铁缺乏与慢性心力衰竭

慢性心力衰竭(chronic heart failure,CHF)是当今最重要的心血管疾病之一,严重影响患者的生活质量.铁缺乏(iron deficiency,ID)是CHF普遍存在的合并症,与其不良预后独立相关,应用铁剂治疗可能改善患者的生活质量和运动能力以及预防再住院,进而降低心衰死亡率.2016欧洲心脏病学会(E...     

The effect of erythroferrone suppression by transfusion on the erythropoietin–erythroferrone–hepcidin axis in transfusion-dependent thalassaemia: A pre–post cohort study

To track post-transfusion changes on the erythropoietin (EPO)–erythroferrone (ERFE)–hepcidin axis, we collected blood samples from 82 regularly transfused patients with β-thalassaemia major (β-TM) immediately before and 4–6 days after transfusion. The post-transfusion haemoglobin, hepcidin, and ferritin levels were increased, while the EPO, ERFE, and soluble transferrin receptor were suppressed. In addition, hepcidin change was inversely associated with erythropoietic change, which was confirmed by an increase in the hepcidin-to-ERFE ratio after transfusion. Age was the main predictor of serum ERFE, followed by EPO, transfusion frequencies, and ferritin. We found ERFE to be a highly sensitive indicator of erythroid activity in β-TM and that the hepcidin-to-ERFE ratio after transfusion may be used as an appropriateness index of serum hepcidin regulation relative to the degree of erythropoiesis.     

Associations between higher plasma ferritin and hepcidin levels with liver stiffness in patients with type 2 diabetes: An exploratory study

Background

Currently, there is no information about the association between circulating levels of ferritin and hepcidin and liver fibrosis in patients with type 2 diabetes mellitus (T2DM) and non-alcoholic fatty liver disease (NAFLD).

Methods

We enrolled 153 patients with T2DM with no known liver diseases, who consecutively attended our diabetes outpatient service and who underwent liver ultrasonography and liver stiffness measurement (LSM) by vibration-controlled transient elastography (Fibroscan^® for the non-invasive assessment of liver fibrosis). Plasma ferritin and hepcidin concentrations were measured with an electrochemiluminescence immunoassay and mass spectrometry-based assay, respectively.

Results

After stratification of patients by LSM tertiles [1st tertile median LSM: 3.6 (interquartile range: 3.3–4.0) kPa, 2nd tertile: 5.3 (4.9–5.9) kPa and 3rd tertile: 7.9 (6.7–9.4) kPa], we found that plasma ferritin and hepcidin concentrations increased across LSM tertiles [median ferritin: 68.7 (interquartile range: 25.1–147) vs. 85.8 (48.3–139) vs. 111 (59.3–203) μg/L, p = 0.021; median hepcidin: 2.5 (1.1–5.2) vs. 4.4 (2.5–7.3) vs. 4.1 (1.9–6.8) nmol/L, p = 0.032]. After adjustment for age, sex, diabetes duration, waist circumference, haemoglobin A1c, HOMA-insulin resistance score, triglycerides, haemoglobin, presence of hepatic steatosis on ultrasonography and patatin-like phospholipase domain-containing-3 (PNPLA3) rs738409 genetic variant, higher plasma ferritin levels were associated with greater LSM values (adjusted-odds ratio 2.10, 95% confidence interval 1.23–3.57, p = 0.005). Higher plasma hepcidin levels were also associated with greater LSM values (adjusted-odds ratio 1.90, 95% confidence interval 1.15–3.13, p = 0.013).

Conclusions

Higher levels of plasma ferritin and hepcidin were associated with greater NAFLD-related liver fibrosis (assessed by LSM) in patients with T2DM, even after adjustment for established cardiometabolic risk factors, diabetes-related variables and other potential confounders.     

益气维血胶囊联合复方硫酸亚铁治疗妊娠期缺铁性贫血的临床研究

目的 探讨益气维血胶囊联合复方硫酸亚铁颗粒治疗妊娠期缺铁性贫血的临床疗效。方法 选取2020年4月-2022年12月邢台市第三医院收治的82例妊娠期缺铁性贫血患者，根据随机数字表法将所有患者分为对照组和治疗组，每组各41例。对照组患者温水冲服复方硫酸亚铁颗粒，1袋/次，1次/d。治疗组在对照组基础上口服益气维血胶囊，4粒/次，3次/d。两组连续治疗2个月。观察两组的临床疗效，比较两组治疗前后红细胞计数（RBC）、血红蛋白（Hb）、平均红细胞血红蛋白浓度（MCHC）、红细胞比容（HCT）、铁调素、可溶性转铁蛋白受体（sTfR）、铁蛋白（SF）水平。结果 治疗后，治疗组患者的总有效率为95.12%，对照组的总有效率为80.49%，组间比较差异显著（P<0.05）。治疗后，两组的RBC、Hb、MCHC、HCT高于治疗前（P<0.05）；治疗后，治疗组的RBC、Hb、MCHC、HCT高于对照组，差异有统计学意义（P<0.05）。治疗后，两组铁调素、SF高于治疗前，sTfR低于治疗前（P<0.05）；治疗组的铁调素、SF高于对照组，sTfR低于对照组，差异有统计学意义（P<0.05）。结论 益气维血胶囊联合复方硫酸亚铁颗粒治疗妊娠期缺铁性贫血的疗效确切，能提高RBC、Hb水平，改善铁代谢水平。     

Elevated erythroferrone distinguishes erythrocytosis with inherited defects in oxygen-sensing pathway from primary familial and congenital polycythaemia

Congenital erythrocytoses represent a heterogenous group of rare defects of erythropoiesis characterized by elevated erythrocyte mass. We performed molecular-genetic analysis of 21 Czech patients with congenital erythrocytosis and assessed the mutual link between chronic erythrocyte overproduction and iron homoeostasis. Causative mutations in erythropoietin receptor (EPOR), hypoxia-inducible factor 2 alpha (HIF2A) or Von Hippel–Lindau (VHL) genes were detected in nine patients, including a novel p.A421Cfs*4 EPOR and a homozygous intronic c.340+770T>C VHL mutation. The association and possible cooperation of five identified missense germline EPOR or Janus kinase 2 (JAK2) variants with other genetic/non-genetic factors in erythrocytosis manifestation may involve variants of Piezo-type mechanosensitive ion channel component 1 (PIEZO1) or Ten-eleven translocation 2 (TET2), but this requires further research. In two families, hepcidin levels appeared to prevent or promote phenotypic expression of the disease. No major contribution of heterozygous haemochromatosis gene (HFE) mutations to the erythrocytic phenotype or hepcidin levels was observed in our cohort. VHL- and HIF2A-mutant erythrocytosis showed increased erythroferrone and suppressed hepcidin, whereas no overproduction of erythroferrone was detected in other patients regardless of molecular defect, age or therapy. Understanding the interplay between iron metabolism and erythropoiesis in different subgroups of congenital erythrocytosis may improve current treatment options.