Is Iron Overload Associated with Worse Outcomes in Patients with Chronic Liver Disease Undergoing Liver Transplantation?

ABSTRACT Background: Iron overload is frequent in patients with chronic liver disease, associated with shorter survival after liver transplantation in patients with hereditary hemochromatosis. Its effect on patients without hereditary hemochromatosis is unclear. The aim of the study was to study the clinical impact of iron overload in patients who underwent liver transplantation at an academic tertiary referral center. Methods: We performed a retrospective cohort study including all patients without hereditary hemochromatosis who underwent liver transplantation from 2015 to 2017 at an academic tertiary referral center in Mexico City. Explant liver biopsies were reprocessed to obtain the histochemical hepatic iron index, considering a score ≥ 0.15 as iron overload. Baseline characteristics were compared between patients with and without iron overload. Survival was estimated using the Kaplan-Meier method, compared with the log-rank test and the Cox proportional hazards model. Results: Of 105 patients included, 45% had iron overload. Viral and metabolic etiologies, alcohol consumption, and obesity were more frequent in patients with iron overload than in those without iron overload (43% vs. 21%, 32% vs. 22%, p = 0.011; 34% vs. 9%, p = 0.001; and 32% vs. 12%, p = 0.013, respectively). Eight patients died within 90 days after liver transplantation (one with iron overload). Complication rate was higher in patients with iron overload versus those without iron overload (223 vs. 93 events/100 person-months; median time to any complication of 2 vs. 3 days, p = 0.043), without differences in complication type. Fatality rate was lower in patients with iron overload versus those without iron overload (0.7 vs. 4.5 deaths/100 person-months, p = 0.055). Conclusion: Detecting iron overload might identify patients at risk of early complications after liver transplantation. Further studies are required to understand the role of iron overload in survival.

Research progress on the role of ferroptosis in the pathogenesis of non-alcoholic fatty liver disease / 基础医学与临床

Ferroptosis is a new type of cell death proposed in recent years,and its main characteristics are iron overload and lipid peroxidation.Ferroptosis is involved in the occurrence and development of non-alcoholic fatty liv-er disease(NAFLD).Iron overload can generate a large amount of reactive oxygen species through the Fenton reac-tion.Under the action of lipoxygenase,the unsaturated fatty acids on the liver cell membrane undergo lipid peroxi-dation,which induces liver cell death and leads to the occurrence of non-alcoholic fatty liver disease/non-alcoholic steatohepatitis.Blocking ferroptosis may provide one of the therapeutic strategies to protect liver cells.

Iron overload regulates atherosclerotic activity of foam cells induced by oxLDL / 实用医学杂志

Objective To explore the roles of iron overload in pro-atherogenic activation of foam cells.Methods RAW264.7 and MOVAS cells were stimulated by oxLDL,ferrimine citrate and deferoxamine respectively.Prussian Blue and Oil Red O staining were used to detect iron deposition and foam cell.CCK-8 test,DHE probe,ELISA,RT-qPCR were performed to detect the cell death rate,reactive oxygen species(ROS)generation,lipid peroxidation molecules[glutathione peroxidase(GSH),glutathione peroxidase 4(GPX4),malondialdehyde(MDA)content]and the mRNA level of ATP binding cassette transporter A1(ABCA1),ATP binding cassette transporter G1(ABCG1),inductible nitris oxide synthase(iNOS),arginase-1(Arg-1),α-smooth muscle actin(α-SMA),smooth muscle 22 alpha(SM22a),osteopontin(OPN),Interleukin-1β(IL-1β),tumor necrosis factor-α(TNF-α).Results Iron overload could reduced reverse cholesterol transporters(ABCA1 and ABCG1),promote foam cells generation,increased cell death rate,induced the expression of lipid peroxidation molecules(GSH,GPX4,MDA),and promoted pro-inflammatory M1 marker of macrophage and synthetic marker expression of vascular smooth muscle cell(VSMC)and inflammatory cytokines(IL-1β,TNF-α).Conclusion Iron overload promotes the generation of foam cells derived from macrophages and smooth muscle cells and transform them into pro-atherosclerotic phenotype,aggravates cell lipid peroxidation and inflammatory reaction,which contributes to the progress of atherosclerosis.

