PENGARUH PEMBELAJARAN KEWIRAUSAHAAN DAN EKSTRAKURIKULER WAJIB KEPRAMUKAAN TERHADAP KARAKTER KEWIRAUSAHAAN (Studi Kasus Peserta Didik Kelas XI SMA YPI Bandung)

Penelitian ini bertujuan untuk memperoleh data mengenai 1. Pembinaan karakter kewirausahaan pada pembelajaran kewirausahaan pokok pembahasan produksi ikan konsumsi peserta didik kelas XI SMA YPI Bandung, 2. Pembinaan karakter kewirausahaan di Gugus depan 03021-03022 pangkalan SMA YPI Bandung, 3. Karakter kewirausahaan peserta didik kelas XI SMA YPI Bandung, 4. Pengaruh pembinaan karakter kewirausahaan melalui pembelajaran kewirausahaan pokok bahasan produksi ikan konsumsi peserta didik kelas XI SMA YPI Bandung, 5. Pengaruh pembinaan karakter kewirausahaan dalam ekstrakurikuler wajib kepramukaan di Gugus depan 03021-03022 pangkalan SMA YPI Bandung. Metode penelitian yang dipergunakan metode survei tingkat eksplanasi asosiatif kausal. Teknik pengumpulan data dengan cara studi pustaka, kuesioner, dan observasi. Populasi pada penelitian ini, yaitu peserta didik kelas XI SMA YPI Bandung semester I yang berjumlah 22 orang. Hipotesis pada penelitian ini, yaitu 1. Terdapat pengaruh pembelajaran kewirausahaan terhadap karakter kewirausahaan peserta didik kelas XI SMA YPI Bandung, 2 Terdapat pengaruh ekstrakurikuler kepramukaan terhadap karakter kewirausahaan peserta didik kelas XI SMA YPI Bandung. Teknik pengolahan data menggunakan uji validitas, uji reliabilitas, uji normalitas data, analisis regresi linier sederhana, dan koefisien determinasi dengan menggunakan program SPSS 20.0 for windows. Hasil pengolahan data menunjukkan bahwa, 1. Pembinaan karakter kewirausahaan pada pembelajaran kewirausahaan peserta didik kelas XI SMA YPI menunjukkan kategori Baik dengan rata-rata 3,85, 2. Pembinaan karakter kewirausahaan di Gugus depan 03021-03022 pangkalan SMA YPI Bandung menunjukkan kategori Baik dengan rata-rata 3,78, 3. Karakter kewirausahaan peserta didik kelas XI SMA YPI Bandung menujukkan kategori Sangat Baik dengan rata-rata 4,04, 4. T¬erdapat pengaruh pembelajaran kewirausahaan terhadap karakter kewirausahaan peserta didik senilai 4,7 % itu berarti Y sebagian kecil dipengaruhi oleh X1, arti Ha1 dapat diterima dan Ho1 ditolak, 5. Terdapat pengaruh ekstrakurikuler kepramukaan terhadap karakter kewirausahaan peserta didik senilai 27,6 % itu berarti Y hampir setengahnya dipengaruhi oleh X2, arti Ha2 dapat diterima dan Ho2 ditolak. Kesimpulan dari penelitian ini yaitu secara parsial terdapat pengaruh positif pembelajaran kewirausahaan dan ekstrakurikuler kepramukaan terhadap karakter kewirausahaan peserta didik kelas XI SMA YPI Bandung. Sebagai akhir penelitian penulis menyampaikan saran kepada sekolah, guru, dan pembina Pramuka agar pembelajaran dan pelatihan dioptimalkan sebagai salah satu strategi untuk meningkatkan karakter kewirausahaan peserta didik. Kata kunci: Pembelajaran Kewirausahaan, Ekstrakurikuler Kepramukaan, Karakter Kewirausahaan

Recommendations for Pregnancy in Rare Inherited Anemias

Rare inherited anemias are a subset of anemias caused by a genetic defect along one of the several stages of erythropoiesis or in different cellular components that affect red blood cell integrity, and thus its lifespan. Due to their low prevalence, several complications on growth and development, and multi-organ system damage are not yet well defined. Moreover, during the last decade there has been a lack of proper understanding of the impact of rare anemias on maternal and fetal outcomes. In addition, there are no clear-cut guidelines outlining the pathophysiological trends and management options unique to this special population. Here, we present on behalf of the European Hematology Association, evidence- and consensus-based guidelines, established by an international group of experts in different fields, including hematologists, gynecologists, general practitioners, medical geneticists, and experts in rare inherited anemias from various European countries for standardized and appropriate choice of therapeutic interventions for the management of pregnancy in rare inherited anemias, including Diamond-Blackfan Anemia, Congenital Dyserythropoietic Anemias, Thalassemia, Sickle Cell Disease, Enzyme deficiency and Red cell membrane disorders

