained swollen mitochondria with largely dissolved cristae, resembling those previously identified in the dysfunctional neurons of MPS IIIC mice. Ex vivo Diffusion Basis Spectrum Imaging revealed compelling signs of demyelination (26% increase in radial diffusivity) and tissue loss (76% increase in hindered diffusivity) in CC of MPS IIIC mice. Discussion: Our findings demonstrate an important role for white matter injury in the pathophysiology of MPS III. This study also defines specific parameters and brain regions for MRI analysis and suggests that it may become a crucial non-invasive method to evaluate disease progression and therapeutic response.
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Background: There is no specific test in the definitive diagnostic approach to Allergic bronchopulmonary aspergillosis (ABPA) especially in the background of cystic fibrosis, but comprehensive and simultaneous clinical, radiological and serological examination will be the basis of ABPA diagnosis. The increasing in attenuation of bronchoceles in imaging has recently been proposed as a valuable diagnostic criterion. Purpose: The present study aimed to assess bronchocele attenuation in pulmonary CT scan of patients with complicated cystic fibrosis for diagnosis of ABPA. Methods: This cross-sectional study was performed on 74 consecutive patients aged 3-18 years suffering cystic fibrosis presented with exacerbation of pulmonary symptoms and were suspected of having ABPA. All were examined by 16 Slice CT Scan and the density of bronchoceles above 5 mm in diameter were measured in Hounsfield unit. The total serum IgE titer, skin prick test for aspergillus and anti-aspergillus IgG and IgE level were obtained for all subjects and both cutoff values of IgE level (>500 IU/mL and >1000 IU/mL) were considered as the criteria for ABPA diagnosis. Results: Considering IgE level of greater than 500 IU/mL and 1000 IU/mL as the diagnostic criteria, 24.3% and 10.8% had evidence of ABPA, respectively. Considering the two pointed diagnostic IgE ranges and based on the analysis of the area under the ROC curve, bronchocele attenuation could effectively predict the presence of ABPA with the best cutoff values of 37.25 (with a sensitivity of 70.6% and a specificity of 66.7%) and 40.00 (with a sensitivity of 85.7% and a specificity of 65.1%), respectively. Conclusion: The presence of bronchocele and an increase in its attenuation on CT scan will be diagnostic for the occurrence of ABPA.
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The incidence of both obesity and inflammatory bowel disease (IBD) is rising globally. The influence of bariatric metabolic surgery (BMS) upon IBD development is largely unknown. This systematic review and meta-analysis aimed to evaluate the relationship between BMS and the risk of de novo IBD development following surgery. A systematic literature search and meta-analysis were performed using PubMed and Scopus databases. Inclusion criteria were any study reporting risk of de novo IBD development following BMS relative to an appropriate control cohort. Pooled odds ratios (POR) were calculated. A total of 31 articles were identified by the literature search. Four studies including 149,385 patients met the inclusion criteria and were included in the meta-analysis. Pooled estimation of a meta-analysis of risk ratios studies demonstrated a POR for the development of IBD following BMS of 1.17 (95% CI, 1.06-1.29). This indicates a 17% increase in relative risk of de novo IBD development for those patients receiving BMS compared to those treated by non-surgical methods. Based on the present data, there appears to be an association between BMS and risk of de novo IBD. Compared to the proven benefits of BMS on other aspects of patient health, this potential risk remains proportionally low but may be an important consideration for patients both pre- and post-operatively.
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Cirugía Bariátrica , Enfermedades Inflamatorias del Intestino , Obesidad Mórbida , Cirugía Bariátrica/efectos adversos , Cirugía Bariátrica/métodos , Humanos , Incidencia , Enfermedades Inflamatorias del Intestino/epidemiología , Obesidad/cirugía , Obesidad Mórbida/cirugíaRESUMEN
This systematic review intends to evaluate incidence and symptoms of post-bariatric splenic complications as well as best available modalities establishing the diagnosis and management protocols. A systematic literature search was performed in electronic database until March 2022. A total of 41 articles were included on the subject of splenic complications following bariatric/metabolic surgery (BMS). Splenic abscess was the most common splenic complications (44.2%) after BMS and leak was the most common reported etiology of the splenic abscess. Fever and abdominal pain were the most common presenting symptom in all splenic complications and CT scan was the most common diagnostic modality. Splenic complications after BMS are relatively rare but may lead to dangerous consequences. Prompt diagnosis and treatment can prevent potentially life-threatening outcomes.
