Management of Priapism 2021 Bring up to date

Population pharmacokinetic analysis is used to estimate pharmacokinetic parameters and their variability from concentration data. Due to data sparseness issues, available datasets often do not allow the estimation of all parameters of the suitable model. The PRIOR subroutine in NONMEM supports the estimation of some or all parameters with values from previous models, as an alternative to fixing them or adding data to the dataset. From a literature review, the best practices were compiled to provide a practical guidance for the use of the PRIOR subroutine in NONMEM. Thirty-three articles reported the use of the PRIOR subroutine in NONMEM, mostly in special populations. This approach allowed fast, stable and satisfying modelling. The guidance provides general advice on how to select the most appropriate reference model when there are several previous models available, and to implement and weight the selected parameter values in the PRIOR function. On the model built with PRIOR, the similarity of estimates with the ones of the reference model and the sensitivity of the model to the PRIOR values should be checked. Covariates could be implemented a priori (from the reference model) or a posteriori, only on parameters estimated without prior (search for new covariates).The original version of this article unfortunately contained mistakes.Deficiency of adenosine deaminase 2 (DADA2) is an autosomal recessive disease resulting from loss-of-function pathogenic variants in ADA2 gene, which might resemble polyarteritis nodosa (PAN). The authors present two pediatric cases of ADA2 deficiency with phenotypic manifestations of PAN, including an unusual presentation with spinal cord ischemia. Also described is an assessment of ADA2 activity and gene expression profiling with description of a previously unreported homozygous variant, c.1226C > A (p.(Pro409His)), detected in a patient with consanguineous parents, confirmed by near-absent ADA2 plasma enzymatic activity. The authors suggest to first obtain enzymatic activity, whenever DADA2 is suspected, before proceeding to genetic testing, due to its excellent cost-effective results. Moreover, physicians must be aware of this monogenic disorder, especially in the case of early-onset PAN-like manifestations, having a family member with similar manifestations or having consanguineous parents suggesting an autosomal recessive inheritance pattern. Given the multi-organ involvement, recognizing the diverse manifestations is a crucial step towards timely diagnosis and management of this potentially fatal but often treatable syndrome.Rituximab (RTX) is an effective treatment for refractory nephrotic syndrome (NS), but may produce human anti-chimeric antibodies (HACA) which can cause severe infusion reaction or rituximab-induced serum sickness (RISS). RISS presents with a fever, rash, and arthralgia, which typically occurs 7-21 days after RTX infusion. Degrasyn cost On the other hand, Kawasaki disease (KD) also presents with fever and rash. There have been no reports of KD developed after RTX infusion. A 6-year-old girl with frequently relapsing NS was admitted to our hospital for fever and rash on day 7 after receiving RTX. Although it was suggestive of RISS at first, she also had conjunctival hyperemia, swelling, and erythema of the hands and feet, and a right coronary artery abnormality on echocardiography. Her symptoms met the diagnostic criteria of KD. We administered intravenous immunoglobulin (IVIg) (2 g/kg), and her symptoms resolved within a few days. The HACA titer determined using the serum collected at admission was very high. This is the first report of KD with a clinical course similar to RISS. It should be noted that a careful follow-up of coronary arteries should be performed in patients suspected of RISS.Here, we present a 67-year-old Japanese man who developed insidious-onset nephrotic syndrome. He had a history of occupational asbestos exposure for about 8 years during his 30s, and was found to have pleural effusion 3 years before his present illness. At that time, repeated cytology testing of his pleural effusion found no malignant cells, and pleural biopsy found fibrous pleuritis without evidence of malignant mesothelioma. Percutaneous kidney biopsy found massive deposits of AA-type amyloid in the glomeruli, small arteries, and medulla. Computed tomography showed a calcified mass in the right lower lung that was positive for 67Ga uptake, but transbronchial lung biopsy and bronchoalveolar lavage found no evidence of malignancy. He was diagnosed with rounded atelectasis and diffuse pleural thickening. As these benign asbestos-related diseases have no standard treatment, we administered low-dose angiotensin II receptor blocker to preserve kidney function. Unfortunately, his nephrotic syndrome persists, with progressive chronic kidney failure. Kidney involvement in patients with asbestos-related disease is rare. To our knowledge, this is the first case to present with secondary amyloidosis. Kidney biopsy should be considered for patients with existing asbestos-related pleuropulmonary diseases who have urinary abnormalities or renal dysfunction, to clarify the incidence and pathophysiology of renal manifestations.To investigate the correlation of epicardial adipose tissue (EAT) characteristics and high-risk plaque features characterized by coronary CT angiography (CCTA) for identifying the presence of thin-cap fibroatheroma (TCFA). Patients who underwent both CCTA and intravascular ultrasound (IVUS) within 4 weeks were retrospectively included. CT-derived quantitative and qualitative parameters, including diameter stenosis, low attenuation plaque (LAP), napkin-ring sign (NRS), positive remodeling and spotty calcification, were recorded. EAT volume and density were also measured. TCFA lesions and non-TCFA lesions were determined by IVUS. Multivariate regression analysis was used to determine