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466425
Cystic fibrosis
Descrição
Paediatrics (Respiratory) Mapa Mental sobre Cystic fibrosis, criado por v.djabatey em 06-01-2014.
Sem etiquetas
paediatrics
respiratory
paediatrics
respiratory
Mapa Mental por
v.djabatey
, atualizado more than 1 year ago
Mais
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Criado por
v.djabatey
quase 11 anos atrás
92
2
0
Resumo de Recurso
Cystic fibrosis
clinical features
newborn
diagnosed through newborn screening
infancy
meconium ileus
newborn period
prolonged neonatal jaundice
failure to thrive
recurrent chest infections
malabsorption, steatorrhoea
young child
bronchiectasis
rectal prolapse
nasal polyp
sinusitis
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
Diagnosis
sweat test
essential diag procedure
to sig elevation in [Cl-] in sweat
normal kids= 10-40 mmol/L
CF= 60-120 mmol/L
stimulate sweating
by pilocarpine iontophoresis
collect sweat
into special capillary tube
absorb it onto weighed filter paper
diag error common if not enough sweat collected
gene abnormalities in CFTR protein
detect to confirm diag
if child homozygote for 2 IDed mutations, then has CF
management
MDT approach
paediatricians
physiotherapists
dieticians
specialist nurses
primary care team
pt & fam, teachers
at least annual review at CF centre
aims of therapy
prevent progression of lung disease
maintain adequate nutrition & growth
Respiratory management
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