scholarly journals Dental Arch Dimensions in Subjects with Beta-thalassemia Major

2011 ◽  
Vol 12 (6) ◽  
pp. 429-433 ◽  
Author(s):  
Othman M Yassin
Keyword(s):  
Thalassemia Major ◽  
Severe Form ◽  
Beta Thalassemia ◽  
Control Group ◽  
Dental Arch ◽  
Mean Values ◽  
Mandibular Arch ◽  
Beta Thalassemia Major ◽  
Males And Females ◽  
And Control

ABSTRACT Aim Thalassemia is a group of inherited hemoglobinopathies with thalassemia major representing the severe form of the disease characterized by craniofacial deformities. The aim of this study is to provide a detailed description of dental arch dimensions in subjects with thalassemia major. Subjects and methods The sample consisted of 43 thalassemic subjects, 24 males and19 females, aged 7.3 to 15.4 years (mean ± SD = 10.6 ± 3.5 years) and control group matched by age and sex. Dental casts of the participants were measured for arch lengths, arch widths, using a digital sliding caliper. Student t-test was used for comparison of mean values between males and females as well as between thalassemic and control groups. Results The results show that all means of maxillary and mandibular arch dimensions in thalassemic males and females were smaller than their controls, with 14 of the 16 comparisons being statistically significant (ranged from p < 0.05 to p < 0.001). The segmental arch lengths in the maxilla and mandible of thalassemic group were reduced by an average of 2.59 and 2.55 mm respectively, compared with the control group. The mean maxillary and mandibular arch depths (lengths) in the thalassemic group were shorter by 3.21 and 2.63 mm respectively, relative to the controls (p < 0.001). All arch widths thalassemic patients were significantly reduced by an average ranged from 1.33 to 1.90 mm in the maxilla and 1.37 to 1.77 mm in the mandible. Conclusion The present study showed that the maxillary and mandibular dental arches dimensions are significantly reduced in patients with thalassemia major compared with healthy control subjects. Clinical significance Changes in the size of dental arches and tooth dimensions in thalassemic patients have an impact on the occlusal relationships. These changes should be taken into account when planning orthodontic treatment and orthognathic surgery. How to cite this article Hattab FN, Yassin OM. Dental Arch Dimensions in Subjects with Beta-thalassemia Major. J Contemp Dent Pract 2011;12(6):429-433.

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 Relationship between Vitamin B9 (Folic Acid), Vitamin B12 (Cobalamin), and Peripheral Neuropathy in Children with Beta-Thalassemia Major

2021 ◽  
Vol 9 (2) ◽  
Author(s):  
Uni Gamayani ◽  
Titin Junaidi ◽  
Nushrotul Lailiyya ◽  
Nur Suryawan ◽  
Nanan Sekarwana
Keyword(s):  
Folic Acid ◽  
Peripheral Neuropathy ◽  
Vitamin B12 ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Control Group ◽  
Case Group ◽  
Beta Thalassemia Major ◽  
Vitamin B9 ◽  
Significant Difference

