An Automated Hb A_2 Analyzer, HLC-723G7, for Diagnosis of β-Thalassemia

We investigated the usefulness of an automated analyzer, the HLC-723G7, for determination of Hb A2 in the diagnosis of β-thalassemia. The analyzer was used for 250 Thai subjects who were either normal or were diagnosed with microcytic hypochromia based on hematological findings. An intra-assay precision test of normal subject (Hb A_2 = 2.78%) and that (Hb A_2 = 5.90%) with β-thalassemia revealed as coefficients of variation (CV%) of 3.2% and 0.95%, respectively. Samples were kept stable at 4℃ for at least 10 days. The Hb A_2 values obtained by the HLC-723G7 showed good correlation with those obtained by DEAE-HPLC with the coefficient of correlation (r) being 0.940, the coefficient of determination (R^2) 0.884, and the equation of the regression line Y_ = 1.057X_-0.248. The subjects with a higher Hb A_2 value than about 4% were suspected of having β-thalassemia based on hematological findings. Most of those with a lower or relatively lower Hb A_2 value than the normal range (2.3-3.5%) or 4% and hematological abnormalities were considered to be suffering from α-thalassemia-1, α-thalassemia-2 or iron deficiency anemia. However, a confirmed diagnosis must be made by the further study of the red cell morphology, clinical manifestations and by the DNA analysis

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

Association of osteoporosis and sarcopenia with fracture risk in transfusion-dependent thalassemia

Abstract Patients with transfusion-dependent thalassemia (TDT) have an increased risk of osteoporosis and fractures. They also have several potential factors associated with sarcopenia. There has been currently no study on sarcopenia and its association with falls and fractures in TDT. This study aims to determine the prevalence of and factors associated with osteoporosis, fragility fractures, and sarcopenia in adults with TDT. A cross-sectional study was conducted at the hematologic clinic at King Chulalongkorn Memorial Hospital, Bangkok, Thailand. Clinical data and laboratory testing were collected. Bone mineral density and morphometric vertebral fracture were assessed. Sarcopenia was defined using the 2014 and 2019 Asian Working Group for Sarcopenia (AWGS) criteria. We included 112 TDT patients aged 35.1 ± 12.5 years. The prevalence of osteoporosis was 38.4%. Fragility fractures were found in 20.5% of patients. Lower BMI (OR 0.29; 95% CI 0.12–0.72, P = 0.007) and hypogonadal state (OR 3.72; 95% CI 1.09–12.74, P = 0.036) were independently associated with osteoporosis. According to the 2014 AWGS criteria, the prevalence of overall sarcopenia and severe sarcopenia was 44.6% and 13.4%, respectively. Severe sarcopenia was strongly associated with fragility fractures (OR 4.59, 95% CI 1.21–17.46, P = 0.025). In conclusion, osteoporosis, fragility fractures, and sarcopenia were prevalent in adults with TDT. Severe sarcopenia was associated with fragility fractures. Early osteoporosis and sarcopenia screening and prevention may reduce fracture risk and its complications in these patients

Limitations of serum ferritin to predict liver iron concentration responses to deferasirox therapy in patients with transfusion-dependent thalassaemia

WOS: 000394910900011PubMed ID: 27859648BackgroundIn transfusion-dependent anaemias, while absolute serum ferritin levels broadly correlate with liver iron concentration (LIC), relationships between trends in these variables are unclear. These relationships are important because serum ferritin changes are often used to adjust or switch chelation regimens when liver magnetic resonance imaging (MRI) is unavailable. Objectives and methodsThis post hoc analysis of the EPIC study compared serum ferritin and LIC in 317 patients with transfusion-dependent thalassaemia before and after 1 yr of deferasirox. ResultsSerum ferritin responses (decreases) occurred in 73% of patients, 80% of whom also have decreased LIC. However, 52% of patients without a serum ferritin response did decrease LIC and by >1 mg Fe/g dw (median 3.9) in 77% of cases. Absolute serum ferritin and LIC values correlated significantly only when serum ferritin was <4000 ng/mL (r = 0.59; P < 0.0001) and not at higher levels (4000 ng/mL; r = 0.19). Serum ferritin response was accompanied by decreased LIC in 89% and 70% of cases when serum ferritin was <4000 or 4000 ng/mL, respectively. ConclusionsAs serum ferritin non-response was associated with LIC decrease in over half of patients, use of liver MRI may be particularly useful for differentiating true from apparent non-responders to deferasirox based on serum ferritin trends alone.Novartis Pharma AG; Novartis PharmaceuticalsThis study was sponsored by Novartis Pharma AG. Medical writing support was provided by Debbi Gorman and Catherine Risebro, PhD, from Mudskipper Business Ltd, funded by Novartis Pharmaceuticals

Defining serum ferritin thresholds to predict clinically relevant liver iron concentrations for guiding deferasirox therapy when MRI is unavailable in patients with non-transfusion-dependent thalassaemia

Liver iron concentration (LIC) assessment by magnetic resonance imaging (MRI) remains the gold standard to diagnose iron overload and guide iron chelation therapy in patients with non-transfusion-dependent thalassaemia (NTDT). However, limited access to MRI technology and expertise worldwide makes it practical to also use serum ferritin assessments. The THALASSA (assessment of Exjade((R)) in non-transfusion-dependent THALASSemiA patients) study assessed the efficacy and safety of deferasirox in iron-overloaded NTDT patients and provided a large data set to allow exploration of the relationship between LIC and serum ferritin. Using data from screened patients and those treated with deferasirox for up to 2years, we identified clinically relevant serum ferritin thresholds (for when MRI is unavailable) for the initiation of chelation therapy (>800g/l), as well as thresholds to guide chelator dose interruption (2000g/l). (clinicaltrials.gov identifier: NCT00873041)

Deferasirox demonstrates a dose‐dependent reduction in liver iron concentration and consistent efficacy across subgroups of non‐transfusion‐dependent thalassemia patients

The 1-year THALASSA study enrolled 166 patients with various non-transfusion-dependent thalassemia (NTDT) syndromes, degrees of iron burden and patient characteristics, and demonstrated the overall efficacy and safety of deferasirox in reducing liver iron concentration (LIC) in these patients. Here, reduction in LIC with deferasirox 5 and 10 mg/kg/day starting dose groups is shown to be consistent across the following patient subgroups—baseline LIC/serum ferritin, age, gender, race, splenectomy (yes/no), and underlying NTDT syndrome (β-thalassemia intermedia, HbE/β-thalassemia or α-thalassemia). These analyses also evaluated deferasirox dosing strategies for patients with NTDT. Greater reductions in LIC were achieved in patients dose-escalated at Week 24 from deferasirox 10 mg/kg/day starting dose to 20 mg/kg/day. Patients who received an average actual dose of deferasirox >12.5–≤17.5 mg/kg/day achieved a greater LIC decrease compared with the ≥7.5–≤12.5 mg/kg/day and >0–<7.5 mg/kg/day subgroups, demonstrating a dose–response efficacy. LIC reduction across patient subgroups was generally consistent with the primary efficacy analysis with a similar safety profile. Am. J. Hematol. 88:503–506, 2013. © 2013 Wiley Periodicals, Inc