466425
Descripción
Mapa Mental por v.djabatey, actualizado hace más de 1 año
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Creado por v.djabatey
hace más de 10 años
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Cystic fibrosis
- clinical features
- newborn
- diagnosed through newborn screening
- diagnosed through newborn screening
- infancy
- meconium ileus
- newborn period
- newborn period
- prolonged neonatal jaundice
- failure to thrive
- recurrent chest infections
- malabsorption, steatorrhoea
- meconium ileus
- young child
- bronchiectasis
- rectal prolapse
- nasal polyp
- sinusitis
- bronchiectasis
- older child and adolescent
- allergic bronchopulmonary aspergillosis (ABPA)
- diabetes mellitus
- cirrhosis & portal hypertension
- distal intestinal obstruction (DIOS, meconium ileus equivalent)
- pneumothorax or recurrent haemoptysis
- sterility in males
- allergic bronchopulmonary aspergillosis (ABPA)
- newborn
- Diagnosis
- sweat test
- essential diag procedure
- to sig elevation in [Cl-] in sweat
- normal kids= 10-40 mmol/L
- CF= 60-120 mmol/L
- normal kids= 10-40 mmol/L
- stimulate sweating
- by pilocarpine iontophoresis
- collect sweat
- into special capillary tube
- absorb it onto weighed filter paper
- into special capillary tube
- by pilocarpine iontophoresis
- diag error common if not enough sweat collected
- essential diag procedure
- gene abnormalities in CFTR protein
- detect to confirm diag
- if child homozygote for 2 IDed mutations, then has CF
- detect to confirm diag
- sweat test
- management
- MDT approach
- paediatricians
- physiotherapists
- dieticians
- specialist nurses
- primary care team
- pt & fam, teachers
- paediatricians
- at least annual review at CF centre
- aims of therapy
- prevent progression of lung disease
- maintain adequate nutrition & growth
- prevent progression of lung disease
- Respiratory management
- MDT approach
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