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

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.

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Quantitative Assessment of Serum Copper Status in Children with Thalassemia Major

Current Journal of Applied Science and Technology ◽

10.9734/cjast/2020/v39i1330685 ◽

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.

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

Journal of Diagnostic Medical Sonography ◽

10.1177/8756479321989662 ◽

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.

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

International Journal of Current Research in Physiology and Pharmacology (IJCRPP) ◽

10.31878/ijcrpp.2020.42.01 ◽

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

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

Children ◽

10.3390/children8020100 ◽

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.

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Nutritional Biomarkers in Children and Adolescents with Beta-Thalassemia-Major: An Egyptian Center Experience

BioMed Research International ◽

10.1155/2014/261761 ◽

2014 ◽

Vol 2014 ◽

pp. 1-7 ◽

Cited By ~ 22

Author(s):

Laila M. Sherief ◽

Sanaa M. Abd El-Salam ◽

Naglaa M. Kamal ◽

Osama El safy ◽

Mohamed A. A. Almalky ◽

...

Keyword(s):

Trace Elements ◽

Folic Acid ◽

Serum Zinc ◽

Serum Levels ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Healthy Children ◽

Multicenter Studies ◽

Thalassemic Patient ◽

Beta Thalassemia Major

Background and Aim. Trace elements and vitamins play a vital role in human body to perform its function properly. Thalassemic patients are at risk of micronutrient deficiency. This study estimated levels of vitamins A, C, E, B12, folic acid, total homocysteine (tHcy), and methylmalonic acid (MMA) along with trace elements, zinc, copper, and selenium in Beta-thalassemia-major patients.Methods. This study included 108 patients with Beta-thalassemia-major and 60 age and sex matched healthy children. Serum levels of vitamin A, E, C, tHcy, and MMA were estimated by high pressure liquid chromatography while serum levels of folic acid and B12 were estimated by thin layer chromatography. Serum zinc, copper, and selenium were determined by atomic absorption spectrometry.Results. There was a significant decrease of vitamins A, C, E, and B12 and trace elements zinc, copper, and selenium in thalassemic patients as compared to controls. tHcy and MMA were significantly elevated in patients. No significant correlations were found between the serum levels of the studied vitamins and trace elements as regards age, frequency of transfusion, duration of transfusion, and serum ferritin.Conclusion. The level of various nutritional biomarkers (vitamins A, C, E, and B12 and trace elements zinc, copper, selenium) was reduced in chronically transfused Egyptian thalassemic patient. These patients should have periodic nutritional evaluation and supplementation. Multicenter studies are highly recommended.

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Pengaruh Multiple Mikro Nutrien (MMN) Terhadap Berat Badan Bayi Baru Lahir Di Desa Pandes Klaten

Jurnal Kebidanan dan Kesehatan Tradisional ◽

10.37341/jkkt.v2i2.56 ◽

2017 ◽

Vol 2 (2) ◽

Author(s):

Dwi Retna Prihati ◽

Gita Kostania

Keyword(s):

Folic Acid ◽

Birth Weight ◽

Treatment Group ◽

Vitamin B12 ◽

Pregnant Women ◽

Control Group ◽

Metabolic System ◽

Cross Sectional ◽

Significant Difference ◽

Newborn Weight

Abstract: MMN, Newborn Baby Weight. During pregnancy food is required with good quality and quantity to meet the nutritional needs of mother and baby. The low nutritional status of pregnant women during pregnancy can lead to various adverse effects for mothers and infants, such as those born with Low Birth Weight (LBW). LBW babies have a 10 to 20 times greater chance of dying than babies born with enough birth weight. Multiple Micro Nutrient (MMN) contains 15 types of vitamins and minerals most important for pregnant women, including vitamin A, vitamin E, vitamin D, vitamin B1, vitamin B2, niacin, Vitamin B6, vitamin B12, folic acid, vitamin C, Fe , folic acid, Zink, Copper, Selenium, and Iodine. MMN is one of the nutrients to prevent the occurrence of anemia because in MMN there are factors forming Hemoglobin ie Fe, Vitamin B12 and folic acid. The availability of adequate hemoglobin makes the metabolic system work well. Lack of hemoglobin not only affects the health of the mother but also affects the health of the fetus it contains, including the growth of the fetal inhibition (such as weight, body length). The purpose of this study was to prove the effect of MMN on newborn weight in Pandes Klaten village. This type of research is arestrospective study with cross sectional design. The subjects of this study were BBL (newborn) whose mother consumed MMN during pregnancy. Different test sing Independent T-test to compare control group and treatment group. Significant value in this study was p <0.05. The results of this study were no significant difference between birth weight between control group and MMN treatment group (P = 0.879). In conclusion MMN has no significant effect on newborn weight gain.

