H63D syndrome is a phenotype of a homozygous mutation of the HFE gene H63D, which is otherwise known to cause at most mild classical hemochromatosis. H63D syndrome leads to an iron overload in the body (especially in the brain, heart, liver, skin and male gonads) in the form of non-transferrin bound iron (NTBI) poisoning. Hallmark symptoms and causal factor for H63D syndrome is a mild hypotransferrinemia with transferrin saturation values >50%. H63D syndrome is an incurable multi-organ disease, leading to permanent disability. Our objective was to detect the prevalence of narcolepsy and narcolepsy with cataplexy in patients with H63D syndrome.

Although detailed experimental investigations would provide insight into viral infections and vaccine production, building a computational framework is necessary to identify the parameters that regulate the budding and packaging of nucleocapsids. This study shows that a predictive model for the complete infection cycle can be built using nonlinear coupled ODEs and parameter estimation using a Genetic algorithm. Specifically, we have used a dataset containing the occluded virus information, budded virus in infected cells obtained by transmission electron microscopy (TEM). A novel parameter estimation strategy is proposed based on the k-medoid clustering of infected cells. Firstly, we show that the parameter estimation framework can be used for model evolution and selection of the feedback structure. Secondly, we show that the model was capable of capturing the distribution of packaged and unpackaged nucleocapsids in the nucleus, cytoplasm, and plasma membrane, the number of packaged and unpackaged ODV, and polyhedra in the nucleus. The proposed framework assumes importance in generating data for achieving quality by design in the optimization of vaccine/recombinant protein yield.

Patients after liver transplantation have an increased risk to develop haematologic neoplasias. Information how to treat these patients in the context of immunosuppression is sparse. Here, we report two patients with polycythemia vera (PV) and myelofibrosis (MF) on ruxolitinib after liver transplantation.

Objective: To characterize viscoelastic testing profiles of children with multisystem inflammatory syndrome in children (MIS-C). Methods: This single-center retrospective review included 30 patients diagnosed with MIS-C from January 1 to September 1, 2020. Thromboelastography (TEG) with platelet mapping was performed in 19 (63%) patients and compared to age- and gender- matched controls via Student’s t-test and Wilcoxon rank sum test. Pearson’s and Spearman correlation were used to assess relationships between TEG parameters and inflammatory markers. Results: Patients with MIS-C had abnormal TEG results compared to controls, including decreased K time (1.1 vs. 1.7 min, P<0.01), increased alpha angle (75.0 vs. 65.7 degrees, P<0.01), increased maximum amplitude (70.8 vs. 58.3 mm, P<0.01), and decreased Ly-30 (1.1 vs. 3.7%, P=0.03); consistent with increased clot formation rate and strength, and slower fibrinolysis. TEG maximum amplitude was moderately correlated with erythrocyte sedimentation rate (r=0.60, P=0.02), initial platelet count (r=0.67, P<0.01), and peak platelet count (r=0.51, P=0.03). TEG alpha angle was moderately correlated with peak platelet count (r=0.54, P=0.02). 17 (57%) patients received aspirin (ASA) and anticoagulation, 5 (17%) received only ASA, and 3 (10%) received only anticoagulation. No patients had a thrombotic event. 6 (20%) patients had a bleeding event, none of which was major. Conclusions: Patients with MIS-C had evidence of hypercoagulability on TEG. Increased erythrocyte sedimentation rate and platelets were associated with higher clot strength. Treatment with ASA or anticoagulation was well tolerated. Further multi-center study is required to characterize the rate of thrombosis and optimal thromboprophylaxis algorithm in this patient population.

A non-equilibrium sediment transport soil erosion model based on finite volume method (FVM) coupled with two-dimensional hydrodynamic process is proposed, application of the GPU techniques in the numerical model, making it possible to simulate the sediment transport and bed evolution in a high resolution but efficient way. The first-order Gudonov format FVM is used to discreting the control equation. The variables on both sides of the unit interface are obtained by limiting slope interpolation. An efficient and robust non-negative depth reconstruction algorithm is used to solve the dry-wet boundary problem. This algorithm makes the model have second-order accuracy in space, and also effectively suppresses the numerical oscillation. Harten, Lax van Leer Contact (HLLC) approximate Riemann solver is used to calculate mass and momentum flux, and the friction source term is calculated by the proposed split point implicit method. These values are evaluated by a novel 2D edge-based MUSCL scheme. The code is programmed using C++ and CUDA, which can be run on GPU to greatly accelerate the calculation speed. In this paper, two numerical experiments show that the model performs well in accuracy and robustness of the algorithm in the process of slope erosion and watershed erosion. The constructed model can simulate the soil erosion of slope and watershed gully under different vegetation coverage, and characterize the erosion process of interaction between slope and gully.