Vitamin B9 (folic acid) and B12 (cobalamin) are essential vitamins that play roles in the process of hematopoiesis and maintaining the function of peripheral nerves. Therefore, these deficiencies may create a risk for peripheral neuropathy in beta-thalassemia major patients. The purpose of this study is to determine the relationship between vitamin B9 level, vitamin B12 level, and peripheral neuropathy in beta-thalassemia major children. It was an observational analytical study with a case-control design has been conducted at Dr. Hasan Sadikin General Hospital Bandung, Indonesia, in May–July 2019. There were 47 beta-thalassemia major children with peripheral neuropathy (case) and 41 healthy children (control). All subjects completed a general demographic questionnaire, underwent neurological examination, and were tested for vitamin B9 and B12 serum levels. Data were then analyzed using the unpaired t test to compare the vitamin levels between both groups and Spearman’s rank correlation test to investigate the correlation between vitamin levels and the number of affected nerves in the case group. Comparison of folic acid levels in the case group (21.52±6.22 ng/mL) and the control group (23.81±7.51 ng/mL) showed no significant difference (p=0.19). In contrast, cobalamin in the case group (288.57±168.61 ng/mL) and the control group (385.95±197.48 ng/mL) showed a significant difference (p=0.01). In addition, there was a moderate correlation (p=0.004, r=0.41) between folic acid level and the number of motoric nerves affected in the case group. In conclusion, cobalamin level correlates with peripheral neuropathy in beta-thalassemia major patients, and folic acid level correlates with the number of affected nerves, especially motoric nerves. HUBUNGAN ANTARA VITAMIN B9 (ASAM FOLAT), VITAMIN B12 (KOBALAMIN), DAN NEUROPATI PERIFER PADA ANAK DENGAN TALASEMIA BETA MAYORVitamin B9 (asam folat) dan B12 (kobalamin) merupakan vitamin esensial yang berperan dalam proses hematopoiesis dan menjaga fungsi saraf tepi. Defisiensi vitamin ini dapat menimbulkan risiko neuropati perifer pada pasien talasemia beta mayor. Tujuan penelitian ini mengetahui hubungan antara kadar vitamin B9, vitamin B12, dan neuropati perifer pada anak talasemia beta mayor. Metode penelitian ini adalah analitik observasional dengan rancangan studi kasus kontrol yang dilakukan di RSUP Dr. Hasan Sadikin Bandung, Indonesia pada Mei–Juli 2019. Terdapat 47 anak talasemia beta mayor dengan neuropati perifer (kelompok kasus) dan 41 anak sehat (kelompok kontrol). Seluruh subjek penelitian mengisi kuesioner demografi umum, menjalani pemeriksaan fisis neurologis, serta dilakukan tes kadar vitamin B9 dan B12 serum. Uji t test tidak berpasangan digunakan untuk membandingkan kadar vitamin pada kedua kelompok dan uji korelasi Spearman untuk membandingkan kadar kedua vitamin tersebut dengan jumlah saraf yang terkena pada kelompok kasus. Perbandingan kadar asam folat kelompok kasus (21,52±6,22 ng/mL) dan kelompok kontrol (23,81±7,51 ng/mL) menunjukkan perbedaan yang tidak bermakna (p=0,19), sedangkan perbandingan kadar kobalamin kelompok kasus (288,57±168,61 ng/mL) dan kelompok kontrol (385,95±197,48 ng/mL) menunjukkan perbedaan yang bermakna (p=0,01). Selain itu, terdapat korelasi sedang (p=0,004; r=0,41) antara kadar asam folat dam jumlah saraf motorik yang terkena pada kelompok kasus. Kesimpulan, kadar kobalamin berhubungan dengan neuropati perifer pada penderita talasemia beta mayor dan kadar asam folat berhubungan dengan jumlah saraf yang terkena, terutama saraf motorik.

scholarly journals Quantitative Assessment of Serum Copper Status in Children with Thalassemia Major

2020 ◽  
pp. 94-100
Author(s):  
Subir Nandy ◽  
A. K. M. Amirul Morshed ◽  
Tanzina Iveen Chowdhury ◽  
Shahnoor Islam ◽  
Bijoy K. Paul ◽  
...  
Keyword(s):  
Thalassemia Major ◽  
Serum Copper ◽  
Dietary Factors ◽  
Chemistry Laboratory ◽  
Beta Thalassemia ◽  
Copper Level ◽  
Control Group ◽  
Normal Children ◽  
Beta Thalassemia Major ◽  
Significant Difference

