Prevalence of low bone mass among adolescents with nontransfusion‐dependent hemoglobin E/β‐thalassemia and its relationship with anemia severity

Nakavachara, Pairunyar; Petchkul, Jaturat; Jeerawongpanich, Krittha; Kiattisakthavee, Pornpimol; Manpayak, Teerarat; Netsakulnee, Parichat; Chaichanwattanakul, Katharee; Pooliam, Julaporn; Srichairatanakool, Somdet; Viprakasit, Vip

doi:10.1002/pbc.26744

Cited by 15 publications

(15 citation statements)

References 37 publications

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“…The mechanism of the iron load is likely to be associated with gastro-intestinal (GI) absorption. With increased GI absorption of iron, iron overload occurs late in NTDT patients, leading to a series of complications linked to endocrine disorders including glucose metabolism disorder,4,8 adrenal insufficiency,28 vitamin D deficiency and low bone mass 29,30. It is known that abnormal glucose homeostasis is a fairly common complication of this disease 6.…”

Section: Discussionmentioning

confidence: 99%

<p>Prevalence of abnormal glucose homeostasis in Chinese patients with non-transfusion-dependent thalassemia</p>

Luo¹,

Bajoria²,

Lai³

et al. 2019

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Purpose: To determine the prevalence and underlying pathology of abnormal glucose homeostasis in Chinese patients with non-transfusion-dependent thalassemia (NTDT). Patients and methods: In this study, we enrolled 211 patients aged 4–63 years with NTDT, including 79 β thalassemia intermedia patients, 114 Hb H disease patients and 18 Hb E/β thalassemia patients. All had oral glucose tolerance test, serum ferritin (SF), homeostasis model assessment (HOMA) and liver iron concentration (LIC) measurement. One hundred and twenty healthy age-matched controls were also used for the comparative purpose. Iron load was assessed by using SF and hepatic load by LIC using validated MRI techniques. Results: The 211 patients were divided into three groups according to their fasting and 2 hrs postprandial blood glucose levels: hypoglycemic, normal glucose tolerance (NGT) and hyperglycemic groups. In this study, 149 patients had NGT, 33 had hypoglycemia, 4 had diabetes and 25 had impaired glucose tolerance (IGT). None had impaired fasting glucose. There was a significant correlation between 2 hrs postprandial blood glucose levels and age, PINS120, HOMA-IR, alanine aminotransferase and LIC ( P <0.05). Risk factors for IGT in NTDT patients were older age (≥24 years) and SF concentration of ≥2,500 ng/mL. Conclusion: Age ≥24 years and SF ≥2,500 ng/mL of NTDT patients were at a greater risk for impaired glucose tolerance.

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Section: Discussionmentioning

confidence: 99%

<p>Prevalence of abnormal glucose homeostasis in Chinese patients with non-transfusion-dependent thalassemia</p>

Luo¹,

Bajoria²,

Lai³

et al. 2019

DMSO

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“…We used low bone mass (osteopenia and osteoporosis) for multivariate analysis and found that a higher ferritin (>1000 ng/mL) was significantly associated with low bone mass (RR = 4.6, P = .043) and higher Hb (>9 g/dL) was protective for low bone mass (RR = 0.07, P = .031, data not shown). Low bone mass has a complex nature involving several factors including vitamin D status, calcium intake, levels of osteoclast activity, iron overload, and history of iron chelation . Recently, our group reported that low bone mass can develop as early as prepubertal and pubertal age in NTDT patients, and baseline Hb level was associated with low bone mass.…”

Section: Discussionmentioning

confidence: 99%

A prospective analysis for prevalence of complications in Thai nontransfusion‐dependent Hb E/β‐thalassemia and α‐thalassemia (Hb H disease)

2018

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Recently, complications in patients with nontransfusion-dependent thalassemia (NTDT), in particular those with β-thalassemia intermedia (β-TI), were found to be significantly different from those in patients with transfusion dependent thalassemia (TDT), mainly β-thalassemia major (β-TM). However, this information is rather limited in other forms of NTDT. In this prospective study, adult Thai NTDT patients were interviewed and clinically evaluated for thalassemia related complications. Fifty-seven NTDT patients (age 18-74 years), 59.6% Hb E/β-thalassemia and 40.4% Hb H disease, were recruited; 26.4% were splenectomized. The most common complications were gallstones (68.4%), osteoporosis (26.3%), and pulmonary hypertension (15.8%). Splenectomy was associated with higher rate of gallstones and serious infection (P = .001 and .052, respectively), consistent with a multivariate analysis (RR = 9.5, P = .044, and RR = 15.1, P = .043, respectively). In addition, a higher hemoglobin level was inversely associated with gallstones in both univariate and multivariate analyses (P = .01 and .022, respectively). Serum ferritin was associated with abnormal liver function (P = .002). In contrast to the previous study, the prevalence of thrombosis was less common in our population (1.7%), probably due to differences in transfusion therapy, ethnicity, and underlying genotypes. For the first time, this prospective study provided the current prevalence of NTDT related complications in a Southeast Asian population with a different underlying genetic basis compared with previous studies. Although individual prevalence of each complication might differ from other studies, several important clinical factors such as splenectomy, degree of anemia, and iron overload seem to be determining risks of developing these complications consistently across different ethnicities.

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“…In measuring the density by Dual-Energy X-ray Absorptiometry each person has three criteria: first index ( Z ): actually indicates Compare between patient׳s bone density and bone density persons are same age and gender. The second index ( T ): the comparison between the patient׳s bone density and bone density persons are same age [5] , [6] , [7] .…”

Section: Experimental Design Materials and Methodsmentioning

confidence: 99%

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

Abbasi

Asl

Zadeh³

et al. 2018

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The aim of the study is determining bone mineral density (BMD) of Patients with beta thalassemia in order to find the prevalence and related factors on the conditions. Z-Score of femoral neck and lumbar vertebrae were reported comparing normal matched subjects. Age and bone mineral density were significantly correlated. Moreover, the disease had significantly higher severity in men than in women. A negative significant correlation was detected between BMD and the mean of hematocrit in the last 5 years. There was significant differences between sex hormone and bone density. A significant correlation between hydroxy urea and BMD were found. A significant relationship between the use of bisphosphonates and bone density were found. Osteopenia and osteoporosis were highly prevalent in our participants. Therefore, regular tests are required to examine bone mineral density in these patients. Furthermore, the exact effect of age on bone mineral density need to be clarified.

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Prevalence of low bone mass among adolescents with nontransfusion‐dependent hemoglobin E/β‐thalassemia and its relationship with anemia severity

Cited by 15 publications

References 37 publications

<p>Prevalence of abnormal glucose homeostasis in Chinese patients with non-transfusion-dependent thalassemia</p>

<p>Prevalence of abnormal glucose homeostasis in Chinese patients with non-transfusion-dependent thalassemia</p>

A prospective analysis for prevalence of complications in Thai nontransfusion‐dependent Hb E/β‐thalassemia and α‐thalassemia (Hb H disease)

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

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