scholarly journals Heart Failure Due to Secondary Haemochromatosis in a Case of Beta Thalassemia Major: A Rare Case Presentation

2021 ◽  
pp. 101-107
Author(s):  
V. V. S. S. Sagar ◽  
Maharshi Patel ◽  
Pallavi Yelne ◽  
Shilpa Gaidhane ◽  
Chitturi Venkata Sai Akhil
Keyword(s):  
Heart Failure ◽  
Cardiac Dysfunction ◽  
Rare Case ◽  
Cardiac Involvement ◽  
Thalassemia Major ◽  
The Body ◽  
Beta Thalassemia ◽  
Case Presentation ◽  
Abnormal Accumulation ◽  
Concurrent Processes

Hemochromatosis is defined as abnormal accumulation of iron in various organs of the body such as liver, pancreas, skin, joints, heart due to raised gut absorption of iron due to low hepcidin yield. Hemochromatosis is secondary to thalassemia major in this case. In the absence of other concurrent processes, cardiac hemochromatosis is identified as the presence of cardiac dysfunction owing to increased iron accumulation in the heart leading to heart failure. Unique: Cardiac involvement in secondary hemochromatosis due to thalassemia major. Take Away lesson: Regular monitoring of serum iron, ferritin, echocardiographic monitoring among thalassemia major patients is essential. Despite the fact that heart failure due to iron overload could be fatal, early diagnosis and intervention could prevent and treat the disease.

scholarly journals The Prevalence of Adrenal, Parathyroid and Cardiac Dysfunction in Patients with Beta Thalassemia Major

2013 ◽  
Vol 04 (07) ◽  
pp. 325-330 ◽  
Author(s):  
Najat E. Mahdi ◽  
Taysir S. Garadah ◽  
Zuheir Hassan ◽  
Ahmed A. Jaradat ◽  
Das S. Nagalla
Keyword(s):  
Cardiac Dysfunction ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Beta Thalassemia Major

Fibroblast Growth Factor 23 (FGF23) and Klotho Protein in Beta-Thalassemia

2020 ◽  
Vol 52 (03) ◽  
pp. 194-201
Author(s):  
Dimitrios Stefanopoulos ◽  
Narjes Nasiri-Ansari ◽  
Ismene Dontas ◽  
Andromachi Vryonidou ◽  
Antonis Galanos ◽  
...  
Keyword(s):  
Growth Factor ◽  
Fibroblast Growth Factor ◽  
Fibroblast Growth Factor 23 ◽  
Iron Chelation ◽  
Thalassemia Major ◽  
The Body ◽  
Beta Thalassemia ◽  
Fibroblast Growth ◽  
Control Subjects ◽  
Number Of Patients

AbstractDerangements in phosphate and calcium homeostasis are common in patients with beta-thalassemia. Fibroblast growth factor 23 (FGF23) is among the main hormones regulating phosphate levels, while several studies underline an interplay between iron (Fe) and FGF23. Herein, we investigated, for the first time, the serum intact molecule (iFGF23) and the carboxyl-terminal fragment (C-FGF23) and Klotho levels simultaneously in patients with beta-thalassemia major receiving iron chelation regimens in comparison to healthy control subjects. We also correlated them with the body iron burden. The observational case-control study included 81 subjects (40 thalassemic patients and 41 healthy controls). Serum iFGF23, C-FGF23 and Κlotho were measured by ELISA. Parathormone, 25-hydroxycholecalciferol, calcium, and phosphorus were measured in blood and/or urine. The degree of hemosiderosis was evaluated by assessing the serum ferritin levels and performing T2* MRI measurements. Serum C-FGF23 levels were significantly lower in patients compared to control subjects (p=0.04), while iFGF23 and Klotho levels did not differ. Serum C-FGF23 levels were negatively correlated with ferritin (r=–0,421, p=0.018), whereas there were no significant correlations of each of the three factors with the iron chelation therapy. Decreased serum C-FGF23 levels were found in βTh patients which may be attributed to inhibition of proteolytic cleavage of iFGF23. Further studies in a greater number of patients will shed more light on the disturbances of the iFGF23, Klotho and C-FGF23 in thalassemia and their possible role in bone disease of such patients.

scholarly journals Zinc Phosphide Poisoning

2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
Author(s):  
Erdal Doğan ◽  
Abdulmenap Güzel ◽  
Taner Çiftçi ◽  
İlker Aycan ◽  
Feyzi Çelik ◽  
...  
Keyword(s):  
Heart Failure ◽  
Congestive Heart Failure ◽  
Intensive Care ◽  
Clinical Symptoms ◽  
Treatment Resistance ◽  
Zinc Phosphide ◽  
The Body ◽  
Care Process ◽  
Circulatory Collapse ◽  
Case Presentation

