Cystic fibrosis

v.djabatey
Mind Map by , created almost 6 years ago

Paediatrics (Respiratory) Mind Map on Cystic fibrosis, created by v.djabatey on 01/06/2014.

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v.djabatey
Created by v.djabatey almost 6 years ago
Laryngeal & tracheal infections
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Upper respiratory tract infection (URTI)
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Pneumonia
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Cystic fibrosis
1 clinical features
1.1 newborn
1.1.1 diagnosed through newborn screening
1.2 infancy
1.2.1 meconium ileus
1.2.1.1 newborn period
1.2.2 prolonged neonatal jaundice
1.2.3 failure to thrive
1.2.4 recurrent chest infections
1.2.5 malabsorption, steatorrhoea
1.3 young child
1.3.1 bronchiectasis
1.3.2 rectal prolapse
1.3.3 nasal polyp
1.3.4 sinusitis
1.4 older child and adolescent
1.4.1 allergic bronchopulmonary aspergillosis (ABPA)
1.4.2 diabetes mellitus
1.4.3 cirrhosis & portal hypertension
1.4.4 distal intestinal obstruction (DIOS, meconium ileus equivalent)
1.4.5 pneumothorax or recurrent haemoptysis
1.4.6 sterility in males
2 Diagnosis
2.1 sweat test
2.1.1 essential diag procedure
2.1.2 to sig elevation in [Cl-] in sweat
2.1.2.1 normal kids= 10-40 mmol/L
2.1.2.2 CF= 60-120 mmol/L
2.1.3 stimulate sweating
2.1.3.1 by pilocarpine iontophoresis
2.1.3.2 collect sweat
2.1.3.2.1 into special capillary tube
2.1.3.2.2 absorb it onto weighed filter paper
2.1.4 diag error common if not enough sweat collected
2.2 gene abnormalities in CFTR protein
2.2.1 detect to confirm diag
2.2.2 if child homozygote for 2 IDed mutations, then has CF
3 management
3.1 MDT approach
3.1.1 paediatricians
3.1.2 physiotherapists
3.1.3 dieticians
3.1.4 specialist nurses
3.1.5 primary care team
3.1.6 pt & fam, teachers
3.2 at least annual review at CF centre
3.3 aims of therapy
3.3.1 prevent progression of lung disease
3.3.2 maintain adequate nutrition & growth
3.4 Respiratory management

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