Notes

Whipping the standard massive reduce under ambient conditions together with solid-state revolves.
The clinical translation of three-dimensionally printed bioceramic scaffolds with tailored architectures holds great promise toward the regeneration of bone to heal critical-size defects. Herein, the long-term in vivo performance of printed hydrogel-ceramic composites made of methacrylated-oligocaprolactone-poloxamer and low-temperature self-setting calcium-phosphates is assessed in a large animal model. Scaffolds printed with different internal architectures, displaying either a designed porosity gradient or a constant pore distribution, are implanted in equine tuber coxae critical size defects. Bone ingrowth is challenged and facilitated only from one direction via encasing the bioceramic in a polycaprolactone shell. After 7 months, total new bone volume and scaffold degradation are significantly greater in structures with constant porosity. Interestingly, gradient scaffolds show lower extent of remodeling and regeneration even in areas having the same porosity as the constant scaffolds. Low regeneration in distal regions from the interface with native bone impairs ossification in proximal regions of the construct, suggesting that anisotropic architectures modulate the cross-talk between distant cells within critical-size defects. The study provides key information on how engineered architectural patterns impact osteoregeneration in vivo, and also indicates the equine tuber coxae as promising orthotopic model for studying materials stimulating bone formation. © 2020 The Authors. Published by WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.BACKGROUND We present a rare case of neonatal cholestasis in a female infant with Gaucher Disease (GD), who received liver transplantation. We review the relevant literature on similar disease presentations. METHODS A chart review of the index case was performed. read more PubMed and Medline databases were searched to identify other cases. RESULTS A 4-day-old female was referred with conjugated hyperbilirubinemia. Physical examination revealed icterus with hepatosplenomegaly and normal neurologic examination. The diagnosis of GD was confirmed through liver biopsy, low glucocerebrosidase enzyme activity, and two pathogenic mutations in GBA gene. Despite early initiation of ERT, the patient had worsening of her liver failure and underwent a left lateral segment liver transplant from a living donor at 7 months of age. She experienced improvement of her liver enzymes and coagulation, but passed away at 8 months due to the late onset of neurologic involvement. Nine other cases of GD presenting with neonatal cholestasis have been reported. Forty-four percent (4/9) of cases received ERT and none were considered for transplant. Overall, the literature suggests a poor prognosis with death reported in 77% (7/9) cases. CONCLUSIONS Neonatal presentation of GD represents a poor prognosis despite early initiation of treatment. Diagnosis remains a challenge as the presentation is rare and multiple tests such as BM biopsy, liver biopsy with both light and electron microscopy, enzymology, and genetic testing may need to be completed to reach a diagnosis. Neurological sequelae may manifest later making the decision to proceed with liver transplantation a difficult one. © 2020 Wiley Periodicals LLC.This case report describes an 8-year-old girl who underwent a segmental LT for a primary diagnosis of citrullinemia at the age of 12 months. She presented with cholangitis secondary to stenosis of the biliary-enteric anastomosis. MRI revealed dilatation of intrahepatic bile ducts associated with multiple stones. An endoscopic approach failed to decompress the bile ducts and remove the stones. A percutaneous approach was then undertaken. After placement of a temporary external biliary drain for 12 days, a 26 French sheath was placed to access the bile ducts. Using a 14Fr flexible cystoscope, 80%-90% of the biliary stones were removed. This was followed by antegrade balloon dilatation of the biliary-enteric anastomosis. Two months later, the procedure was repeated, resulting in complete clearance of the biliary stones. An internal-external biliary drain was maintained in placed for 10 months. The patient has been asymptomatic, with no evidence of stone recurrence for 13 months after drain removal. Percutaneous biliary stone removal is commonly performed in adults with non-transplanted livers, especially in complex cases, and has also been shown to be successful in the pediatric population. However, it is rarely reported in transplanted livers in adults, and to the best of our knowledge, no pediatric cases have been reported. This case illustrates that this technique can be successfully utilized in pediatric LT patients. © 2020 Wiley Periodicals LLC.BACKGROUND AND PURPOSE Comprehensive imaging characterization of the morphology and luminal patency of cerebral aneurysms are cornerstones of their successful treatment and subsequent appropriate management. Giant cerebral aneurysms (GCAs), a distinct subgroup of aneurysms, are defined by large size (≥ 25 mm in greatest diameter), complex blood flow dynamics, and a high risk of rupture. The purpose of this study is to explore compare multiple imaging modalities in the assessment of GCAs. METHODS This study retrospectively evaluated CT angiography (CTA), 3D time-of-flight (TOF) MR angiography (MRA), contrast-enhanced MRA (CEMRA), and digital subtraction angiography (DSA) in characterizing GCAs in 21 patients. RESULTS Aneurysm size ranged from 26 to 58 mm (mean 31.3 ± 12.2) and 18/21 (85.7%) had intraluminal thrombus. No significant difference was found between the aneurysmal sizes of any two modalities regarding comparisons of CTA, 3D TOFMRA, and CEMRA. However, there were significant differences in the aneurysmal patency visibility grade between CTA versus TOFMRA and CTA versus CEMRA. Moreover, the patent luminal size measured on CTA was significantly