scholarly journals Gingival enlargement in thalassemia patient – A Conjectural Association

2020 ◽  
Vol 6 (3) ◽  
pp. 74-77
Author(s):  
CS BAIJU ◽  
Gunjan Gupta ◽  
Karuna Joshi ◽  
Shagufta ◽  
N.D Gupta
Keyword(s):  
Single Gene ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Thalassemia Patient ◽  
Anterior Maxilla ◽  
Facial Region ◽  
Periodontal Tissues ◽  
Beta Thalassemia Major ◽  
Gingival Enlargement ◽  
Maxilla And Mandible

Thalassemia is a single gene inherited blood disease. Beta thalassemia major is life threating. It causes abnormality in various organs and oral-facial region. Thalassemia patients are immune-deficient because of iron-overload. Immune system abnormality includes neutrophilic dysfunction and impairment of phagocytosis by the monocyte-macrophage system. Iron accumulation also affects periodontal tissues, which seems to increase the level of cytokines and thus have an enhancing effect on gingival inflammation. This article highlights a peculiar case of gingival enlargement in anterior maxilla and mandible. The patient was known case of a beta-thalassemia major. Blood investigation revealed a lower level of hemoglobin. The patient underwent non-surgical periodontal therapy. Proper periodontal care improves the quality of life in these patients. This case report reinforces the significance of proper history taking with all minor details and the role of patient education in phase I therapy

A Report of Transplantation of 224 Patients with Beta Thalassemia Major in Tehran, 1991– 2004.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3772-3772
Author(s):  
Ardeshir Ghavamzadeh ◽  
Mohamad Jahani ◽  
Kamran Alimoghaddam ◽  
Masoud Iravani ◽  
Babak Bahar ◽  
...  
Keyword(s):  
Single Gene ◽  
Chronic Gvhd ◽  
Conditioning Regimen ◽  
Disease Free Survival ◽  
Thalassemia Major ◽  
Class I ◽  
Beta Thalassemia ◽  
Class Iii ◽  
Beta Thalassemia Major ◽  
Gvhd Prophylaxis

Abstract Introduction: Beta Thalassemia Major is one of the most common single gene disorders and the most common type of hemoglobinopathies in Iran, with more than 20,000 patients. North and South parts of Iran have the highest prevalence, although it exists all over the country. In Iran, the first thalassemic patient was transplanted in our center, in 1991. Methods and patients: Till now (August 2004) 224 Beta Thalassemia Major Patients have been transplanted in this center. Age range =2–17 years, M/F=123/101, Class I=99, class II=81 and class III= 44. 124 patients (55.4%) received Bone Marrow, 94 (42%) Peripheral Blood stem cell and 6 (2.7%) Cord Blood for transplantation. Their conditioning regimen in class I and II included Busulfan 3.5mg/kg for 4days, Cyclophosphamide (CY) 50mg/kg for 4 days and their GVHD prophylaxis regimen was Cyclosporine (CYX) 3mg/kg (IV from day −2 till day +5) and 12.5mg/kg (PO after then) and in class III, included Busulfan 4mg/kg for 4days and CY 40mg/kg for 4days and their GVHD prophylaxis regimen was CYX plus MTX 10mg/m2 on day +1 and 6mg/m2 on days +3 and +6. Results:188 (84%) patients are alive and 36(16.1%) deceased.188 (84%) patients have passed 100 days after transplantation.168 (75%) patients had developed clinical acute GVHD after transplantation (grade II–IV= 57.1%). 67(35.6%) patients developed chronic GVHD (44 limited, 23 extensive). Three to ten year overall survival was 81% and disease free survival was 70 %. Conclusion: Blood and marrow transplantation (based on other documented studies and our experience) is the treatment of choice for class I& II and is indicated in class III thalassemic patients.

scholarly journals Posttransfusion hyperhemolysis syndrome in beta thalassemia major: Postulation of various mechanisms

2021 ◽  
Author(s):  
Ganesh Kasinathan ◽  
Jameela Sathar
Keyword(s):  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Thalassemia Patient ◽  
Beta Thalassemia Major

Hyperhemolysis syndrome (HS) should be considered in a multiply transfused thalassemia patient. HS in this patient was attributed to a combination of factors including multiple transfusions, presence of anti-Cw, macrophage hyperactivity, hypersplenism and suppression of erythropoiesis.

scholarly journals STUDY OF SERUM LIPID PROFILE IN BETA-THALASSEMIA MAJOR PATIENTS

2021 ◽  
Vol 5 (8) ◽  
Author(s):  
Sandeep Kulhari ◽  
Deepak Choudhary
Keyword(s):  
Lipid Profile ◽  
Total Cholesterol ◽  
Single Gene ◽  
Globin Gene ◽  
Density Lipoprotein ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Healthy Children ◽  
Cross Sectional ◽  
Beta Thalassemia Major

Background: Beta-thalassemia is the commonest single-gene disorder in the Indian population. Beta thalassemia major is caused by complete absence of beta globin chain production resulting from reduced synthesis of one or more globin chains which can be caused by different globin gene mutation resulting in ineffective hematopoiesis, increased hemolysis and early onset anemia. Methods: A cross-sectional; case control study was done on 50 diagnosed Cases of beta-thalassemia major in the age group of 1 year to 18 years receiving regular blood transfusions; not suffering from any ailment or any other disease leading to deranged lipid profile were included. In controls; 50 normal healthy children were included. Results: Lipid analyses of controls and thalassemic children. It is clear from the results that beta thalassemia major patients had significantly lower total cholesterol (TC), high-density lipoprotein cholesterol (HDL) and low-density lipoprsotein cholesterol (LDL) compared with controls (p<0.001). Conclusion: Lipid profile in Beta thalassmia patients show significantly low levels of total cholesterol, LDLC and HDL-C. Keywords: Beta Thalassemia Major, Lipid Profile, Hypocholesterolemia.

