Preliminary data on COVID-19 in patients with hemoglobinopathies : A multicentre ICET-A study

Objectives: This study aims to investigate, retrospectively, the epidemiological and clinical characteristics, laboratory results, radiologic findings, and outcomes of COVID-19 in patients with transfusion-dependent β thalassemia major (TM), β-thalassemia intermedia (TI) and sickle cell disease (SCD). Design: A total of 17 Centers, from 10 countries, following 9,499 patients with hemoglobinopathies, participated in the survey. Main outcome data: Clinical, laboratory, and radiologic findings and outcomes of patients with COVID-19 were collected from medical records and summarized. Results: A total of 13 patients, 7 with TM, 3 with TI, and 3 with SCD, with confirmed COVID-19, were identified in 6 Centers from different countries. The overall mean age of patients was 33.7±12.3 years (range:13-66); 9/13 (69.2%) patients were females. Six patients had pneumonia, and 4 needed oxygen therapy. Increased C-reactive protein (6/10), high serum lactate dehydrogenase (LDH; 6/10), and erythrocyte sedimentation rate (ESR; 6/10) were the most common laboratory findings. 6/10 patients had an exacerbation of anemia (2 with SCD). In the majority of patients, the course of COVID-19 was moderate (6/10) and severe in 3/10 patients. A 30-year-old female with TM, developed a critical SARS-CoV-2 infection, followed by death in an Intensive Care Unit. In one Center (Oman), the majority of suspected cases were observed in patients with SCD between the age of 21 and 40 years. A rapid clinical improvement of tachypnea/dyspnea and oxygen saturation was observed, after red blood cell exchange transfusion, in a young girl with SCD and worsening of anemia (Hb level from 9.2 g/dl to 6.1g/dl). Conclusions: The data presented in this survey permit an early assessment of the clinical characteristics of COVID 19 in different countries. 70% of symptomatic patients with COVID-19 required hospitalization. The presence of associated co-morbidities can aggravate the severity of COVID- 19, leading to a poorer prognosis irrespective of age

Differential response of Zea mays L. in relation to weed control and different macronutrient combinations

Time of weed control and fertilizer application usually decide the profitability of crop production. The effects of weed control and macronutrients on maize crop were investigated. The study was undertaken in March 2009, using a RCBD design with split plot arrangements. The experimental set up was established at the Agricultural University Peshawar and seedbeds were prepared with the proper moisture regime. Maize was planted with one plot left weed free for first six weeks while another infested with weed. The combinations of macronutrients used were nitrogen, phosphorus, potassium, nitrogen-phosphorus, nitrogen-potassium, phosphorus-potassium and nitrogen-phosphorus-potassium. Control (no fertilizer) was included for comparison. The observations revealed that when a comparison was made between the application of fertilizers and weed control, the latter proved more important because weed infested plots had no harvestable maize plants. The role of main nutrients in crop production is well known and cannot be left aside, however weed infestation does not provide us a fair choice of fertilizers application. The maximum maize grain yield was recorded under nitrogen-phosphorus combination and promising results were obtained. The weeds and maize benefited equally in terms of fresh and dry weed biomass with an application of fertilizer in particular N singly or together with P. In view of this, application of fertilizer should be changed from broadcast to band and/or placement. In general, a positive interaction was seen between N and P promoting the growth of maize and weeds. It can be said that herbicide application for weed control is important because of the fact that hand weeding is not economical, difficult, time consuming because of perennial weeds and hot weather conditions in the month of June

Predicting body weight from body and testicular characteristics of balochi male sheep in Pakistan using different statistical analyses