An insight into the mechanism of iron overload in knee osteoarthritis under the theory of blood stasis / 中国组织工程研究

BACKGROUND:Knee osteoarthritis is a common disease in middle-aged and elderly people.It is a kind of disease that seriously affects the quality of life of patients and even has the risk of disability.Therefore,the pathogenesis and treatment of knee osteoarthritis have become the focus of research.In Chinese medicine,knee osteoarthritis is often treated as"biness,"which is closely related to"biness"caused by blood stasis and blood vessels blocking collaterals in the theory of"blood stasis"in traditional Chinese medicine.Iron overload is a kind of pathological state caused by iron metabolism disorder,which highly coincides with the pathogenic characteristics and clinical manifestations of the"blood stasis"theory of traditional Chinese medicine,and is a risk factor that promotes the development of knee osteoarthritis. OBJECTIVE:Based on the"blood stasis"theory,to summarize the effects of iron overload on cartilage metabolism and subchondral bone reconstruction,to lay a new theoretical foundation for the treatment of knee osteoarthritis with traditional Chinese medicine,and to explore the therapeutic effect of traditional Chinese medicine for promoting blood circulation after interfering with bone tissue. METHODS:CNKI,WanFang database,PubMed and Web of Science databases were searched for relevant literature.The Chinese search terms were"ferroptosis,iron,iron overload,osteoarthritis,blood stasis"and the English search terms were"ferroptosis,iron,iron overload,osteoarthritis,TCM."In the end,76 articles were included for further review. RESULTS AND CONCLUSION:First of all,we explored the potential of the"blood stasis"theory in treating knee osteoarthritis,and found that"blood stasis"is a crucial part in the progress of knee osteoarthritis,indicating that the"blood stasis"theory is the key to the treatment of knee osteoarthritis in traditional Chinese medicine.Secondly,"blood stasis"and iron overload have a high degree of similarity in pathogenic factors,clinical manifestations,and pathogenic characteristics,suggesting the possibility of"blood stasis"theory in treating iron overload.This finding reminds us that iron overload may be an important mechanistic basis for the"blood stasis"theory in the treatment of knee osteoarthritis.The extracts of blood-activating drugs can relieve iron overload and treat knee osteoarthritis,but the specific mechanism is still unclear.Therefore,we believe that the relationship between"blood stasis"theory and iron overload and related mechanisms are important research directions for knee osteoarthritis in the future.The related mechanism of"blood stasis"theory to alleviate iron overload and then treat knee osteoarthritis also provides a theoretical basis for the modernization of traditional Chinese medicine,such as the development of new drugs and innovative usage,and has certain guiding significance for clinical practice.