Objective To compare the cumulative live birth rate per egg retrieval between time lapse imaging (TLI) incubators and standard culture (SC) incubators both using a single-step culture medium Design Retrospective cohort study Setting A tertiary level fertility-centre Population Women undergoing an IVF cycle between November 2015 and December 2017 Methods Comparison was done between 1219 IVF cycles using TLI and 1039 cycles using SC after accounting for confounding factors such as age and number of oocytes retrieved. Main outcome measure Cumulative live birth rate per egg retrieval Results The live birth rate per egg retrieval following fresh embryo transfer was noted to be higher for TLI cycles (TLI 39.87% vs SC 38.02%, aOR 1.20, 95% CI 1.01 to 1.44). More embryos were available for cryopreservation in the TLI arm (MD 0.08 embryos, 95% CI 0.10 to 0.41). The live birth rate per frozen embryo transfer was not significantly different. The cumulative live birth rate per egg retrieval was significantly higher in the TLI arm (TLI 50.29% vs SC 46.78%, aOR 1.24, 95% CI 1.04 to 1.48) Conclusions With the use of single step medium, there appears to be a greater benefit of TLI through a reduced interruption in embryo culture conditions, resulting in a higher number of embryos available for cryostorage which in turn appears to improve the cumulative live birth rate. Funding No funding was obtained for this study Keywords Time lapse imaging, cumulative live birth rate, single step culture medium, embryo utilization rate

Most but not all observational studies of statin treatment of COVID-19 patients suggest that treatment improves outcomes. However, almost all observational studies fail to consider what cardiovascular investigators have known for 15-20 years: withdrawing statins after hospital admission has detrimental effects on patient outcomes. Continuing (or starting) statin treatment after hospital admission consistently improves COVID-19 patient outcomes, whereas discontinuing treatment does not. Thus, observational studies of the effectiveness of statin treatment of COVID-19 patients must consider the consequences of statin withdrawal.

Porcine epidemic diarrhea virus (PEDV) produces infection in pigs characterized by vomiting and diarrhea. PEDV is transmitted via oral-fecal and a very low oral dose is enough to infect susceptible pigs, causing devastating consequences in production. A 10,000-sow farrow-to-wean farm located in northwest Mexico was infected with PEDV. After the observation of the first clinical signs, an outbreak investigation take into place to determine the most probably source of infection. A systematic collection of samples including rectal swabs, gestation and lactation feed, surface swabs from the interior or feed bins and many points of the feed truck delivering the implicated feed was performed. Samples were tested for PEDV polymerase chain reaction (PCR). Positive PCR results showed the evidence of PEDV RNA in lactating feed, the interior walls of the feed bins and in the interior of the auger boom of the feed truck. This, connected with the location of first clinical signs point that the most probably incursion of PEDV in to this breeding herd was contaminated feed. This paper shows how feed or feed transport can be a potential source of PEDV infection in farms and highlight the importance of stablishing biosecurity programs to mitigate the risk of PEDV infections.

Ravitch technique of chest correction has been considered, although invasive, as safe and efficacious surgical method. We describe a case of 35-year-old woman with cardiac tamponade and in cardiogenic shock due to exceptional late complication after pectus excavatum reconstruction by means of classic Ravitch technique 19 years earlier. This very late adverse event was caused by broken metal sternal wire that injured the wall of the ascending aorta. Patient underwent salvage repair of this segment of aorta in cardiopulmonary bypass. Postoperative course and post-discharge 3-year follow-up have been uneventful.