The aim of this study was to determine the suitable statistical analysis for the prediction of body weight from biometrical and testicular traits in Balochi male sheep. For this aim, statistical performances of Stepwise Regression Analysis, use of factor analysis scores with multiple regression model analysis and Ridge Regression analysis were evaluated on data of 131 Balochi male sheep. The measured characteristics were: body weight (BW), testicular length (TL), scrotal length (SL), scrotal circumference (SC), body length (BL), withers height (WH) and heart girth (HG). In order to determine the best model, determination coefficient (R2%), Root of Mean Square Error (RMSE) and Variance Inflation Factor (VIF) values were used. Stepwise Regression and Ridge Regression analyses produced multicollinearity problem due to high VIF and RMSE values. In comparison with these two analyses, use of factor analysis scores with multiple regression model analysis offering optimal solution with very low VIF and RMSE values was adopted for the prediction of body weight of the Balochi male sheep. In the factor analysis, the 3 new-uncorrelated variables derived from eight explanatory variables were used as explanatory variables with the multiple regression analysis. Results reflected without multicollinearity problem that 91.1 % of variation in body weight was perfectly explained by the 3 new uncorrelated variables

Dopamine transporter deficiency syndrome: phenotypic spectrum from infancy to adulthood

Dopamine transporter deficiency syndrome is an SLC6A3-related progressive infantile-onset parkinsonism-dystonia that mimics cerebral palsy. Ng et al. describe clinical features and molecular findings in a new cohort of patients. They report infants with classical disease, as well as young adults manifesting as atypical juvenile-onset parkinsonism-dystonia, thereby expanding the disease spectrum.Dopamine transporter deficiency syndrome due to SLC6A3 mutations is the first inherited dopamine 'transportopathy' to be described, with a classical presentation of early infantile-onset progressive parkinsonism dystonia. In this study we have identified a new cohort of patients with dopamine transporter deficiency syndrome, including, most significantly, atypical presentation later in childhood with a milder disease course. We report the detailed clinical features, molecular genetic findings and in vitro functional investigations undertaken for adult and paediatric cases. Patients presenting with parkinsonism dystonia or a neurotransmitter profile characteristic of dopamine transporter deficiency syndrome were recruited for study. SLC6A3 mutational analysis was undertaken in all patients. The functional consequences of missense variants on the dopamine transporter were evaluated by determining the effect of mutant dopamine transporter on dopamine uptake, protein expression and amphetamine-mediated dopamine efflux using an in vitro cellular heterologous expression system. We identified eight new patients from five unrelated families with dopamine transporter deficiency syndrome. The median age at diagnosis was 13 years (range 1.5-34 years). Most significantly, the case series included three adolescent males with atypical dopamine transporter deficiency syndrome of juvenile onset (outside infancy) and progressive parkinsonism dystonia. The other five patients in the cohort presented with classical infantile-onset parkinsonism dystonia, with one surviving into adulthood (currently aged 34 years) and labelled as having 'juvenile parkinsonism'. All eight patients harboured homozygous or compound heterozygous mutations in SLC6A3, of which the majority are previously unreported variants. In vitro studies of mutant dopamine transporter demonstrated multifaceted loss of dopamine transporter function. Impaired dopamine uptake was universally present, and more severely impacted in dopamine transporter mutants causing infantile-onset rather than juvenile-onset disease. Dopamine transporter mutants also showed diminished dopamine binding affinity, reduced cell surface transporter, loss of post-translational dopamine transporter glycosylation and failure of amphetamine-mediated dopamine efflux. Our data series expands the clinical phenotypic continuum of dopamine transporter deficiency syndrome and indicates that there is a phenotypic spectrum from infancy (early onset, rapidly progressive disease) to childhood/adolescence and adulthood (later onset, slower disease progression). Genotype-phenotype analysis in this cohort suggests that higher residual dopamine transporter activity is likely to contribute to postponing disease presentation in these later-onset adult cases. Dopamine transporter deficiency syndrome remains under-recognized and our data highlights that dopamine transporter deficiency syndrome should be considered as a differential diagnosis for both infantile- and juvenile-onset movement disorders, including cerebral palsy and juvenile parkinsonism