scholarly journals Assessment of Subclinical Renal Glomerular and Tubular Dysfunction in Children with Beta Thalassemia Major

Children ◽  
2021 ◽  
Vol 8 (2) ◽  
pp. 100
Author(s):  
Asmaa A. Mahmoud ◽  
Doaa M. Elian ◽  
Nahla MS. Abd El Hady ◽  
Heba M. Abdallah ◽  
Shimaa Abdelsattar ◽  
...  
Keyword(s):  
Cystatin C ◽  
Kidney Injury ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Good Survival ◽  
Control Group ◽  
Healthy Children ◽  
Serum Cystatin C ◽  
Serum Cystatin ◽  
Beta Thalassemia Major

Background: A good survival rate among patients with beta thalassemia major (beta-TM) has led to the appearance of an unrecognized renal disease. Therefore, we aimed to assess the role of serum cystatin-C as a promising marker for the detection of renal glomerular dysfunction and N-acetyl beta-D-glucosaminidase (NAG) and kidney injury molecule 1 (KIM-1) as potential markers for the detection of renal tubular injury in beta-TM children. Methods: This case-control study was implemented on 100 beta-TM children receiving regular blood transfusions and undergoing iron chelation therapy and 100 healthy children as a control group. Detailed histories of complete physical and clinical examinations were recorded. All subjected children underwent blood and urinary investigations. Results: There was a significant increase in serum cystatin-C (p < 0.001) and a significant decrease in eGFR in patients with beta-TM compared with controls (p = 0.01). There was a significant increase in urinary NAG, KIM-1, UNAG/Cr, and UKIM-1/Cr (p < 0.001) among thalassemic children, with a significant positive correlation between serum cystatin-C, NAG and KIM-1 as regards serum ferritin, creatinine, and urea among thalassemic patients. A negative correlation between serum cystatin-C and urinary markers with eGFR was noted. Conclusion: Serum cystatin-C is a good marker for detection of glomerular dysfunction. NAG and KIM-1 may have a predictive role in the detection of kidney injury in beta-TM children.

scholarly journals Left Atrial Strain Identifies Increased Atrial Ectopy in Patients with Beta-Thalassemia Major

Diagnostics ◽  
2020 ◽  
Vol 11 (1) ◽  
pp. 1
Author(s):  
Maria Vlachou ◽  
Vasileios Kamperidis ◽  
Efthymia Vlachaki ◽  
Georgios Tziatzios ◽  
Despoina Pantelidou ◽  
...  
Keyword(s):  
Blood Transfusion ◽  
Left Atrial ◽  
Systolic Pressure ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Tape Recording ◽  
Blood Transfusions ◽  
Systolic Strain ◽  
Peak Systolic Strain ◽  
Beta Thalassemia Major

Patients with beta-thalassemia major (β-ΤΜ) may develop cardiac arrhythmias through a multifactorial mechanism. The current study evaluated the association of cardiac structure and function on echocardiography with atrial ectopic burden on 24-hour tape recording in β-ΤΜ patients. This prospective study included consecutive β-ΤΜ patients. Demographic, laboratory, echocardiographic, cardiac magnetic resonance (CMR) T2* and 24-hour tape recording data were prospectively collected. The patients were classified according to the median value of premature atrial contractions (PACs) on 24-hour tape. In total, 50 β-TM patients (37.6 ± 9.1 years old, 50% male) were divided in 2 groups; PACs ≤ 24/day and > 24/day. Patients with PACs > 24/day were treated with blood transfusion for a longer period of time (39.0 ± 8.6 vs. 32.0 ± 8.9 years, p < 0.007), compared to their counterparts. Older age (OR: 1.121, 95% CI: 1.032–1.217, p = 0.007), longer duration of blood transfusion (OR:1.101, 95% CI:1.019–1.188, p = 0.014), larger LV end-diastolic diameter (OR: 4.522, 95% CI:1.009–20.280, p = 0.049), higher values of LA peak systolic strain (OR: 0.869, 95% CI: 0.783–0.964, p = 0.008), higher MV E/E′ average (OR: 1.407, 95% CI: 1.028–1.926, p = 0.033) and higher right ventricular systolic pressure (OR: 1.147, 95% CI: 1.039–1.266, p = 0.006) were univariably associated with PACs > 24/day. LA peak systolic strain remained significantly associated with PACs > 24/day after adjusting for the duration of blood transfusions or for CMR T2*. The multivariable model including blood transfusion duration and LA peak systolic strain was the most closely associated with PACs > 24/day. Receiver operating characteristic curve analysis identified a left atrial peak systolic strain of 31.5%, as the best cut-off value (83% sensitivity, 68% specificity) for prediction of PACs > 24/day. In β-TM patients, LA peak systolic strain was associated with the atrial arrhythmia burden independently to the duration of blood transfusions and CMR T2*.

Sign in / Sign up

Export Citation Format

Share Document