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Summary: Primary hypertrophic osteoarthropathy (PHO) is a hereditary bone disease that is grouped into PHO autosomal recessive 1 (PHOAR1) and PHO autosomal recessive 2 (PHOAR2) due to different causative genes. Data comparing bone microstructure between the two subtypes are scarce. This is the first study to find that PHOAR1 patients had inferior bone microstructure compared with PHOAR2 patients. Purpose: The primary goal of this study was to assess bone microarchitecture and strength in PHOAR1 and PHOAR2 patients and to compare them with age- and sex-matched healthy controls (HCs). The secondary goal was to assess the differences between PHOAR1 and PHOAR2 patients. Methods: Twenty-seven male Chinese PHO patients (PHOAR1 = 7; PHOAR2 = 20) were recruited from Peking Union Medical College Hospital. The areal bone mineral density (aBMD) was assessed by dual-energy X-ray absorptiometry (DXA). Peripheral bone microarchitecture at the distal radius and tibia were evaluated by high-resolution peripheral quantitative computed tomography (HR-pQCT). Biochemical markers of PGE2, bone turnover, and Dickkopf-1 (DKK1) were investigated. Results: Compared with HCs, PHOAR1 and PHOAR2 patients had distinctively larger bone geometry, substantially lower vBMD at the radius and tibia, and compromised cortical microstructure at the radius. For trabecular bone, PHOAR1 and PHOAR2 patients showed different changes at the tibia. PHOAR1 patients had significant deficits in the trabecular compartment, resulting in lower estimated bone strength. Conversely, PHOAR2 patients showed a higher trabecular number, narrower trabecular separation, and lower trabecular network inhomogeneity than HCs, translating into preserved or slightly high estimated bone strength. Conclusion: PHOAR1 patients had inferior bone microstructure and strength compared with PHOAR2 patients and HCs. Additionally, this study was the first to find differences in the bone microstructure between PHOAR1 and PHOAR2 patients. [ABSTRACT FROM AUTHOR]

Arteriovenous fistula (AVF) strategy has been recommended in clinical guidelines for a long time due to the survival benefits associated with it. However, the underlying mechanism still needs to be explored. This retrospective cohort study included 611 patients who received hemodialysis in West China Hospital Medical Center between January 1, 2014 and December 31, 2014. Patient characteristics, dialysis parameters, and 1-year blood pressure records were collected at baseline. Echocardiographic changes and clinical outcomes were assessed during the 59-month follow-up. Our study showed that fistulas were associated with lower long-term systolic blood pressure (SBP) standard deviation (SD) (P < 0.0001), lower long-term SBP residual metric (P < 0.0001), and lower intradialytic SBP residual (P = 0.001). Fistulas were also associated with a higher but non-significant proportion of the newly developed left ventricular (LV) hypertrophy (8.29% vs. 6.78%, P = 0.116) and increased LV volume (8.29% vs. 4.52%, P = 0.139), as well as a lower proportion of the newly developed left ventricular ejection fraction (LVEF) dysfunction (1.62% vs. 2.82%, P = 0.586). After a median of 59-month follow-up, catheter group showed a higher risk of cardiovascular events (hazard ratio [HR] 1.21; 95% confidence interval [95%CI] 1.01-1.52), all-cause infection (HR 1.25; 95%CI 1.07-1.47), and access-related infection (HR 2.88; 95%CI 1.76-4.68). However, the advantage of fistulas only retained in low-albumin subgroup (serum albumin < 40 g/l) except for access-related infections. Our results suggested the possible attribution of BPV and other patient factors to fistula-associated survival benefits.