Background: Medical care has shifted from a paternalistic model towards one centered around patient autonomy and shared decision-making (SDM), yet the role of the pediatric patient in decision-making is unclear. Studies suggest that many children with chronic disease are capable of participating in and even making medical decisions at a young age, and yet we do not standardly involve them. Methods: This is a single center survey study investigating physician attitudes towards involvement of children in decisions regarding lung transplantation, utilizing a hypothetical case scenario with systematic manipulation of age and maturity level. We evaluated physician belief regarding ultimate decision-making authority, attempts at reconciliation of parent-child discordance, and views towards utilizing ethics and psychiatry consultation services. Results: The majority of pediatric pulmonologists believe decision-making authority rests with the parents. The effects of age and maturity are unclear. In instances of parent-child disagreement, physician are more likely to try to convince parents to defer to the child if the child is both older and more mature. Physicians are divided on the utility of ethics and psychiatry consultations. Conclusion: Involvement of children in shared decision-making is broadly supported but poorly implemented. Despite evidence that children with chronic disease may have decisional capacity starting at a young age, the majority of physicians still grant decisional authority to parents. There are numerous barriers to involving children in decisions, including legal considerations. The role of age and maturity level in influencing these decisions appears small and warrants further investigation.

The FLNA gene encodes filamin A, an actin filament cross-linking protein that is ubiquitously expressed within the body. FLNA mutation causes periventricular nodular heterotopia (PVNH) and congenital heart disease. Interstitial lung disease (ILD) related to FLNA mutation has also been reported from 2011 and can be lethal. However, there are no reports of how to combine the treatment of heart disease with the conflicting treatment of lung disease. We herein report cases of two girls with FLNA mutation and both ILD and left-to-right shunts due to congenital heart disease. They presented with respiratory symptoms in early infancy and required management with long-term intubation and ventilation. However, their respiratory status improved subsequent to the closure of their left-to-right shunts even though they were small shunts with improvement in pulmonary hypertension. This suggests that early intervention with closure of cardiac shunts can prevent further deterioration of lung damage.

We are experienced a challenge for the surgeon for tracheocutaneous fistula closure in managing such a complication such as chronic cough, infection and other co-morbidities. The need for a secondary closure is also warranted when the stoma does not close on itself within a specified time.

We hypothesized that the combination therapy of SGLT-2(Sodium-glucose co-transporter-2)inhibitor and Glucagon-like peptide-1 receptor agonist(GLP-1RA) provide renal protection in adults with T2D with estimated GFR (eGFR) below 30ml/minute/1.73 m2. We hereby describe four consecutively treated high renal risk patients with T2D with eGFR between 21 to 30ml/minute/1.73m2.

Newcastle disease (ND), caused by avian orthoavulavirus type-1 (NDV), is endemic in poultry in the Middle East causing continuing outbreaks in poultry populations despite efforts to vaccinate. In the past, genotype 2.XXI (former 2.VI) was present in poultry in Egypt but has been replaced by genotype 2.VII. We investigated whether virus evolution contributed to superseding, and focused on the antigenic sites within the Heamagglutinin-Neuramindase (HN) spike protein. Full length sequences of a NDV genotype 2.VII isolate currently circulating in Egypt was compared to a genotype 2.XXI isolate that was present as co-infection with vaccine type viruses (2.II) in an historical isolate of the year 2011. Amino acid differences in the HN glycoprotein for both 2.XXI and 2.VII viruses amounted to 11,7% and 11,9 % compared to LaSota vaccine type. However, mutations within the globular head (aa 126-570), bearing relevant antigenic sites, were underrepresented (aa divergence of 8,8% and 8,1 % compared to 22,4% and 25,6% within the fragment encompassing cytoplasmic tail, transmembrane part and stalk regions (aa 1-125) for genotypes 2.XXI and 2.VII, respectively. Nevertheless, reaction patterns of HN-specific monoclonal antibodies revealed differences between vaccine type viruses and genotype 2.XXI and 2.VII viruses for specific epitopes. Accordingly, compared to Egyptian vaccine type isolates and the LaSota vaccine reference strain, single aa substitutions in 6 of 10 described neutralizing epitopes were found within the attachment protein. However, the same alterations in neutralization sensitive epitopes were present in old genotype 2.XXI as well as in newly emerged genotype 2.VII isolates. In addition, isolates were indistinguishable by polyclonal chicken sera raised against different genotypes including vaccine viruses. These findings suggest, that factors other than antigenic differences within the HN-protein account for facilitating spread of genotype 2.VII while displacing genotype 2.XXI viruses in Egypt.

