Pamidronate is an effective treatment for osteoporosis in patients with beta‐thalassaemia

Voskaridou, Ersi; Terpos, Evangelos; Spina, Giovanna; Palermos, J.; Rahemtulla, Amin; Loutradi, Aphroditi; Loukopoulos, Dimitris

doi:10.1046/j.1365-2141.2003.04657.x

Cited by 91 publications

(83 citation statements)

References 23 publications

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“…11,12 Our study suggests the potential benefits of BMT in reversing the bone complications in TM. Although several studies reported the usefulness of bisphosphonates in treating bone complications for TM, 20,21 there has not been any report on whether BMD in these patients would improve spontaneously after BMT. Our post transplant TM patients had a significantly lower rate of BMD deficits in their left hips.…”

Section: Discussionmentioning

confidence: 99%

Bone mineral density in children with thalassaemia major: determining factors and effects of bone marrow transplantation

Leung

Hung

Lam

et al. 2005

Bone Marrow Transplant

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Summary:Osteoporosis and osteopenia affect up to half of patients with thalassaemia major (TM). We investigate the effects of acquired factors and BMT on bone mineral density (BMD) in these patients. In all, 53 patients on regular transfusion (BT group) and 33 patients at 5.771.9 years post transplant (BMT group) were recruited. BMD was measured by dual energy X-ray absorptiometry. Serum concentrations of osteocalcin, bone-specific alkaline phosphatase (ALP), beta-crossLap and urinary cross-linking deoxypyridinoline (DPD) were measured by chemiluminescence and enzyme immunoassay, respectively. Severe BMD deficit (Z-score oÀ2.5) at spine and hip were noted in 62 and 35% of BT group. Serum osteocalcin (b ¼ À0.463; P ¼ 0.006) was predictive of spine BMD, whereas age (b ¼ À0.843; P ¼ 0.007) and urine DPD (b ¼ À0.439; P ¼ 0.037) were associated with hip BMD in BT group. Among BMT patients, post transplant duration (b ¼ 0.450; P ¼ 0.009) and serum bone-specific ALP (b ¼ À0.495; P ¼ 0.013) were associated with spine BMD. Severe BMD deficit was less common among BMT than BT patients (6 vs 35%; P ¼ 0.036). The mean (s.d.) osteocalcin levels in BMT and BT groups were 96.4 (72.7) lg/l and 68.9 (40.3) lg/l, respectively (P ¼ 0.037).In conclusion, severe BMD deficit is common in Chinese TM patients and BMT may reverse BMD deficit in these patients. Osteoporosis and osteopenia are major long-term complications of thalassaemia major (TM). A number of studies have been performed using dual energy X-ray absorptiometry (DEXA) to measure bone mineral density (BMD) in patients with transfusion-dependent thalassaemia. [1][2][3][4][5] The prevalence of reduced BMD varied, but Jensen et al 1 reported that half of their 82 patients had severely low BMD whereas the other 45% had low bone mass. Angastiniotis et al 5 showed that patients with thalassaemia intermedia were not spared, and 75% of them had BMD below À2 s.d. even with transfusion started only later in life. BMD correlates with the risk of fractures. DEXA is currently the most reliable and widely used method for measuring BMD, and this technique assesses bone mass classically at the lumbar spine and proximal femur. 6,7 There are well-established normal standards for DEXA measurements for Caucasian and Chinese children. [6][7][8] Both genetic and acquired factors may contribute to the reduction in BMD in TM patients. Concerning the genetic regulation, Sp1 polymorphism in the gene encoding type 1 collagen (major) protein (COLIA1) was found to be associated with the severity of osteopenia at spine and hip in male thalassaemic patients, and the same genetic factor also predicted treatment failure to bisphosphonate. 3 Acquired factors contributing to skeletal morbidity include endocrinopathy, direct iron-induced toxicity, bone marrow expansion and the use of iron-chelating therapy. [9][10][11][12] However, the effects of BMT, being the only curative treatment for TM, on BMD measurements have not been reported. We hypothesise that the diminished BMD seen in TM patients may improve wh...

