Normal lumbar bone mineral density in optimally treated children and young adolescents with ?-thalassaemia major

Christoforidis, Athanasios; Kazantzidou, Eirini; Tsatra, Ioanna; Tsantali, Haido; Koliakos, George; Hatzipantelis, Emmanuel; Katzos, George; Athanassiou‐Metaxa, Miranda

doi:10.14310/horm.2002.1111030

Cited by 21 publications

(14 citation statements)

References 27 publications

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“…Also the study showed no significant difference between males and females as far as bone mineral density is concerned. This was in agreement with Karimi, et al 17 and Shamshirsaz, et al 18 , but not with others, [19][20][21][22][23][24][25] who reported lower BMD in males. This may indicate more severe changes in females in our study, which could be due to delayed puberty in patients as amenorrhea and hypogonadism have a greater impact on osteoporosis in females than in males.…”

Section: Discussionsupporting

confidence: 90%

Bone mineral density in beta thalassemia syndrome in Mosul city

Al-Khero¹,

Salih²

2013

Ann Coll Med Mosul

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Bone mineral density in beta thalassemia.. ABSTRACTObjectives: To assess bone mineral density in βthalassemia major (TM) patients and its relation with gender, age, hemoglobin (Hb), calcium, ferritin, body mass index (BMI), chelation therapy, and splenectomy. Patients and methods: Randomised cross-sectional study of 52 patients with beta thalassemia major (TM) from Thalassemia Center in Ibn-Alatheer Teaching Hospital (32 males and 20 females) with age between 3 and 30 years scanned for bone mineral density (BMD) at lumbar spine with dual-energy X-ray absorptiometry (DEXA) scan at DEXA unit in Ibn-Sena Teaching Hospital from September 2010 to December 2010. The information about chelation therapy and splenectomy were obtained from the patients with measurement of height and weight and blood samples for hemoglobin (Hb), calcium and ferritin were taken. Results: All 52 patients had T-score in osteoporotic range (100%), however, Z-score osteoporosis was seen in 26 (50%) and osteopenia in 19 (36.5%). Bone density was normal in only 7 (13.4%). All patients had elevated ferritin levels (100%), 36 (69.2%) had low body mass index (BMI) and 32 (61.5%) had low hemoglobin (Hb) levels. Twenty six (50%) had low calcium levels and 2 (3.8%) were not using chelation therapy. Sixteen (30.7%) had delayed puberty and the rest 36 (69.2%) were in prepubertal stage. Twelve patients (23%) had splenectomy. Conclusion: There is high incidence of low BMD in beta thalassemia (100% by T-score and 86.5% by Zscore) with significant association with age (P-value0.000), low BMI (P-value 0.038), low Hb (P-value 0.022), delayed sexual maturity (P-value 0.000), and splenectomy (P-value 0.000).Bone mineral density in beta thalassemia..

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Section: Discussionsupporting

confidence: 90%

Bone mineral density in beta thalassemia syndrome in Mosul city

Al-Khero¹,

Salih²

2013

Ann Coll Med Mosul

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“…Adolescent patients with transfusion-dependent thalassemia had suboptimal BMD with 61.3% of patients demonstrating a Z score of less than -2 and 22.6% having a Z score between -1 and -2 (56). In contrast, in a study of children and adolescents (5 -20 years) with ␤-thalassemia major who received regular transfusion and chelation therapy, Z scores were within normal range for all subjects with a mean Z score of 0.42 for females and -0.41 for males (P ϭ .018) (57).…”

Section: A Low Bone Mass and Strengthmentioning

confidence: 83%

Bone Disease in Thalassemia: A Molecular and Clinical Overview

et al. 2016

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Thalassemia bone disease is a common and severe complication of thalassemia-an inherited blood disorder due to mutations in the α or β hemoglobin gene. In its more severe form, severe anemia is present, and treatment with frequent red blood cell transfusion is necessary. Because the body has limited capacity to excrete iron, concomitant iron chelation is required to prevent the complications of iron overload. The effects of chronic anemia and iron overload can lead to multiple end-organ complications such as cardiomyopathy, increased risks of blood-borne diseases, and liver, pituitary, and bone disease. However, our understanding of thalassemia bone disease is incomplete and is composed of a complex piecemeal of risk factors that include genetic factors, hormonal deficiency, marrow expansion, skeletal dysmorphism, iron toxicity, chelators, and increased bone turnover. The high prevalence of bone disease in transfusion-dependent thalassemia is seen in both younger and older patients as life expectancy continues to improve. Indeed, hypogonadism and GH deficiency contribute to a failure to achieve peak bone mass in this group. The contribution of kidney dysfunction to bone disease in thalassemia is a new and significant complication. This coincides with studies confirming an increase in kidney stones and associated accelerated bone loss in the thalassemia cohort. However, multiple factors are also associated with reduced bone mineral density and include marrow expansion, iron toxicity, iron chelators, increased bone turnover, GH deficiency, and hypogonadism. Thalassemia bone disease is a composite of not only multiple hormonal deficiencies but also multiorgan diseases. This review will address the molecular mechanisms and clinical risk factors associated with thalassemia bone disease and the clinical implications for monitoring and treating this disorder.

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“…These findings are in parallel with the data published by Christoforidis et al . [22], who demonstrated a delay in bone mass acquisition with advancing age in the thalassaemic group compared to controls.…”

Section: Discussionmentioning

confidence: 99%

Predictors of bone disease in Egyptian prepubertal children with β-thalassaemia major

Tantawy¹,

El-Bostany²,

Matter³

et al. 2010

aoms

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IntroductionThalassaemic osteopathy is a multifactorial disorder and limited information exists about bone accrual and bone mineral density (BMD) in prepubertal thalassaemic children. The study aimed to investigate some potential genetic and biochemical bone markers as possible early predictors of BMD variations in children with β-thalassaemia major (TM) before puberty.Material and methodsThirt-one prepubertal children with β-TM, and 43 matched controls were subjected to BMD assessment by dual energy X-ray absorptiometry (DEXA). Vitamin D receptor (VDR) gene polymorphisms (Bsm1, Fok1) and the biochemical bone markers serum osteocalcin and propeptide I procollagen (CPIP) and urinary deoxypyridinoline (DPD) excretion were assessed.ResultsBone mineral density was reduced in 25% of thalassaemics at the spine and 15.4% at the hip region. Significantly higher levels of urinary DPD and lower serum osteocalcin and CPIP levels were found in the studied thalassaemic children compared to controls (p < 0.001). A significant negative correlation was present between BMD in spine and hip and the patients’ age (r = −0.6367, p = 0.0002 and r = −0.616, p = 0.00079, respectively). There was a significant reduction in BMD in males compared to females. Reduced BMD was more frequent in male patients with genotypes bb and Ff but not in females. Bone mineral density was not related to the studied biochemical bone markers, mean pre-transfusion haemoglobin or serum ferritin.ConclusionsRoutine BMD screening with DEXA is proposed to be a sensitive predictor for early bone changes, particularly at the lumbar spine. DR gene polymorphisms of Bsm1 and Fok1 polymorphisms may be determinants of BMD in Egyptian prepubertal male thalassemics

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Normal lumbar bone mineral density in optimally treated children and young adolescents with ?-thalassaemia major

Cited by 21 publications

References 27 publications

Bone mineral density in beta thalassemia syndrome in Mosul city

Bone mineral density in beta thalassemia syndrome in Mosul city

Bone Disease in Thalassemia: A Molecular and Clinical Overview

Predictors of bone disease in Egyptian prepubertal children with β-thalassaemia major

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