Objective: Support of ordinary degrees of copper, has a significant influence in reducing the morbidities related with thalassemia major. Levels of this component has been seen as modified in this incessant transfusion subordinate infection from everywhere throughout the world, however with broadly factor results. Other than rehashed blood transfusions and utilization of chelating operators, the level of copper in thalassemia patients are influenced by hereditary and dietary factors likewise, convincing us to embrace this investigation in our population. The Objectives of this study was to appraise of serum levels of copper in children with beta-thalassemia major. Methods: In this observational comparative research design, all children between 5 to 15 years affected by beta thalassemia major (30 patients) and 30 normal children were evaluated for serum copper levels in the Department of Pediatrics, DMCH and attended at Thalassemia Center, DSH (Dhaka Shishu Hospital). Serum measurements for copper were performed using Atomic Absorption Spectrophotometer (Varian, AA 240 FS, USA) in Analytical Chemistry Laboratory, Chemistry Division, Atomic Energy Centre, Dhaka, Bangladesh. Results: The minimum, maximum, and the mean concentration of serum copper in patients were 103.50, 239.94, and 147.55 ± 35.17 µg/dl and in control group, those were 58.75, 192.43, and 123.85± 33.19 µg/dl respectively. There was no significant difference in serum copper level in the β thalassemia group and the control group (p=0.54) (P=0.3). Conclusion: This investigation revealed that there is no copper inadequacy. Further assessment in such manner is suggested.

scholarly journals Evaluation of Erythroferrone, Hepcidin, and Iron Overload Status in Iraqi Transfusion-dependent β-Thalassemia Major Patients

2019 ◽  
Author(s):  
Hasan Smesam ◽  
Hasan Qazmooz ◽  
Sareh Arjmand ◽  
Hussein Kadhem Al-Hakeim ◽  
Seyed Omid Ranaei‐Siadat,
Keyword(s):  
Iron Overload ◽  
Iron Status ◽  
Iron Chelation ◽  
Fold Increase ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Control Group ◽  
Beta Globin ◽  
Beta Thalassemia Major ◽  
Globin Chains

Beta thalassemia major (&beta;-TM) disorder characterized by the lack, or severe reduction in the production of hemoglobin &beta;-globin chains. The standard protocol for the management of &beta;-TM is blood transfusion and iron chelation therapy to reduce the iron overload state. The present study aimed to investigate the relationships between two iron regulatory hormones, hepcidin (HEPC) and erythroferrone (ERFE) levels and iron status parameters (ISPs) in Iraqi patients with &beta;-TM. ISPs and hormones were measured in sixty patients and compared with thirty healthy controls. The results indicated significant changes in different iron status parameters, while ferritin (FRT) with the ~11 fold increase showed the most change. Significant reduction in HEPC and increase in ERFE levels were detected in patients as compared to the control group, while no direct correlation was identified with the other measured ISPs. Receiver operating characteristic (ROC) analysis showed that the z-score of the composite of ERFE+FRT has a full diagnostic ability for &beta;-TM. In conclusion, our finding indicated the correlation between different ISPs, FRT as the leading predictor of iron overload and tow main iron regulatory hormones.

Investigation of Pancreatic Sonography Findings in Patients With Beta-Thalassemia Major

2021 ◽  
pp. 875647932198966
Author(s):  
Razieh Behzadmehr ◽  
Iraj Shahramian ◽  
Pouya Ostad Rahimi ◽  
Mahboobeh Sheikh ◽  
Soosan keikha ◽  
...  
Keyword(s):  
Statistical Tests ◽  
Pancreatic Head ◽  
Thalassemia Major ◽  
Cross Sectional Study ◽  
Beta Thalassemia ◽  
Control Group ◽  
P Value ◽  
Case Group ◽  
Cross Sectional ◽  
Beta Thalassemia Major

