在囊性纤维化中骨病的特征
Victoria Contreras-Bolívar1, María Carmen Andreo-López2, Manuel Muñoz-Torres3
1Endocrinology and Nutrition Unit, University Hospital Clínico San Cecilio, 18016 Granada, Spain; Instituto de Investigación Biosanitaria de Granada (Ibs. Granada), 18014 Granada, Spain; CIBER on Frailty and Healthy Aging (CIBERFES), Instituto de Salud Carlos III, 18012 Granada, Spain.
概括
囊性纤维化 (CF) 患者面临骨疾病,增加骨折风险. 像DXA,TBS,VFA,QCT,MRI和QUS这样的先进成像技术有助于超越传统方法监测骨健康.
科学领域:
- 医学成像医学成像
- 骨的健康 骨的健康
- 囊性纤维化研究研究
背景情况:
- 囊性纤维化 (CF) 的预期寿命增加需要关注非肺部并发症.
- 与CF相关的骨病 (CFBD) 很普遍,导致骨折发生率更高.
- 早期发现和治疗CFBD对于患者的治疗结果至关重要.
研究的目的:
- 审查当前和补充的成像技术,以评估CF患者的骨健康.
- 为了突出标准骨密度测量的局限性.
- 讨论高级成像模式在CFBD管理中的实用性.
主要方法:
- 审查双能X射线吸收计 (DXA) 作为骨健康评估的黄金标准.
- 补充技术的评估:脊椎骨分数 (TBS),脊椎骨折评估 (VFA).
- 探索新兴的定量计算机断层扫描 (QCT),磁共振成像 (MRI) 和定量超声波 (QUS) 应用.
主要成果:
- 虽然DXA是标准的,但由于其二维性质而存在局限性.
- TBS和VFA为骨质和脊椎完整性提供了宝贵的见解.
- QCT,MRI和QUS显示出在CF中进行全面骨评估的潜力.
结论:
- 多模式成像方法有利于管理CF相关的骨病.
- 先进的技术补充了DXA,提供了更详细的了解CF骨健康.
- 对QCT,MRI和QUS的进一步研究可以优化CFBD监测和治疗.
相关概念视频
Cystic Fibrosis: Pathogenesis
202
Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
202
Cystic Fibrosis: Management
154
Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic...
Sinus disease and chronic...
154
Bone Disorders
3.5K
Aging and its effect on bone remodeling is the most common cause of bone disorders. In young and healthy people, bone deposition and resorption happen at an equal rate to maintain optimal bone health.
Bone deposition is also affected by the levels of sex hormones like estrogen and testosterone that promote osteoblast activity and bone matrix synthesis. When the level of these hormones decreases due to aging, it causes a reduction in bone deposition. As a result, bone resorption by osteoclasts...
Bone deposition is also affected by the levels of sex hormones like estrogen and testosterone that promote osteoblast activity and bone matrix synthesis. When the level of these hormones decreases due to aging, it causes a reduction in bone deposition. As a result, bone resorption by osteoclasts...
3.5K
What is the Skeletal System?
52.4K
Overview
52.4K
Bone Remodeling
38.2K
Bone remodeling is a continuous and balanced process of bone resorption by osteoclasts and bone formation by osteoblasts. In adults, it helps maintain bone mass and calcium homeostasis. While mechanical stress can stimulate turnover as part of the normal maintenance and reparative process, several hormones also regulate bone remodeling.
38.2K
Osteoclasts in Bone Remodeling
2.9K
Osteoclasts are cells responsible for bone resorption and remodeling. They originate from hematopoietic progenitor cells present in the bone marrow. Numerous progenitor cells fuse to form multinucleated cells, each with 10-20 nuclei. A single osteoclast has a diameter of 150 to 200 µM. These cells have ruffled borders that break down the underlying bone tissue and release minerals such as calcium into the blood in bone resorption. Osteoclasts cling to bones with their ruffled edges during...
2.9K


