Swedish Cystic Fibrosis (CF) care follows international guidelines in general. The only difference in our CF care package since 25 years is the physiotherapy regimen. Airway clearance therapy has since the early 1980s, from the day of diagnosis, been based upon daily physical exercise in conjunction with techniques for transporting and evacuating mucus. Postural Drainage+/-percussion and vibration has not been used in any age. The aim of this study was to evaluate our CF care package. Lung function data from the start of the study and with a 3-year interval were collected in the entire Swedish CF population > or =7 years old. Data were analysed for the age groups 7-17 and > or =18 years of age. Change of lung function over the study period was calculated. The impact of chronic Pseudomonas aeruginosa (Pa) colonisation and basal FEV(1)%p was also evaluated in a linear mixed model. Data from 99% of the country-wide CF population were available at inclusion. Mean FEV(1)%p was 90+/-21 vs 73+/-26 in the different age groups and mean VC%p was 94+/-18 vs 91+/-20. Forty percent of the adult group was > or =30 years old. Overall, 41% were chronically Pa colonised. Mean annual FEV(1)%p rate of decline was 0.77 and 0.64 in the different age groups. Lung function among Swedish CF patients is good and annual rate of decline low, even in an old cohort. The large proportion of adult patients emphasises future demands on CF care.
Background and aim: Repeat hepatectomy is increasingly performed for the management of recurrent colorectal liver metastases (CRLM). The aim of this study was to evaluate long-term functional liver volume (FLV) after a second hepatic procedure and to measure survival outcome. Methods: In this retrospective cohort study, patients treated for recurrent CRLM in the years 2005–2015 at two liver centers were included. Total FLV was calculated before the first procedure and before and after the second procedure. Overall survival was calculated. Results: Eighty-two patients were identified. The median follow-up was 53 (40–71) months from the first procedure. The median interval between first and second procedure was 13 (8–22) months. The initial FLV was 1584 (1313–1927) mL. The FLV was 1438 (1204–1896) mL after the first procedure and 1470 (1172–1699) mL after the second procedure ( P <0.001). After the second procedure, a total of ten patients (12%) had a residual liver volume of less than 75% of the initial liver volume. The 5-year overall survival was 37 (26–54)% after the second procedure. Conclusion: Small changes in FLV were found after two hepatic procedures but with considerable inter-individual variation. Patients selected for a repeated hepatic procedure for recurrent CRLM had an acceptable survival.
The state-of-the-field in complex disorder genetics is marked by large-scale, biobank-ascertained data sets that integrate broad demographic, health, survey, and genetic data. Leveraging the richness of this data can advance our understanding of complex disorders by improving predictions, describing etiology, and augmenting gene-mapping. These themes are especially, but not exclusively, relevant to Major Depressive Disorder (MDD), a leading cause of disability for which genetic predictors underperform, clinical heterogeneity is etiologically enigmatic, and missing heritability persists. The iPSYCH 2015 case-cohort presents a unique opportunity to integrate genotypes, register-based clinical outcomes, and extended genealogies of 30,949 MDD cases and 39,655 random population controls. To make full use of our data, we introduce the Pearson-Aitken framework for Family Genetic Risk Scores (PA-FGRS) to estimate individuals' liabilities for major depressive disorder from extended genealogies of partially observed relatives. PA-FGRS extends previous methods by accounting for censoring, leveraging distant relatives, and utilizing a flexible, model-based approach amenable to analysis and extension, advantages we highlight in simulations. Combining PA-FGRS with genotype data improves classification, replicates and extends known genetic contributions to clinical heterogeneity, and increases power for genome-wide association studies. This study combines novel analyses of unique data and can serve as a model for studies of other outcomes.
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