Vitamin D Deficiency in Children with Beta Thalassemia Major and Intermedia

2013 ◽  
Vol 33 (4) ◽  
pp. 1058-1063 ◽  
Author(s):  
Canan ALBAYRAK ◽  
Davut ALBAYRAK
Keyword(s):  
Vitamin D ◽  
Vitamin D Deficiency ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Beta Thalassemia Major

Fok‐I gene polymorphism of vitamin D receptor in patients with beta‐thalassemia major and its effect on vitamin D status

Hematology ◽  
2011 ◽  
Vol 16 (1) ◽  
pp. 54-58 ◽  
Author(s):  
Meropi Dimitriadou ◽  
Athanasios Christoforidis ◽  
Liana Fidani ◽  
Marina Economou ◽  
Vassilios Perifanis ◽  
...  
Keyword(s):  
Vitamin D ◽  
Gene Polymorphism ◽  
Vitamin D Receptor ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Vitamin D Status ◽  
Beta Thalassemia Major ◽  
I Gene

scholarly journals Assessment of serum vitamin D levels in Egyptian children with beta-thalassemia major

2021 ◽  
Vol 69 (1) ◽  
Author(s):  
Ghada Saad Abdelmotaleb ◽  
Ola Galal Behairy ◽  
Khalid Eid Abd El Azim ◽  
Dalia Mohamed Abd El-Hassib ◽  
Tasbeh Mohamed Hemeda
Keyword(s):  
Body Mass Index ◽  
Vitamin D ◽  
Body Mass ◽  
Serum Calcium ◽  
Serum Levels ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Beta Thalassemia Major ◽  
Vitamin D Levels ◽  
25 Oh Vitamin D

Abstract Background Beta-thalassemia major patients are at increased risk of complications including endocrinopathies and bone disease due to iron overload. So, this study aimed to assess the growth parameters, serum levels of 25-OH-vitamin D, calcium, and phosphorous in children with beta-thalassemia major. This was a case-control study that included 55 children with beta-thalassemia major compared with 30 sex- and age-matched healthy children that served as a control group. All enrolled children were subjected to full history taking, clinical examination including anthropometric measurements, and laboratory investigations including complete blood count, serum ferritin, levels of serum calcium, phosphorus, and 25-OH-vitamin D. Results Body mass index was statistically significantly lower in the thalassemic group (P < 0.001). It was observed that vitamin D levels were significantly lower in thalassemic patients than in controls (P value < .0001). The mean serum 25-OH-vitamin D levels were 19.84 ± 5.79 ng/ml and 44.98 ± 5.77 ng/ml, respectively; 22 cases (40%) had insufficient vitamin D, and 5 cases (9%) had deficient vitamin D. Regarding serum calcium and phosphorous, there was no significant difference between the thalassemic and control groups. Conclusion Children with beta-thalassemia major had low body mass index and metabolic abnormality in the form of lower serum levels of vitamin D that signify the importance of therapeutic interventions.

scholarly journals Correlation between 25-Hydroxyvitamin D and Lipid Profile among Children with Beta Thalassemia Major

2018 ◽  
Vol 6 (10) ◽  
pp. 1790-1794
Author(s):  
Christian Nasir ◽  
Nelly Rosdiana ◽  
Aridamuriany Dwiputri Lubis
Keyword(s):  
Vitamin D ◽  
Lipid Profile ◽  
Total Cholesterol ◽  
Thalassemia Major ◽  
Atherogenic Index ◽  
Beta Thalassemia ◽  
P Value ◽  
Healthy Children ◽  
Positive Correlation ◽  
Beta Thalassemia Major

BACKGROUND: Beta thalassemia major is associated with lipid profile abnormalities, presented as a lower level of total cholesterol (TC), low-density lipoproteins (LDL), high-density lipoprotein (HDL), and higher triglyceride level; increasing risk for cardiovascular complications. The previous studies indicated that Vitamin D give a positive impact on the lipid profile in healthy children population. However, its role needs to be determined in a high-risk group of children with beta-thalassemia major. AIM: To determine the correlation between vitamin D (25-OHD) and lipid profile among children with beta-thalassemia major. METHODS: A cross-sectional study was conducted in a general tertiary hospital in Medan, Sumatera Utara, Indonesia from January to March 2018. Subjects were children aged below 18-year-old with beta-thalassemia major. The measurement of vitamin D (25-OHD) level and 10-12 hour overnight fasting serum lipid profile including total cholesterol, triglyceride, HDL, and LDL were performed. The analysis was done using Pearson’s correlation and Fisher test. P value < 0.05 was considered significant. RESULTS: Forty-five subjects were enrolled in this study, with serum ferritin level ranged from 1017 to 13372 ng/mL. The prevalence of vitamin D deficiency (a 25-OHD level less than 20 ng/mL) in this study was 40%, with mean value at 20.6 (SD 5.3) ng/mL. The markers for cardiovascular risk were observed to be elevated, both in Atherogenic Index Plasma (0.32 ± 0.25) and TC: HDL ratio (4.2 ± 1.5). Statistical analysis revealed that Vitamin D had positive correlation with total cholesterol (r = 0.302, p = 0.044) and HDL (r = 0.297, p = 0.048). There was no significant correlation between both vitamin D and triglyceride (p = 0.305), or vitamin D and LDL (p = 0.727). CONCLUSION: Vitamin D correlated positively with total cholesterol and HDL in children with beta-thalassemia major. Positive correlation to HDL indicated a beneficial effect of vitamin D to reduce the risk of cardiovascular complication.

