A Comparison of Lumbar Spine and Lateral Distal Femur Bone Density in Girls With Rett Syndrome.

INTRODUCTION/BACKGROUND: Patients with Rett syndrome (RS) are at risk for low bone mineral density (BMD) and femoral fractures. In patients with RS, assessment with lateral distal femur (LDF) dual-energy X-ray absorptiometry (DXA) is recommended and clinically relevant. This study is the first to assess LDF BMD in girls with RS, and to compare LDF BMD results with lumbar spine BMD results in RS. Method Eleven girls (mean age 8.4 yr) with molecularly diagnosed RS and clinical DXA scan(s) were identified; medical charts were retrospectively reviewed. Baseline and serial lumbar spine and LDF BMD Z-scores were evaluated based on patients' ambulation status, presence of epilepsy, and mutation type. Results At the time of first scan, 8 of 11 patients had normal lumbar spine BMD and low LDF BMD Z-scores. Two patients had fracture history. Fully ambulatory (3) patients had higher lumbar spine and LDF BMD than partially (5) and nonambulatory (3) patients. Patients with epilepsy had lower average BMD at all sites. No difference was seen in lumbar spine or LDF BMD in patients with high-risk BMD mutations. Seven patients had serial DXA scans with an average observation of 5.1 yr (range 3.1 yr to 6.2 yr). Lumbar spine BMD over time was variable, while LDF bone mass accrual occurred at a lower rate than typically developing girls. Conclusion Females with RS exhibited lower BMD Z-scores at the LDF than at the lumbar spine. LDF and lumbar spine results were discordant. Ambulatory status and the presence of epilepsy were related to BMD. LDF BMD accrual deviated from normal as patients aged.

Utility of Respiratory Pathogen Panels in the Outpatient Oncology Setting.

Oncology patients presenting for outpatient evaluation of a respiratory tract infection (RTI) are often tested for a variety of viruses with a respiratory pathogen panel (RPP) in addition to influenza and SARS-CoV-2. This triad of testing is expensive and uncomfortable because it requires 2 nasal swabs. Little evidence supports the use of an RPP in outpatient settings, but it is routinely ordered. This retrospective chart review analyzed 183 RPPs performed at Jefferson between April 2020 and November 2021 in outpatient oncology patients presenting with RTI. Data collected included patient demographics, symptoms, and exam findings at time of RPP, additional testing completed, results of RPP, antibiotic and antiviral use before and after RPP results, and patient outcomes 30 days after RPP. Descriptive statistics were calculated. Of the 183 RPPs analyzed, 16.9% (31) were positive for at least 1 respiratory virus. Fifty-two patients (28.4%) started antibiotics before results of the RPP. Of those, 2 patients (3.8%) had a change in antibiotic plan after RPP results returned. Zero patients were started on antiviral medication before results of the RPP. One patient started antiviral treatment after RPP results returned. In total, only 3 patients (1.6%) had an RPP-driven change in medication management. This study suggests limited utility in use of RPPs for oncology patients presenting to the office with RTI symptoms. Targeted testing with a single nasal swab for influenza, RSV, and SARS-CoV-2 may be more clinically relevant. The authors hope to use these data to implement a quality improvement initiative to reduce RPP utilization in this population.

Adult Tourniquet for Use in School-Age Emergencies.

BACKGROUND: Gunshot injuries are a leading cause of morbidity and mortality in the pediatric population. The Pediatric Trauma Society supports the use of tourniquets for exsanguinating hemorrhage in severe extremity trauma. The Combat Application Tourniquet (CAT) used with success in adults has not been prospectively tested in children. Our objective with this study was to determine if the CAT is successful in arresting extremity arterial blood flow in school-aged children. METHODS: Sixty school-aged volunteers (ages 6-16 years) recruited by age cohort had the CAT applied to an upper arm and thigh while peripheral pulse was monitored by Doppler. The number of windlass turns (maximum allowed: 3 [1080°]) required to arrest arterial pulse was recorded. Success was analyzed by BMI percentile for age and extremity circumference. RESULTS: The CAT was successful in occluding arterial blood flow as detected by Doppler pulse in all 60 (100%) of the upper extremities tested. In the lower extremity, 56 (93%) had successful occlusion. The 3-turn maximum allowed by the protocol was not adequate in some obese, older subjects (BMI >30). In both the upper and lower extremity, the number of turns required to occlude blood flow gradually increased with an increase in arm and thigh circumference. CONCLUSIONS: Prospective testing of a cohort of school-aged children 6 to 16 years revealed the CAT tourniquet to be suitable for use in both the upper and lower extremity.