PHTY205 Lecture Notes - Lecture 6: Pharyngitis, Haemophilus Influenzae, Osteoporosis

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Describe the respiratory pathophysiology of cystic fibrosis using the clinical reasoning model: definition. Inherited genetic disease: autosomal recessive pattern 1:4 chance of inheriting if both parents are carriers of the c. Increased goblet cells: destruction of airway walls (bronchiectasis, cyst formation, nacl transport dysfunction, exocrine gland dysfunction. "d/i(cid:373)pai(cid:396)ed ci se(cid:272)(cid:396)etio(cid:374) f(cid:396)o(cid:373) epithelial (cid:272)ells of (cid:396)espi(cid:396)ato(cid:396)(cid:455) (cid:373)u(cid:272)osa. Increased goblet cells: destruction of airway walls (bronchiectasis, cyst formation, diagnosis, new born screening. Incurable disease: scientific promise of significant improvements with gene modulators, longer term: a cure with gene therapy, a well informed demanding group of patients, clinical reasoning model. Initial problem is mucus (sticky: sticky mucus -> cilia don"t work properly -> damage. List the signs and symptoms of a patient presenting with cystic fibrosis. Stenotrophomonas maltophilia: mrsa, achromobacter xylosoxidans, non tuberculous mycobacteria (m. abcessus, aspergillus, endocrine, pancreatic involvement leads to the insufficient secretion of enzymes which leads to malabsorption of fats and some proteins.

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