Measurement bone mineral density (BMD) of patients with beta thalassemia

Abbasi, Saeed; Asl, Jafar Fatahi; Zadeh, Leila Moein; Mirdoraghi, Mohammad

doi:10.1016/j.dib.2018.05.120

Cited by 7 publications

(7 citation statements)

References 6 publications

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“…However, the findings of this study are corroborative of other Indian studies where either adolescents or a combination of adolescents and adult patients were included as the sample [ 6 - 8 ]. Our results support the findings on BMD in thalassemia patients by Aslan et al from Turkey [ 11 ]; Abbasi et al, Bekheirnia et al, and Shamshirsaz et al from Iran [ 12 - 14 ]; Domrongkitchaiporn et al from Thailand [ 15 ]; Soliman et al from Egypt [ 16 ]; Vogiatzi et al from the US [ 17 ]; and Tharwat et al from Saudi Arabia [ 18 ].…”

Section: Discussionsupporting

confidence: 91%

Bone Mineral Density, Serum Calcium, and Vitamin D Levels in Adult Thalassemia Major Patients: Experience From a Single Center in Eastern India

Santra¹,

Sharma²,

Dash³

et al. 2022

Cureus

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Background and objectivePatients suffering from thalassemia major are at higher risk of osteoporosis. Due to their decreased life expectancy, the number of adult patients is low. However, their bone health is rarely checked in developing countries like India. There is no data available in the literature on the bone mineral density (BMD) of adult (aged ≥18 years) thalassemia major patients in eastern India. In this study, we aimed to measure the BMD and serum calcium and vitamin D levels in adult thalassemia major patients and to compare them with healthy controls. Materials and methodsWe conducted this cross-sectional observational study at a tertiary care hospital in eastern India. We recruited adult thalassemia major patients who were not on calcium or vitamin D supplements. Their BMD was measured by dual-energy X-ray absorptiometry (DXA) on the lumbar spine (L1-L4). Venous blood was tested for serum calcium and vitamin D levels. We compared the parameters between the cases and controls by using the Mann-Whitney U test. ResultsA total of 31 (male = 19, female = 12) patients with a median age of 28 years comprised the case group. Ageand sex-matched controls showed similar height but higher weight and BMI. The serum calcium level was similar (p = 0.43) in the case and control groups but T-score (p = 0.0003) and vitamin D levels (p: <0.0001) were significantly lower in thalassemia major patients. ConclusionBased on our findings, adult thalassemia major patients have lower BMD and vitamin D levels. Although the serum calcium may be normal in these patients, they should still be screened both for BMD and vitamin D for prompt and early detection of risks and complications so that a proper management strategy can be implemented.

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Section: Discussionsupporting

confidence: 91%

Bone Mineral Density, Serum Calcium, and Vitamin D Levels in Adult Thalassemia Major Patients: Experience From a Single Center in Eastern India

Santra¹,

Sharma²,

Dash³

et al. 2022

Cureus

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“…Hypogonadism was related to a lower Z-score in the lumbar spine, while low serum IGF-1 levels and changes in serum phosphate levels only affected the femoral neck. These results were consistent with those of previous studies (5,(15)(16)(17)(18).…”

Section: Discussionsupporting

confidence: 94%

“…Therefore, recognition of the significant risk factors of osteoporosis is crucial for identification of high-risk patients. Features previously discovered to have a role in bone metabolism include serum ferritin, type of iron chelator, zinc levels, levels of insulin-like growth factor-1 (IGF-1), hypothyroidism and calcium levels, physical activity and high caloric intake measured indirectly by body mass index (BMI) (14)(15)(16)(17). However, the relationships between IGF-1, hematocrit, vitamin D and BMD are still being debated (15)(16)(17)(18).…”

Section: Introductionmentioning

confidence: 99%

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“…As the therapy of choice for secondary OP, we mention bisphosphonates and denosumab; as primary prevention, the hormonal replacement therapy for hypogonadism is helpful [ 104 , 105 ]. One randomised, placebo-controlled, phase 2b study on denosumab (N1 = 32 patients treated with 60 mg denosumab every 6 months versus N2 = 31 individuals with placebo, for 1 year) evaluated for the first time in subjects with transfusion-dependent TH noggin levels, an antagonist of bone morphogenetic protein as promotor of bone formation.…”

