Wang Jiajia, Yang Yi, Song An, Chen Yingying, Jiang Yan, Li Mei, Xing Xiaoping, Xia Weibo, Wang Ou
Department of Endocrinology, Key Laboratory of Endocrinology, National Health Commission, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, No. 1 Shuaifuyuan Wangfujing, Beijing, 100730, China.
Calcif Tissue Int. 2024 Dec 14;116(1):4. doi: 10.1007/s00223-024-01313-1.
The present study aimed to assess bone strength and microstructure in patients with pseudohypoparathyroidism (PHP), nonsurgical HP (NS-HP), and healthy controls. A total of 54 PHP1 patients (14 PHP1a, 40 PHP1b), 54 age-/sex- matched NS-HP patients and 27 age-/sex-matched controls were enrolled. Clinical characteristics, biochemical indices and HR-pQCT indices were respectively collected. PHP1 patients had higher serum Ca and PTH level than NS-HP patients at the time of both diagnosis and HR-pQCT measurement. A higher BMI and lower serum P level were observed in PHP1 patients than NS-HP patients at the time of HR-pQCT measurement. Bone microstructure analysis showed PHP1 patients had decreased Tb.N, increased Tb.Sp, and increased Tb.1/N.SD at tibia compared to NS-HP patients. Subgroup analysis showed no significant difference in Tb.N and Tb.Sp in PHP1b patients compared to NS-HP patients. Multiple linear regression analysis showed increased Tb.N and decreased Tb.Sp at tibia were associated with longer treatment duration in PHP1b patients. The microarchitecture evaluated by HR-pQCT in this study further supported that bone tissue had a preserved sensitivity to PTH in PHP1 patients and trabecular bone was more susceptible. Moreover, at least, bone status in PHP1b patients might benefit from regular treatment.
本研究旨在评估假性甲状旁腺功能减退症（PHP）、非手术性甲状旁腺功能减退症（NS-HP）患者及健康对照者的骨强度和微观结构。共纳入54例PHP1患者（14例PHP1a，40例PHP1b）、54例年龄/性别匹配的NS-HP患者和27例年龄/性别匹配的对照者。分别收集临床特征、生化指标和高分辨率外周定量CT（HR-pQCT）指标。在诊断时和进行HR-pQCT测量时，PHP1患者的血清钙和甲状旁腺激素（PTH）水平均高于NS-HP患者。在进行HR-pQCT测量时，观察到PHP1患者的体重指数（BMI）较高，血清磷水平低于NS-HP患者。骨微观结构分析显示，与NS-HP患者相比，PHP1患者胫骨的骨小梁数量（Tb.N）减少、骨小梁间距（Tb.Sp）增加、骨小梁分离度（Tb.1/N.SD）增加。亚组分析显示，与NS-HP患者相比，PHP1b患者的Tb.N和Tb.Sp无显著差异。多元线性回归分析显示，PHP1b患者胫骨Tb.N增加和Tb.Sp降低与治疗时间延长有关。本研究中通过HR-pQCT评估的微观结构进一步支持，PHP1患者骨组织对PTH仍具有敏感性，且小梁骨更易受累。此外，至少PHP1b患者的骨状态可能受益于规律治疗。
