2.Efficacy and safety of combined oral iron chelation therapy with deferasirox and deferiprone in a patient with beta-thalassemia major and persistent iron overload.
Samin ALAVI ; Elham SADEGHI ; Azin ASHENAGAR
Blood Research 2014;49(1):72-73
No abstract available.
beta-Thalassemia*
;
Chelation Therapy*
;
Humans
;
Iron Overload*
;
Iron*
3.Effect of Desferrioxamine Therapy in Patients with Transfusional Hemosiderosis Due to Severe Aplastic Anemia.
Jung Hyun LEE ; Bin CHO ; Dae Chul JEONG ; Hack Ki KIM
Korean Journal of Pediatric Hematology-Oncology 1997;4(1):62-69
BACKGROUND: This study was carried out to evaluate the efficacy of desferrioxamine as a chelating agent in iron overloaded patients with severe aplastic anemia due to multiple transfusion. METHODS AND MATERIALS: From Oct. 1995 to Aug. 1996, 15 patients with aplastic anemia, diagnosed from May 1995 to Jan. 1996 at St. Mary's Hospital, who had a transfusional hemosiderosis were included in this study. They received 19 courses of high-dose desfer-rioxamine therapy for 6 days(20 to 30 mg/kg daily as a 24-hour intravenous infusion) . Before and after treatment, we measured serum ferritin, iron, TIBC, 24-hour urinary excretion of iron. RESULTS: 1) The range of iron load before treatment was between 4.5 and 20.0 gram. 2) Because of limit of detection(1,800 microgram/L), it was difficult to compare the changes of serum ferritin level after therapy to those of before therapy. 3) There was no significant differences between the levels of serum iron before and after therapy(214.3+/-62.8 vs 220.0+/-53.3). And there was no significant differences between TIBC before and after therapy(235.8+/-64.6 vs 259.4+/-60.1). 4) Iron/TIBC ratios were significantly deceased after desferrioxamine treatment compared to those of before therapy(0.90+/-0.04 vs 0.85+/-0.04, P<0.001) and mean urinary excretions of iron were increased by high-dose desferrioxamine compared to those by test dose(6.5+/-7.6 vs 29.1+/-14.3, P<0.001) CONCLUSION: High-dose desferrioxamine therapy is very effective for chelating and excretion of iron in iron overloaded patients with severe aplastic anemia due to multiple transfusion. A repeat administration of desferrioxamine is necessary for the iron overloaded patient to eliminate the risk of a transfusional hemosidersis.
Anemia, Aplastic*
;
Deferoxamine*
;
Ferritins
;
Hemosiderosis*
;
Humans
;
Iron
;
Iron Overload
5.Change of Hepcidin in Patients with Iron Overload at the Tibet Plateau.
Shu-Yao SUN ; Su-Yuan WANG ; Zeng-Mei SUN ; Ming-Xia LI ; Yun-Hong WU
Journal of Experimental Hematology 2020;28(4):1406-1409
OBJECTIVE:
To explore the possible etiological factors of iron overload through detecting plasma hepcidin level of adult males at Tibet plateau.
METHODS:
81 Tibetan male adult patients hospitalized in our department during January 2017 - December 2018 were selected, and divided into iron overload group and non-iron overload group. The difference in serum ferritin, serum iron, total iron binding capacity, hemoglobin, HBSAg, ALT, AST, albumin, creatinine and hepcidin of patients in each group were tested. To analyze the differences between groups. The regression analysis was applied to analyze the relationship between laboratory index and hepcidin.
RESULTS:
The plasma hepcidin of iron overload group was significantly higher than that of the non-iron overload group [93.69 (65.57-133.92) ng/ml vs 63.93 (40.01-90.65) ng/ml] (P=0.005). And there was a positive correlation between plasma hepcidin and ferritin (β=0.03 ng/ml,95%CI 0.01-0.05) (P<0.01) and BMI (β=5.71 ng/ml,95%CI 0.54-10.88) (P<0.05).