Application of deferoxamine in bone tissue regeneration / 中国组织工程研究

BACKGROUND:Aside from iron chelating,deferoxamine is also considered as an effective hypoxia mimetic agent and hypoxia inducible factor-1α stabilizer.Deferoxamine has played a favorable effect on bone regeneration in both basic and clinical research recently.Deferoxamine solutions or deferoxamine loaded bio-scaffolds have been locally applied in bone tissue engineering,and their promotion of bone repair involves various functional properties and molecular mechanisms which have not been entirely clarified.Moreover,their advances in research of bone regeneration lack comprehensive summary as well. OBJECTIVE:To review the functional properties,relative merits and advances in basic research and clinical practice of deferoxamine applied in bone regeneration,attempting to provide references and strategies for further studies. METHODS:Relevant articles were searched with the key words of"deferoxamine OR desferrioxamine OR desferal OR DFO,""bone tissue engineering OR bone regeneration OR bone remodeling OR bone repair OR bone healing OR osteogenesis,""angiogenesis OR vascularized bone regeneration OR angiogenic-osteogenic coupling"in English and Chinese by using PubMed,WanFang and CNKI databases.Eventually,88 articles were selected for review. RESULTS AND CONCLUSION:Deferoxamine can recruit stem cells and regulate their function,activate relevant signaling pathways to advance hypoxia adaptation of the cells,exert anti-inflammatory and antioxidant properties to improve local inflammatory environment,and promote bone regeneration by coupling osteogenesis and angiogenesis as well as inhibiting bone resorption.Compared with growth factors or peptides loaded in conventional bone tissue engineering,deferoxamine has its unique advantages as a small molecule drug,while it also has toxic reactions and application limitations.Therefore,it is necessary to optimize its loading form and dosagey.The unique angiogenic-osteogenic coupling ability of deferoxamine can be used in different types of bone injuries including fractures,osteonecrosis,distraction osteogenesis,bone grafting,oral related osteogenesis,and bone defects.Due to the enhancement of angiogenesis,this ability enables deferoxamine to better adapt and solve the difficulties in bone repair caused by the complex and variable clinical situations and individual differences.However,it is also necessary to compare and optimize the application methods and safe dosage of deferoxamine to expand its application scope and enhance its clinical value.

Shear wave elastography for evaluating hepatic iron overload in children with β-thalassemia major:Correlations with MR T2* value and serum ferritin / 中国医学影像技术

Objective To observe the value of shear wave elastography(SWE)for evaluating hepatic iron overload in children with β-thalassemia major(β-TM),as well as the correlations of relative parameters with MR T2*value and serum ferritin.Methods Totally 96 children with β-TM and 100 healthy children(control group)were retrospectively enrolled.Children with β-TM were divided into hematopoietic stem cell transplantation(HSCT)group(n=41)or non-HSCT group(n=55)according to underwent HSCT or not.SWE parameters were compared among groups.Spearman correlation was performed to observe the correlations of liver shear wave velocity with MR T2*value and serum ferritin,as well as Young's modulus with MR T2*value and serum ferritin in children with β-TM.Results Liver shear wave velocity(LSWV)and Young's modulus in HSCT group and non-HSCT group were all higher than those in control group(all P＜0.001).No significant difference of LSWV nor Young's modulus was found between HSCT group and non-HSCT group(both P＞0.05).SWE parameters of children with β-TM were moderately and negatively correlated with MR T2*value(r=-0.501,P＜0.05;r=-0.514,P＜0.05),while weakly and positively correlated with serum ferritin(r=0.488,P＜0.05;r=0.470,P＜0.05).Conclusion SWE was helpful for evaluating hepatic iron overload in children with β-TM,with parameters being negatively correlated with MR T2*value and positively correlated with serum ferritin.

Clinico-Hematological Profile and Management of Children With Non-Transfusion Dependent Thalassemia (NTDT) at a Pediatric Center in Northern India

Objective: To study the clinico-hematological profile, complications, and management of children with non-transfusion dependent thalassemia (NTDT) in northern India. Method: We retrieved and analyzed the data of 69 children with NTDT diagnosed between January, 2006 to December, 2018, aged under 18 years from our unit’s records. Result: The participants mean (SD) age was 4.4 (3.1) years, and they presented with anemia (29%), jaundice (13%), hemolytic facies (13%), splenomegaly (87%), thromboembolism (2.9%) and pathological short stature (28.5%). The most common cause of NTDT was ?-thalassemia (45%), followed by either compound-heterozygous or homozygous for E?-thalassemia mutation. The most frequent single genotype observed was compound heterozygous for IVS1-5 (G>C) and codon 26 (G>A). The mean (SD) follow-up duration was 3.5 (2.4) years. On follow-up, 27 children (%) remained transfusion free, and 30 (%) needed occasional transfusions. 63% of patients initially presenting with pathological short stature showed improvement in growth. Amongst children older than 10 years (n=20), subclinical hypothyroidism was detected in 6 children and impaired glucose tolerance test in 1 child. Conclusion: Eß-thalassemia was the commonest cause of NTDT in this population.

