Bronchiectasis - Learning：支气管扩张症-学习.ppt

Ion transport → absorption of sodium ions from mucosal surface → movement of water into epithelial cells. Balance between movement of sodium and chloride → volume and composition of of airway surface liquid and mucociliary clearance Newborn screening DNA testing Symptoms of respiratory and GI symptoms Failure to pass meconium (meconium ileus) Healthy apeptite, but failure to thrive (malabsorption and hyposecretion of enzymes by pancreas) Streatorrhoea (fatty and offensive stools) ↑ concentration of sweat chloride Productive cough Chest pain – musculo-skeletal or pleuritic Dyspnoea (infection or as disease progresses) Pneumothorax Haemoptysis Clubbing Coarse crepitations Pleural rub Nasal polyps Chronic sinusitis Bronchial wall thickening Hyperinflation Nodular shadows Pulmonary function – initially obstructive, later restrictive Ventilation/perfusion imbalance Hypoxaemia, CO2 retention Pulmonary hypertension ABPA Obstruction of small bowel with Abdominal distension and discomfort Vomiting and ↓ or no bowel signs CFRD Biliary cirrhosis Portal hypertension Bleeding Liver transplant Puberty delayed Normal or near normal fertility in women Men infertile Rheumatic symptoms Joint pain, swelling, ↓ ROM of knees, ankles and wrists Low bone mineral density Fractures, rib fractures Pulmonary function and nutrition important Interdisciplinary team Morbidity and mortality related to chronic infection → oral, nebulized and intravenous antibiotics Important to wash hands between patients, contamination of nebulizers Inhaled bronchodilators and steroids Hypertonic saline High energy intake Fat-soluble vitamins and vitamin K, pancreatic enzymes Cortcosteroid nasal spray Haemoptysis – will stop spontaneously, embolization Pneumothorax – resolve without Rx or with ICD Heart-lung and double lung transplant Palliative care Home treatment less disrupting than hospitalisation IV antibiotics at home Home visits Physio doing home Rx Patient must take responsibility for own Rx F