Use of the SNOT-22 and UPSIT to appropriately select pediatric patients with cystic fibrosis who should be referred to an otolaryngologist: cross-sectional study.

IMPORTANCE: Sinonasal disease and, specifically, nasal polyps, occur frequently in children with cystic fibrosis (CF). As survival rates have improved, it has become imperative that otolaryngologists become involved in the care of patients with CF to provide appropriate medical and surgical interventions for sinonasal disease. Despite significant variability in the subjective reporting of clinical symptoms, previous work has suggested there may be a relationship between clinical indicators and sinonasal disease in this population. OBJECTIVE: To determine whether the 22-item Sino-Nasal Outcome Test (SNOT-22), the University of Pennsylvania Smell Identification Test (UPSIT), and other measures of sinonasal disease could be used to predict the presence of subclinical nasal polyps in children with CF. DESIGN, SETTING, AND PARTICIPANTS: This was a cross-sectional study performed from May 2012 through April 2013 at a cystic fibrosis clinic at BC Children's Hospital in Vancouver, British Columbia, Canada. There were 72 eligible children with CF for this study (with a confirmed diagnosis of CF based on genetic testing; their ages ranged from 6 to 18 years, and they were not actively being treated by an otolaryngologist). Thirty-seven of these patients (23 males, 14 females) consented to participate in this study. Twenty-three declined participation, and 12 could not be contacted. MAIN OUTCOMES AND MEASURES: Potential clinical predictors for the presence of subclinical nasal polyps were determined a priori. All 37 recruited participants completed a full study assessment. Nasal endoscopy (the gold standard) was performed to determine the presence of nasal polyps. Potential predictors that were assessed included age, sex, genotype, pancreatic function, SNOT-22 and UPSIT scores, oral culture swab result, and severity of forced expiratory volume in 1 second (FEV(1)). RESULTS: A SNOT-22 score of greater than 11 was the only statistically significant predictor of nasal polyps (P = .04). The positive predictive value was 68.1%, the negative predictive value was 66.7%, and the positive likelihood ratio was 1.82. CONCLUSIONS AND RELEVANCE: Given that the SNOT-22 is easy to administer and inexpensive, this sinus disease-specific questionnaire seems to be an appropriate tool for routine use by respirologists when assessing patients with CF to help predict subclinical nasal polyps.

Visuomotor integration deficits precede clinical onset in Huntington's disease.

OBJECTIVES: Visuomotor integration deficits have been documented in Huntington disease (HD), with disproportionately more impairment when direct visual feedback is unavailable. Visuomotor integration under direct and indirect visual feedback conditions has not been investigated in the stage before clinical onset ('premanifest'). However, given evidence of posterior cortical atrophy in premanifest HD, we predicted visuomotor integration would be adversely affected, with greater impairment under conditions of indirect visual feedback. METHODS: 239 subjects with the HD CAG expansion, ranging from more than a decade before predicted clinical onset until early stage disease, and 122 controls, completed a circle-tracing task, which included both direct and indirect visual feedback conditions. Measures included accuracy, speed, and speed of error detection and correction. Using brain images acquired with 3T magnetic resonance imaging (MRI), we generated grey and white matter volumes with voxel-based morphometry, and analyzed correlations with circle-tracing performance. RESULTS: Compared with controls, early HD was associated with lower accuracy and slower performance in both circle-tracing conditions. Premanifest HD was associated with lower accuracy in both conditions and fewer rotations in the direct condition. Comparing performance in the indirect condition with the direct condition, HD gene expansion-carriers exhibited a disproportionate increase in errors relative to controls. Premanifest and early HD groups required longer to detect and correct errors, especially in the indirect condition. Slower performance in the indirect condition was associated with lower grey matter volumes in the left somatosensory cortex in VBM analyses. CONCLUSIONS: Visuomotor integration deficits are evident many years before the clinical onset of HD, with deficits in speed, accuracy, and speed of error detection and correction. The visuomotor transformation demands of the indirect condition result in a disproportionate decrease in accuracy in the HD groups. Slower performance under indirect visual feedback was associated with atrophy of the left-hemisphere somatosensory cortex, which may reflect the proprioceptive demands of the task.