larger than DSA. CONCLUSIONS CTA, 3D TOFMRA, and CEMRA are equivalent in the delineation of size of GCAs. Nevertheless, 3D TOFMRA and CEMRA seem to be inferior to CTA in demonstrating luminal size/patency, likely because of the signal loss resulting from the presence of intraluminal thrombus and flow turbulence. Moreover, CTA is superior to DSA in determining lumen patency in GCAs, probably due to CTA's multipass-related luminal enhancement while DSA general fills the lesion via the first pass of enhancement or soon thereafter. In addition, CTA may also better demonstrate intraluminal thrombus, adjacent anatomical structures, and calcified rims. © 2020 by the American Society of Neuroimaging.Clearance (CL) is the major pharmacokinetic parameter for evaluating systemic exposure of drugs in the body and thus for developing new drugs. To predict in vivo CL, the Vmax /KM ratio estimated from in vitro metabolism study has been widely used. This canonical approach is based on the Michaelis-Menten equation, which is valid only when the KM value of a drug is much higher than the hepatic concentration of the enzymes, especially cytochrome P450, involved in its metabolism. Here we find that such a condition does not hold for many drugs with low KM , and thus the canonical approach leads to considerable error. Importantly, we propose an alternative approach, which incorporates the saturation of drug metabolism when concentration of the enzymes is not sufficiently lower than KM . This new approach dramatically improves the accuracy of prediction for in vivo CL of high-affinity drugs with low KM . This indicates that the proposed approach in this study rather than the canonical approach should be used to predict in vivo hepatic CL for high-affinity drugs such as midazolam and propafenone. This article is protected by copyright. All rights reserved.Coronavirus disease 2019 (Covid-19) pneumonia has been poorly reported in solid-organ transplanted patients; prognosis is uncertain and best management unclear. We describe the case of a 61-year-old kidney transplant recipient with several co-morbidities who was hospitalized and later received a diagnosis of Covid-19 pneumonia; the infection was successfully managed with the use of hydroxychloroquine and a single administration of Tocilizumab, after immunosuppression reduction; the patient did not require mechanical ventilation. During the rapid spread of SARS-CoV-2 pandemic, transplant clinicians should be readily informed about new cases of Covid-19 pneumonia in solid-organ transplant recipients, with focus on therapeutic strategies employed and their outcome. This article is protected by copyright. All rights reserved.The present study uses a parent-offspring adoption design to examine the dual roles of heritable and environmental influences on children's mathematics achievement. Linked sets (N = 195) of adopted children, adoptive parents, and birth parents each completed a measure of mathematics fluency (i.e., simple computational operations). Birth parent mathematics achievement and adoptive father mathematics achievement positively correlated with child achievement scores at age 7, whereas adoptive mother and adopted child mathematics achievement scores were not significantly associated with one another. Additionally, findings demonstrated no significant effects of gene-environment interactions on child mathematics achievement at age 7. These results indicate that both heritable and rearing environmental factors contribute to children's mathematics achievement and identify unique influences of the paternal rearing environment on mathematics achievement in middle childhood. © 2020 John Wiley & Sons Ltd.To evaluate the curative effect of one-stage posterior debridement and osteotomy parallel to the endplates for reconstruction, deformity correction, and tuberculosis control on treating the spinal tuberculosis of graded GATA III. From July of 2012 to December of 2017, there were 36 cases from the Second Hospital of Shanxi Medical University with thoracic and lumbar tuberculosis graded GATA III, in which we used osteotomy parallel to the endplates and reconstruction for treatment,16 for males and 20 for females. The local Cobb angles of kyphosis of all patients are greater than or equal to 20.The age varied from 28 months to 72 years with an average of 38.8 years. There were 15 cases of thoracic segment, 12 cases of thoracolumbar segment (T11 -L2 ), 9 cases of lumbar segment. Preoperative results of ASIA were 3 cases of grade C,5 cases of grade D and 28 cases of grade E with an average kyphosis Cobb angle of 37.21 ± 3.28. The visual analogue scale(VAS) scores preoperatively were 0-8 points (averaged 5.58 ± 1.6he last ASIA grades were 1 case of grade D and 35 cases of grade E. The one-stage posterior debridement and osteotomy parallel to the endplates for patients with spinal tuberculosis of graded GATA III are not only beneficial to spinal reconstruction, but also obtain ideal reconstuction effects. © 2020 The Authors. Orthopaedic Surgery published by Chinese Orthopaedic Association and John Wiley & Sons Australia, Ltd.It has been almost 40 years since HIV emerged in the human population with an alarming impact in 1981, quickly reaching pandemic proportions. Reaching the goal of eradication, or at least ending the pandemic, however, has not been as easy as hoped. To better understand and therefore better address the persistence and often devastating effects of this now chronic disease, the heterogeneity of HIV-in the virus-human and human-human relationships it engages-is parsed in discussions of the groups affected and the multiple factors that drive the diverse effects of the disease, both of which make treatment and prevention of the disease highly challenging. The construct of time cognition is then considered as a heretofore unexplored factor that may inform our understanding of HIV-relevant behaviors. © 2020 Wiley Periodicals, Inc.
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