Early childhood lung function is a stronger predictor of adolescent lung function in cystic fibrosis than early Pseudomonas aeruginosa infection

Pseudomonas aeruginosa has been suggested as a major determinant of poor pulmonary outcomes in cystic fibrosis (CF), although other factors play a role. Our objective was to investigate the association of early childhood Pseudomonas infection on differences in lung function in adolescence with CF

Three-dimensional computed tomography for evaluation and management of children with complex chest wall anomalies: useful information or just pretty pictures?

Shaded Surface Display (SSD) technology, with 3-D CT reconstruction, has been reported in a few small series of patients with congenital or acquired chest wall deformities. SSD images are visually attractive and educational, but many institutions are hesitant to utilize these secondary to cost and image data storage concerns. This study was designed to assess the true value of SSD to the patient, family, and operating surgeon, in the evaluation and management of these children

Histo-Blood Group Gene Polymorphisms as Potential Genetic Modifiers of Infection and Cystic Fibrosis Lung Disease Severity

The pulmonary phenotype in cystic fibrosis (CF) is variable; thus, environmental and genetic factors likely contribute to clinical heterogeneity. We hypothesized that genetically determined ABO histo-blood group antigen (ABH) differences in glycosylation may lead to differences in microbial binding by airway mucus, and thus predispose to early lung infection and more severe lung disease in a subset of patients with CF. infection in the severe or mild groups. Multivariate analyses of other clinical phenotypes, including gender, asthma, and meconium ileus demonstrated no differences between groups based on ABH type. infection, nor was there any association with other clinical phenotypes in a group of 808 patients homozygous for the ΔF508 mutation

Validation of a septoplasty deformity grading system for the evaluation of nasal obstruction.

OBJECTIVES/HYPOTHESIS: We developed and validated a septal deformity grading (SDG) system that accounts for anatomic location and grading of deformity severity. STUDY DESIGN: Retrospective cohort study. METHODS: Subjects were patients with nasal obstruction presenting to University of California, Irvine Medical Center. Subjects were given pre- and postoperative Nasal Obstruction Symptom Evaluation (NOSE) questionnaires and were evaluated by a facial plastic surgeon using our septal deformity grading (SDG) system. Validity and reliability analyses were conducted on the SDG results. Statistical analyses were conducted on SDG and NOSE data to assess and compare instruments, and to validate the SDG instrument using the NOSE instrument. RESULTS: One hundred thirty-five patients met inclusion criteria. Cronbachs α was ≥ 0.7 for SDG and pre- and postoperative NOSE scores. There was a significant difference in pre- and postoperative NOSE scores (Z score = -7.21, P < .001). Correlations between postoperative NOSE and SDG scores were significant (P = .014), and convergent construct validity was achieved. There was a significant difference in SDG scores between primary versus revision operations (P < .001), history versus no history of nasal trauma, and nasal/septal surgery (P = .025, P = .003, respectively). The odds of having a revision operation were 2.3 times higher for high SDG scores (P < .001), of having a history of nasal trauma were 1.33 times higher for high SDG scores (P = .014), and of having a history of nasal/septal surgery were 2.9 times higher for low SDG scores. CONCLUSIONS: Our SDG system addresses the challenge of providing objective anatomic information on the severity of nasal septal deformities, and may be valuable when used in conjunction with subjective data gathered from the NOSE questionnaire. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:586-593, 2019

Validation of a septoplasty deformity grading system for the evaluation of nasal obstruction

Objectives/hypothesisWe developed and validated a septal deformity grading (SDG) system that accounts for anatomic location and grading of deformity severity.Study designRetrospective cohort study.MethodsSubjects were patients with nasal obstruction presenting to University of California, Irvine Medical Center. Subjects were given pre- and postoperative Nasal Obstruction Symptom Evaluation (NOSE) questionnaires and were evaluated by a facial plastic surgeon using our septal deformity grading (SDG) system. Validity and reliability analyses were conducted on the SDG results. Statistical analyses were conducted on SDG and NOSE data to assess and compare instruments, and to validate the SDG instrument using the NOSE instrument.ResultsOne hundred thirty-five patients met inclusion criteria. Cronbach's α was ≥ 0.7 for SDG and pre- and postoperative NOSE scores. There was a significant difference in pre- and postoperative NOSE scores (Z score = -7.21, P < .001). Correlations between postoperative NOSE and SDG scores were significant (P = .014), and convergent construct validity was achieved. There was a significant difference in SDG scores between primary versus revision operations (P < .001), history versus no history of nasal trauma, and nasal/septal surgery (P = .025, P = .003, respectively). The odds of having a revision operation were 2.3 times higher for high SDG scores (P < .001), of having a history of nasal trauma were 1.33 times higher for high SDG scores (P = .014), and of having a history of nasal/septal surgery were 2.9 times higher for low SDG scores.ConclusionsOur SDG system addresses the challenge of providing objective anatomic information on the severity of nasal septal deformities, and may be valuable when used in conjunction with subjective data gathered from the NOSE questionnaire.Level of evidence4 Laryngoscope, 129:586-593, 2019

Population characteristics of GMS and NBS subjects.

<p>Population characteristics of GMS and NBS subjects.</p

Colorado NBS Population: Absolute change and annual rate of change (percent predicted per year) in FEV₁ percent predicted from early childhood to adolescence by adolescent lung function quartile.

<p>Absolute change in FEV₁ was defined as mean FEV₁ percent predicted_{age 6–8} minus mean FEV₁ percent predicted_adolescent. Annual rate of change was defined as absolute change in FEV/ (mean age adolescent PFTs–mean age early childhood PFTs).</p