the independent predictors of TCFA lesions. Sixty-eight patients (mean age 68.6 ± 9.7 years; 40 males) with 91 lesions were finally included in our study. For CT-derived plaque features, LAP (77.8% versus 25%, p less then 0.001) and NRS (40.7% versus 9.4%, p less then 0.001) was more frequently presented in TCFA lesions than was in non-TCFA lesions. For EAT characteristics, EAT volume (110 ± 14 cm3 versus 98 ± 12 cm3, p less then 0.001) was significantly larger whereas EAT density (-77 ± 4 HU versus -80 ± 5, p = 0.003) was markedly higher in TCFA lesions. According to multivariate logistic regression analysis, LAP, EAT volume and EAT density were significant predictors (odds ratio 9.758, 1.095 and 1.202, all p value less then 0.05) for the presence of TCFA lesions. EAT volume and density was greater in patients with TCFA lesions whereas LAP and NRS was more frequently presented. In addition, EAT characteristics and LAP were independent predictors of vulnerable plaques as determined by IVUS.Accurate quantification of mitral regurgitation (MR) severity is critical for appropriate clinical decision making regarding surgical intervention. General imaging three-dimensional quantification (GI3DQ) method allows for direct measurement of mitral regurgitant jet volume (MRJvol) with the help of three-dimensional (3D) color flow Doppler imaging. The aim of this study was to evaluate diagnostic value of MRJvol by GI3DQ for MR grading severity, using the guideline recommended integrated approach as a reference. The study included ninety-seven patients with varying degree of MR, and all MR cases were divided into central MR group (n = 44) and eccentric MR group (n = 53). The MRJvol was measured by GI3DQ. The severity of MR was graded on the basis of recommended integrated approach as mild, moderate, or severe. As assessed by receiver operating characteristic analysis, MRJvol by GI3DQ at a cutoff value of 43.4 ml yielded 76.9% of sensitivity and 86.9% of specificity to differentiate moderate from severe MR in all cases, a cutoff value of 47.5 ml yielded 98.9% of sensitivity and 94.4% of specificity to differentiate moderate from severe MR in central MR, and a cutoff value of 40.7 ml yielded 80.0% of sensitivity and 78.6% of specificity to differentiate moderate from severe MR in eccentric MR. MRJvol measured by GI3DQ could assess MR severity, especially in central MR group, which has higher sensitivity and specificity to differentiate moderate from severe MR.The human visual system is capable of processing visual information from fovea to the far peripheral visual field. Recent fMRI studies have shown a full and detailed retinotopic map in area prostriata, located ventro-dorsally and anterior to the calcarine sulcus along the parieto-occipital sulcus with strong preference for peripheral and wide-field stimulation. Here, we report the anatomical pattern of white matter connections between area prostriata and the thalamus encompassing the lateral geniculate nucleus (LGN). To this end, we developed and utilized an automated pipeline comprising a series of Apps that run openly on the cloud computing platform brainlife.io to analyse 139 subjects of the Human Connectome Project (HCP). We observe a continuous and extended bundle of white matter fibers from which two subcomponents can be extracted one passing ventrally parallel to the optic radiations (OR) and another passing dorsally circumventing the lateral ventricle. Interestingly, the loop travelling dorsally connects the thalamus with the central visual field representation of prostriata located anteriorly, while the other loop travelling more ventrally connects the LGN with the more peripheral visual field representation located posteriorly. We then analyse an additional cohort of 10 HCP subjects using a manual plane extraction method outside brainlife.io to study the relationship between the two extracted white matter subcomponents and eccentricity, myelin and cortical thickness gradients within prostriata. Our results are consistent with a retinotopic segregation recently demonstrated in the OR, connecting the LGN and V1 in humans and reveal for the first time a retinotopic segregation regarding the trajectory of a fiber bundle between the thalamus and an associative visual area.The present study investigated the short-term and long-term synaptic plasticity of excitatory synapses formed by the nucleus reuniens (RE) and entorhinal cortex (EC) on the distal apical dendrites of CA1 pyramidal cells. RE-CA1 synapses are implicated in memory involving the hippocampus and medial prefrontal cortex. Current source density (CSD) analysis was used to identify excitatory and inhibitory currents following stimulation of RE or medial perforant path (MPP) in urethane-anesthetized mice in vivo. At the distal apical dendrites, RE evoked an initial excitatory sink followed by inhibitory sources at short (~ 30 ms) and long (150-200 ms) latencies, and often showing gamma (25-40 Hz) oscillations. Both RE-evoked and spontaneous gamma-frequency local field potentials displayed the same CSD depth profile. Paired-pulse facilitation (PPF) of the distal excitatory sink at 20-200 ms interpulse intervals was observed following RE stimulation, generally higher than that following MPP stimulation. Theta-frequency burst stimulation (TBS) of RE induced input-specific long-term potentiation (LTP) at the distal dendritic CA1 synapses, accompanied by reduction of PPF. After TBS of the MPP, the MPP-CA1 distal dendritic synapse could manifest LTP or long-term depression, but the non-tetanized RE-CA1 synapse was typically potentiated. Heterosynaptic potentiation of the RE to CA1 distal synapses may occur after repeated activity of EC afferents, or spread of MPP stimulus currents to coursing RE afferents. The results indicate a propensity of RE-CA1 distal excitatory synapses to show PPF, LTP and gamma oscillations, all of which may participate in memory processing by RE and EC.