Objective: Some structural abnormalities have been reported on sonography of pancreas in patients with beta-thalassemia which can indicate that the patient is developing diabetes. In this study, the aim was to investigate the findings of pancreatic sonography in patients, with beta-thalassemia major. Methods: This cross-sectional study included information extracted from the medical record and entered into an information form. A cohort of 190 people were split into case and control groups, retrospectively. The required data were extracted from patient’s documents. Data were analyzed with SPSS software, version 22; with the chi-square statistical tests. A P value of < .05 was considered statistically significant. Results: This study consisted of 33 males and 61 females in the case group and 32 males and 64 females in the control group. The most common echogenicity observed during pancreatic sonography, in the control group, was an isoechoic feature (72.3%), followed by hyper and hypoechogenicity (18.1% and 9.6%, respectively). The prevalence of an isoechoic texture in the patient case group was 58.3%, while hyper and hypoechogenicity were seen in 26% and 2.1%, respectively ( P = .070). There was a poor negative correlation between diabetes and pancreatic echogenicity ( P = .002 and r = −.226). Also, there was a poor positive correlation between fasting serum glucose levels and pancreatic echogenicity ( P = .034, n = 96 and r = .217). Conclusion: This study demonstrates, in this patient group, that there was a poor relationship between pancreatic head size with serum ferritin levels and pancreatic echogenicity with diabetes. These results may suggest using sonography to diagnose early stages of diabetes in patients with thalassemia.

The Study of G/T Polymorphism in COLIA1 Gene and Osteoporosis in b-Thalassemia Patients.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4078-4078
Author(s):  
Amal M. El-Beshlawy ◽  
Mona Mohamed Hamdy ◽  
Ibtesam Ramzy Hussein ◽  
Hala Fathy Sheba ◽  
Mona Abdel Gawad
Keyword(s):  
Negative Correlation ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Control Group ◽  
Type I ◽  
Control Groups ◽  
Significant Difference ◽  
High Prevalence ◽  
S Allele ◽  
And Control

Abstract Abstract 4078 Poster Board III-1013 Introduction Osteoporosis is an important cause of morbidity in patients with β thalassemia major. Several genes are thought to be involved in the pathogenesis of osteoporosis. Collagen type I alpha 1 (COLIA1) is one of the most prominent candidate genes, which has been consistently associated with osteoporosis in different populations. Polymorphism at the Sp1 binding site within a key regulatory region of COLIA 1 has been reported to be associated with susceptibility to osteoprotic fractures. Our objective was to detect the allelic distribution of COLIA1 gene in beta thalassemia patients and its relation to bone mineral density (BMD). Patients and methods The study included 25 beta thalassemia major patients (12-28 years, mean = 18.12±5.077 years) and 20 controls (mean age = 23.05±2.3 years) with no family history of thalassemia. Anthropometric measurements were done to all patients .Femoral and lumbar BMD was measured in all patients and control groups using dual energy absorbiometry (DXA). Assessment of the COLIA1 genotypes (SS, Ss, ss) and G/T polymorphism were done using PCR amplification and restriction enzyme digestion (Bal1) of DNA amplified products to thalassemic patients and control groups. Results High prevalence of growth retardation was observed in β thalassemia major patients in the form of short stature (36%), truncal shortening (48%) and under weight (8%). Osteopenia and osteoporosis were detected in 68% of the thalassemia patients with a highly significant lower lumbar and femoral BMD compared to controls (P<0.01) (Table 1). There was a non significant difference between genders regarding BMD, significant negative correlation was observed between long duration of desferroxamine (DFO) intake and low femoral and lumbar BMD (R=-0.571& P<0.01,R=-0.571&P<0.05) in β thalassemia patients. A negative correlation was observed between ferritin level and fat free mass% (FFM%) in thalassemic patients (R=-0.48& P<0.05) and low lumbar BMD in female patients (R=-0.55& P<0.05). There was a positive correlation between age of starting DFO and decreased sitting height in male patients (R=0.74&P<0.01). In the thalassemic group, frequency of COLIA1 alleles was S (94%), s (6%), 12% showed the G/T polymorphism while 88% had the G/G polymorphism (Table 2). Non significant difference was observed in most of the parameters between the 2 different groups of polymorphism. In the control group allele frequencies were S (87.5%) and s (12.5%), G/T polymorphism was present in 25%.and G/G in 75%. No association was detected between COLIA1 gene polymorphism and BMD in the lumbar spine or in femur bone in the thalassemia patients (P>0.05) while the s allele was associated with femoral and lumbar BMD in the control group (P<0.05, P<0.01) (Table 3). Conclusion High prevalence of growth impairment, osteopenia and osteoporosis in our β thalassemia major patients. Desferroxamine duration therapy was related to osteoporosis in our patients. The absence of association between BMD and COLIA 1 polymorphism and the low frequency of s allele in our thalassemic group may be due to the small sample size of the thalassemia patients together with the fact that BMD is determined by a variety of genetic and environmental factors. Further studies including larger number of patients are needed to evaluate these findings. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Comparison of Blood Pressure in Beta Thalassemia Major Patients with that of Control