This paper is concerned with the initial-boundary value problem for a nonlinear parabolic equation with a small parameter. The existence of a boundary layer as the parameter goes to zero is obtained together with the estimation on the thickness of the boundary layer. The main result extends an earlier work of Frid and Shelukhin (1999).

Objective To determine the relative risk of suicide and accidental death among patients with ovarian cancer to that of the general population, and to identify risk factors associated with suicide and accidental death. Design The surveillance, epidemiology, and end results (SEER) registry provided ovarian cancer data from 18 registries. Setting Surveillance, Epidemiology, and End Results database. Population The study population comprised 149,204 patients. Methods The standardized mortality ratios (SMRs) were calculated and Fine-Gray models were fitted, with stratifications on demographic and tumor-related characteristics. Main outcome measures The standardized mortality ratios. Results Women with ovarian cancer had a higher risk of suicide and accidental death than the cancer-free group (SMR=1.86; 95% CI [1.54-2.25] and SMR=1.54; 95% CI [1.39-1.71]). Subgroup analysis indicated that only patients with type II epithelial ovarian cancer (SMR=2.31; 95% CI [1.83-2.91]) had an increased risk of suicide, and those with type I and type II epithelial ovarian cancer (SMR=1.65; 95% CI [1.39-1.97] and SMR=1.49; 95% CI [1.30-1.70]) were at a higher risk of accidental death. Patients with ovarian cancer who were younger, white, diagnosed with high-grade, non-metastatic cancer and pelvic exenteration were at a higher risk of suicide. Additionally, pelvic exenteration increased the risk of suicide but not the risk of accidental death among these women. Conclusion Women with ovarian cancer had a higher risk of suicide and accidental death compared with the general population. Clinicians should identify high-risk subgroups of ovarian cancer patients for suicide and accidental death as early as possible, with appropriate prevention strategies.

Introduction: High uric acid levels are commonly encountered in kidney transplant recipients, and can be associated with allograft dysfunction. Our study aims to examine the relationship between UA levels and graft function in patients discontinuing steroids. Methods: In this single-center-retrospective study, 56 patients from among 678 RT patients transplanted from living donors between 1999-2020 were included. Causes of steroid discontinuation, creatinine levels concurrent with uric acid levels before and after steroid discontinuation (mean 3.9 ± 2.1 years), acute rejection numbers, demographics, durations of dialysis and transplantation, medications (d.a. use of immunosuppressive, antihypertensive), laboratory data, human leukocyte antigen (HLA) mismatch numbers, blood-pressure [BP], body mass index, late acute rejection (LAR) numbers (3 months post-transplantation) were all recorded. Results: Creatinine and uric acid levels were seen to have increased after steroid discontinuation, there was a significant relationship between them (p<0.001). Statistically significant correlation was found between increased creatinine levels after steroid discontinuation and graft survival with higher HLA mismatch; 39 (69.6%) patients with mismatch ≥2, and 17 patients with mismatch <2 (30.4%) (p=0.049) . No significant relationship was found between LAR numbers before and after steroid discontinuation, and creatinine levels after steroid discontinuation. In conclusion, per model obtained as a result of multivariate linear analysis, hyperuricemia and HLA mismatch numbers (p= 0.048 and p= 0.044, respectively) are independent predictive factors for graft dysfunction in patients discontinuing steroids. Accordingly, negative effects of modeling should be kept in mind for long-term graft survival in patients who plan to continue with steroid-sparing regimens.

Background: Laparoscopic proximal gastrectomy with an anti-reflux procedure, as a function-preserving surgery, has been a common treatment strategy for proximal early gastric cancer. This study aimed to develop a new surgical technique for these patients. Methods: Laparoscopic proximal gastrectomy with vagus nerve preservation and double-flap technique was performed in patients who could retain two-thirds of the stomach. The hepatic branches and the celiac branches were both preserved. A seromuscular double-flap was created through the auxiliary incision, and the anastomosis was performed under laparoscopy. The detailed surgical procedure and preliminary results were presented. Results: Six patients underwent this procedure with no conversion to open surgery. No intraoperative and postoperative complications occurred during the perioperative period. No patients showed diarrhea and appetite loss, and body weight regained preoperative level 6 month later. No food residue and reflux esophagitis were observed via endoscopic examination. Gallbladder contraction was observed in all the patients by ultrasonography after high-fat diet. Conclusion: Although long-term follow up and a larger number of patients are required to evaluate the functional outcomes, this new technique provides a minimally invasive surgical option for proximal early gastric cancer, especially in the cardiac area.

