Differences in the prevalence of growth, endocrine and vitamin D abnormalities among the various thalassaemia syndromes in North America.
Differences in the prevalence of growth, endocrine and vitamin D abnormalities among the various thalassaemia syndromes in North America.
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DOI：
10.1111/j.1365-2141.2009.07793.x
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发表时间：
2009-09
影响因子：
6.5
通讯作者：
Thalassemia Clinical Research Network
中科院分区：
文献类型：
--
作者：
Vogiatzi MG;Macklin EA;Trachtenberg FL;Fung EB;Cheung AM;Vichinsky E;Olivieri N;Kirby M;Kwiatkowski JL;Cunningham M;Holm IA;Fleisher M;Grady RW;Peterson CM;Giardina PJ;Thalassemia Clinical Research Network
目的：确定北美目前治疗的不同地中海贫血综合征患者的生长、内分泌和钙相关异常的比率的差异。地中海贫血临床研究网络中所有6岁及以上地中海贫血综合征患者的病史、体检和血液和尿液采集均来自地中海贫血临床研究网络。361名受试者中，49%为男性，平均年龄23.2岁(6.1~75岁)。大约25%的儿童和成人，无论是地中海贫血综合征，都有矮小的身材。儿童的总体发育受到轻微影响。终末身高接近中亲身高(z=-0.73±1.24)。重度β地中海贫血(TM)患者性腺功能减退、多种内分泌疾病、高血糖、亚临床甲状旁腺功能减退、高钙尿发生率较高。性腺功能减退症仍然是最常见的内分泌疾病，而且经常得不到治疗。无论地中海贫血综合征如何，12.8%的受试者维生素D浓度低于27nmol/L，82%的受试者维生素D浓度低于75nmol/L。青少年的维生素D水平低于儿童和成人。与其他地中海贫血综合征患者相比，βTM患者的多发性内分泌疾病、钙代谢异常和高钙尿症的发生率更高。维生素D异常在青少年中很高。
To determine differences in the rates of growth, endocrine and calcium related abnormalities in the various thalassemia syndromes in North America with current therapy. Medical history, physical examinations and blood and urine collections were obtained from patients with all thalassemia syndromes age 6 years and older in the Thalassemia Clinical Research Network. 361 subjects, 49% male, mean age 23.2 years (range 6.1 to 75 years) were studied. Approximately 25% of children and adults, regardless of the thalassemia syndrome, had short stature. Overall growth in children was mildly affected. Final height was close to midparental height (z = -0.73 ± 1.24). Patients with beta thalassemia major (TM) had higher rates of hypogonadism, multiple endocrinopathies, worse hyperglycemia, subclinical hypoparathyroidism and hypercalciuria. Hypogonadism remained the most frequent endocrinopathy and was frequently under-treated. 12.8% of the subjects had 25 vitamin D concentrations less than 27nmol/L and 82% less than 75nmol/L, regardless of the thalassemia syndrome. Adolescents had lower 25 vitamin D levels than children and adults. Compared to patients with other thalassemia syndromes, those with beta TM suffer from higher rates of multiple endocrinopathies, abnormal calcium metabolism and hypercalciuria. Vitamin D abnormalities are high among adolescents.
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影响因子：
3.6
作者：
Roth, C;Pekrun, A;Schroter, W
通讯作者：
Schroter, W
thalalsyaine骨骼疾病：一个频繁且仍未解决的问题。
DOI：
10.1359/jbmr.080505
发表时间：
2009-03
期刊：
Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research
影响因子：
--
作者：
Vogiatzi MG;Macklin EA;Fung EB;Cheung AM;Vichinsky E;Olivieri N;Kirby M;Kwiatkowski JL;Cunningham M;Holm IA;Lane J;Schneider R;Fleisher M;Grady RW;Peterson CC;Giardina PJ;Thalassemia Clinical Research Network
通讯作者：
Thalassemia Clinical Research Network
影响因子：
3.1
作者：
Shalitin, S;Carmi, D;Tamary, H
通讯作者：
Tamary, H
影响因子：
4.1
作者：
Vogiatzi, MG;Macklin, EA;Giardina, PJ
通讯作者：
Giardina, PJ
影响因子：
20.3
作者：
Cunningham, MJ;Macklin, EA;Cohen, AR
通讯作者：
Cohen, AR