Section: Thalassemic Endocrine Diseasementioning

confidence: 99%

New Entity—Thalassemic Endocrine Disease: Major Beta-Thalassemia and Endocrine Involvement

et al. 2022

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Beta-thalassemia (BTH), a recessively inherited haemoglobin (Hb) disorder, causes iron overload (IO), extra-medullary haematopoiesis and bone marrow expansion with major clinical impact. The main objective of this review is to address endocrine components (including aspects of reproductive health as fertility potential and pregnancy outcome) in major beta-thalassemia patients, a complex panel known as thalassemic endocrine disease (TED). We included English, full-text articles based on PubMed research (January 2017–June 2022). TED includes hypogonadism (hypoGn), anomalies of GH/IGF1 axes with growth retardation, hypothyroidism (hypoT), hypoparathyroidism (hypoPT), glucose profile anomalies, adrenal insufficiency, reduced bone mineral density (BMD), and deterioration of microarchitecture with increased fracture risk (FR). The prevalence of each ED varies with population, criteria of definition, etc. At least one out of every three to four children below the age of 12 y have one ED. ED correlates with ferritin and poor compliance to therapy, but not all studies agree. Up to 86% of the adult population is affected by an ED. Age is a positive linear predictor for ED. Low IGF1 is found in 95% of the population with GH deficiency (GHD), but also in 93.6% of persons without GHD. HypoT is mostly pituitary-related; it is not clinically manifested in the majority of cases, hence the importance of TSH/FT4 screening. HypoT is found at any age, with the prevalence varying between 8.3% and 30%. Non-compliance to chelation increases the risk of hypoT, yet not all studies confirmed the correlation with chelation history (reversible hypoT under chelation is reported). The pitfalls of TSH interpretation due to hypophyseal IO should be taken into consideration. HypoPT prevalence varies from 6.66% (below the age of 12) to a maximum of 40% (depending on the study). Serum ferritin might act as a stimulator of FGF23. Associated hypocalcaemia transitions from asymptomatic to severe manifestations. HypoPT is mostly found in association with growth retardation and hypoGn. TED-associated adrenal dysfunction is typically mild; an index of suspicion should be considered due to potential life-threatening complications. Periodic check-up by ACTH stimulation test is advised. Adrenal insufficiency/hypocortisolism status is the rarest ED (but some reported a prevalence of up to one third of patients). Significantly, many studies did not routinely perform a dynamic test. Atypical EM sites might be found in adrenals, mimicking an incidentaloma. Between 7.5–10% of children with major BTH have DM; screening starts by the age of 10, and ferritin correlated with glycaemia. Larger studies found DM in up to 34%of cases. Many studies do not take into consideration IGF, IGT, or do not routinely include OGTT. Glucose anomalies are time dependent. Emerging new markers represent promising alternatives, such as insulin secretion-sensitivity index-2. The pitfalls of glucose profile interpretation include the levels of HbA1c and the particular risk of gestational DM. Thalassemia bone disease (TBD) is related to hypoGn-related osteoporosis, renal function anomalies, DM, GHD, malnutrition, chronic hypoxia-induced calcium malabsorption, and transplant-associated protocols. Low BMD was identified in both paediatric and adult population; the prevalence of osteoporosis/TBD in major BTH patients varies; the highest rate is 40–72% depending on age, studied parameters, DXA evaluation and corrections, and screening thoracic–lumbar spine X-ray. Lower TBS and abnormal dynamics of bone turnover markers are reported. The largest cohorts on transfusion-dependent BTH identified the prevalence of hypoGn to be between 44.5% and 82%. Ferritin positively correlates with pubertal delay, and negatively with pituitary volume. Some authors appreciate hypoGn as the most frequent ED below the age of 15. Long-term untreated hypoGn induces a high cardiovascular risk and increased FR. Hormonal replacement therapy is necessary in addition to specific BTH therapy. Infertility underlines TED-related hormonal elements (primary and secondary hypoGn) and IO-induced gonadal toxicity. Males with BTH are at risk of infertility due to germ cell loss. IO induces an excessive amount of free radicals which impair the quality of sperm, iron being a local catalyser of ROS. Adequate chelation might improve fertility issues. Due to the advances in current therapies, the reproductive health of females with major BTH is improving; a low level of statistical significance reflects the pregnancy status in major BTH (limited data on spontaneous pregnancies and growing evidence of the induction of ovulation/assisted reproductive techniques). Pregnancy outcome also depends on TED approach, including factors such as DM control, adequate replacement of hypoT and hypoPT, and vitamin D supplementation for bone health. Asymptomatic TED elements such as subclinical hypothyroidism or IFG/IGT might become overt during pregnancy. Endocrine glands are particularly sensitive to iron deposits, hence TED includes a complicated puzzle of EDs which massively impacts on the overall picture, including the quality of life in major BTH. The BTH prognostic has registered progress in the last decades due to modern therapy, but the medical and social burden remains elevated. Genetic counselling represents a major step in approaching TH individuals, including as part of the pre-conception assessment. A multidisciplinary surveillance team is mandatory.

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Measurement bone mineral density (BMD) of patients with beta thalassemia

Cited by 7 publications

References 6 publications

Bone Mineral Density, Serum Calcium, and Vitamin D Levels in Adult Thalassemia Major Patients: Experience From a Single Center in Eastern India

Bone Mineral Density, Serum Calcium, and Vitamin D Levels in Adult Thalassemia Major Patients: Experience From a Single Center in Eastern India

Table_1.docx

New Entity—Thalassemic Endocrine Disease: Major Beta-Thalassemia and Endocrine Involvement

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