CONCLUSION
Iron overload at Tibet plateau can not be attributed to hepcidin deficiency in Tibetan adult male patients. Iron metabolism disorders in Tibetan population may be associated with metabolic syndrome.
Adult
;
Ferritins
;
Hepcidins
;
Humans
;
Iron
;
Iron Overload
;
Male
;
Tibet
6.Chelating effects of siderophore in reducing organ dysfunction caused by iron overload in ICR Mice
Thucydides L. Salunga ; Isabella R. Panelo ; Joel C. Cornista
Philippine Journal of Health Research and Development 2019;23(4):47-57
Background and Objectives:
Iron is an essential element that plays a vital role in a wide variety of cellular
processes. But when present in excess concentration in organs, it may increase the risk for liver disease, heart
failure, and diabetes. Recently, siderophores, which are iron-chelating agents produced by microorganisms,
have attracted tremendous attention because of their strong binding and high selectivity to the ferric form of
iron. Thus, the use of siderophore in sequestering excess iron in the body as a form of therapy is very attractive.
This study determined the effects of commercially available siderophore in sequestering excess iron in organs
such as liver, heart, and pancreas under excess iron conditions.
Methodology:
First, iron-overload was induced by injecting iron dextran (20 mg) into male ICR mice for three
consecutive days. The effects of iron to the liver, heart, and pancreas and the possible sequestration by
siderophore were determined by scoring histological sections. The liver iron concentration was also assessed
by atomic absorption spectroscopy (AAS).
Results and Conclusion
The study showed that iron-overloaded mice exhibited skin hyperpigmentation and
hemosiderosis in liver, heart, and pancreas. Significant changes in the liver include hepatomegaly and
development of tumor. Iron-overloaded mice had 2,935% increase in liver iron content compared to the salinetreated mice. However, when iron-overloaded mice were treated with either 100 µg or 200 µg siderophore,
there was a 77% and 84% decrease in liver iron content, respectively. Moreover, the treatment of ironoverloaded mice with siderophore prevented the development of hemosiderosis, tumor, and structural
changes in the tissues studied. The results showed that siderophore can effectively reduce excess iron and
organ damage in iron-overloaded mice and can be potentially employed in chelation therapy of iron-overload
diseases. Further studies on the possible mechanisms of siderophore aside from decreasing iron excess and
lowering organ dysfunction are recommended.
Siderophores
;
Iron Overload
;
Iron Chelating Agents
;
Hemosiderosis
;
Hepatomegaly
7.Effect of GDF15 on iron overloading and erythropoiesis.
You-Shan ZHAO ; Chun-Kang CHANG
Journal of Experimental Hematology 2011;19(2):537-541
Ineffective erythropoiesis is recognized as the principal reason of non-transfusional iron overload. In the process of expanded erythropoiesis, the apoptosis of erythroblasts induces the up-regulation of GDF15. GDF15 suppresses hepcidin production by the hepatocytes. Subsequently, low hepcidin levels increase iron absorption from the intestine resulting in iron overload. Physiological dose of GDF15 can promote the growth and differentiation of erythroid progenitors, but the high dose of GDF15 can suppress the secretion of hepcidin. The regulation of GDF15 may also be related to iron levels, epigenetic regulation and hypoxia. In this article the GDF15 and its expression and distribution, roles of GDF15 in erythropoiesis and iron overload, as well as the regulation factors of GDF15 are reviewed.
Erythropoiesis
;
Growth Differentiation Factor 15
;
metabolism
;
Humans
;
Iron Overload
8.Hepatic iron overload in hemochromatosis: a case report.
Gen-dong YANG ; Pu-xuan LU ; Bo-ping ZHOU ; Ru-xin YE ; Jian ZANG ; Jian SU
Chinese Journal of Hepatology 2006;14(8):634-634
Hemochromatosis
;
metabolism
;
Humans
;
Iron Overload
;
Liver
;
metabolism
;
Male
;
Middle Aged
9.The Clinical Guidelines for Myelodysplastic Syndrome.
