Long-Term Follow-up of Untreated Adult Patients with Spondylothoracic Dysostosis (Jarcho-Levin Syndrome).

BACKGROUND: Spondylothoracic dysostosis (STD), also known as Jarcho-Levin syndrome (JLS), is a rare autosomal recessive disorder affecting the formation of the spine, characterized by a complete bilateral fusion of the ribs at the costovertebral junction, producing a "crablike" appearance of the thorax. Despite being declared a core indication for a V-osteotomy vertical expandable prosthetic titanium rib (VEPTR) expansion thoracoplasty of the posterior thorax, the natural history of STD in untreated subjects remains poorly documented. In this study, we report radiographic and pulmonary function findings and Patient-Reported Outcomes Measurement Information System (PROMIS) and 24-Item Early Onset Scoliosis Questionnaire (EOSQ-24) scores for untreated adult subjects with STD to gain insights into the natural history. METHODS: We identified 11 skeletally mature, untreated subjects with STD. Findings on medical evaluation, demographics, radiographic parameters, pulmonary function, genetic testing results, PROMIS measures, and EOSQ-24 scores were assessed. RESULTS: Five male and 6 female subjects (mean age, 32.3 years [range, 15 to 70 years]) with a confirmed STD diagnosis based on radiographs and genetic testing were evaluated. Mean body mass index (BMI) was 24.4 kg/m 2 (range, 18 to 38.9 kg/m 2 ), and mean thoracic height was 16 cm (range, 12 to 17 cm). Pulmonary function tests (PFTs) showed a mean forced vital capacity (FVC) of 22% of predicted, mean forced expiratory volume in 1 second (FEV1) of 24% of predicted, and FEV1/FVC ratio of 107% of predicted. The mean PROMIS dyspnea score was 40 ± 8 points (range, 27.7 to 52.1 points). The mean total EOSQ-24 score was 77.3 ± 18 points (range, 43.9 to 93.2 points). CONCLUSIONS: Our study characterizes the natural history of STD in untreated subjects. We confirmed the expected restrictive pattern in pulmonary function, but interestingly, our subjects exhibited better EOSQ scores compared with those reported in neuromuscular populations. PFT results and thoracic height did not correspond to PROMIS and EOSQ scores, questioning the use of those parameters as a surgical indication. We therefore suggest that the STD diagnosis as an absolute indication for VEPTR expansion thoracoplasty surgery be reconsidered. LEVEL OF EVIDENCE: Therapeutic Level IV . See Instructions for Authors for a complete description of levels of evidence.

Opioid-Sparing Multimodal Analgesia Efficacy in Hispanic Patients Undergoing Total Knee Arthroplasty.

