Carbonic anhydrase and soluble adenylate cyclase regulation of cystic fibrosis cellular phenotypes.

Several aspects of the cell biology of cystic fibrosis (CF) epithelial cells are altered including impaired lipid regulation, disrupted intracellular transport, and impaired microtubule regulation. It is unclear how the loss of cystic fibrosis transmembrane conductance regulator (CFTR) function leads to these differences. It is hypothesized that the loss of CFTR function leads to altered regulation of carbonic anhydrase (CA) activity resulting in cellular phenotypic changes.

Divergence of bacterial communities in the lower airways of CF patients in early childhood.

Rationale: Chronic airway infection and inflammation resulting in progressive, obstructive lung disease is the leading cause of morbidity and mortality in cystic fibrosis. Understanding the lower airway microbiota across the ages can provide valuable insight and potential therapeutic targets.

Objectives: To characterize and compare the lower airway microbiota in cystic fibrosis and disease control subjects across the pediatric age spectrum.

Relationship between sleep and pain in adolescents with juvenile primary fibromyalgia syndrome.

Study Objectives: To investigate sleep quality in adolescents with juvenile primary fibromyalgia syndrome (JPFS) and determine whether sleep abnormalities, including alpha-delta sleep (ADS), correlate with pain intensity. We hypothesized that successful treatment for pain with exercise therapy would reduce ADS and improve sleep quality.

Design: Single-center preintervention and postintervention (mean = 5.

Accuracy of computer algorithms and the human eye in scoring actigraphy.

Purpose: The purpose of this study is to determine the optimal scoring method and parameter settings of actigraphy by comparison to simultaneous polysomnography (PSG).

Methods: Fifteen studies of simultaneous PSG and actigraphy were completed in adolescents (mean age = 16.3 years) and analyzed.