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Investigates psychosocial issues concerning puberty and motherhood among adult women with cystic fibrosis (CF) to see how they had obtained information on these matters and how they would like information to be given. Results reveal problems with destructive behavior during puberty. Information about puberty and fertility should be given individually and in small discussion groups with teenage girls. (Author/MKA)

This literature review points out that the three major respiratory disorders prevalent in school children are asthma, cystic fibrosis, and tuberculosis. The review notes the incidence and medical management of these disorders, briefly describes related disorders, and outlines the implications for educators. (Author/JDD)

Discussion of automatic indexing in information retrieval systems focuses on attempts to improve the indexing representation produced by the FASIT system. Concept selection and concept grouping are explained; improving disambiguation is discussed; and a retrieval experiment to test the effectiveness of the disambiguation using the cystic fibrosis database is described. (26 references) (LRW)

This study, with 10 adult patients with cystic fibrosis, found that the improvement in lung function and ergometry parameters obtained by a short in-patient training program could be maintained on an out-patient basis through a voluntary self-treatment program. (DB)

Describes results of a study that investigated the effect of variations in indexing exhaustivity on retrieval performance in a vector space retrieval system. The test collection of documents in the National Library of Medicine's Medline file indexed under cystic fibrosis is described, and use of the SMART information retrieval system is discussed. (20 references) (LRW)

Social learning theory formed the basis of a program to develop self-management skills in cystic fibrosis patients. Strategies for practical learning activities for patients and their families included goal setting, reinforcement, modeling, skill training, and self-monitoring. (SK)

Two articles discuss the clustering of composite representations in the Cystic Fibrosis Document Collection from the National Library of Medicine's MEDLINE file. Clustering is evaluated as a function of the exhaustivity of composite representations based on Medical Subject Headings (MeSH) and citation indexes, and evaluation of retrieval performance is based on effectiveness measures. (53 references) (LRW)

Describes the files contained in the Cystic Fibrosis (CF) database and discusses educational and research opportunities using this database. Topics discussed include queries, evaluating the relevance of items retrieved, and use of the database in an online searching course in the School of Information and Library Science at the University of North Carolina at Chapel Hill. (26 references) (LRW)

Discusses four chronic diseases (cystic fibrosis, congenital heart disease, rheumatoid arthritis, and asthma) that affect American children. Many have their physical activities unnecessarily restricted, though sports and exercise can actually alleviate symptoms and improve their psychosocial development. Physicians are encouraged to prescribe exercise programs and advocate for these children. (SM)

These two articles discuss clustering structure in the Cystic Fibrosis Document Collection, which is derived from the National Library of Medicine's MEDLINE file. The exhaustivity of four subject representations and two citation representations is examined, and descriptor-weight thresholds and similarity thresholds are used to compute cluster-based retrieval effectiveness. (53 references) (LRW)

Examines effect of cystic fibrosis (CF) on structure and social climate of the family using self-report scales and independent observations of family functioning. Families in which the child with CF was not the firstborn were found to be functioning more healthily than those in which the child was firstborn. (Author/NRB)

An assessment of the impact of cystic fibrosis (CF) was conducted with 43 families. The impact of hospitalization upon parents was the most prevalent major problem. Parental communication was a "major problem" for 28% of the mothers but only one father. (Author/CL)

Parents of children with neuromuscular disease, cystic fibrosis, and renal disease were compared with parents of control subjects matched by age to the clinical cases. The three clinical groups exhibited different patterns of stressful response, consistent with the nature of their illnesses and the requirements for care imposed on the families. (Author/ABB)

Administered several psychiatric inventories to 30 cystic fibrosis and 30 matched control children and their parents. Data analysis revealed few differences in either psychopathological symptoms or psychiatric diagnoses between groups. Differences were either physical in nature or did not depart enough from normal scores to merit label of high psychopathology. (Author/NB)

Explored vocational development, self-concept, and vocational adjustment of persons with cystic fibrosis. The following measures of vocational development correlated with work adjustment: vocational plans, educational plans, initiative, occupational information, and average vocational development score. Vocational development did not correlate with severity of disability or self-concept. (Author)

Examined the relationship between family stress and changes in health of a child with cystic fibrosis. Data from parents (N=100) and clinic records revealed that a decline in pulmonary functioning was associated with family life changes, especially in family development and relationships, family management and decisions, and family finances. (Author/JAC)

Studied coping patterns in families (N=100) of children with cystic fibrosis. Three coping patterns emerged: (1) maintaining family integration and optimism; (2) maintaining social support, self-esteem, and psychological stability; and (3) understanding the medical situation. These patterns were validated against measures of the child's health and family adaptation. (Author/JAC)

Cystic Fibrosis and its effects on children are described as well as one child's means of coping with a chronic illness such as this in a school setting. (JMF)

Salmonella translocate to the gut epithelium via microfold cells lining the follicle-associated epithelium (FAE). How Salmonella localize to the FAE is not well characterized. Here we use live imaging and competitive assays between wild-type and chemotaxis-deficient mutants to show that Salmonella enterica serotype Typhimurium (S. Typhimurium) localize to the FAE independently of chemotaxis in an ex vivo mouse caecum infection model. Electrical recordings revealed polarized FAE with sustained outward current and small transepithelial potential, while the surrounding villus is depolarized with inward current and large transepithelial potential. The distinct electrical potentials attracted S. Typhimurium to the FAE while Escherichia coli (E. coli) localized to the villi, through a process called galvanotaxis. Chloride flux involving the cystic fibrosis transmembrane conductance regulator (CFTR) generated the ionic currents around the FAE. Pharmacological inhibition of CFTR decreased S. Typhimurium FAE localization but increased E. coli recruitment. Altogether, our findings demonstrate that bioelectric cues contribute to S. Typhimurium targeting of specific gut epithelial locations, with potential implications for other enteric bacterial infections.

The cystic fibrosis transmembrane conductance regulator (CFTR) is a crucial ion channel whose loss of function leads to cystic fibrosis, whereas its hyperactivation leads to secretory diarrhea. Small molecules that improve CFTR folding (correctors) or function (potentiators) are clinically available. However, the only potentiator, ivacaftor, has suboptimal pharmacokinetics and inhibitors have yet to be clinically developed. Here, we combine molecular docking, electrophysiology, cryo-EM, and medicinal chemistry to identify CFTR modulators. We docked ∼155 million molecules into the potentiator site on CFTR, synthesized 53 test ligands, and used structure-based optimization to identify candidate modulators. This approach uncovered mid-nanomolar potentiators, as well as inhibitors, that bind to the same allosteric site. These molecules represent potential leads for the development of more effective drugs for cystic fibrosis and secretory diarrhea, demonstrating the feasibility of large-scale docking for ion channel drug discovery.