Disease activity and treatment-related toxicity are the most important contributors to poor prognosis in patients with ANCA-associated vasculitis (AAV). Sometimes, it is difficult to distinguish them. We present a case to highlight the challenges in the management of AAV and the complications with immunosuppression.

Background: In children and adolescents, isolated cholestasis rarely is a first clinical sign of severe sepsis. Case: A previously healthy 14-year-old adolescent had severe S. aureus sepsis. Delayed diagnosis required a prolonged antimicrobial treatment. Conclusion: This unique case illustrates the importance of considering jaundice as an early sign of sepsis.

Under-expanded stents in calcific coronary stenoses are a common challenge for interventional cardiologists as we undertake more complex coronary interventions for older population cohorts. It results in short-term and long-term stent failure and adverse patient outcomes. These complex lesions can be treated after many years with intravascular lithotripsy (IVL-Shockwave).

Background: Flaxseed may be beneficial for the management of obesity due to its high content of alpha-linolenic acid, fiber, and lignans. Objective: We aimed to evaluate the effects of Flaxseed consumption on serum lipids, adiponectin, leptin, and weight loss in overweight or obese women. Methods: This randomized controlled clinical trial involved 60 overweight or obese women. Participants were randomly allocated to two groups; a) a balanced diet plus 30 g/day milled Flaxseed and b) a balanced diet plus 30g/day milled rice (as control group), for 12 weeks. Anthropometric indices, serum lipids, leptin, and adiponectin levels were measured at baseline and at the end of intervention. Results: After 12 weeks of intervention, there were significantly higher reductions in waist circumference (WC) and waist to hip ratio (WHR) (both P<0.05) in the flaxseed group compared to the controls. Moreover, adiponectin level was significantly increased in the flaxseed group (17.15±6.1) compared to the controls (16.83±10.5), (P=0.001). However, there were no significant differences in serum lipid levels between the study groups before and after the intervention (all p>0.05). Conclusion: Flaxseed consumption may improve adiposity markers, as well as adiponectin levels Thus, flaxseed consumption could be an adjunctive therapy to attenuate central obesity. Serum lipid profile has not changed significantly after flaxseed consumption. Keywords: obesity, flaxseed, adiponectin, Leptin, blood lipids

Actinomycosis is a rare, indolent and invasive infection caused by Actinomyces species. Pulmonary actinomycosis is very rarely seen in the paediatric population. The classic radiological presentation of thoracic involvement of actinomycosis includes lower lobe consolidation, empyema and periostitis of the ribs. We report a case of endobronchial pulmonary actinomycosis in a child diagnosed on endobronchial biopsy and broncho-alveolar lavage taken during bronchoscopy. Bronchoscopy can be dangerous when performed on these cases, as there is a risk of severe bleeding and large airway obstruction, as was the case with this patient.

African swine fever (ASF), is a serious global concern from an ecological and economic point of view. While it is well established that its main transmission routes comprise contact between infected and susceptible animals and transmission through contaminated carcasses, the specific mechanism leading to its long-term persistence is still not clear. Among others, a proposed mechanism involves the potential role of convalescent individuals, which would be able to shed the virus after the end of the acute infection. Using a spatially explicit, stochastic, individual-based model, we tested: 1) if ASF can persist when transmission occurs only through infected wild boars and infected carcasses; 2) if the animals that survive ASF can play a relevant role in increasing ASF persistence chances; 3) how hunting pressure can affect the ASF probability to persist. The scenario in which only direct and carcass-mediated transmission were contemplated had 52% probability of virus persistence 10 years after the initial outbreak. The inclusion of survivor-mediated transmission corresponded to slightly higher persistence probabilities (57%). ASF prevalence during the endemic phase was generally low, ranging 0.1-0.2%. The proportion of seropositive individuals gradually decreased with time and ranged 4.5 – 6.6%. Our results indicate that direct and carcass-mediated infection routes are sufficient to explain and justify the long-term persistence of ASF at low wild boar density and the ongoing geographic expansion of the disease front in the European continent. During the initial years of an ASF outbreak, hunting should be carefully evaluated as a management tool, in terms of potential benefits and negative side-effects, and combined with an intensive effort for the detection and removal of wild boar carcasses. During the endemic phase, further increasing hunting effort should not be considered as an effective strategy. Additional effort should be dedicated to finding and removing as many wild boar carcasses as possible.