June Won CHEONG ; Hoon KOOK ; Soo Mee BANG ; Je Hwan LEE ; Yong Don JOO ; Inho KIM ; Hyeoung Joon KIM ; Chan Jeoung PARK ; Hyeon Jin PARK ; Jin Seok AHN ; Sung Soo YOON ; Jong Ho WON ; Mark Hong LEE ; Chul Won JUNG ; Deog Yeon JO ; Bin CHO ; Kyoung Ja HAN ; Yoo Hong MIN ; Sun Hee KIM
Korean Journal of Hematology 2007;42(2):71-90
The myelodysplastic syndromes (MDS) are characterized by ineffective hematopoiesis associated with multilineage cytopenias leading to serious morbidity or mortality, and the additional risk of leukemic transformation. The management of patients with MDS can be very complex and varies according to both the clinical manifestations in individual patients as well as the presence of complicating medical conditions. However, therapeutic dilemmas still exist for MDS due to the multifactorial pathogenetic features of the disease, its heterogeneous stages, and the elderly patient population. For these reasons, proper guidelines for management are necessary. This review describes the proper diagnosis for MDS, decision-making approaches for optimal therapeutic options that are based on a consideration of patient clinical factors and risk-based prognostic categories, and the use of recently available biospecific drugs such as hypomethylating agents that are potentially capable of abrogating the abnormalities associated with MDS. Proper indications and methods for transplantation, response criteria, management for iron overload for highly transfused patients and specific considerations for MDS in childhood are also described. All of these topics were discussed at the third symposium of AML/MDS working party on 3 March, 2007.
Aged
;
Diagnosis
;
Hematopoiesis
;
Humans
;
Iron Overload
;
Mortality
;
Myelodysplastic Syndromes*
;
Transplantation
10.Luteoloside protects the vascular endothelium against iron overload injury via the ROS/ADMA/DDAH II/eNOS/NO pathway.
Shu-Ping CHEN ; Tian-Hong HU ; Qing ZHOU ; Tian-Peng CHEN ; Dong YIN ; Huan HE ; Qing HUANG ; Ming HE
Chinese Journal of Natural Medicines (English Ed.) 2022;20(1):22-32
Iron overload injury is considered to be a part of blood stasis syndrome of arthralgia in traditional Chinese medicine. Its primary therapies include clearing heat and detoxification, activating blood circulation, and removing blood stasis. Lonicera japonica flos (LJF) has long been known as an excellent antipyretic and antidote. Luteoloside (Lut) is one of the main components of LJF and exhibits antioxidant, anti-inflammatory, and cytoprotective properties. However, the protection of Lut against iron overload injury and its underlying mechanisms remain unclear. Therefore, HUVECs were exposed to 50 μmol·L-1 iron dextran for 48 h to establish an iron overload damage model and the effects of Lut were assessed. Our results showed that 20 μmol·L-1 Lut not only increased cell viability and weakened LDH activity, but also significantly up-regulated DDAHⅡ expression and activity, increased p-eNOS/eNOS ratio and NO content, and reduced ADMA content in HUVECs exposed to iron overload. Furthermore, Lut significantly attenuated intracellular/mitochondrial ROS generation, improved SOD, CAT, and GSH-Px activities, reduced MDA content, maintained MMP, inhibited mPTP opening, prevented cyt c from mitochondria released into cytoplasm, reduced cleaved-caspase3 expression, and ultimately decreased cell apoptosis induced by iron overload. The effects of Lut were similar to those of L-arginine (an ADMA competitive substrate), cyclosporin A (a mPTP blocker agent), and edaravone (a free radical scavenger) as positive controls. However, addition of pAD/DDAH II-shRNA adenovirus reversed the above beneficial effects of Lut. In conclusion, Lut can protect HUVECs against iron overload injury via the ROS/ADMA/DDAH II/eNOS/NO pathway. The mitochondria are the target organelles of Lut's protective effects.
Endothelium, Vascular
;
Glucosides
;
Humans
;
Iron Overload
;
Luteolin
;
Reactive Oxygen Species