[1]蒋洁,徐潮,赵家军.骨硬化症的诊疗现状[J].国际内分泌代谢杂志,2023,43(04):301-304.[doi:10.3760/cma.j.cn121383-20220207-02003]
 Jiang Jie,Xu Chao,Zhao Jiajun.Diagnosis and treatment of osteosclerosis[J].International Journal of Endocrinology and Metabolism,2023,43(04):301-304.[doi:10.3760/cma.j.cn121383-20220207-02003]
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骨硬化症的诊疗现状()
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《国际内分泌代谢杂志》[ISSN:1673-4157/CN:12-1383/R]

卷:
43
期数:
2023年04期
页码:
301-304
栏目:
综述
出版日期:
2023-07-20

文章信息/Info

Title:
Diagnosis and treatment of osteosclerosis
作者:
蒋洁徐潮赵家军
山东大学齐鲁医学院附属省立医院内分泌科,山东省糖尿病与代谢疾病临床医学研究中心,山东省内分泌与脂代谢重点实验室,山东省内分泌与代谢性疾病防治工程实验室,济南 250021
Author(s):
Jiang Jie Xu Chao Zhao Jiajun
Department of Endocrinology, Shandong Provincial Hospital, Cheeloo College of Medicine, Shandong University; Shandong Clinical Research Center of Diabetes and Metabolic Diseases; Shandong Key Laboratory of Endocrinology and Lipid Metabolism; Shandong Prevention and Control Engineering Laboratory of Endocrine and Metabolic Diseases, Jinan 250021, China
关键词:
骨硬化症 诊断 治疗
Keywords:
Osteopetrosis Diagnosis Treatment
DOI:
10.3760/cma.j.cn121383-20220207-02003
摘要:
骨硬化症是一种罕见的遗传代谢性骨病,临床表现呈显著异质性,从无症状到表现为致命性临床特征。骨硬化症的诊断主要根据临床表现和影像学诊断相结合,基因检测有助于分型和制定个体化治疗方案。造血干细胞治疗是部分类型骨硬化症的根治方法,但可能出现致死性并发症。基因治疗有望作为造血干细胞治疗的根治疗法,目前仅限于临床前研究。本文将结合近年来国内外研究,对骨硬化症的诊治现状进行总结,以供参考。
Abstract:
Osteopetrosis is a rare hereditary metabolic bone disease with significant clinical heterogeneity, and the clinical feature of osteopetrosis ranges from asymptomatic to fatal. The diagnosis of osteopetrosis is mainly decided according to the combination of clinical manifestations and imaging examinations. In addition, genetic testing is helpful for the confirming of the classification of osteopetrosis and more individualized treatment strageties for specified patient. Hematopoietic stem cell therapy(HSCT)is the radical cure for part of osteopetrosis, while fatal complications may occur. Gene therapy is a promising replacement therapy for HSCT, but it is limited to preclinical research currently. This article summarizes the status of diagnosis and treatment of osteopetrosis for reference by combining the domestic and foreign research in recent years.

参考文献/References:

[1] Penna S,Villa A,Capo V.Autosomal recessive osteopetrosis: mechanisms and treatments[J].Dis Model Mech,2021,14(5):dmm048940.DOI:10.1242/dmm.048940.
[2] Stark Z,Savarirayan R.Osteopetrosis[J].Orphanet J Rare Dis,2009,4:5. DOI:10.1186/1750-1172-4-5.
[3] Kim JM,Lin C,Stavre Z,et al.Osteoblast-osteoclast communication and bone homeostasis[J].Cells,2020,9(9):2073.DOI:10.3390/cells9092073.
[4] Charoenngam N,Cevik MB,Holick MF.Diagnosis and management of pediatric metabolic bone diseases associated with skeletal fragility[J].Curr Opin Pediatr,2020,32(4):560-573.DOI:10.1097/MOP.0000000000000914.
[5] Teti A,Econs MJ.Osteopetroses,emphasizing potential approaches to treatment[J].Bone,2017,102:50-59.DOI:10.1016/j.bone.2017.02.002.
[6] Gregson CL,Duncan EL.The genetic architecture of high bone mass[J].Front Endocrinol(Lausanne),2020,11:595653. DOI:10.3389/fendo.2020.595653.
[7] Shaik NA,Bokhari HA,Masoodi TA,et al.Molecular modelling and dynamics of CA2 missense mutations causative to carbonic anhydrase 2 deficiency syndrome[J].J Biomol Struct Dyn,2020,38(14):4067-4080.DOI:10.1080/07391102.2019.1671899.
[8] Li L,Lv SS,Wang C,et al.Novel CLCN7 mutations cause autosomal dominant osteopetrosis type II and intermediate autosomal recessive osteopetrosis[J].Mol Med Rep,2019,19(6):5030-5038.DOI:10.3892/mmr.2019.10123.
[9] Wu CC,Econs MJ,DiMeglio LA,et al.Diagnosis and Management of Osteopetrosis:Consensus Guidelines From the Osteopetrosis Working Group[J].J Clin Endocrinol Metab,2017,102(9):3111-3123.DOI:10.1210/jc.2017-01127.
[10] Stepensky P,Grisariu S,Avni B,et al.Stem cell transplantation for osteopetrosis in patients beyond the age of 5 years[J].Blood Adv,2019,3(6):862-868.DOI:10.1182/bloodadvances.2018025890.
[11] Capo V,Penna S,Merelli I,et al.Expanded circulating hematopoietic stem/progenitor cells as novel cell source for the treatment of TCIRG1 osteopetrosis[J].Haematologica,2021,106(1):74-86.DOI:10.3324/haematol.2019.238261.
[12] Sabir AH,Cole T.The evolving therapeutic landscape of genetic skeletal disorders[J].Orphanet J Rare Dis,2019,14(1):300.DOI:10.1186/s13023-019-1222-2.
[13] Jensen TL,Gøtzsche CR,Woldbye DPD.Current and future prospects for gene therapy for rare genetic diseases affecting the brain and spinal cord[J].Front Mol Neurosci,2021,14:695937.DOI:10.3389/fnmol.2021.695937.
[14] Moscatelli I,Almarza E,Schambach A,et al.Gene therapy for infantile malignant osteopetrosis:review of pre-clinical research and proof-of-concept for phenotypic reversal[J].Mol Ther Methods Clin Dev,2020,20:389-397.DOI:10.1016/j.omtm.2020.12.009.
[15] Moscatelli I,Löfvall H,Schneider Thudium C,et al.Targeting NSG mice engrafting cells with a clinically applicable lentiviral vector corrects osteoclasts in infantile malignant osteopetrosis[J].Hum Gene Ther,2018,29(8):938-949.DOI:10.1089/hum.2017.053.
[16] Löfvall H,Rothe M,Schambach A,et al.Hematopoietic stem cell-targeted neonatal gene therapy with a clinically applicable lentiviral vector corrects osteopetrosis in oc/oc mice[J].Hum Gene Ther,2019,30(11):1395-1404.DOI:10.1089/hum.2019.047.
[17] Okur FV,Cevher ,Özdemir C,et al.Osteopetrotic induced pluripotent stem cells derived from patients with different disease-associated mutations by non-integrating reprogramming methods[J].Stem Cell Res Ther,2019,10(1):211.DOI:10.1186/s13287-019-1316-8.
[18] Hennig AF,Rössler U,Boiti F,et al.Generation of a human induced pluripotent stem cell line(BIHi002-A)from a patient with CLCN7-related infantile malignant autosomal recessive osteopetrosis[J].Stem Cell Res,2019,35:101367.DOI:10.1016/j.scr.2018.101367.
[19] Chen W,Twaroski K,Eide C,et al.TCIRG1 transgenic rescue of osteoclast function using induced pluripotent stem cells derived from patients with infantile malignant autosomal recessive osteopetrosis[J].J Bone Joint Surg Am,2019,101(21):1939-1947.DOI:10.2106/JBJS.19.00558.
[20] Maurizi A,Capulli M,Patel R,et al.RNA interference therapy for autosomal dominant osteopetrosis type 2.Towards the preclinical development[J].Bone,2018,110:343-345.DOI:10.1016/j.bone.2018.02.031.

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备注/Memo

备注/Memo:
基金项目:国家自然科学基金项目(81974124); 泰山学者计划专项资金项目(tsqn20161071)
通信作者:徐潮,Email:doctorxuchao@163.com; 赵家军,Email:jjzhao@sdu.edu.cn
更新日期/Last Update: 2023-08-15