2020 ◽  
pp. 1-3
Author(s):  
Sneha Mahadev Bhatkar ◽  
Surendra S Shivalkar ◽  
Sachin H Mulkutkar
Keyword(s):  
Blood Pressure ◽  
Systolic Blood Pressure ◽  
Diastolic Blood Pressure ◽  
Normal Subjects ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Control Group ◽  
Study Group ◽  
Beta Thalassemia Major ◽  
Significant Difference

Introduction and Objectives : β-thalassemia major patients need to have repeated blood transfusions throughout their life for survival, which leads to so many complications. Heart disease is the most important complication and the main determinant of survival in these patients. Our study shows comparison of blood pressure between these patients and that of normal subjects. Material and Methods : Present study was a cross sectional type of study and consisted of 100 normal subjects (control group) and 100 patients of beta thalassemia major (study group). Blood pressure was recorded in both the groups using mercury sphygmomanometer and statistical analysis of the observations was done using Z test. Results : There was no statistically significant difference in the Systolic blood pressure (SBP) of study group as compared to control group whereas there was a difference in the Diastolic blood pressure (DBP) of study group and control group which was statistically significant (p value <0.0001). Conclusion : There was an overall reduction in the blood pressure of study group as compared to control group but the difference in SBP was statistically not significant whereas that of DBP was statistically significant. Key Words: Beta thalassemia major, Diastolic blood pressure, Systolic blood pressure

scholarly journals Cytokine Dependent Hematopoietic Cell Linker (CLNK) is Highly Elevated in Blood Transfusion Dependent Beta-Thalassemia Major Patients

2019 ◽  
Author(s):  
Hussein Kadhem Al-Hakeim ◽  
Hawraa Hussein Al-Mayali ◽  
Michael Maes
Keyword(s):  
Iron Status ◽  
Globin Gene ◽  
Adaptor Protein ◽  
Thalassemia Major ◽  
Severe Form ◽  
Hematopoietic Cell ◽  
Beta Thalassemia ◽  
Beta Globin ◽  
Beta Thalassemia Major ◽  
Globin Chains

Beta-thalassemia major (&beta;-TM) is a severe form of thalassemia caused by mutations in the &beta;-globin gene, resulting in partial or complete deficiency of &beta;-globin chains. This deficiency results in oxidative stress, dyserythropoiesis, and chronic anemia. Cytokine dependent hematopoietic cell linker (CLNK) belongs to the adaptor protein family and has the capacity to interact with multiple signaling proteins thereby modulating signal transduction. The aim of the present study was to examine CLNK in sera of &beta;-TM patients and examine its association with iron overload biomarkers. Sixty &beta;-TM patients, aged 3&ndash;12 years old and undergoing blood transfusions, and 30 healthy control children were recruited and CLNK, ferritin and iron status parameters were measured. The results showed a significant increase (p &lt; 0.001) in serum CLNK levels in &beta;-TM patients as compared with normal controls. The increased levels of CLNK were significantly associated with increased ferritin levels. Increased CLNK levels in &beta;-TM may be explained by reciprocal effects between immune signaling and immature erythrocytes, which, release soluble receptors and signaling molecules, including CLNK, in the blood.

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