RESUMO
Background: The pediatric/adolescent shoulder survey (PASS) score is a subjective measure of shoulder symptomology in younger patients. Purpose: To establish the minimal clinically important difference (MCID) and minimal detectable change (MDC) for the PASS score in adolescents after surgical treatment for shoulder instability. Study Design: Case series; Level of evidence, 4. Methods: Included were patients aged 12.5 to 23 years who underwent surgical treatment for shoulder instability and who had completed PASS forms preoperatively and at 3 months postoperatively. The MCID was established using an anchor-based approach, with the Single Assessment Numeric Evaluation (SANE) and shortened version of the Disabilities of the Arm, Shoulder and Hand (QuickDASH) as anchors. Change in PASS score between anchor groups was determined using receiver operating characteristic curve analysis. MDC with 90% confidence (MDC90) was also calculated. Range of motion and strength data at 3-month follow-up were evaluated to identify the optimal postoperative PASS score. Factors associated with improvement in PASS score beyond the MDC90 and MCID were determined in a subset of patients with ≥6-month follow-up data. Results: A total of 95 patients were included. The mean PASS score improved significantly from preoperatively to postoperatively (57 ± 15 to 75 ± 16; P < .001). The anchor-based MCID ranged from 12.5 to 13.2 points, with an area under the receiver operating characteristic (AUC) curve of 0.87 for the SANE and 0.99 for the QuickDASH. The MDC90 was 16.5 points. The optimal PASS score at 3 months after surgery was ≥85 (AUC, 0.66). Shorter duration of symptoms, lower preoperative forward elevation, and higher preoperative external rotation were associated with improvement in PASS score above the MDC90 and/or MCID for the subset of patients (n = 25) with ≥6-month follow-up data. Increased number of suture anchors, less preoperative external rotation deficit, and number of previous dislocations had a moderate effect on improvement in outcomes. Conclusion: A postoperative increase in PASS score of ≥16.5 points had a 90% chance of being a true-positive change, while a score change of approximately 13 points was likely clinically relevant. The optimal PASS score after surgery was ≥85. Shorter duration of symptoms, preoperative range of motion, number of surgical anchors, and number of previous dislocations were associated with achieving a clinically relevant improvement in PASS score at minimum 6 months postoperatively.
RESUMO
AIM: To describe the population of young people in Ireland diagnosed with narcolepsy with regards to vaccine exposure, symptomatology, investigation results and experience of medical treatment. METHOD: Retrospective review of medical records at the single tertiary referral centre for young people with narcolepsy in Ireland. RESULTS: Sixty-seven patients were diagnosed with narcolepsy between July 2006 and July 2017. Sixty-one (91%) of these developed symptoms after receiving the Pandemrix vaccine. The population was largely homogeneous with low hypocretin (87.5%), HLA DQB1∗0602 positivity (95%) and unremarkable findings on MRI Brain (100%). 77.6% experienced cataplexy; we also measured high levels of obesity, school non-attendance and psychosocial complexity. Symptoms often continued despite treatment, with multiple medications prescribed in 76.1% of patients. Prescription of sodium oxybate was associated with a significant reduction in BMI standard deviation scores at 6 months, with improved IOTF obesity scores seen at 36 month follow-up. CONCLUSIONS: This paper describes the experience of narcolepsy in children and young people in Ireland from 2006 - 2017 at the national tertiary referral centre. Narcolepsy in children and young people in Ireland carries a significant burden of illness, with impact on participation in education as well as physical and mental health. Symptoms can be refractory to medical treatment. Referral to tertiary centres for prompt treatment and multidisciplinary input is essential.
