Characterization and management of hypercalcemia following transplantation for osteopetrosis.

Characterization and management of hypercalcemia following transplantation for osteopetrosis.
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DOI:
10.1038/bmt.2009.277
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发表时间:
2010-05
影响因子:
4.8
通讯作者:
Orchard PJ
Orchard PJ
中科院分区:
医学3区
文献类型:
--
作者:
Martinez C;Polgreen LE;DeFor TE;Kivisto T;Petryk A;Tolar J;Orchard PJ

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常染色体隐性遗传性石骨症(OP)的特征是破骨细胞活性不足,导致骨吸收缺陷和骨骼质量和密度显著增加。OP已成功地用造血细胞移植(HCT)治疗,继发于供体来源的功能性破骨细胞的植入,导致骨重建和正常造血的建立。虽然高钙血症是OP的常见表现特征,但由于从造血前体分化的破骨细胞的植入,在HCT后可能会观察到高钙血症。为了描述HCT后高钙血症的特征--风险、发病、持续时间和对治疗的反应--我们评估了2000年至2009年在明尼苏达大学接受治疗的15例OP患者。40%的患者出现高钙血症,定义为首次移植后任何单一钙>11.0 mg/100 ml。高钙血症的中位发病时间为23天,持续时间为2-24天。高钙血症在HCT时年龄大于2岁的患者中更常见。水化、呋塞米和s.c.治疗。降钙素解决了高钙血症,没有导致严重的不良事件。总之,高钙血症在HCT后前4周内的OP患者中很常见,更可能发生在老年患者中。等渗盐水、呋塞米和s.c.降钙素在我们的研究人群中耐受性良好且有效。
Autosomal recessive osteopetrosis (OP) is characterized by insufficient osteoclast activity resulting in defective bone resorption and marked increase in skeletal mass and density. OP has been successfully treated with hematopoietic cell transplantation (HCT), secondary to engraftment of donor-derived functioning osteoclasts resulting in remodeling of bone and establishment of normal hematopoiesis. Although hypercalcemia is a common presenting feature of OP, it may be observed following HCT due to engraftment of osteoclasts differentiated from the hematopoietic precursors. To characterize hypercalcemia after HCT—who is at risk, onset, duration and response to treatment—we evaluated 15 patients with OP treated at the University of Minnesota from 2000 to 2009. Hypercalcemia, defined as any single calcium >11.0 mg/100 ml after the first transplant, was found in 40% of patients. Median onset of hypercalcemia was 23 days and the duration was 2–24 days. Hypercalcemia was more common in patients older than 2 years of age at the time of HCT. Treatment with hydration, furosemide and s.c. calcitonin resolved hypercalcemia and resulted in no severe adverse events. In conclusion, hypercalcemia is common in patients with OP within the first 4 weeks after HCT, and more likely in older patients. Isotonic saline, furosemide and s.c. calcitonin were well-tolerated and effective treatments in our study population.
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