Fabry Disease Rat Model Develops Age- and Sex-Dependent Anterior Segment Ocular Abnormalities.

Purpose: Fabry disease is an X-linked lysosomal storage disorder that results in multi-systemic renal, cardiovascular, and neuropathological damage, including in the eyes. We evaluated anterior segment ocular abnormalities based on age, sex (male and female), and genotype (wild-type, knockout [KO] male, heterozygous [HET] female, and KO female) in a rat model of Fabry disease. Methods: The α-Gal A KO and WT rats were divided into young (6-24 weeks), adult (25-60 weeks), and aged (61+ weeks) groups. Intraocular pressure (IOP) was measured. Eyes were clinically scored for corneal and lens opacity as well as evaluated for corneal epithelial integrity and tear break-up time (TBUT). Anterior chamber depth (ACD) and central corneal thickness (CCT) using anterior segment-optical coherence tomography (AS-OCT). Results: The Fabry rats showed an age-dependent increase in IOP, predominantly in the male genotype. TBUT was decreased in both male and female groups with aging. Epithelial integrity was defective in KO males and HET females with age. However, it was highly compromised in KO females irrespective of age. Corneal and lens opacities were severely affected irrespective of sex or genotype in the aging Fabry rats. AS-OCT quantification of CCT and ACD also demonstrated age-dependent increases but were more pronounced in Fabry versus WT genotypes. Conclusions: Epithelial integrity, corneal, and lens opacities worsened in Fabry rats, whereas IOP and TBUT changes were age-dependent. Similarly, CCT and ACD were age-related but more pronounced in Fabry rats, providing newer insights into the anterior segment ocular abnormalities with age, sex, and genotype in a rat model of Fabry disease.

Infection Rates After Aseptic Revision Total Hip Arthroplasty With Extended Oral Antibiotic Prophylaxis.

INTRODUCTION: Few US studies have investigated the efficacy of extended oral antibiotic prophylaxis (EOAP) in the prevention of periprosthetic joint infection (PJI) after aseptic revision total hip arthroplasty (R-THA). This study compared PJI rates in aseptic R-THA performed with EOAP with PJI rates in published studies of aseptic R-THA patients not receiving EOAP. METHODS: Prospectively documented data on 127 consecutive aseptic R-THAs were retrospectively reviewed. Evidence-based perioperative infection prevention protocols were used, and all patients were discharged on 7-day EOAP. Superficial and deep infections at 30 and 90 days postoperatively and at mean latest follow-up of 27.8 months were statistically compared with all US studies reporting the prevalence of PJI after aseptic R-THA. Complications related to EOAP within 120 days of the index procedure also are reported. RESULTS: No superficial or deep infections were observed at 30 and 90 days postoperatively when 7-day postdischarge EOAP was used. Superficial and deep infection rates were 1.57% (two patients) and 3.15% (four patients) at mean latest follow-up, respectively. Comparisons with published 30-day PJI rates of 1.37% ( P = 0.423) and 1.85% ( P = 0.257) were not statistically significant. Two of four comparisons with published 90-day PJI rates of 3.43% ( P = 0.027) and 5.74% ( P = 0.001) were statistically different. The deep PJI rate of 3.15% at mean latest follow-up was significantly lower than two of three published rates at equivalent follow-up including 10.10% ( P = 0.009) and 9.12% ( P = 0.041). No antibiotic-related complications were observed within 120 days of the index procedure. DISCUSSION: Study findings possibly support the use of EOAP after aseptic R-THA to prevent catastrophic PJI with revision implants, indicating that the efficacy of EOAP cannot be definitively ruled-in or ruled-out based on available evidence.