Worldwide survey of T2* cardiovascular magnetic resonance in Thalassaemia

Introduction Thalassaemia major (TM) affects hundreds of thousands of patients worldwide but only a minority have access to regular blood transfusion and chelation therapy. Cardiovascular magnetic resonance (CMR) T2* measurement provides an accurate, reproducible measurement of cardiac iron which is the cause of heart failure and early death in many transfused TM patients. This technique has been adopted as part of routine management in many countries where survival is now approaching normal but little is known about the severity and effects of myocardial iron loading in different geographical regions. Purpose The aim of this study was to describe the burden of disease of myocardial siderosis (measured by T2*) in different populations throughout the world and to assess the relationship between T2* and outcome such as heart failure and cardiac death. Methods 34 worldwide centres were involved in this survey of 3376 patients from Europe, the Middle East, North America, South America, North Africa, Australia and Asia. Anonymised data on myocardial T2* values were analysed in conjunction with clinical outcomes (heart failure and death). Results Overall, 57.5% of patients had no significant iron loading (T2* >20ms), 22.6% had moderate cardiac iron (10ms50%) in South-East Asia had cardiac iron (T2* >20ms) at baseline. At the time of the first scan, 100 patients (3.3%) had confirmed heart failure, the majority of whom (77.0%) had myocardial T2* <10ms with almost all (99%) having T2* <20ms. There were 113 patients who subsequently developed heart failure. 92.0% of these had T2* <10ms and 99.1% had a T2* <20ms. There were 39 deaths. Cardiac T2* values were <10ms in 79.5%, with 92.3% <20ms. Conclusions Even in this well-treated cohort with access to transfusion, chelation and CMR, there is a large proportion of TM patients with moderate to severe cardiac iron loading. Low T2* (<10ms) is associated with cardiac failure and death. There is a huge unmet worldwide need in terms of access to specialist medical care (including transfusion and chelation therapy) together with advanced monitoring techniques (such as CMR)

Iron Chelation Therapy as a Modality of Management

WOS: 000428462100010PubMed ID: 29458731Introduction of MRI techniques for identifying and monitoring tissue iron overload and the current understanding of iron homeostasis in transfusion-dependent (TDT) and non transfusion-dependent thalassemia have allowed for a more robust administration of iron chelation therapies. The development of safe and efficient oral iron chelators and the insights gained from large-scale prospective studies using these agents have improved iron overload management. A significant reduction in iron toxicity induced morbidity and mortality and improvements in quality of life were observed in TDT. The appropriate management of tissue-specific iron loading in TDT has been portrayed using evidence-based data obtained from investigational studies.Novartis Pharmaceuticals; Cerus; Celgene; ShireReceiving research grant funding, consulting fees, and lecture fees from Novartis Pharmaceuticals, research grant funding and lecture fees from Cerus, research grant funding from Celgene and Shire

Current approach to iron chelation in children

Transfusion-dependent children, mostly with thalassaemia major, but also and occasionally to a more significant degree, with inherited bone marrow failures, can develop severe iron overload in early life. Moreover, chronic conditions associated with ineffective erythropoiesis, such as non-transfusion-dependent thalassaemia (NTDT), may lead to iron overload through increased gut absorption of iron starting in childhood. Currently, the goal of iron chelation has shifted from treating iron overload to preventing iron accumulation and iron-induced end-organ complications, in order to achieve a normal pattern of complication-free survival and of quality of life. New chelation options increase the likelihood of achieving these goals. Timely initiation, close monitoring and continuous adjustment are the cornerstones of optimal chelation therapy in children, who have a higher transfusional requirements compared to adults in order to reach haemoglobin levels adequate for normal growth and development. Despite increased knowledge, there are still uncertainties about the level of body iron at which iron chelation therapy should be started and about the appropriate degree of iron stores’ depletion

Optimising management of deferasirox therapy for patients with transfusion-dependent thalassaemia and lower-risk myelodysplastic syndromes