Nephrolithiasis in Transfusion-Dependent Pediatric Patients

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4895-4895
Author(s):  
Joanne Yacobovich ◽  
Irit Krause ◽  
Rotem Semo-Oz ◽  
Hannah Tamary
Keyword(s):  
Vitamin D ◽  
Pediatric Patients ◽  
Renal Stones ◽  
Thalassemia Major ◽  
Small Sample ◽  
Beta Thalassemia ◽  
Thalassemia Intermedia ◽  
Urine Calcium ◽  
Beta Thalassemia Major ◽  
Vitamin D Levels

Abstract Background: The prolonged survival in patients with beta thalassemia major and other transfusion dependent disorders has exposed the multi-organ effects of multiple transfusions and chelation therapy. Renal deficits were described with the focus on tubular and glomerular function. Nephrolithiasis has not been systematically explored in these patients. Contributing factors to stone formation may include hypercalcuria, abnormal vitamin D homeostasis, and proximal tubulopathy. Objective: We analyzed hematologic and nephrologic parameters of 22 transfusion- dependent pediatric patients to investigate contributors to nephrolithiasis. Methods: The study included patients with beta thalassemia major (TM) (13), transfusion-dependent beta thalassemia intermedia (TDTI) (4), Diamond-Blackfan anemia (DBA) (3), other transfusion-dependent hemoglobinopathies (2). Patients' ages were from 4-20 years (overall median -11 years, TM/TDTI median - 11.5, DBA median - 7.5 years). At the time of analysis 11 received iron chelation with deferasirox, 8 with combined desferoxamine & deferiprone, 2 with combined desferoxamine & deferasirox and one patient received desferoxamine alone. All patients had previously been treated with deferasirox alone and due to either significant adverse events or failure to control iron overload were switched to an alternate regimen. Seven patients were treated with vitamin D supplements for a median of 3 years (range: 0.2-4.3), 6 patients received vitamin D+calcium for a median of 4.5 years (range: 4.3-10). Venous blood samples, 24-hour urine collections and spot urine samples were collected to assess urine chemistry, vitamin D metabolism, electrolytes and renal function. Renal sonography and directed computerized tomography were used to diagnose nephrolithiasis/nephrocalcinosis. Results: Three patients, all with TM/TDTI, had symptomatic and radiographically identified renal stones, an additional TM patient had symptomatic nephrocalcinosis (Table 1). Among the entire cohort hypercalcuria was identified in 75% with increased Ca/Cr in 68%. 25(OH)D3 was abnormal in 81% of patients, mostly in the "insufficient" range (25-75 mmol/L). No significant relationship between presence of nephrolithiasis and chelation regimen, Ca mg/kg/d, Ca/Cr, renal fractional excretion (Fe) of phosphate (FePhos(, FeNa, FeUA, oxalate, cystein, citrate, 25(OH)D3, or treatment with vitamin D± calcium was identified, possibly in part due to the small sample size. Conclusions: The incidence rate of nephrolithiasis in our pediatric transfusion-dependent population was 1000x that published in various pediatric populations. Hypercalcuria and relatively low vitamin D levels were found in the majority of our patients, notably in all with nephrolithiasis. All 4 patients with nephrolithiasis/calcinosis were ≥ 13 years old, receiving vitamin D±calcium supplements and had received deferasirox chelation. Of note none of the patients not receiving vitamin D±calcium therapy presented with renal stones or calcinosis. The pathophysiology of nephrolithiasis/calcinosis is unclear among these adolescents; however the role of vitamin D±calcium supplemental therapy and the contribution of deferasirox must be further studied to find the proper formulation needed to maintain bone health without increasing the risk for renal stones. Abstract 4895. Table 1- Characteristics of patients with nephrolithiasis/nephrocalcinosis Patient Diagnoses Age at onset (years) Chelation (at onset) Duration of vitamin D+Ca supplement (years) Low vitamin D levels (mmol/L) Urine calcium (mg/kg/d) 1 TM/NL 16 DFX 2.3 + 7.6 2 TM/NL 16 DFX 5 - 6.9 3 TDTI/NL 13 DFO&DFP (prior DFX) 0.25 (vitamin D only) + 4.4 4 TM/NC 18 DFX 4 + 2.7 TM-thalassemia major, TDTI-transfusion-dependent thalassemia intermedia, NL-nephrolithiasis, NC-nephrocalcinosis, DFX-deferasirox, DFO-desferoxamine, DFP-deferiprone, Ca-calcium, normal values: vitamin D>75mmol/L, urine calcium<4mg/kg/d Disclosures No relevant conflicts of interest to declare.

Evaluation of osteopathy and vitamin D status in patients with beta-thalassemia major

2019 ◽  
Vol 0 (0) ◽  
pp. 0-0
Author(s):  
mohamed qwader ◽  
laila sherief ◽  
Adel Sherif ◽  
Ahmed Baraka
Keyword(s):  
Vitamin D ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Vitamin D Status ◽  
Beta Thalassemia Major
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