A higher frequency (25%) of gastrooesophageal reflux (GOR) has been previously reported in patients over 5 years old with cystic fibrosis compared with controls without cystic fibrosis. It was believed that GOR was caused by the complications of cystic fibrosis. We looked for GOR in all 26 children younger than 60 months who had cystic fibrosis diagnosed. They had a classical genetic profile an...

1 Nocturnal Oxygen Therapy Trial Group. Continuous or nocturnal oxygen therapy in hypoxemic chronic obstructive lung disease. Ann Intern Med 1980; 93:391-8 2 Zinman R, Corey M, Coates AL, Canny GJ, Connolly J, Levison H, et al. Nocturnal home oxygen in the treatment of hypoxemic cystic fibrosis patients. J Pediatr 1989; 114:368-77 3 Orenstein DA, Franklin BA, Doershuk CF. Hellerstem HK, Germann...

RATIONALE Cystic fibrosis airways are recurrently exposed to noxious stimuli, leading to epithelial injury. Previous reports suggest that cystic fibrosis airway epithelia may respond to injury by increasing proliferation. OBJECTIVES We sought to determine the characteristics of the proliferating cell population in cystic fibrosis airways. METHODS Six cystic fibrosis and six normal lung sect...

Depletion of the periciliary liquid in "Cystic Fibrosis" airway disease results in reduced mucociliary transport, persistent mucus hypersecretion and consequently increased height of the luminal mucus layer, so hypoxic gradients in the mucus plugs are developed. Because of anaerobic lung zones, it is highly probable that anaerobic bacteria not detected by routine bacteriologic culture methods a...

Cystic fibrosis is the most common lethal autosomal recessive disorder in the Caucasian population. Although the survival rate in patients constantly improves, lung damage is still the major cause of morbidity and mortality in patients with cystic fibrosis. In clinical practice, evaluation of patients' pulmonary state is made by combination of monitoring of lung function and more directly by as...

Cystic fibrosis is a life-threatening genetic disease that causes persistent lung infections and progressively limits the ability to breathe. The median predicted survival age of the patient with cystic fibrosis is 40 years. In the terminal care of the patient with cystic fibrosis, the role of the bedside nurse is critical in providing seamless, interdisciplinary care in order to promote a ‘‘go...

Background: Cystic fibrosis (CF) is the most common lethal genetic disorder of Cystic Fibrosis Trans-membrane Conductance (CFTR) Regulator gene mutations. We aimed to investigate common mutations in CF patients and to assess its possible relationship with clinical presentations. Materials and Methods: This cross sectional study was conducted on 36 CF patients who were referred to a tertiary ped...

plantation for cystic fibrosis. Proc Am Thorac Soc 2009;6:619–633 [13] Bech B, Pressler T, Iversen M, Carlsen J, Milman N, Eliasen K, Perko M, Arendrup H. Long-term outcome of lung transplantation for cystic fibrosis—Danish results. Eur J Cardiothorac Surg 2004;26:1180–1186 [14] Algar FJ, Cano JR, Moreno P, Espinosa D, Cerezo F, Alvarez A, Baamonde C, Santos F, Vaquero JM, Salvatierra A. Result...

metabolic alkalosis is characterized by high hco3- as it is seen in chronic respiratory acidosis, but ph differentiates the two disorders. there is no characteristic symptom or sign. orthostatic hypotension may be encountered. weakness and hyporeflexia occur if serum k+ is markerdly low. tetany and neuromuscular irritability occur rarely. we report the results of retrospective data analysis of ...