RESUMO
Aim: This study aimed to evaluate the imbalance of erythropoiesis and iron metabolism in patients with thalassemia. Methods: 192 patients with non-transfusion-dependent thalassemia (NTDT), 94 patients with transfusion-dependent thalassemia (TDT) and 101 healthy controls were recruited between June 2013 and December 2016 in the Hematology Department, the First Affiliated Hospital of Guangxi Medical University. The groups were compared in terms of levels of erythropoiesis biomarkers [growth differentiation factor 15 (GDF15), erythropoietin (EPO) and soluble transferrin receptor (sTfR)] and of iron overload biomarkers [serum ferritin (SF), liver iron concentration (LIC) and cardiac T2*] and hepcidin. Results: The levels of GDF15, EPO, sTfR, LIC and SF were significantly higher in patients with thalassemia. The levels of GDF15 and EPO were significantly higher in patients with TDT compared to NTDT. Those with iron overload had higher EPO, GDF15, SF and sTfR levels compared with non-iron overload patients. Hepcidin levels and ratios of hepcidin to erythropoietic activity and to iron biomarker levels were lower in patients with ß-thalassemia intermedia or hemoglobin (Hb) E/ß-thalassemia than in patients with HbH disease. The hepcidin levels were correlated negatively with the levels of EPO, GDF15 and sTfR in patients with NTDT and TDT, but correlated positively with SF and Hb levels only in patients with TDT. Conclusions: Patients with thalassemia showed iron overload, reduced hepcidin levels, and a greater extent of ineffective erythropoiesis. The hepcidin levels were more strongly related to ineffective erythropoiesis compared with iron overload. The imbalance between erythropoiesis and iron metabolism differed across different thalassemia types.
Assuntos
Eritropoese , Talassemia/sangue , Adolescente , Adulto , Estudos de Casos e Controles , Criança , Pré-Escolar , Estudos Transversais , Eritropoetina/sangue , Feminino , Ferritinas/sangue , Fator 15 de Diferenciação de Crescimento/sangue , Coração/diagnóstico por imagem , Hepcidinas/sangue , Humanos , Lactente , Fígado/diagnóstico por imagem , Fígado/metabolismo , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Miocárdio/metabolismo , Receptores da Transferrina/sangue , Talassemia/diagnóstico por imagem , Adulto JovemRESUMO
Hb Southampton [ß106(G8)LeuâPro; HBB: c.320T>C] is a rare, unstable hemoglobin (Hb), variant. The main clinical presentation of this variant is hemolytic anemia. We detected this mutation in a 2-year-old Chinese girl with a history of regular transfusions. To the best of our knowledge, this is the first time that this variant has been found in the Asian population.
Assuntos
Anemia Hemolítica/genética , Povo Asiático/genética , Hemoglobinas Anormais/genética , Anemia Hemolítica/terapia , Pré-Escolar , China , Feminino , HumanosRESUMO
Due to the different weed characteristics in peanut fields at different weeding periods, there is an urgent need to study a general model of peanut and weed detection and identification applicable to different weeding periods in order to adapt to the development of mechanical intelligent weeding in fields. To this end, we propose a BEM-YOLOv7-tiny target detection model for peanuts and weeds identification and localization at different weeding periods to achieve mechanical intelligent weeding in peanut fields at different weeding periods. The ECA and MHSA modules were used to enhance the extraction of target features and the focus on predicted targets, respectively, the BiFPN module was used to enhance the feature transfer between network layers, and the SIoU loss function was used to increase the convergence speed and efficiency of model training and to improve the detection performance of the model in the field. The experimental results showed that the precision, recall, mAP and F1 values of the BEM-YOLOv7-tiny model were improved by 1.6%, 4.9%, 4.4% and 3.2% for weed targets and 1.0%, 2.4%, 2.2% and 1.7% for all targets compared with the original YOLOv7-tiny. The experimental results of positioning error show that the peanut positioning offset error detected by BEM-YOLOv7-tiny is less than 16 pixels, and the detection speed is 33.8 f/s, which meets the requirements of real-time seedling grass detection and positioning in the field. It provides preliminary technical support for intelligent mechanical weeding in peanut fields at different stages.
RESUMO
Background: Iron overload is one of the main factors that increase morbidity and mortality in patients with non-transfusion dependent thalassemia (NTDT). Aim: This study aimed at investigating the prevalence and severity of iron overload in Chinese NTDT patients. Methods: we analyzed serum ferritin (SF), liver iron concentration (LIC) and cardiac T2* in 178 Chinese NTDT in this cross-sectional study. Results: The median SF level was 996.00(27.15-19704.00) ng/ml and the median LIC value was 8.90(0.60-43.00) mg Fe/g dry weight (dw). The youngest patient with liver iron overload was 5 years old with 5.6 mg Fe/g dw in LIC. The median cardiac T2* was 33.06(7.46-75.08) ms. 6 patients had cardiac T2*⩽20ms. The patients with ß thalassemia intermedia and HbE/ß thalassemia showed a statistically significant lower Hb and higher values of SF and LIC than those of hemoglobin H disease patients. On multivariate logistic regression analysis, patients inâ⩾âage 30-year old had a significant higher risk for iron overload (OR: 77.75, 95% CI: 8.76-690.49) in the age group. The detailed analysis of proportions of different LIC indicate in > 30-year old group, 76.8% patients suffered from moderate and severe LIC. Conclusion: Our study provides a strong support for the novel findings that Chinese NTDT patients have a high prevalence of iron overload. The first assessment of MRI LIC should be performed as early as 5 years old. Then, NTDT patients > 30 years old may suffer with a high burden of iron overload.
RESUMO
BACKGROUND: Extramedullary hematopoiesis (EMH) is common in non-transfusion-dependent thalassemia (NTDT) patients. Clinical presentations of EMH vary as MRI screening is not feasible. Hence, serum biomarkers are used to predict the risk of EMH. MATERIALS AND METHODS: 52 NTDT patients, including 26 EMH (+) and 26 EMH (-), together with 26 healthy controls, were enrolled in this case-control study from 2013 to 2016. EMH was confirmed by computed tomography or MRI. Demographic, transfusion, genetic, laboratory, and liver iron concentration (LIC) data, as well as clinical complications, were analyzed. RESULTS: EMH (+) patients had significantly higher serum ferritin (SF), growth differentiation factor 15 (GDF15), and erythropoietin (EPO) levels compared with EMH (-) patients and controls. The levels of erythroferrone (ERFE), hepcidin, and sTfR did not differ significantly between EMH (+) and EMH (-) patients (p>0.05). In NTDT patients, serum ERFE was not related to SF, LIC, hepcidin, sTfR, EPO, GDF15, and Hb levels. GDF15, EPO concentrations, and GDF15 to sTfR and GDF15 to EPO ratios are able to determine the presence of EMH with considerable sensitivity and specificity. CONCLUSIONS: GDF15, EPO, and GDF15 to EPO and GDF15 to sTfR ratios are potential biomarkers for the early prediction of NTDT in patients who are at risk for EMH.