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Study on Serum Vitamin B12 And Folic Acid in Patients of Ischaemic Stroke

Journal of Dhaka Medical College ◽

10.3329/jdmc.v19i2.7080 ◽

1970 ◽

Vol 19 (2) ◽

pp. 115-117

Author(s):

K Kirtania ◽

S Ahmed ◽

N Sultana ◽

MZ Hossain ◽

MM Rahman

Keyword(s):

Folic Acid ◽

Vitamin B12 ◽

Ischaemic Stroke ◽

Large Population ◽

Serum Vitamin ◽

Developed Countries ◽

Control Group ◽

Case Group ◽

Key Words Ischaemic Stroke ◽

The Mean

Context: Stroke is the third commonest cause of death in developed countries and is responsible for the physical disability of a large population. Of two types, ischaemic stroke covers 85% and haemorrhagic stroke is only 15%. Methods: A case control study was designed to see the association of serum vitamin B12 and folic acid level with ischemic stroke. The study was done from January to December 2009 in the Department of Biochemistry, Dhaka Medical College, Dhaka. A total of 60 subjects were selected as study population. Among them 30 were diagnosed case of ischaemic stroke and 30 were age and sex matched healthy control. Results: The mean vitamin B12 and folic acid levels in case group were 231.02±10.81 pg/ml and 2.29±0.54 ng/ml respectively. For control group, the mean vitamin B12 and folic acid levels were 278.72±15.88 pg/ml and 7.24±2.19 ng/ml respectively. Conclusion: The study suggests that low levels of serum vitamin B12 and folic acid are associated with ischaemic stroke. Key words: Ischaemic stroke; vitamin B12; folic acid. DOI: 10.3329/jdmc.v19i2.7080J Dhaka Med Coll. 2010; 19(2) : 115-117

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The effect of bone marrow-derived mesenchymal stem cell co-transplantation with hematopoietic stem cells on liver fibrosis alleviation and survival in patients with class III β-thalassemia major

Stem Cell Research & Therapy ◽

10.1186/s13287-021-02242-8 ◽

2021 ◽

Vol 12 (1) ◽

Author(s):

Tahereh Rostami ◽

Amir Kasaeian ◽

Nasrollah Maleki ◽

Mohsen Nikbakht ◽

Azadeh Kiumarsi ◽

...

Keyword(s):

Stem Cells ◽

Liver Fibrosis ◽

Chronic Gvhd ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Class Iii ◽

Mixed Effect ◽

Hematopoietic Stem ◽

Beta Thalassemia Major ◽

Significant Difference

Abstract Background Hepatic fibrosis is a common complication in transfusion-dependent thalassemia patients. Data on the co-transplantation of mesenchymal stem cells (MSCs) with hematopoietic stem cells (HSCs) in beta-thalassemia major patients are scarce. Therefore, we aimed to evaluate the effect of co-transplantation of bone marrow-derived MSC with HSCs on the liver fibrosis alleviation and transplant outcomes in class III beta-thalassemia major. Methods Between April 1998 and January 2017, a total of 224 consecutive patients with class III beta-thalassemia major underwent allogeneic HSCT in the Research Institute for Oncology, Hematology and Cell Therapy, Tehran University of Medical Sciences, Tehran, Iran. To assess liver fibrotic changes after transplantation, 47 patients participated in the MSC plus HSC group and 30 patients in the HSC only group at the end of the follow-up period. All patients underwent laboratory tests, especially serum ferritin and liver function testing, hepatic T2* MRI, liver biopsy, and FibroScan before and 2 years after transplantation. Kaplan-Meier curves were derived to determine survival and were compared using the log-rank test. Repeated-measure, mixed-effect linear regression models were used to examine the changes in liver fibrosis over time. Results The 10-year OS rate was 71.84% in the mesenchymal group and 61.89% in the non-mesenchymal group (P value = 0.294), while the 10-year TFS rate was 63.64% in the mesenchymal group and 52.78% in the non-mesenchymal group (P value = 0.285). No significant difference was observed in the 10-year NRM, rejection rate, ANC engraftment, platelet engraftment, acute GvHD, and chronic GvHD between the two groups. In addition, the results of repeated-measure, mixed-effect linear regression models showed that none of the variables determining hepatic fibrosis had a significant difference between patients receiving MSCs and patients who did not receive MSCs. Conclusions Based on the results of this study, a single infusion of MSCs at the time of HSCT to patients with class III beta-thalassemia major could not significantly improve the liver fibrosis alleviation and transplantation outcomes, including OS, TFS, TRM, rejection rate, ANC engraftment, platelet engraftment, acute GvHD, and chronic GvHD.

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Dental Arch Dimensions in Subjects with Beta-thalassemia Major

The Journal of Contemporary Dental Practice ◽

10.5005/jp-journals-10024-1071 ◽

2011 ◽

Vol 12 (6) ◽

pp. 429-433 ◽

Cited By ~ 2

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.

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

10.20944/preprints201912.0356.v1 ◽

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 (β-TM) disorder characterized by the lack, or severe reduction in the production of hemoglobin β-globin chains. The standard protocol for the management of β-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 β-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 β-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.

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