The patients' discouragement from presentation due to the COVID-19 pandemic resulted in the progression of many manageable diseases to a critical condition. we report a case of carbuncle promotion to the necrotizing fasciitis in the context of undiagnosed diabetes mellitus and delay in visiting physicians due to fear of COVID-19.

Patient with a long history of CLL and known trisomy 12 presented with rapidly rising lymphocytosis, bulky adenopathy, and splenomegaly. Peripheral blood and bone marrow exam showed preponderance of prolymphocytes. Cytogenetic analysis showed an abnormal male karyotype with trisomy 12 and a new t(8;21) translocation in the same 6 metaphases.

A document by Josefina Cernadas. Click on the document to view its contents.

Stress KillsBerhane Worku MD1, Shudhanshu Alishetti2, Kumudha Ramasubbu21. New York Presbyterian Brooklyn Methodist Hospital/Weill Cornell Medical Center Department of Cardiothoracic Surgery, Brooklyn, NY 112152. New York Presbyterian Brooklyn Methodist Hospital Division of Cardiology, Brooklyn NY 11215Corresponding AuthorBerhane Worku MDDepartment of Cardiothoracic SurgeryNew York Presbyterian Brooklyn Methodist Hospital506 6th StreetBrooklyn, NY 11215The medical, economic, and social consequences of the COVID-19 pandemic have been profound. Severe respiratory failure as well as inflammatory and thrombotic complications have resulted in hundreds of thousands of deaths. Political controversy continues regarding optimal strategies for large scale control of the pandemic. Social distancing policies have led to reduced transmission rates but the economic effects have been devastating. Optimal treatment strategies continue to evolve, and vaccine solutions are on the horizon. In addition to these more obvious issues, other severe consequences of the pandemic are slowly being recognized.In the current report, Kir et. al. describe two postmenopausal women presenting with signs and symptoms of acute coronary syndrome in the setting of severe psychological stress related to social isolation during the COVID-19 pandemic (1). Both were COVID negative and both had unremarkable coronary angiograms. Both were diagnosed with takotsubo cardiomyopathy based on the characteristic findings of angina, mild troponin elevation, electrocardiographic changes, and apical akinesis on echocardiogram. Both admitted to severe anxiety and stress in the days prior to the onset of symptoms. Both improved with conservative management including beta-blockers and anxiolytics with resolution of apical akinesis on follow up echocardiogram.Takotsubo or stress cardiomyopathy is a now well recognized entity typically presenting as angina or dyspnea in the setting of a severe emotional or physical stressor. Postmenopausal women are more frequently affected and a history of psychiatric disorders is frequently noted. Electrocardiographic abnormalities and mild troponin elevations are common. Diagnosis is based on the InterTAK diagnostic score. Echocardiography classically demonstrates apical ballooning with basal hyperkinesis, but other wall motion abnormalities are described usually extending beyond a traditional coronary artery distribution. Coronary angiography is frequently performed to rule out acute coronary syndrome but is unremarkable. The syndrome is typically self-limited, requiring conservative supportive management, but in severe cases can lead to heart failure and shock requiring high-dose pharmacologic support, mechanical circulatory support, and in ~5% of cases can be fatal (2).Emotional and physical stress are risk factors for a variety of conditions including cardiovascular disease. Furthermore, psychiatric disorders such as depression and anxiety are associated with poorer outcomes in the setting of cardiovascular disease. Proposed mechanisms for this include behavioral factors such as noncompliance with medications and lifestyle modifications (diet, exercise, smoking cessation). Biological factors are also suggested, including altered autonomic nervous system activity with elevations in catecholamine levels and inflammatory responses amongst others (3). Similar hypotheses have been put forth regarding the mechanism of takotsubo stress cardiomyopathy and perhaps some overlap exists between the cardiovascular manifestations of psychiatric disorders and overt stress cardiomyopathy.The COVID-19 pandemic has had several medical consequences beyond those related to viral infection itself. The suspension of certain medical and surgical services potentially allows for the natural history of various diseases to take their course. Unemployment impairs the ability of many to access what medical services remain available. Psychiatric disorders are inflamed in the setting of social, economic, and other stressors. A four to five-fold increase in the incidence of stress cardiomyopathy has been noted during the months following the COVID-19 outbreak unrelated to COVID-19 infection itself, presumably the consequence of stress related to the abovementioned effects of the pandemic and our response to it. (4). COVID-19 has taught us that stress kills.REFERENCESKir D, Beer N, De Marchena EJ. Takutsobo cardiomyopathy caused by emotional stressors in the Coronavirus Disease 2019 (COVID-19) pandemic era. J Card Surg in pressDe Chazal HM, Del Buono MG, Keyser-Marcus L, Ma L, Moeller FG, Berrocal D, Abbate A. Stress cardiomyopathy diagnosis and treatment. J Am Coll Cardiol 2018;72:1955-71Takagi H, Ando T, Umemoto. Perioperative depression or anxiety and postoperative mortality in cardiac surgery: a systematic review and meta-analysis. Heart Vessels 2017;32:1458-1468Jabri A, Kalra a, Kumar A, Alameh A, Adroja S, Bashir H, Nowacki AS, Shah R, Khubber S, Kanaa’N A, Hedrick DP, Sleik KM, Mehta N, Chung MK, Khot UN, Kapadia SR, Puri R, Reed GW. Incidence of stress cardiomyopathy during the coronavirus disease 2019 pandemic. JAMA Netw Open 2020 Jul 1;3(7):e2014780. doi: 10.1001/jamanetworkopen.2020.14780

