cystic fibrosis/アルブミン

The study aims are: - Task 1: pathophysiological characterization of CFRD with assessment of the relative contribution of the insulin resistance and the β-cellular secretion impairment through the use of a Minimum Model applied to OGTT for the evaluation of insulin sensitivity and secretion - Task

INTRODUCTION & STATE-OF-ART Cystic Fibrosis (CF) is the most common autosomal recessive lethal disorders affecting 1 in 2.500 newborns among Caucasians. CF lung disease reflects a failure in the capacity of airway epithelia to normally hydrate their surface. Poor hydration of airways surfaces leads

3 Background The use of cardiopulmonary bypass (CPB) during cardiac surgery elicits generalized non-specific systemic inflammatory response syndrome (SIRS) and subsequent activation of the cytokine, complement, and coagulation-fibrinolytic cascades (1). In approximately 11% of the patients SIRS may

Ambulatory adult CF patients are recruited at time of their yearly evaluation. Phenotypic patients' characteristics Age, gender, CFTR genotype, pancreatic status (presence or absence of exocrine pancreatic insufficiency), number of IV antibiotic courses and number of hospitalization over the

Prospective controlled study including patients with cow's milk allergy(6-36 months of age.During the first examination, the diagnosis will be assessed by pediatricians with great experience in food allergy, checking that the patient had undergone a standardized procedure. All cases with an unclear

Background: Lung transplantation has been established as a viable treatment for cystic fibrosis (CF) patients who have end-stage lung disease. CF is a genetic disorder which is caused by mutations in the cystic fibrosis transmembrane conductance regulator gene. These mutations cause abnormal

Background: Cystic fibrosis (CF) affects an estimated 30,000 people in the United States. It is caused by a mutation in the gene encoding a protein called cystic fibrosis transmembrane regulator (CFTR). This protein functions as a chloride channel in epithelial cells of multiple organ systems. The

The etiology of Primary Sclerosing Cholangitis (PSC) is unknown. There are no proven effective therapies and no early markers of the disease to predict which patients with colitis may be at risk to develop PSC. Our group has demonstrated an increased prevalence of CFTR alleles (the gene responsible

Patients will be treated with growth hormone in the form of Nutropin (a powder formulation that is reconstituted by the patient) or Nutropin AQ (a ready-to-use liquid) for 1 year. Both of these products are manufactured by Genentech, Inc. The starting dose will be 0.006 mg/kg given as a daily

Background: Hypochloremic hypovolemic metabolic alkalosis contributes to hypercapnia in acute exacerbations of cystic fibrosis. Treatment of the metabolic alkalosis with volume and sodium chloride (NaCl) replacement could reduce hypoventilation and hypercapnia, thereby improving symptoms, sleep