Trasplante cardíaco por hemocromatosis. Caso pediátrico / Cardiac transplantation due to hemochromatosis. A pediatric case report

La hemocromatosis es una enfermedad caracterizada por el excesivo depósito de hierro en múltiples órganos, entre ellos hígado, páncreas, piel y corazón. La infiltración de este último es un importante factor en morbilidad y mortalidad. Presentamos un caso de un paciente pediátrico con insuficiencia cardíaca terminal que ameritó trasplante cardíaco, que resultó sin complicaciones. Posterior a la cirugía, mostró mejoría bioquímica y clínica, lo que influyó positivamente en su calidad de vida y prolongó su supervivencia.

Hemochromatosis is a disease characterized by excess iron stores in multiple organs, including the liver, pancreas, skin, and heart. The infiltration of the heart is an important factor in morbidity and mortality. Here we describe the case of a pediatric patient with end-stage heart failure who required a heart transplantation, with no complications. After the surgery, she showed biochemical and clinical improvement, with a positive impact on her quality of life and a prolonged survival.

Iron profile of pregnant sickle cell anemia patients in Odisha, India

ABSTRACT Introduction: During pregnancy, the iron requirement increases to meet the optimal growth of the fetus and prevent iron deficiency anemia-related complications in the mother. However, in sickle cell disease (SCD) primarily due to repeated blood transfusions and hemolysis-induced recycling of iron, its supplementation during pregnancy remains questionable and may be harmful. Methods: Twenty-five pregnant women with homozygous SCD and 25 pregnant women with normal hemoglobin variants were included as cases and control, respectively. Pregnancy and sickle cell anemia (SCA) were diagnosed using standard protocols. The serum iron, serum ferritin, total iron-binding capacity (TIBC), percentage transferrin saturation and C-reactive protein were estimated, as per the manufacturer's protocol. The complete blood count was performed. The unpaired 't-test' was performed using the SPSS v23.0 and the principal component analysis (PCA) was performed using the online software MetaboAnalyst for statistical analysis. Main Results: The studied cases had significantly lower mean hemoglobin and higher mean corpuscular volume (MCV), compared to controls. The mean serum-iron, serum-ferritin and percentage transferrin-saturation in the cases were significantly higher than that of the controls, while the TIBC was lower in the cases (p < 0.0001). The mean level of serum iron, ferritin, percentage transferrin saturation and TIBC were 309.44 ± 122.40mcg/dl, 860.36 ± 624.64ng/ml, 42.6 ± 17.30% and 241.32 ± 96.30 mcg/dl, respectively, in the cases and 95.36 ± 41.90mcg/dl, 122.28 ± 49.70ng/ml, 15.83 ± 3.10% and 492.6 ± 149.40mcg/dl in the controls, respectively. Higher MCV, mean corpuscular hemoglobin (MCH) and mean corpuscular hemoglobin concentration (MCHC) with lower hemoglobin (Hb) were noted in the cases. The PCA revealed that the cases were more heterogeneous in terms of the variability of the iron status and hematological indices than the controls. Conclusion: The current study shows iron sufficiency in most cases of pregnancy with SCA and suggests that evaluation of iron status must be made before initiating iron prophylaxis in pregnant women with SCA, especially in regions having a high prevalence of sickle cell hemoglobinopathy.

Cardiac iron overload evaluation in thalassaemic patients using T2* magnetic resonance imaging following chelation therapy: a multicentre cross-sectional study