Mini-commentary on BJOG-20-1841.R1: Cervical dilatation and preparation prior to outpatient hysteroscopy: A systematic-review and meta-analysis

The field of optogenetics is rapidly growing in relevance and number of developed tools. Amongst other things, the optogenetic repertoire includes light-responsive ion channels and methods for gene regulation. This review will be confined to the optogenetic control of gene expression in mammalian cells as suitable models for clinical applications. Here optogenetic gene regulation might offer an excellent method for spatially and timely regulated gene and protein expression in cell therapeutic approaches. Well-known systems for gene regulation, such as the LOV-, CRY2/CIB-, PhyB/PIF-systems, as well as other, in mammalian cells not yet fully established systems will be described. Advantages and disadvantages with regard to clinical applications are outlined in detail. Among the many unanswered questions concerning the application of optogenetics, we discuss items such as the use of exogenous chromophores and their effects on the biology of the cells and methods for a gentle, but effective gene transfection method for optogenetic tools for in vivo applications.

Farmlands contamination with heavy metals (HMs) can be considered as a global issue especially in developing countries. The current study investigates the various pollution indices, potential ecological risk index (PERI) and human health risk caused by HMs in some selected regions of Punjab Province, Pakistan. Farmlands soil samples were collected, acid extracted and analyzed via ICP-MS (Agilent 7500c) for selected HMs. All the HMs were found within permissible limits set by worldwide regulatory authorities except Cd which exceeded its limit in 68% agricultural soils of the study area. The CF values for Cd showed high (CF≥6) degree of contamination to considerable contamination (3≤CF≤6). The CD values indicated low (CD≤6) contamination to moderate (6dermal>inhalation. Furthermore, the RI values were observed higher than 1.0×10–4 for Cd (Lahore and Faisalabad regions) and Cr (Multan and Faisalabad regions) in children working in farmlands and likely exposed to high cancer risk. So, minimization of pollutants must be the top priority of the state to reduce contaminants inputs and immobilization in soil through environmental protection laws and regulations

Through reducing the problem to rational orthogonal system (Takenaka-Malmquist system), this note gives a proof for existence of n-best rational approximation to functions in the Hardy H^2(D) space by using pseudohyperbolic distance.