Zinc phosphide has been used widely as a rodenticide. Upon ingestion, it gets converted to phosphine gas in the body, which is subsequently absorbed into the bloodstream through the stomach and the intestines and gets captured by the liver and the lungs. Phosphine gas produces various metabolic and nonmetabolic toxic effects. Clinical symptoms are circulatory collapse, hypotension, shock symptoms, myocarditis, pericarditis, acute pulmonary edema, and congestive heart failure. In this case presentation, we aim to present the intensive care process and treatment resistance of a patient who ingested zinc phosphide for suicide purposes.

scholarly journals RATIONAL EMOTIVE THERAPY IBU YANG MEMILIKI ANAK DENGAN THALASSAEMIA BETA MAYO

2019 ◽  
Vol 12 (1) ◽  
pp. 81-90
Author(s):  
Aprilia Putri Rahmadini
Keyword(s):  
Group Counseling ◽  
Rational Emotive Behavior Therapy ◽  
Thalassemia Major ◽  
The Body ◽  
Beta Thalassemia ◽  
Rational Beliefs ◽  
Rational Emotive Therapy ◽  
Beta Thalassemia Major ◽  
Blood Disorder ◽  
Rational Emotive

Beta Thalassaemia Major is a genetic blood disorder that causes blood cells red rapidly destroyed in 20-30 days so that the body is deprived of blood. Treatment can be done during this time is a lifetime of blood transfusions. Their conditions of denial of information mother about the child's condition, as was the condition of self is not a carrier of thalassemia trait, blaming the pair as a nature, thinking irrational that the child's illness is a punishment from God for their sins in the past, the child has no future again, resulting in the emergence of maternal behavior that may endanger the lives of children like too late to bring the child to transfusion. Research Purposes is to produce a group counseling program with Rational Emotive Behavior Therapy (REBT) approach in order to increasing acceptance of mothers of children with beta thalassemia major. Research Design is one group pretest-posttest. Group counseling with REBT approach is done in 6 meetings. In the process, the mother will discuss issues with members of the group and counselors who help mothers to detect the irrational belief that appears, discriminating rational beliefs, and challenging that belief, to be replaced by a rational belief. The subject are mothers who have children with beta thalassemia major amounted to 2 people. Results  known to both participants are still in bargaining phase. However, prevalence increased mother’s acceptance of children with beta thalassemia major, marked by decreased aspects of denial and anger on both mother. Conclusion Group counseling with REBT approach can improve mother’s acceptance of children with beta thalassemia major.

scholarly journals Left ventricular dysfunction in transfusion dependent beta Thalassemia major patients in Bangladesh

2016 ◽  
Vol 9 (1) ◽  
pp. 31-35
Author(s):  
Simu Saha ◽  
Tapash Saha ◽  
AKM Amirul Morshed ◽  
Md Lutful Ehsan Fatmi ◽  
Nazneen Umme Zakia ◽  
...  
Keyword(s):  
Diastolic Dysfunction ◽  
Serum Ferritin ◽  
Cardiac Dysfunction ◽  
Serum Ferritin Level ◽  
Ventricular Dysfunction ◽  
Early Stage ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
Left Ventricular ◽  
Beta Thalassemia

Background: Thalassemia major is an inherited haemoglobin disorder resulting in chronic haemolytic anaemia. Patients with beta thalassemia major are maintained on continuous blood transfusion regimens resulting in iron overload that adversely affects both the structure and function of the heart and other vital organs which can be easily prevented with iron chelating therapy. The aim of the study was to detect left ventricular dysfunction at an early stage so that early effective intervention can be done.Methods: A total of 50 patients with beta thalassemia were included in the study by non randomized qualitative purposive sampling from July 2013 to June 2014. Their total body iron status was be assessed by doing serum ferritin level. Left ventricular systolic and diastolic function was assessed by echocardiographyResults: Cardiac dysfunction was present in 11 patients with high incidence in patients with low pre-transfusional haemoglobin group (p=0.4) and in patients having high serum ferritin level (p=0.02). Systolic cardiac dysfunction was present in 7(14%) of patients and diastolic dysfunction was present in 4(8%) of patients. There was a weak but significant correlation between left ventricular ejection fraction and serum ferritin concentration (r=-0.22; p=0.03). Only few (8%) patients had diastolic dysfunction.Conclusion: Patients with beta thalassaemia on an adequate transfusion showed an abnormal left ventricular systolic function. In early stage of disease diastolic function was normal but after repeated transfusion there were impaired relaxation indicating diastolic dysfunction. These findings seem mainly to be related to chronic anaemia and serum ferritin levelCardiovasc. j. 2016; 9(1): 31-35

scholarly journals A Rare Case of Facial Multiple Epidermal Cysts: Successfully Treated by Surgical Excision

