learning支气管扩张症学习课件

Permanent dilatation of one or more bronchi Elastic and muscular tissue of bronchial walls destroyed by acute and chronic infection Impaired drainage of secretions Secretions chronically infected Chronic inflammatory response Progressive destructive lung disease Common causes of Bronchiectasis Post-infectiveTuberculosis Measles Whooping cough Mucociliary clearance defectCF PCD Youngs syndrome Immune defectsImmunoglobulin deficiency Cellular defects ABPA Localized bronchial obstructionForeign body Benign tumour External compression Gastric aspiration Totally asymptomatic to severe disease Productive cough with large amounts of purulent secretions, sometimes haemoptysis Frequently admitted to hospital Exacerbations chest pain, dyspnoea, fever If accompanied by CF or PCD sinus disease with nasal blockage, purulent discharge, and facial pain Auscultation coarse crepitations, wheezing Usually no clubbing Assessment X-ray CT Sputum specimen Bronchoscopy Lung function Serum immunoglobulins ABPA Gene mutation analysis Electron microscopy Physiotherapy Antibiotics Oral, intravenous, nebulized Clearance of infected secretions Treating infections Influenza vaccination Treatment of rhinosinusitis Immunoglobulin replacement therapy Surgical resection Inhaled human deoxyribonuclease (rhDNase) Inhaled steroids and bronchodilators Problems: Excess bronchial secretions Dyspnoea exercise tolerance Chest wall pain (musculoskeletal) Patient must understand pathology and reason for treatment ACBT, AD, Flutter Becareful of head-down tip - GOR Self treatment important daily Time of day? Physio techniques reassessed Improved ventilation Hypertonic saline Hospitalised - secretions or more purulent, dehydrated, dyspnoea. Haemoptysis and pleuritic pain Nebulized bronchodilator and humidification IPPB - work of breathing Post-resection changed anatomy of bronchial tree find optimal position Blood streaking in sputum continue Rx. Frank haemoptysis discontinue Continue Rx when secretions mildly bloodstained Inhalation with bronchodilator Relaxation positions and breathing control Exercise to fitness and secretions Group pulmonary rehab programme IMT Anti-inflammatory drugs and analgesics Heat IF TENS Acupuncture Manual therapy Effective treatment: amount and purulence of sputum no fever spirometry exercise tolerance energy levels dyspnoea chest wall pain Infections in nose, ears, sinuses and lungs Fertility affected (fallopian tubes and sperm motility) Dextrocardia or situs inversus Previously immotile cilia syndrome Chronic sputum production and nasal symptoms Pneumonia, rhinitis, asthma Otitis media GOR Infertility and ectopic pregnancy Investigations: nasal mucociliary clearance test genetic testing Antibiotics Assess and monitor hearing Inhaled B2-agonist GOR proton pump inhibitor Daily physio Teach parents early signs of infection Lethargy, “off colour”, fever Secretions mostly in dependant areas Airway clearance techniques Huffing games Exercises Nasopharyngeal suctioning Effective Rx:minimal coughing on exertion dyspnoea, coughing, wheezing fever secretions (back to usual amount) Autosomal recessive Caucasian populations Life expectancy was 2 years, now 31 years Faulty gene - CFTR 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 Future: Gene therapy Stem cell therapy Accurate assessment and Rx for every individual patient secretions, exercises Education with regards to inhalation therapy / oxygen therapy Musculoskletal pain, low bone density Urinary incontinence Work with patient and family / carers realistic Rx plan Before feeds for 10-15 minutes frequency and duration during infection PEP facemask AD Physical activity Head-down tip - GOR Routine daily airway clearance not required if no symptoms Physical activity very important something they would enjoy Play active role in Rx Encourage child to expectorate Learn to blow nose Main aim CF secretions - viscoelastisity, dehydrated, hyperadhesive Mobilize secretions without obstruction or fatigue airflow, long volumes, alter properties of secretions Huffing Rather ventilation than drainage Patient preference Airway clearance once a day with exercise Some patients may require Rx 2-3x a day exercise tolerance Make a given level of exercise more comfortable and ADL Endurance: swimming, cycling, running Strength training: weights Interval training Intensity 20-30 min, 3-4x per week Weight that can be lifted comfortably 10-15x, progress to 20-30x and then weight 15-30 minutes, every second day Warm-up, stretches and cool down Be careful with strengthening training in children 8-12 repititions without fatigue No absolute contraindications but exercise should not be done if patient has: Abdominal obstruction Acute bronchopulmonary exacerbation with fever Arthralgia and athritis Pneumothorax Persistent haemoptysis Surgery Exercise induced bronchoconstriction Hot climates DM Sport:contact sports bungee jumping parachute jumping scuba altitude (skiing) Not excluded Maintenance Oxygen before and after exercise Beta-adrenergic drugs B2-agoniste Hypertonic saline with ultrasonic nebulizer Bronchoconstriction test dose cough and sputum, in spirometry exercise tolerance Weight loss Lack of energy Dyspnoea Fever Chest pain duration and frequency of Rx manual techniques Positioning than normal driv