Rationale, Aims and Objectives: Alignment between patients’ treatment choices and treatments received is acknowledged as an important outcome of shared decision-making (SDM), yet recent research suggests that patients’ choices do not always align with their actual treatment trajectories. This paper explores the alignment of patient-expressed treatment choices (for surgery or medical management) after exposure to decision aids and treatments received among patients with hip or knee osteoarthritis within High Value Healthcare Collaborative (HVHC) systems as the collaborative integrating decision aids intended to support SDM into routine clinical practice. Method: This retrospective cohort study examines data from adult (>18 years) patients with hip or knee osteoarthritis who received decision aids as part of orthopedic consultations within HVHC systems between 2012-2015. Multivariate logistic regression explored the association between patient-level characteristics with the odds of treatment choice-receipt alignment. Results: The majority of patients with knee osteoarthritis (68.3%) and hip osteoarthritis (71.9%) received treatments aligned with their choices following exposure to decision aids, but analyses reveal important differences in the odds of such alignment across patient characteristics. In adjusted models, African American patients with knee osteoarthritis had 50% lower odds of receiving treatment aligned with their choices compared with white patients (OR = 0.52, p<0.05). Medicare- or Medicaid-insured knee patients had 49% and 59% lower odds (respectively) of receiving choice-aligned treatments relative to privately insured patients. Patients with knee (OR = 0.40, p<0.01) or hip (OR = 0.75, p<0.05) osteoarthritis at earlier decision-making stages had lower odds of receiving treatments congruent with their choices. Conclusion: This work elucidates the odds of treatment choice-aligned care for patients within health care systems attempting to routinely integrate decision aids to support SDM into clinical practice and underscores the gaps in achieving this alignment among African American patients, those with public insurance and those at early decision-making stages.

We in this paper improve a method of establishing the existence of finite time blow-up solutions, and then apply it to study the finite time blow-up, the blow-up time and the blow-up rate of the weak solutions on the initial boundary problem of u_t - \Delta u_{t} - \Delta u_{t} = |u|^{p - 1}u. By applying this improved method, we prove that I(u_{0}) < 0 is a sufficient condition of the existence of the finite time blow-up solutions and \frac{2(p - 1)^{-1}\|u_{0}\|_{H_{0}^{1}}^{2}}{(p - 1) \|\nabla u_{0}\|_{2}^{2} - 2(p + 1)J(u_{0})} is an upper bound for the blow-up time, which generalize the blow-up results of the predecessors in the sense of the variation. Moreover, we estimate the upper blow-up rate of the blow-up solutions, too.

Giant cell arteritis (GCA) is rarely associated with cancer. The association paraneoplastic vasculitis and tumors appears to be more frequent with hematologic cancers. The authors report the case of 83-years-old Caucasian man, diagnosed with prostatic adenocarcinoma who developed a paraneoplastic GCA responsive to corticosteroids associated with disease progression.

The main aim of the current study is to evaluate the compressive quasi-static and fatigue properties of titanium alloy (Ti6Al4V) cellular materials, with different topologies, manufactured via Laser Powder Bed Fusion (LPBF) process. The topologies herein considered are lattice based regular and irregular configurations of cubic, star and cross shaped unit cell along with trabecular based topology. The results have indicated that the effective stiffness of all configurations are in the range of 0.3 – 20 GPa, which is desirable for implant applications. The morphological irregularities in the structures induce bending dominated behavior affecting more the topologies with vertical struts. The S – N curves normalized with respect to the yield stress indicate that the behavior of star regular structures is between purely stretching dominated cubic and purely bending dominated cross based structures. Trabecular structures have shown desirable quasi-static and fatigue properties despite the random distribution of struts.

The growing use of new generation sequencing technologies on genetic diagnosis has produced an exponential increase in the number of Variants of Uncertain Significance (VUS). In this manuscript we compare three machine learning methods to classify VUS as Pathogenic or No pathogenic, implementing a Random Forest (RF), a Support Vector Machine (SVM), and a Multilayer Perceptron (MLP). To train the models, we extracted 82,463 high quality variants from ClinVar, using 9 conservation scores, the loss of function tool and allele frequencies. For the RF and SVM models, hyperparameters were tuned using cross validation with a grid search. The three models were tested on a set of 5,537 variants that had been classified as VUS any time along the last three years but had been reclassified in august 2020. The three models yielded superior accuracy on this set compared to the benchmarked tools. The RF based model yielded the best performance across different variant types and was used to create VusPrize, an open source software tool for prioritization of variants of uncertain significance. We believe that our model can improve the process of genetic diagnosis on research and clinical settings.