WOS: 000443114700002PubMed ID: 29904950Effective iron chelation therapy is an important part of treatment in patients with transfusion-dependent thalassaemia and lower-risk myelodysplastic syndromes (MDS). Key strategies for optimising iron chelation therapy include ensuring good adherence and preventing and managing adverse events (AEs). Good adherence to iron chelation therapy with deferoxamine and deferasirox has been linked to improved survival and/or reductions in complications related to iron overload; however, maintaining good adherence to iron chelators can be challenging. Patients with transfusion-dependent thalassaemia or lower-risk MDS showed better adherence to the deferasirox film-coated tablet (FCT) formulation than to the deferasirox dispersible tablet formulation in the ECLIPSE trial, reflecting in part the improved palatability and convenience of deferasirox FCT. As well as affecting adherence, AEs may lead to dose reduction, interruption or discontinuation, resulting in suboptimal iron chelation therapy. Preventing and successfully managing AEs may help limit their impact on adherence, and following dosage and administration recommendations for iron chelators such as deferasirox may help minimise AEs and optimise treatment in patients with transfusion-dependent thalassaemia and lower-risk MDS.Novartis Pharmaceuticals; Celgene; RocheRoche HoldingAntonis Kattamis reports research funding and honoraria from Novartis Pharmaceuticals and Celgene, and honoraria from Apopharma. Yesim Aydinok has nothing to disclose. Ali Taher reports research funding and honoraria from Novartis Pharmaceuticals, research funding from Celgene, and research funding from Roche

Current approach to iron chelation in children

WOS: 000337520600003PubMed ID: 24646011Transfusion-dependent children, mostly with thalassaemia major, but also and occasionally to a more significant degree, with inherited bone marrow failures, can develop severe iron overload in early life. Moreover, chronic conditions associated with ineffective erythropoiesis, such as non-transfusion-dependent thalassaemia (NTDT), may lead to iron overload through increased gut absorption of iron starting in childhood. Currently, the goal of iron chelation has shifted from treating iron overload to preventing iron accumulation and iron-induced end-organ complications, in order to achieve a normal pattern of complication-free survival and of quality of life. New chelation options increase the likelihood of achieving these goals. Timely initiation, close monitoring and continuous adjustment are the cornerstones of optimal chelation therapy in children, who have a higher transfusional requirements compared to adults in order to reach haemoglobin levels adequate for normal growth and development. Despite increased knowledge, there are still uncertainties about the level of body iron at which iron chelation therapy should be started and about the appropriate degree of iron stores' depletion.Shire; Shire and Novartis; Government Pharmaceutical Organization (GPO), ThailandYA has received research funding from Shire and Novartis; consultancy from Novartis and is a member of the speaker bureau for Novartis. AK has received research funding from Novartis and is a member of the speaker bureau for Novartis and ApoPharma. V.V. has received research funding from The Government Pharmaceutical Organization (GPO), Thailand, Shire and Novartis; consultancy from Shire and Novartis and is a member of the speaker bureau for Novartis

Central Line-Associated Bloodstream Infections in Children With Hematologic and Oncologic Diseases: First Prevalence Results From a University Hospital

###EgeUn###Central line-associated bloodstream infections (CLABSIs) are still a major cause of morbidity and mortality in pediatric hematology-oncology patients in many countries. This cross-sectional study was a retrospective review of CLABSI in inpatient pediatric hematology-oncology cases with long-term central venous catheter at the Pediatric Hematology Department from January 2013 to June 2014. Characteristics of CLABSI events in pediatric patients with hematologic malignancies and related nonmalignant hematologic conditions are documented. CLABSI developed in 61.8% (n = 21) of the 34 hospitalized patients included in the study. The CLABSI rate was 7.8 per 1,000 inpatient central venous catheter days. Coagulase-negative staphylococci was the predominant pathogen in 47.6% of the patients with CLABSI. The high rate of CLABSI requires prevention strategies to reduce CLABSI immediately. This study provides guidance in prioritizing strategies for reducing rates of infection

Thalassaemia

Thalassaemia is a diverse group of genetic disorders with a worldwide distribution affecting globin chain synthesis. The pathogenesis of thalassaemia lies in the unbalanced globin chain production, leading to ineffective erythropoiesis, increased haemolysis, and deranged iron homoeostasis. The clinical phenotype shows heterogeneity, ranging from close to normal without complications to severe requiring lifelong transfusion support. Conservative treatment with transfusion and iron chelation has transformed the natural history of thalassaemia major into a chronic disease with a prolonged life expectancy, albeit with co-morbidities and substantial disease burden. Curative therapy with allogeneic haematopoietic stem cell transplantation is advocated for suitable patients. The understanding of the pathogenesis of the disease is guiding therapeutic advances. Novel agents have shown efficacy in improving anaemia and transfusion burden, and initial results from gene therapy approaches are promising. Despite scientific developments, worldwide inequality in the access of health resources is a major concern, because most patients live in underserved areas