Bone mineral density, biochemical and hormonal profiles in suboptimally treated children and adolescents with β-thalassaemia disease

被引:57
作者
Mahachoklertwattana, P [1 ]
Chuansumrit, A
Sirisriro, R
Choubtum, L
Sriphrapradang, A
Rajatanavin, R
机构
[1] Mahidol Univ, Ramathibodi Hosp, Fac Med, Dept Pediat, Bangkok 10400, Thailand
[2] Mahidol Univ, Ramathibodi Hosp, Fac Med, Res Ctr, Bangkok 10400, Thailand
[3] Mahidol Univ, Ramathibodi Hosp, Fac Med, Dept Radiol, Bangkok 10400, Thailand
[4] Mahidol Univ, Ramathibodi Hosp, Fac Med, Dept Med, Bangkok 10400, Thailand
关键词
D O I
10.1046/j.1365-2265.2003.01707.x
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
OBJECTIVE Thalassaemia/haemoglobinopathy is a hereditary disease causing increased erythropoiesis and expansion of the bone marrow cavity. As a consequence, there is a reduction in trabecular bone tissue resulting in osteopenia/osteoporosis. The present study was performed to assess bone mineral density (BMD) in children and adolescents with beta-thalassaemia disease and to determine biochemical and hormonal changes that may affect BMD. METHODS Forty-eight children and adolescents with beta-thalassaemia were divided into two groups, transfusion-dependent (TD) (n = 16) and transfusion-independent (TI) (n = 32). All patients were treated suboptimally. BMD was determined by dual-energy X-ray absorptiometry. Bone maturation was assessed by radiographic bone age (BA). Blood and urine samples were obtained for the determination of biochemical and hormonal profiles, which included PTH, 25-hydroxyvitamin D (25-OHD), osteocalcin, bone-specific alkaline phosphatase, IGF-1, fT4, TSH and urine deoxypyridinoline. RESULTS Most of the patients were short and underweight, and they had delayed BA with mean Z- scores of -2.77 in the TD and -2.04 in TI groups. The mean Z- scores of BMD in the TD vs. TI groups of total body, radius, femoral neck and lumbar spine were -2.09 vs. -1.49, -0.73 vs. -0.54, -1.93 vs. -1.17 and -3.45 vs. -2.43, respectively. Although the means BMD values in the TD group were lower than those in the TI group, they were not significantly different. Mean serum IGF-1 levels were lower in the TD than the TI groups, 11.6 and 24.9 nmol/l, respectively (P < 0.05). Other biochemical and hormonal profiles did not differ between these two groups. CONCLUSIONS Patients with undertransfused severe beta-thalassaemia had more bone marrow expansion, lower serum IGF-1 levels and more delayed bone age than did patients with untransfused moderately severe beta-thalassaemia. Therefore, the severity of the disease appeared to be a primary factor for low bone mineral density in undertransfused patients in association with bone age delay and low serum IGF-1.
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页码:273 / 279
页数:7
相关论文
共 30 条
[1]   COMBINED VITAMIN-D PARATHYROID DEFECT IN THALASSEMIA MAJOR [J].
ALOIA, JF ;
OSTUNI, JA ;
YEH, JK ;
ZAINO, EC .
ARCHIVES OF INTERNAL MEDICINE, 1982, 142 (04) :831-832
[2]   THE CONTRIBUTION OF HYPOGONADISM TO THE DEVELOPMENT OF OSTEOPOROSIS IN THALASSEMIA MAJOR - NEW THERAPEUTIC APPROACHES [J].
ANAPLIOTOU, MLG ;
KASTANIAS, IT ;
PSARA, P ;
EVANGELOU, EA ;
LIPARAKI, M ;
DIMITRIOU, P .
CLINICAL ENDOCRINOLOGY, 1995, 42 (03) :279-287
[3]   CRITICAL YEARS AND STAGES OF PUBERTY FOR SPINAL AND FEMORAL BONE MASS ACCUMULATION DURING ADOLESCENCE [J].
BONJOUR, JP ;
THEINTZ, G ;
BUCHS, B ;
SLOSMAN, D ;
RIZZOLI, R .
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 1991, 73 (03) :555-563
[4]   IRON DISTRIBUTION IN THALASSEMIC BONE BY ENERGY-LOSS SPECTROSCOPY AND ELECTRON SPECTROSCOPIC IMAGING [J].
BORDAT, C ;
CONSTANS, A ;
BOUET, O ;
BLANC, I ;
TRUBERT, CL ;
GIROT, R ;
COURNOT, G .
CALCIFIED TISSUE INTERNATIONAL, 1993, 53 (01) :29-37
[5]   DEFEROXAMINE-INDUCED BONE DYSPLASIA IN PATIENTS WITH THALASSEMIA MAJOR [J].
BRILL, PW ;
WINCHESTER, P ;
GIARDINA, PJ ;
CUNNINGHAMRUNDLES, S .
AMERICAN JOURNAL OF ROENTGENOLOGY, 1991, 156 (03) :561-565
[6]   SERUM 1,25 DIHYDROXYVITAMIN-D AND OSTEOCALCIN CONCENTRATIONS IN THALASSEMIA MAJOR [J].
DANDONA, P ;
MENON, RK ;
HOULDER, S ;
THOMAS, M ;
HOFFBRAND, AV ;
FLYNN, DM .
ARCHIVES OF DISEASE IN CHILDHOOD, 1987, 62 (05) :474-477
[7]  
DEVIRGILIIS S, 1988, J PEDIATR-US, V113, P661
[8]   SHORT-TERM EFFECTS OF RECOMBINANT HUMAN INSULIN-LIKE GROWTH-FACTOR-I ON BONE TURNOVER IN NORMAL WOMEN [J].
EBELING, PR ;
JONES, JD ;
OFALLON, WM ;
JANES, CH ;
RIGGS, BL .
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 1993, 77 (05) :1384-1387
[9]   Longitudinal monitoring of bone mineral density in thalassemic patients. Genetic structure and osteoporosis [J].
Filosa, A ;
DiMaio, S ;
Vocca, S ;
Saviano, A ;
Esposito, G ;
Pagano, L .
ACTA PAEDIATRICA, 1997, 86 (04) :342-346
[10]   OSTEOPENIA IN MEN WITH A HISTORY OF DELAYED PUBERTY [J].
FINKELSTEIN, JS ;
NEER, RM ;
BILLER, BMK ;
CRAWFORD, JD ;
KLIBANSKI, A .
NEW ENGLAND JOURNAL OF MEDICINE, 1992, 326 (09) :600-604