Romosozumab-aqqg in the Treatment of Osteoporosis in a Patient With Hypophosphatasia.

Hypophosphatasia is a rare, inherited condition that causes osteomalacia and recurrent fractures. Therapeutic options for osteoporosis in patients with hypophosphatasia are limited because of concerns for a greater likelihood of atypical femoral fractures with antiresorptive agents. We report here the case of a patient with hypophosphatasia and osteoporosis who was treated with romosozumab-aqqg (Romo). An 81-year-old woman presented for management of osteoporosis with multiple fractures. She experienced a decline in bone mineral density over 20 years despite sequential osteoporosis treatment with oral bisphosphonates, hormone replacement therapy, teriparatide, and denosumab. Hypophosphatasia was suspected because of low serum alkaline phosphatase levels and was confirmed by genetic testing. After diagnosing hypophosphatasia, bone mineral density continued to decline and a trial of Romo was begun. After 1 year of Romo therapy, bone mineral density improved by 21%, and 10% at the lumbar spine and total hip, respectively. These changes were substantially greater than what she had experienced with prior teriparatide therapy. Blood alkaline phosphatase remained low on Romo. To our knowledge, this is the first report of a patient with hypophosphatasia and osteoporosis treated with Romo. In our patient, Romo did not significantly impact serum alkaline phosphatase, but improved bone mineral density significantly. In conclusion, Romo is a potential treatment option for osteoporosis in patients with hypophosphatasia for whom limited alternatives exist.

Evaluation of Adherence to Guideline-Based Bone Mineral Density Screening in Veterans with HIV.

People with HIV (PWH) have a higher prevalence of bone mineral density (BMD) loss compared to people without HIV. The Infectious Diseases Society of America (IDSA) recommends BMD screening through dual energy X-ray absorptiometry (DXA) in PWH starting at age 50. We aimed to evaluate adherence to this recommendation in a population of Veterans with HIV (VWH). Retrospective cross-sectional analysis of VWH followed from 2014 to 2018 at the Michael E. DeBakey VA Medical Center Infectious Diseases Clinic, Houston, Texas. We collected data through registry extraction and chart review. We calculated the percentage of VWH with timely BMD loss screening by DXA within 5 years of turning 50. Secondary outcomes included prevalence of osteopenia, osteoporosis, and vitamin D deficiency. We included data from 1,243 VWH. Their average age was 52 years (range 18-86). Most were male (95%), and 59% were black. Of the 346 VWH who turned 50 years old during the study period, 78 (22.5%) underwent DXA within 5 years. Of these, 42 (53.8%) had normal BMD, 28 (35.9%) had osteopenia, and 8 (10.3%) had osteoporosis. Nine hundred ninety-three (79.9%) VWH had available 25-hydroxyvitamin D levels; of these, 453 (45%) had normal levels, 304 (30.6%) had vitamin D insufficiency, 184 (18.5%) had vitamin D deficiency, and 52 (5.2%) had severe vitamin D deficiency. Fewer than 25% of eligible VWH underwent timely BMD loss screening by DXA per IDSA guidelines. Almost half of screened VWH showed evidence of BMD loss. Although limited by lack of follow-up and fracture data, this study emphasizes the importance of improving BMD loss screening in this vulnerable population.

Ultrasound-determined landmarks decrease pressure pain at epidural insertion site in immediate post-partum period.

BACKGROUND: Women have blamed epidurals for their post-partum back pain for decades. Survey-based studies have shown similar incidence of chronic back pain between women who delivered with epidurals compared to those who did not. However, epidural insertion site pain has yet to be evaluated by a quantitative measure: pressure pain threshold (PPT). Algometer measured PPT has been shown to be accurate and reproducible in acute, chronic, and postoperative pain studies. This study determines the effect of ultrasound-based landmarks on the PPT at the epidural insertion site in the post-partum period. METHODS: Participants were randomized into either the ultrasound or sham groups. In addition, a non-randomized control group (no epidural) participated. Ultrasound of the lumbar region was used to mark mid intervertebral levels in the US group but not in the sham group. Epidural were placed using the marks in the US group or palpated bony landmarks in the sham group. PPT at each intervertebral space measured before and after the use of epidural. RESULTS: Epidural placement did significantly decreased PPT in US (68%) and US sham (79%) groups and less in the control group (21%). US group showed decreased PPT only at insertion site whereas US sham group also showed decreased PPT at insertion site and adjacent levels. CONCLUSIONS: We showed that epidural placed with ultrasound-determined landmarks not only improves the success of epidural placement but also minimizes the number of intervertebral levels with decreased PPT.