Abstract Introduction Magnetic resonance imaging (MRI) T2* technique is used to assess iron overload in the heart, liver and pancreas of thalassaemic patients. Optimal iron chelation and expected tissue iron response rates remain under investigation. The objective of this study was to analyse serum ferritin and the iron concentration in the heart, liver and pancreas measured by MRI T2*/R2* during regular chelation therapy in a real-world cohort of patients with thalassemia. Methods We evaluated thalassaemic patients ≥ 7 years old undergoing chelation/transfusion therapy by MRI and assessed serum ferritin at baseline and follow-up from 2004-2011. Results We evaluated 136 patients, 92% major thalassaemic, with a median age of 18 years, and median baseline ferritin 2.033ng/ml (range: 59-14,123). Iron overload distribution was: liver (99%), pancreas (74%) and heart (36%). After a median of 1.2 years of follow-up, the iron overload in the myocardium reduced from 2,63 Fe mg/g to 2,05 (p 0.003). The optimal R2* pancreas cut-off was 148 Hertz, achieving 78% sensitivity and 73% specificity. However, when combining the R2* pancreas cut off ≤ 50 Hertz and a ferritin ≤ 1222 ng/ml, we could reach a negative predictive value (NPV) of 98% for cardiac siderosis. Only 28% were undergoing combined chelation at baseline assessment, which increased up to 50% on follow up evaluation. Conclusions Chelation therapy significantly reduced cardiac siderosis in thalassaemic patients. In patients with moderate/severe liver iron concentration undergoing chelation therapy, ferritin levels and myocardium iron improved earlier than the liver siderosis.

Recognition of Iron Overload Cardiomyopathy / 罕见病研究

Iron overload cardiomyopathy (IOC) is a type of cardiac dysfunction caused by several factors resulting in iron overload in the myocardium. Two major causes of IOC include hereditary hemochromatosis and transfusion-dependent anemia. IOC significantly reduces long-term survival of patients. Since IOC is a rare disease in Asian populations that also lacks etiology-specific manifestations, early diagnoses in clinical practice are challenging. Two groups of patients with high risk of IOC should be further investigated: those who present heart failure of unknown origin will be screened for iron overload followed by confirmation of IOC; and those who have high risk of iron overload or an established diagnosis will be monitored for the development of IOC. Serum ferritin is recommended as the first-line screening test for iron overload, while cardiac magnetic resonance T2* should be used to confirm iron overload in the myocardium. Phlebotomy and iron chelating agents can effectively remove the extra iron from the body, preventing IOC, as well as reverse the disease at an early stage and slow down its progession. Timely diagnosis and treatment is critical in improving the prognosis of patients with IOC. Therefore, this review aims to help clinicians to understand IOC in multiple dimensions including pathogenesis, clinical manifestations, diagnostic methods and treatment choices.

Discussion on scientific connotation of "cold coagulation and blood stasis" for endometriosis pathogenesis based on iron overload / 国际中医中药杂志

This article analyzed the specific path of iron overload affecting EMT "blood countercurrent cell survival/adhesion/invasion/angiogenesis ectopic lesions" from the three aspects of mediating NF-κB and inflammatory reaction, stimulation of abnormal activation of macrophages, and induction of oxidative stress injury; introduced the theoretical connotation of the pathogenesis of "cold coagulation and blood stasis", and briefly described the progress of the pathogenesis of EMT from the perspective of "blood out of menstruation - injury caused by cold pathogenic factors - cold coagulation and blood stasis - long-term accumulation syndrome"; based on the mutual confirmation of "menstruation countercurrent - iron overload microenvironment - cell survival/adhesion/invasion/angiogenesis - ectopic focus" and "blood out of menstruation - cold pathogen injury - cold coagulation and blood stasis - chronic accumulation syndrome", it was believed that starting from iron overload could tap the scientific connotation and microscopic materials of the pathogenesis of "cold coagulation and blood stasis" in EMT, so as to provide new directions and theoretical references for the research of the pathogenesis of EMT and the mechanism of drugs.