The understanding of dysplastic (also known as atypical) nevi as a precursor lesion of malignant melanoma is hampered by incongruities on different levels. Neither epidemiological data nor clinical and histomorphological features nor biological aspects are suitable to prove a sequential tumor progression from nevi to melanoma. With respect to basic mechanisms of malignant transformation such as proliferation rate and telomerase activity, no significant differences between dysplastic and other nevi could be found. Thus, the dysplastic nevus represents a type of benign nevi and is to be distinguished from early forms of malignant melanoma in diagnostics. Their use as an easy to take "emergency exits" for pathologists must come to an end.

Methods: Twenty-two French nursing homes were included. COVID-19 had been diagnosed with real-time reverse-transcriptase polymerase chain reaction (RT-PCR) for SARS-CoV-2. Blood S-protein IgG and nucleocapsid (N) IgG protein (N-protein IgG) were measured 21-24 days after the first jab (1,004 residents) and 6 weeks after the second (820 residents). Results: Among the 735 residents without prior COVID-19, 41.7% remained seronegative for S-protein IgG after the first jab vs 2.1% of the 270 residents with a previous positive RT-PCR (p<0.001). After the second jab, only 3% of the 586 residents without prior COVID-19 remained seronegative. However, 26.5% of them had low S-protein IgG levels (50-1050 UA/mL) vs 6.4% of the 222 residents with prior COVID-19. Residents with old infection (first wave), or seropositive for N-protein IgG at the time of vaccination, had the highest S-protein IgG levels. Residents with a prior COVID-19 infection had higher S-protein IgG levels after one dose than those without two jabs. Interpretation: A single vaccine jab is sufficient to reach immunity in residents with prior COVID-19. Most residents without prior COVID-19 are seropositive for S-protein IgG after the second jab, but around 30% have low levels of S-protein IgG. Whether residents with no or low post-vaccine immunity are at higher risk of symptomatic COVID-19 requires further analysis.

Guillain-Barré syndrome is an immune-mediated neuropathy that was reported following multiple vaccines. We present the case of a gentleman who developed GBS 20 days after the second dose of COVID-19 vaccination. It is important to mention that more research is needed to establish an association between COVID-19 vaccine and GBS.

Background It is important to ensure access to hydroxyurea (HU) for patients with sickle cell anemia (SCA) living in rural areas without easy access to experts in sickle cell management. The UAB Pediatric Sickle Cell program’s satellite clinics reduce the barrier of transportation to the university-based clinic. However, as compared to the university clinic, these satellite clinics do not offer immediate access to HU dosing laboratory results. Therefore, a nurse clinician calls families with HU dose adjustments after the completion of the clinic visit. This study evaluated the impact of telehealth dosing adjustments on HU laboratory and clinical response as compared to university-based patients. Methods A one year retrospective chart reviewed was performed to evaluate HU laboratory and clinical response based on clinic location and socioeconomic status for patients with SCA. We identified the number of clinic and acute care visits for one year and calculated the mean CBC and HbF values for each patient. Results We identified 107 academic center participants with SCA prescribed HU and 65 satellite clinic participants. We identified no difference in HbF, Hb, MCV, or ANC by clinic location. We also identified no difference in hospital admissions based on clinic location. Finally, mean socioeconomic indicators by zip code were lower in satellite clinic patients but not associated with a difference in HbF response. Conclusions The use of telehealth did not negatively impact laboratory response to HU. Future studies should identify novel approaches to improve access to HU among patients with SCA living in rural areas.

Pulmonary embolism is a major cause of death worldwide. Recurrences are mainly observed in patients with coagulation disorders or cancers. We describe the case of a brain tumor (Glioblastoma) revealed by behavioral disorder in a young patient admitted for a recurrent pulmonary embolism