Adequate pain control after total knee arthroplasty (TKA) has been associated with improved patient mobility and satisfaction, and is a task that has historically relied on opioids. Multimodal analgesic regimens can be considered a therapeutic alternative for postoperative pain control to avoid opioid consumption and its adverse side effects. This study aimed to evaluate the use of a multimodal analgesia protocol based on nonsteroidal anti-inflammatory drugs (NSAIDs) and acetaminophen as an alternative to reduce opioid consumption in Hispanic patients undergoing primary TKA. Methods: An institutional review board-approved, randomized controlled trial evaluated postoperative pain control after TKA in a Hispanic population. The experimental opioid-sparing group received 30-mg ketorolac intravenously every 6 hours and 1-g acetaminophen orally every 6 hours as the patient requested. The experimental group had the option to use morphine and oxycodone combined with acetaminophen as a rescue therapy. The control group received opioids in the form of 0.1-mg/kg morphine intravenously every 6 hours and/or oxycodone combined with acetaminophen (2.5 and 325 mg), 2 tablets orally every 6 hours, as the patient requested. Sociodemographic, operative, and immediate postoperative data and pain intensity measurements according to the numerical rating scale (NRS) were compared between groups. Analgesic efficacy was assessed according to the NRS scores at 12, 24, and 48 hours postoperatively. Results: Eighty-one Hispanic patients met inclusion criteria: 42 patients in the experimental group and 39 patients in the control group. None of the patients in the experimental group requested oxycodone combined with acetaminophen as a rescue therapy. Only 2 patients in the experimental group used a mean of 3.0 ± 1.4 mg of rescue morphine; in comparison, the control group consumed a mean of 28.0 ± 7.9 mg of morphine and 64.8 ± 26.0 mg of oxycodone per patient. A pain intensity decrease was observed in the experimental and control groups, with no postoperative evaluation differences at 12 hours (6.7 ± 2.9 compared with 5.9 ± 2.8; p = 0.209), 24 hours (6.2 ± 2.0 compared with 6.1 ± 2.2; p = 0.813), and 48 hours (4.7 ± 2.1 compared with 4.6 ± 1.7; p = 0.835). Conclusions: The use of a multimodal analgesic regimen based on intravenous ketorolac and oral acetaminophen was adequate in reducing postoperative pain and opioid consumption in Hispanic patients undergoing TKA. Level of Evidence: Therapeutic Level I. See Instructions for Authors for a complete description of levels of evidence.

Is the numerical rating scale an adequate predictor for assessing MRI underlying pathologies among painful adolescent idiopathic scoliosis patients?

The use of constant pain and night pain as clinical markers for predicting the presence of underlying pathology in painful adolescent idiopathic scoliosis (AIS) patients has been questioned. Pain intensity has been recognized as an important domain in pain assessment. The numerical rating scale (NRS) is one of the most commonly validated tools to assess pain intensity in children above 8 years of age. The aim of this study was to assess the NRS as a predictor of underlying pathologies found by MRI in painful AIS patients. A cross-sectional study comprising of all AIS patients with back pain lasting >4 weeks from April 2015 to April 2019 was performed. An MRI was performed on each AIS patient presenting with back pain after a nondiagnostic history, physical examination and spinal X-ray. The pain was graded using the NRS, ranging from 0 to 10. Patients were divided into three groups: NRS (1-3), NRS (4-6) and NRS (7-10). Variables, including gender, age, scoliosis magnitude and presence of underlying pathology, were compared between the groups. A total of 186 AIS patients were evaluated in the study, were 66/186 (35.5%) of them had underlying pathologies. The mean age of subjects was 14.7 years, 85% being female. An underlying pathology was identified in 4/11 (36.4%) with NRS (1-3), 28/82 (34.2%) with NRS (4-6) and 34/93 (36.6%) with NRS (7-10) (P = 0.94). The use of an NRS was not found to be an adequate predictor for identifying an underlying pathology via MRI in painful AIS patients.

Practice Variation Among Hispanic American Orthopedic Surgeons in the Management of Geriatric Distal Radius Fracture.

INTRODUCTION: There is a controversy in the management of distal radius fractures (DRF) and its criteria for surgical intervention on geriatric patients. The American Academy of Orthopedic Surgeons (AAOS) developed evidence-based guidelines for treatment of DRF. The aim of this study was to evaluate the current practice of Hispanic orthopedic surgeons in the management of geriatric DRF and examine their adherence to AAOS guidelines based on years of surgical experience. MATERIAL & METHODS: A survey was emailed to all orthopedic surgeons who live in Puerto Rico and treated DRF in their daily practice. Responses concerning demographic, management and clinical scenarios were evaluated. For each clinical scenario, treatment of choice was selected with the same fracture in a geriatric and young adult patient. Comparison between years of surgical experience and adherence to the AAOS guidelines was performed. RESULTS: A total of 65 surgeons responded the survey with 65% having >15 years in practice. A high consensus with AAOS guidelines for DRF was found. Use of preoperative radiographs was reported in all respondents, with an additional 12% routine use of preoperative computed tomography scans. Seventy-seven percent of respondents did not allow any range of motion (ROM) at immediate postoperative period, while 23% allowed active or passive ROM. Use of postoperative therapy was reported in 72.3%. Correlation between years of surgical experience showed a higher use of Vitamin C postoperatively for prophylaxis of Complex Regional Pain Syndrome among surgeons <15 years (P = 0.01). A general consensus trend toward operative fixation was noted among geriatric and young adult patients with the same fracture type in all clinical scenarios. DISCUSSION AND CONCLUSIONS: This survey demonstrates a practice variation toward surgical management of geriatric DRF among Hispanic orthopedic surgeons; despite their compliance with the AAOS AUC guidelines. The geriatric DRF management does not vary significantly among years of surgical experience.