Research progress of ferroptosis involved in the pathogenesis of diabetic retinopathy / 中华眼底病杂志

Diabetic retinopathy (DR) constitutes a major retinal vascular disorder leading to blindness in adults. Current therapeutic approaches for DR exhibit certain degrees of efficacy but are constrained by a spectrum of limitations. Hence, there is a pressing need to deeply investigate the underlying pathogenesis of DR and explore novel therapeutic targets. Ferroptosis, a distinctive form of programmed cell death, has emerged as a pertinent phenomenon in recent years. Notably, ferroptosis has been implicated in the progression of DR through mechanisms involving the induction of retinal oxidative stress, provocation of anomalous retinal vascular alterations, exacerbation of retinal neural damage, and elicitation of immune dysregulation. Thus, elucidating the mechanistic role of ferroptosis in DR holds the potential to establish a robust foundational rationale. This could potentially facilitate the clinical translation of ferroptosis inhibitors as promising agents for the prevention and treatment of DR, thereby forging novel avenues in the landscape of DR management.

Research progress on the role and mechanism of ferroptosis in renal fibrosis / 中华肾脏病杂志

Ferroptosis is a recently identified type of non-apoptotic cell death, mainly caused by disruption of cellular metabolic pathways such as iron metabolism and reactive oxygen species metabolism, characterized by intracellular iron overload and reactive oxygen species accumulation leading to lipid peroxidation. Ferroptosis is closely related to renal diseases. The role of ferroptosis in diseases such as acute kidney injury and renal cell carcinoma has been extensively studied, and new discoveries and advances have been made in its relationship with renal fibrosis. The paper systematically reviews the relationship between ferroptosis and renal fibrosis in terms of the latest regulatory mechanisms of ferroptosis and its role in renal fibrosis, and explores the potential clinical application of targeted inhibition of ferroptosis to prevent renal fibrosis.

Hiperferritinemia y sospecha de sobrecarga de hierro en pacientes del Hospital Pablo Tobón Uribe, entre los años 2010 a 2020 / Hyperferritinemia and suspected iron overload in patients at the Pablo Tobón Uribe Hospital, between 2010 and 2020

Introducción. El objetivo de esta investigación fue comparar el perfil bioquímico y clínico de los pacientes con hiperferritinemia secundaria a hemocromatosis hereditaria (HH), frente a aquellos con hiperferritinemia por causas sospechosas de sobrecarga de hierro (Fe) diferentes a la HH. Metodología. Se estudiaron 92 pacientes (61 hombres y 31 mujeres), remitidos tras la detección de valores de ferritina >300 µg/L en hombres y >200 µg/L en mujeres. En todos se analizaron datos demográficos generales, comorbilidades, motivo de remisión para estudios de hiperferritinemia, manifestaciones clínicas, antecedente familiar de HH y tratamiento reci-bido. Los resultados de las pruebas de laboratorio, imagenología, hallazgos histopatológicos y estudios genéticos, se describieron según la disponibilidad. Resultados. El 96,74 % de los pacientes fueron evaluados en consulta externa, 86,96 % procedían de Medellín o de otros municipios de Antioquia, Colombia. La edad promedio de los participantes fue de 52 años, la principal razón para ser derivados para estudios fue la elevación de los marcadores de Fe sérico, la causa más frecuente de hiperferritinemia fueron los diagnósticos diferentes a la HH (64,13 %) y entre quienes no tenían HH, la etiología metabólica fue la más común (59,32 %). Los pacientes con HH tuvieron niveles más elevados de ferritina y Fe sérico, mientras que en el grupo sin HH se presentaron mayores elevaciones en la saturación de transferrina, transfe-rrina y transaminasas. En pacientes con sobrecarga de Fe, la mutación más frecuentemente encontrada fue la homocigota H63D (36,67 %). Finalmente, 93,94 % de los pacientes con HH recibieron tratamiento con flebotomías, mientras que los cambios en el estilo de vida fueron indicados en el 55,93 % de los pacientes sin HH. Conclusiones. La hiperferritinemia es una presentación clínica frecuente y es importante hacer un abordaje sistemático para identificar sus causas. Aunque la HH es una causa importante de elevación persistente de ferritina, en el enfoque de los pacientes con esta condición, se deben descartar etiologías más frecuentes como la hiperferritinemia de etiología metabólica.