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Absceso Abdominal , Cirugía Bariátrica , Obesidad Mórbida , Enfermedades del Bazo , Absceso Abdominal/diagnóstico , Absceso Abdominal/etiología , Absceso Abdominal/terapia , Absceso/complicaciones , Cirugía Bariátrica/efectos adversos , Humanos , Obesidad Mórbida/cirugía , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/terapia , Enfermedades del Bazo/diagnóstico , Enfermedades del Bazo/etiología , Enfermedades del Bazo/cirugíaRESUMEN
The majority of mucopolysaccharidosis IIIC (MPS IIIC) patients have missense variants causing misfolding of heparan sulfate acetyl-CoA:α-glucosaminide N-acetyltransferase (HGSNAT), which are potentially treatable with pharmacological chaperones. To test this approach, we generated a novel HgsnatP304L mouse model expressing misfolded HGSNAT Pro304Leu variant. HgsnatP304L mice present deficits in short-term and working/spatial memory 2-4 mo earlier than previously described constitutive knockout Hgsnat-Geo mice. HgsnatP304L mice also show augmented severity of neuroimmune response, synaptic deficits, and neuronal storage of misfolded proteins and gangliosides compared with Hgsnat-Geo mice. Expression of misfolded human Pro311Leu HGSNAT protein in cultured hippocampal Hgsnat-Geo neurons further reduced levels of synaptic proteins. Memory deficits and majority of brain pathology were rescued in mice receiving HGSNAT chaperone, glucosamine. Our data for the first time demonstrate dominant-negative effects of misfolded HGSNAT Pro304Leu variant and show that they are treatable by oral administration of glucosamine. This suggests that patients affected with mutations preventing normal folding of the enzyme can benefit from chaperone therapy.
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Mucopolisacaridosis , Mucopolisacaridosis III , Acetiltransferasas , Animales , Glucosamina , Heparitina Sulfato , Humanos , Ratones , Ratones Noqueados , Mucopolisacaridosis III/genética , Mucopolisacaridosis III/patologíaRESUMEN
The majority of patients affected with lysosomal storage disorders (LSD) exhibit neurological symptoms. For mucopolysaccharidosis type IIIC (MPSIIIC), the major burdens are progressive and severe neuropsychiatric problems and dementia, primarily thought to stem from neurodegeneration. Using the MPSIIIC mouse model, we studied whether clinical manifestations preceding massive neurodegeneration arise from synaptic dysfunction. Reduced levels or abnormal distribution of multiple synaptic proteins were revealed in cultured hippocampal and CA1 pyramidal MPSIIIC neurons. These defects were rescued by virus-mediated gene correction. Dendritic spines were reduced in pyramidal neurons of mouse models of MPSIIIC and other (Tay-Sachs, sialidosis) LSD as early as at P10. MPSIIIC neurons also presented alterations in frequency and amplitude of miniature excitatory and inhibitory postsynaptic currents, sparse synaptic vesicles, reduced postsynaptic densities, disorganized microtubule networks, and partially impaired axonal transport of synaptic proteins. Furthermore, postsynaptic densities were reduced in postmortem cortices of human MPS patients, suggesting that the pathology is a common hallmark for neurological LSD. Together, our results demonstrate that lysosomal storage defects cause early alterations in synaptic structure and abnormalities in neurotransmission originating from impaired synaptic vesicular transport, and they suggest that synaptic defects could be targeted to treat behavioral and cognitive defects in neurological LSD patients.