儿童中囊性纤维化相关的骨病:它可以预测吗?
Halime Nayir Buyuksahin1, Deniz Dogru1, Onur Gözmen2
1Division of Pulmonology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Clinical nutrition (Edinburgh, Scotland)
|July 24, 2023
概括
患有囊性纤维化 (CF) 的儿童的骨密度无法通过常见的骨周转生物标志物进行预测. 无脂肪质量指数 (FFMI) 和手握强度 (HGS) 是这些患者骨矿物质密度 (BMD) 的最佳指标.
科学领域:
- 儿科内分泌学 儿科内分泌学
- 骨的新陈代谢 骨的新陈代谢
- 囊性纤维化研究 囊性纤维化研究
背景情况:
- 囊性纤维化 (CF) 相关的骨病 (CFBD) 是一个显著的并发症.
- 儿童CF的低骨矿物质密度 (BMD) 预测了未来的骨病.
- 在患有CF的儿童中,研究骨循环生物标志物和BMD的预测因素至关重要.
研究的目的:
- 为了研究与CF (cwCF) 儿童的BMD相关的骨循环生物标志物 (骨素,RANKL,OPG).
- 评估影响骨周转的因素,包括无脂肪质量 (FFM),肺功能 (FEV1) 和手握力 (HGS).
主要方法:
- 将16名患有CF和中度低BMD的儿童 (组1) 与64名患有CF和正常BMD的儿童 (组2) 进行比较.
- 测量了骨质卡尔 (OC),核因子卡帕B连接体 (RANKL) 的受体激活剂和骨质保护素 (OPG) 的血清水平.
- 评估的身体质量指数 (BMI),无脂肪质量指数 (FFMI),1秒内强制呼气体积 (FEV1) 和手握力 (HGS).
主要成果:
- 血清RANKL,OC,OPG或RANKL/OPG比率在两组之间没有显著差异.
- 在群体之间观察到BMI z-score,FFMI z-score,FEV1 z-score和HGS %pred的显著差异.
- 多变量回归确定了FFMI z-score和HGS %pred作为BMD的唯一预测因素.
结论:
- 血清OC,OPG,RANKL和RANKL/OPG比率不能预测患有CF的儿童的BMD.
- 在这个人群中,FFMI z-score 和 HGS %pred 是最有效的,非侵入性的 BMD 预测指标.
相关概念视频
Cystic Fibrosis: Pathogenesis
290
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,...
290
Cystic Fibrosis: Management
194
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...
194
Bone Disorders
3.7K
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...
3.7K
Hormones and Bone Tissue
2.7K
The endocrine system produces and secretes hormones, which interact with the skeletal system. These hormones control bone growth, maintain bone once it is formed, and remodel it.
Hormones That Influence Osteoblasts and/or Maintain the Matrix
Several hormones are necessary for controlling bone growth and maintaining the bone matrix. The pituitary gland secretes growth hormone (GH), which, as its name implies, controls bone growth. This happens in several ways: first, it triggers chondrocyte...
Hormones That Influence Osteoblasts and/or Maintain the Matrix
Several hormones are necessary for controlling bone growth and maintaining the bone matrix. The pituitary gland secretes growth hormone (GH), which, as its name implies, controls bone growth. This happens in several ways: first, it triggers chondrocyte...
2.7K
Essential Minerals for Bone Health
4.1K
The minerals contained in all of the food we consume are essential for our organ systems. However, certain essential minerals, such as calcium, phosphorus, magnesium, manganese, and fluoride, largely affect bone health.
Calcium and Phosphorus
Calcium is a critical component of bones, especially in the form of calcium phosphate and calcium carbonate. Since the body cannot make calcium, it must be obtained from the diet. However, calcium cannot be absorbed from the small intestine without...
Calcium and Phosphorus
Calcium is a critical component of bones, especially in the form of calcium phosphate and calcium carbonate. Since the body cannot make calcium, it must be obtained from the diet. However, calcium cannot be absorbed from the small intestine without...
4.1K
Changes in the Appendicular Skeleton with Age
2.1K
The upper and lower limb initially develops as a small bulge called a limb bud, which appears on the lateral side of the early embryo. The upper limb bud appears near the end of the fourth week of development, with the lower limb bud appearing shortly after.
Initially, the limb buds consist of a core of mesenchyme covered by a layer of ectoderm. The ectoderm at the end of the limb bud thickens to form a narrow crest called the apical ectodermal ridge. This ridge stimulates the underlying...
Initially, the limb buds consist of a core of mesenchyme covered by a layer of ectoderm. The ectoderm at the end of the limb bud thickens to form a narrow crest called the apical ectodermal ridge. This ridge stimulates the underlying...
2.1K