Introduction. The aim of this investigation was to compare the biochemical and clinical profile of patients with secondary hyperferritinemia caused by hereditary hemochromatosis (HH), versus those with hyperferritinemia due to suspected causes of iron (Fe) overload other than HH. Methodology. A total of 92 patients (61 men and 31 women) referred after the detection of ferritin values >300 µg/L in men and >200 µg/L in women were studied. General demographic data, comorbidities, referral reasons for hyperferritinemia studies, clinical manifestations, family history of HH, and treatment received were analyzed in all patients. The results of laboratory tests, medical imaging, histopatho-logical findings, and genetic studies were described based on availability. Results. Of all patients, 96.74% were evaluated as outpatients, 86,96% from the municipality of Medellin in Antioquia, Colombia. The average age of the participants was 52 years, the main reason for being referred for studies was the elevation of serum Fe markers, the most frequent cause of hyperferritinemia in the population studied were conditions other than HH (64.13%), and among those who did not have HH, the metabolic etiology was the most common cause (60%). Patients with HH had higher levels of ferritin and serum Fe, while in the group without HH there were greater elevations of transferrin saturation, transferrin and transaminases. In patients with iron overload, the most frequently found mutation was the homozygous H63D (36.67%). Finally, 93.94% of the patients with HH received phlebotomy treatment, while changes in lifestyle were indicated in 55.93% of patients without HH. Conclusions. Hyperferritinemia is a frequent clinical presentation and it is important to make a systematic approach to identify its causes. Although HH is an important cause of persistent ferritin elevation, in the approach to patients with this condition, more frequent etiologies such as hyperfe-rritinemia of metabolic etiology should be ruled out.

Correlation of Transient Elastography With MRI T2* and Serum Ferritin Levels in Children With Transfusion-Dependent Thalassemia

Objectives: We investigated the correlation of transient elastography (TE) with MRI R2* values and serum ferritin in patients with transfusion-dependent thalassemia (TDT) Methods: We reviewed hospital records of 59 patients with TDT aged ?8 years without any evidence of chronic liver disease and who had fibroscan within 3 months of MRI T2*, who seen at our center between January, 2014 and December, 2019. Spearman correlation and linear regression analysis were used to evaluate the correlation between TE liver stiffness measurements and R2* MRI values and with serum ferritin. Results: Mean (SD) age of the subjects was 13.0 (3.1) years and body mass index was 16.6 (2.3) kg/m2. Mean liver stiffness measurement, MRI T2*(3T), corresponding MRI R2*(3T), and ferritin values were 6.55 (3.10) kPa, 3.4 (4.6) milliseconds, 616.20 (383.9) Hz, and 2874.69 (1570.7) ng/ mL, respectively. TE measurements correlated with MRI R2* values (r=0.61; P=0.001) and with serum ferritin level (r=0.59, P=0.001). Conclusion: TE is a reliable tool to estimate hepatic iron overload in patients with TDT.

Mucormycosis and Its Co-Relation with Raised Ferritin Levels

Background: Mucormycosis has emerged as an epidemic within the COVID-19 pandemic due to widespread use of corticosteroids and immune-modulators like Tocilizumab, in the management of COVID-19 pneumonia. It is an invasive fungal disease which spreads by angioinvasion and rapidly spreads to adjacent tissues. If untreated its outcomes are dangerous and often fatal. Uncontrolled diabetes, malignancies and dialysis are predisposing factors. Raised blood iron is an important factor in pathogenesis and rapid progression of fungal invasion that needs to be investigated. Aim: To establish a correlation between raised serum ferritin level and aggressiveness of Mucormycosis. Methodology: Aretrospective study was done from February 2021 to February 2022 of patients diagnosed with mucormycosis by middle meatal biopsy and microscopy along with CT& MRI scan of PNS with brain. They were treated either surgically or conservatively. All the blood parameters including serum ferritin level, were carried out. Acomparison was done on extent of disease in patients in-relation to their serum ferritin level. Results: Our study suggested that higher levels of ferritin are often associated with aggressive form the disease. Zygomycetes are dependent on environmental iron for their growth. Higher the serum ferritin level, more aggressive & widespread is mucormycosis. Conclusion: Serum free iron aggravates mucormycosis. Measures should take to control the serum ferritin in patients under risk for mucormycosis. Iron chelating agents or novel methods like anti-ftr1 immune serum should be developed for controlling the disease at its earlier stages