2021 ◽  
Author(s):  
Qian Yu ◽  
Yanxian Wang ◽  
Yuling Shi ◽  
Jun Gu
Keyword(s):  
Rare Case ◽  
Surgical Excision ◽  
The Body ◽  
First Line ◽  
Case Presentation ◽  
Common Skin ◽  
The Face ◽  
Stratified Squamous Epithelium ◽  
Gland Function ◽  
Multiple Cysts

Abstract Background Epidermal cysts are common skin tumors that are composed of a keratinocytic cyst wall and central keratin material, which can occur anywhere in the body, especially on the face. However, there are no relevant reports of multiple epidermal cysts coexisting at the same location.Case presentation Here, we report one rare case of facial multiple epidermal cysts, who underwent sequential resection of all cysts. A 45-year-old male presented with facial multiple masses for over 2 years. Physical examination showed multiple cysts with varied sizes on the face. All cysts were smooth, dome-shaped, freely movable, and some of them were attached to the skin by a central pore. The histopathology revealed cysts in the dermis, the wall of which were composed of stratified squamous epithelium, and the cavity were filled with keratin. Therefore, the patient was diagnosed as facial multiple epidermal cysts.Conclusions This is the first well-documented case of multiple epidermal cysts on the face. We report this rare case to highlight that although epidermal cysts usually appear single, they may also appear multiple in the same location, especially in those patients with vigorous sebaceous gland function. Surgical excision is the first-line effective treatment for epidermal cysts.

scholarly journals Thalassemia, a human blood disorder

2023 ◽  
Vol 83 ◽  
Author(s):  
F. Shafique ◽  
S. Ali ◽  
T. Almansouri ◽  
F. Van Eeden ◽  
N. Shafi ◽  
...  
Keyword(s):  
Hemolytic Anemia ◽  
Developmental Stages ◽  
Gene Mutations ◽  
Thalassemia Major ◽  
Marrow Transplant ◽  
The Body ◽  
Beta Thalassemia ◽  
Globin Genes ◽  
Blood Disorder ◽  
Alpha Thalassemia

Abstract A group of inherited blood defects is known as Thalassemia is among the world’s most prevalent hemoglobinopathies. Thalassemias are of two types such as Alpha and Beta Thalassemia. The cause of these defects is gene mutations leading to low levels and/or malfunctioning α and β globin proteins, respectively. In some cases, one of these proteins may be completely absent. α and β globin chains form a globin fold or pocket for heme (Fe++) attachment to carry oxygen. Genes for alpha and beta-globin proteins are present in the form of a cluster on chromosome 16 and 11, respectively. Different globin genes are used at different stages in the life course. During embryonic and fetal developmental stages, γ globin proteins partner with α globin and are later replaced by β globin protein. Globin chain imbalances result in hemolysis and impede erythropoiesis. Individuals showing mild symptoms include carriers of alpha thalassemia or the people bearing alpha or beta-thalassemia trait. Alpha thalassemia causes conditions like hemolytic anemia or fatal hydrops fetalis depending upon the severity of the disease. Beta thalassemia major results in hemolytic anemia, growth retardation, and skeletal aberrations in early childhood. Children affected by this disorder need regular blood transfusions throughout their lives. Patients that depend on blood transfusion usually develop iron overload that causes other complications in the body systems like renal or hepatic impairment therefore, thalassemias are now categorized as a syndrome. The only cure for Thalassemias would be a bone marrow transplant, or gene therapy with currently no significant success rate. A thorough understanding of the molecular basis of this syndrome may provide novel insights and ideas for its treatment, as scientists have still been unable to find a permanent cure for this deadly disease after more than 87 years since it is first described in 1925.

scholarly journals The Role of Heart Rate Variability and Fragmented QRS for Determination of Subclinical Cardiac Involvement in Beta-Thalassemia Major

Pulse ◽  
2020 ◽  
Vol 8 (1-2) ◽  
pp. 15-20
Author(s):  
Mozhgan Parsaee ◽  
Amir Farjam Fazelifar ◽  
Elham Ansaripour ◽  
Azita Azarkeyvan ◽  
Behshid Ghadrdoost ◽  
...  
Keyword(s):  
Heart Rate ◽  
Heart Rate Variability ◽  
Cardiac Involvement ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Fragmented Qrs ◽  
Beta Thalassemia Major

scholarly journals Lingguizhugan decoction attenuates doxorubicin-induced heart failure in rats by improving TT-SR microstructural remodeling

2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Xueping Li ◽  
Guangmin Xu ◽  
Shujun Wei ◽  
Baocheng Zhang ◽  
Huan Yao ◽  
...  
Keyword(s):  
Heart Failure ◽  
Left Ventricular Ejection Fraction ◽  
Ejection Fraction ◽  
Cardiac Dysfunction ◽  
Left Ventricular ◽  
The Body ◽  
Ventricular Ejection ◽  
Left Ventricular Ejection ◽  
Heart Weight ◽  
Ventricular Ejection Fraction

