Zubidat Dalia, Hanna Christian, Randhawa Amarjyot K, Smith Byron H, Chedid Maroun, Kaidbay Daniel-Hasan N, Nardelli Luca, Mkhaimer Yaman G, Neal Reem M, Madsen Charles D, Senum Sarah R, Gregory Adriana V, Kline Timothy L, Zoghby Ziad M, Broski Stephen M, Issa Naim S, Harris Peter C, Torres Vicente E, Sfeir Jad G, Chebib Fouad T
Division of Nephrology and Hypertension, Department of Medicine, Mayo Clinic, Rochester, MN, USA.
Division of Pediatric Nephrology and Hypertension, Department of Pediatric Adolescent Medicine, Mayo Clinic, Rochester, MN, USA.
Bone Rep. 2023 Jan 11;18:101655. doi: 10.1016/j.bonr.2023.101655. eCollection 2023 Jun.
ADPKD is caused by pathogenic variants in or , encoding polycystin-1 and -2 proteins. Polycystins are expressed in osteoblasts and chondrocytes in animal models, and loss of function is associated with low bone mineral density (BMD) and volume. However, it is unclear whether these variants impact bone strength in ADPKD patients. Here, we examined BMD in ADPKD after kidney transplantation (KTx). This retrospective observational study retrieved data from adult patients who received a KTx over the past 15 years. Patients with available dual-energy X-ray absorptiometry (DXA) of the hip and/or lumbar spine (LS) post-transplant were included. ADPKD patients (n = 340) were matched 1:1 by age (±2 years) at KTx and sex with non-diabetic non-ADPKD patients (n = 340). Patients with ADPKD had slightly higher BMD and T-scores at the right total hip (TH) as compared to non-ADPKD patients [BMD: 0.951 vs. 0.897, p < 0.001; T-score: -0.62 vs. -0.99, p < 0.001] and at left TH [BMD: 0.960 vs. 0.893, p < 0.001; T-score: -0.60 vs. -1.08, p < 0.001], respectively. Similar results were found at the right femoral neck (FN) between ADPKD and non-ADPKD [BMD: 0.887 vs. 0.848, p = 0.001; T-score: -1.20 vs. -1.41, p = 0.01] and at left FN [BMD: 0.885 vs. 0.840, p < 0.001; T-score: -1.16 vs. -1.46, p = 0.001]. At the LS level, ADPKD had a similar BMD and lower T-score compared to non-ADPKD [BMD: 1.120 vs. 1.126, p = 0.93; T-score: -0.66 vs. -0.23, p = 0.008]. After adjusting for preemptive KTx, ADPKD patients continued to have higher BMD T-scores in TH and FN. Our findings indicate that BMD by DXA is higher in patients with ADPKD compared to non-ADPKD patients after transplantation in sites where cortical but not trabecular bone is predominant. The clinical benefit of the preserved cortical bone BMD in patients with ADPKD needs to be explored in future studies.
常染色体显性多囊肾病（ADPKD）由编码多囊蛋白 -1 和 -2 蛋白的 或 中的致病变体引起。在动物模型中，多囊蛋白在成骨细胞和软骨细胞中表达，功能丧失与低骨矿物质密度（BMD）和骨体积相关。然而，尚不清楚这些变体是否会影响 ADPKD 患者的骨强度。在此，我们研究了肾移植（KTx）后 ADPKD 患者的 BMD。这项回顾性观察性研究检索了过去 15 年接受 KTx 的成年患者的数据。纳入移植后有可用的髋部和/或腰椎（LS）双能 X 线吸收测定（DXA）的患者。ADPKD 患者（n = 340）在 KTx 时按年龄（±2 岁）和性别与非糖尿病非 ADPKD 患者（n = 340）进行 1:1 匹配。与非 ADPKD 患者相比，ADPKD 患者右侧全髋（TH）的 BMD 和 T 评分略高[BMD：0.951 对 0.897，p < 0.001；T 评分：-0.62 对 -0.99，p < 0.001]，左侧 TH 也有类似情况[BMD：0.960 对 0.893，p < 0.001；T 评分：-0.60 对 -1.08，p < 0.001]。在右侧股骨颈（FN），ADPKD 和非 ADPKD 患者之间也有类似结果[BMD：0.887 对 0.848，p = 0.001；T 评分：-1.20 对 -1.41，p = 0.01]，左侧 FN 也是如此[BMD：0.885 对 0.840，p < 0.001；T 评分：-1.16 对 -1.46，p = 0.001]。在 LS 水平，与非 ADPKD 患者相比，ADPKD 患者的 BMD 相似但 T 评分较低[BMD：1.120 对 1.126，p = 0.93；T 评分：-0.66 对 -0.23，p = 0.008]。在调整了先发 KTx 因素后，ADPKD 患者在 TH 和 FN 的 BMD T 评分仍然较高。我们的研究结果表明，移植后，在以皮质骨而非小梁骨为主的部位，ADPKD 患者通过 DXA 测得的 BMD 高于非 ADPKD 患者。ADPKD 患者保留的皮质骨 BMD 的临床益处需要在未来的研究中进行探索。