A rare case of bronze diabetes in a patient with thalassemia

Secondary haemochromatosis (also known as bronze diabetes) is a perilous medical condition that can occur as a complication of frequent blood transfusions. Thalassemia major which occurs due to a decrease in the beta globulin chain can lead to severe anemia, extramedullary hematopoiesis and splenomegaly. Becauseof this, the affected patients requiredcontinuous blood transfusions throughout their life and as a consequence, it may lead to iron overload. A 26-year-old male presented with a complaint of darkening skin, joint pain and fever. He was a known case of thalassemia major and was undergoing blood transfusions three times a week. Further laboratory findings revealed decreased hemoglobin, abnormal liver function tests and increased blood glucose levels. The patient was managed with IV insulin and chelation therapy. The patient responded to treatment and was better on subsequent follow-up. The diagnostic and therapeutic challenges along with the epidemiological dataemphasize the need of raising the awareness of physicians to this devastating condition.

HFE hemochromatosis: an overview about therapeutic recommendations

Abstract Hemochromatosis is currently characterized by the iron overload caused by hepcidin deficiency. Large advances in the knowledge on the hemochromatosis pathophysiology have occurred due to a better understanding of the protein of the iron metabolism, the genetic basis of hemochromatosis and of other iron overload diseases or conditions which can lead to this phenotype. In the present review, the main aims are to show updates on hemochromatosis and to report a practical set of therapeutic recommendations for the human factors engineering protein (HFE) hemochromatosis for the p.Cys282Tyr (C282Y/C282Y) homozygous genotype, elaborated by the Haemochromatosis International Taskforce.

Deferasirox for the treatment of iron overload in thalassemia ：a rapid health technology assessment / 中国药房

OBJECTIVE To eval uate the effectiveness ，safety and economy of deferasir ox for the treatment of iron overload in thalassemia with rapid health technology assessment ，and to provide evidence-based basis for rational clinical use. METHODS Retrieved from Chinese and English database/website as PubMed ，Embase，Cochrane Library ，NHS EED ，CADTH，CNKI and Wanfang database ，health technology assessment （HTA），systematic evaluation/meta-analysis and pharmacological studies about deferasirox versus deferoxamine/deferiprone for the treatment of iron overload in thalassemia were collected from the inception to June 2021. Based on literature screening and data extraction ，the quality of literature about HTA reports ，systematic evaluation/ Meta-analysis and pharmacoeconomic research were evaluated with HTA checklist ，A Measurement Tool to As sess Systematic Reviews，standard scale of economic evaluation report. The effectiveness and safety results were described quantitatively ，and the economic evaluation results were described qualitatively. RESULTS One HTA report ，five systematic evaluation/meta-analysis and five pharmacoeconomic studies were selected from 1 569 literature. Included HTA reports ， systematic evaluation/meta-analysis，pharmacoeconomic studies were high in quality. Most studies reported that 30 mg/（kg·d） deferasirox was E-mail：aydgs@126.com better than deferoxamine in reducing the levels of s erum ferritin and liver iron overload ；ADR induced by deferasirox were mainly gastrointestinal irritation symptoms ，skin itching ，joint pain，transaminase elevation ，etc.，which generally did not affect subsequent treatment. There was no statistical significance in severe ADR between deferoxamine group and deferasirox group [RR ＝0.96，95％CI（0.85，1.08），P＝0.52]. Compared with deferoxamine，deferasirox had higher cost-effectiveness ；but deferasirox was less likely to be cost-effective than deferiprone. CONCLUSIONS Deferasirox has good effectiveness and safety for iron overload in thalassemia ，and has good economic advantages in Britain and Iran ，compared with deferoxamine.