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Section: Discussionmentioning

confidence: 99%

Bone mineral density in children with thalassaemia major: determining factors and effects of bone marrow transplantation

Leung

Hung

Lam

et al. 2005

Bone Marrow Transplant

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“…During the last decade, there was sufficient data supporting that increased osteoclast activation is present in TM patients. Patients with TM and osteoporosis have elevated markers of bone resorption, such as N-terminal cross-linking telopeptide of collagen type-I (NTX) and tartrate-resistant acid phosphatase type 5b (TRACP-5b) (37,38) that correlated with BMD of the lumbar spine in these patients (38,39). This increased osteoclast activity seems to be at least partially due to an imbalance in the receptor-activator of nuclear factor-kappa B ligand (RANKL)/osteoprotegerin (OPG) system and the overproduction of cytokines that are involved in the osteoclast differentiation and function (39).…”

Section: Acquired Factorsmentioning

confidence: 99%

Bone Disease in Haemoglobin Disorders

Voskaridou

Terpos

2013

Thalassemia Reports

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Bone disease represents a prominent cause of morbidity in patients with thalassaemia and other haemoglobin disorders. The delay in sexual maturation, the presence of diabetes and hypothyroidism, the parathyroid gland dysfunction, the haemolytic anaemia, the progressive marrow expansion, the iron toxicity on osteoblasts, the iron chelators, and the deficiency of growth hormone or insulin growth factors have been identified as major causes of osteoporosis in thalassaemia. Adequate hormonal replacement, effective iron chelation, improvement of hemoglobin levels, calcium and vitamin D administration, physical activity, and smoking cessation are the main to-date measures for the management of the disease. During the last decade, novel pathogenetic data suggest that the reduced osteoblastic activity, which is believed to be the basic mechanism of bone loss in thalassemia, is accompanied by a comparable or even greater increase in bone resorption. Therefore, potent inhibitors of osteoclast activation, such as the aminobisphosphonates, arise as key drugs for the management of osteoporosis in thalassaemia patients and other haemoglobin disorders.

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“…Pamidronat ile yapılan çalışmalarda ise aylık 15 mg, 30 mg veya 60 mg pamidronat kullanımı ile KMY'de artış saptanmıştır (10,14). Türkiye'de yapılan bir çalışmada Patıroğlu ve arkadaşları (15) 23 hastaya bir yıl süreyle 3 ayda bir 15 mg pamidronat tedavisi uygulamış, bir yıllık tedaviden sonra sadece kalsiyum ve vitamin D desteği sağlanmıştır.…”

Section: Discussionunclassified

Talasemi Osteoporozunda Zoledronik Asit Kullanımı: İki Olgu Sunumu ve Literatürün Gözden Geçirilmesi

...

2012

Tftr

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ÖzetTalasemi majör hastalarında, erken tanı ve uygulanan tedaviler ile yaşam süresinin uzamasıyla birlikte, osteoporozla ilişkili sorunlar belirgin şekilde artmıştır. Bu hastalarda optimal transfüzyonlarla bile kemik iliği hiperaktif kalmaktadır. Bu nedenle transfüzyonlar, demir şelasyonu ve seks hormonlarıyla optimal tedaviye rağmen jeneralize osteoporoz ve osteopeni talasemide sık görülmektedir. Talasemiye bağlı osteoporozda kemik rezorbsiyonunun artmış olması osteoklast fonksiyonunun potent inhibitörleri olan bifosfonatların kullanımını gündeme getirmiştir. Bu makalede talasemi major ve sekonder osteoporoz tanılarıyla takip edilen iki kızkardeş tartışılmakta ve 6 yıl süre ile uygulanan 4 mg aralıklı zoledronik asit tedavisinin sonuçları sunulmaktadır. Talasemide gelişen osteoporozun etyopatogenezi ve uygulanan tedavi yaklaşımları gözden geçirilmektedir. Türk Fiz T›p Re hab Derg 2012;58:154-8. Anah tar Ke li me ler: Talasemi, osteoporoz, zoledronik asit Sum maryThe life span of thalassemia major patients has been extended with early diagnosis and treatment modalities. This condition has resulted in an increase in osteoporosis-related problems. Even with optimal transfusions, the bone marrow of these patients remains hyperactive. For this reason, despite blood transfusion, iron chelation, and sex hormone replacement therapy, generalized osteoporosis and osteopenia are still frequent in thalassemia patients. In thalassemia-induced osteoporosis, bone resorption increases. This condition makes biphosphonates, which are potent inhibitors of osteoclast function, the drug of choice. In this article, two sisters with thalassemia major and secondary osteoporosis are discussed and the results of treatment with intermittent zoledronic acid (iv. 4 mg ) for 6 years are presented. We also performed a review of the literature on the etiopathogenesis of thalasemia-induced osteoporosis and the treatment modalities. Turk J Phys Med Re hab 2012;58:154-8.

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Pamidronate is an effective treatment for osteoporosis in patients with beta‐thalassaemia

Cited by 91 publications

References 23 publications

Bone mineral density in children with thalassaemia major: determining factors and effects of bone marrow transplantation

Bone mineral density in children with thalassaemia major: determining factors and effects of bone marrow transplantation

Bone Disease in Haemoglobin Disorders

Talasemi Osteoporozunda Zoledronik Asit Kullanımı: İki Olgu Sunumu ve Literatürün Gözden Geçirilmesi

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