Military Medicine Implements In-home Virtual Health in Europe.

OBJECTIVE: This report outlines a multispecialty implementation effort which included 12 specialty practices and 28 clinicians within Regional Health Command Europe (RHCE) and Landstuhl Regional Medical Center (LRMC) to pilot an in-home virtual health (VH) program using existing resources. METHODS AND MATERIALS: Synchronous VH encounters were performed using an Acano desktop conferencing client (Cisco Systems, Inc, San Jose, CA) and a USB web camera at the provider (distant) site and the patient's own computer or device in the home. A web real-time conferencing (Web RTC) server provided the connections. RESULTS: Between October 2016 and May 2018, 310 synchronous VH appointments to patients' homes in 23 geographic locations in 9 countries on 3 different continents were completed; 28 skill type I and II specialty providers at LRMC, SHAPE Belgium Army Health Clinic (AHC), and Vilseck AHC, Germany Primary Care Clinic participated. The providers represented 9 distinct specialties and primary care. Appointment types were as follows: 85 (39%) follow-up type appointments; 70 (32%) group type appointments; 65 (30%) initial specialty care appointments. The 3 most active clinics were Pediatric Gastroenterology with 88 (28%), the Nutrition Clinic with 82 (26%), and the Traumatic Brain Injury Clinic with 63 (20%) encounters. Full audio and video connectivity rate was 97%, excluding reconnects after dropped calls which occasionally occurred. Patient satisfaction scores were high 16/17 (94%) with 5% of patients surveyed. CONCLUSION: Low complexity synchronous VH appointments were successfully accomplished across a broad spectrum of health care services and appointment types. Landstuhl RMC specialists received consults from sites across a vast geographic area including Europe, the Middle East, and Africa. An in-home VH option gives providers a special tool to extend services far beyond traditional boundaries. This pilot project helped RHCE and LRMC providers gain valuable experience extending care to the home and will provide foundational knowledge for future VH efforts targeting groups and outcomes.

Two Na+ Sites Control Conformational Change in a Neurotransmitter Transporter Homolog.

In LeuT, a prokaryotic homolog of neurotransmitter transporters, Na(+) stabilizes outward-open conformational states. We examined how each of the two LeuT Na(+) binding sites contributes to Na(+)-dependent closure of the cytoplasmic pathway using biochemical and biophysical assays of conformation. Mutating either of two residues that contribute to the Na2 site completely prevented cytoplasmic closure in response to Na(+), suggesting that Na2 is essential for this conformational change, whereas Na1 mutants retained Na(+) responsiveness. However, mutation of Na1 residues also influenced the Na(+)-dependent conformational change in ways that varied depending on the position mutated. Computational analyses suggest those mutants influence the ability of Na1 binding to hydrate the substrate pathway and perturb an interaction network leading to the extracellular gate. Overall, the results demonstrate that occupation of Na2 stabilizes outward-facing conformations presumably through a direct interaction between Na(+) and transmembrane helices 1 and 8, whereas Na(+) binding at Na1 influences conformational change through a network of intermediary interactions. The results also provide evidence that N-terminal release and helix motions represent distinct steps in cytoplasmic pathway opening.