Abstract Background Lingguizhugan decoction (LGZG), an ancient Chinese herbal formula, has been used to treat cardiovascular diseases in eastern Asia. We investigated whether LGZG has protective activity and the mechanism underlying its effect in an animal model of heart failure (HF). Methods A rat model of HF was established by administering eight intraperitoneal injections of doxorubicin (DOX) (cumulative dose of 16 mg/kg) over a 4-week period. Subsequently, LGZG at 5, 10, and 15 mL/kg/d was administered to the rats intragastrically once daily for 4 weeks. The body weight, heart weight index (HWI), heart weight/tibia length ratio (HW/TL), and serum BNP level were investigated to assess the effect of LGZG on HF. Echocardiography was performed to investigate cardiac function, and H&E staining to visualize myocardial morphology. Myocardial ultrastructure and T-tubule-sarcoplasmic reticulum (TT-SR) junctions were observed by transmission electron microscopy. The JP-2 protein level was determined by Western blotting. The mRNA level of CACNA1S and RyR2 and the microRNA-24 (miR-24) level were assayed by quantitative RT-PCR. Results Four weeks after DOX treatment, rats developed cardiac damage and exhibited a significantly increased BNP level compared with the control rats (169.6 ± 29.6 pg/mL versus 80.1 ± 9.8 pg/mL, P < 0.001). Conversely, LGZG, especially at the highest dose, markedly reduced the BNP level (93.8 ± 17.9 pg/mL, P < 0.001). Rats treated with DOX developed cardiac dysfunction, characterized by a strong decrease in left ventricular ejection fraction compared with the control (58.5 ± 8.7% versus 88.7 ± 4.0%; P < 0.001). Digoxin and LGZG improved cardiac dysfunction (79.6 ± 6.1%, 69.2 ± 2.5%, respectively) and preserved the left ventricular ejection fraction (77.9 ± 5.1, and 80.5 ± 4.9, respectively, P < 0.01). LGZG also improved the LVEDD, LVESD, and FS and eliminated ventricular hypertrophy, as indicated by decreased HWI and HW/TL ratio. LGZG attenuated morphological abnormalities and mitochondrial damage in the myocardium. In addition, a high dose of LGZG significantly downregulated the expression of miR-24 compared with that in DOX-treated rats (fold change 1.4 versus 3.4, P < 0.001), but upregulated the expression of JP-2 and antagonized DOX-induced T-tubule TT-SR microstructural remodeling. These activities improved periodic Ca2+ transients and cell contraction, which may underly the beneficial effect of LGZG on HF. Conclusions LGZG exerted beneficial effects on DOX-induced HF in rats, which were mediated in part by improved TT-SR microstructural remodeling.

scholarly journals Estimation of iron overloads using oral exfoliative cytology in beta-thalassemia major patients

CytoJournal ◽  
2016 ◽  
Vol 13 ◽  
pp. 6 ◽  
Author(s):  
Swati Leekha ◽  
Amit Kumar Nayar ◽  
Preeti Bakshi ◽  
Aman Sharma ◽  
Swati Parhar ◽  
...  
Keyword(s):  
Iron Overload ◽  
Prussian Blue ◽  
Serum Ferritin ◽  
Thalassemia Major ◽  
The Body ◽  
Transport Cost ◽  
Beta Thalassemia ◽  
Safe Procedure ◽  
Beta Thalassemia Major ◽  
Mucosal Cells

Background: Iron overload is a medical condition that occurs when too much of the mineral iron builds up inside the body and produces a toxic reaction. Thalassemia is a genetic disorder of hemoglobin synthesis, which requires regular blood transfusion therapy, and the lack of specific excretory pathways for iron in humans leads to iron overload in the body tissues. It is a major cause of morbidity and mortality in these patients. The estimation of iron levels in exfoliated buccal mucosal cells may provide a simple, noninvasive, and a safe procedure for estimating the iron overload by using the Perls’ Prussian blue stain. Methods: Smears were obtained from buccal mucosa of 40 randomly selected beta-thalassemia major patients and 40 healthy subjects as controls. Smears were stained with Perls’ Prussian blue method. Blood samples were taken for estimation of serum ferritin levels. Images of smears were analyzed using the software image J software version 1.47v and correlated with serum ferritin. Results: Perls’ positivity was observed in 87.5% of thalassemic patients with a positive correlation to serum ferritin levels. Conclusion: The use of exfoliative buccal mucosal cells for the evaluation of iron overloads in the body provides us with a diagnostic medium that is noninvasive, easy to collect, store, and transport, cost effective, and above all reliable.

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