Mineral bone disease in autosomal dominant polycystic kidney disease.
Mineral bone disease in autosomal dominant polycystic kidney disease.
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DOI：
10.1016/j.kint.2020.07.041
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发表时间：
2021-04
影响因子：
19.6
通讯作者：
Chonchol M
中科院分区：
文献类型：
--
作者：
Gitomer B;Pereira R;Salusky IB;Stoneback JW;Isakova T;Cai X;Dalrymple LS;Ofsthun N;You Z;Malluche HH;Maddux F;George D;Torres V;Chapman A;Steinman TI;Wolf M;Chonchol M
成骨细胞中Pkd 1破坏的小鼠表现出骨矿物质密度、骨小梁体积和皮质厚度降低。迄今为止，尚未研究常染色体显性遗传性多囊肾病（ADPKD）伴I期和II期慢性肾病成人患者的骨表型。为了验证这一点，我们对ADPKD患者矿物质代谢的生化标志物进行了表征，检查了骨转换和生物学，并估计了骨折的风险。在944例ADPKD和其他肾脏疾病患者中测量了矿物质代谢标志物。对20例ADPKD患者和17例健康人的骨活检组织进行组织形态计量学和免疫组织化学比较，平均eGFR为97 ml/ min/1.73m2。此外，还检查了2002-2013年期间开始血液透析的终末期肾病（ESKD）成人患者，并估计了与ADPKD相关的骨折风险，与其他肾病病因相比。与无ADPKD的慢性肾病患者相比，ADPKD患者的完整成纤维细胞生长因子23较高，总碱性磷酸酶较低。与健康个体相比，ADPKD患者的类骨质体积/骨体积（0.61 vs. 1.21%）和骨形成率/骨表面（0.012 vs. 0.026 μm3/μm2/天）显著降低。与糖尿病引起的ESKD相比，ADPKD引起的ESKD与更高的骨折风险无关（年龄校正的发生率比：0.53（95%置信区间0.31，0.74）），或与其他肾脏疾病病因相比。因此，患有ADPKD的个体具有较低的碱性磷酸酶，较高的循环完整成纤维细胞生长因子23和降低的骨形成率。然而，ADPKD与ESKD的骨折发生率较高无关。
Mice with disruption of Pkd1 in osteoblasts demonstrate reduced bone mineral density, trabecular bone volume and cortical thickness. To date, the bone phenotype in adult patients with autosomal dominant polycystic kidney disease (ADPKD) with stage I and II chronic kidney disease has not been investigated. To examine this, we characterized biochemical markers of mineral metabolism, examined bone turnover and biology, and estimated risk of fracture in patients with ADPKD. Markers of mineral metabolism were measured in 944 patients with ADPKD and other causes of kidney disease. Histomorphometry and immunohistochemistry were compared on bone biopsies from 20 patients with ADPKD with a mean eGFR of 97 ml/ min/1.73m2 and 17 healthy individuals. Furthermore, adults with end stage kidney disease (ESKD) initiating hemodialysis between 2002–2013 and estimated the risk of bone fracture associated with ADPKD as compared to other etiologies of kidney disease were examined. Intact fibroblast growth factor 23 was higher and total alkaline phosphatase lower in patients with compared to patients without ADPKD with chronic kidney disease. Compared to healthy individuals, patients with ADPKD demonstrated significantly lower osteoid volume/bone volume (0.61 vs. 1.21%) and bone formation rate/bone surface (0.012 vs. 0.026 μm3/μm2/day). ESKD due to ADPKD was not associated with a higher risk of fracture as compared to ESKD due to diabetes (age adjusted incidence rate ratio: 0.53 (95% confidence interval 0.31, 0.74) or compared to other etiologies of kidney disease. Thus, individuals with ADPKD have lower alkaline phosphatase, higher circulating intact fibroblast growth factor 23 and decreased bone formation rate. However, ADPKD is not associated with higher rates of bone fracture in ESKD.
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影响因子：
56.9
作者：
Mochizuki, T;Wu, GQ;Somlo, S
通讯作者：
Somlo, S
影响因子：
8
作者：
Dalagiorgou, Georgia;Piperi, Christina;Georgopoulou, Urania;Adamopoulos, Christos;Basdra, Efthimia K.;Papavassiliou, Athanasios G.
通讯作者：
Papavassiliou, Athanasios G.
DOI：
10.1152/ajprenal.1997.272.4.f451
发表时间：
1997-04-01
影响因子：
4.2
作者：
Geng, L;Segal, Y;Zhou, J
通讯作者：
Zhou, J
影响因子：
3.8
作者：
Leys, Charles M.;Nomura, Sachiyo;Goldenring, James R.
通讯作者：
Goldenring, James R.
影响因子：
8
作者：
Dalagiorgou, Georgia;Piperi, Christina;Papavassiliou, Athanasios G.
通讯作者：
Papavassiliou, Athanasios G.