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Features of Cystic Fibrosis
"CF PANCREAS"
- Chronic respiratory diseases occur*
- Failure to thrive, poor weight gain
- Pancreatic exocrine and endocrine insufficiency, Pulmozyme as treatment
- Autosomal recessive inheritance, metabolic Alkalosis
- Neonatal intestinal obstruction, meconium ileus, intussusception, volvulus
- Clubbing of fingers, Cholestasis, biliary Cirrhosis, ...**
- Rectal prolapse
- Electrolyte excess in sweat like sweat Na+ and Cl-
- Azoospermia and infertility
- Staph aureus and pSeudomonas in sputum, Salty sweat
*Chronic respiratory diseases include recurrent respiratory tract infections, pneumonia, pneumothorax, bronchiectasis, haemoptysis, chronic sinusitis, nasal polyps etc.
** CFTR-cAMP mediated chloride channelopathy, Chloride in sweat >40 mEq/L is suggestive of CF, Creon (pancrelipase) is used in treatment
"Woe is the child who tastes salty from a kiss on
the brow for he is cursed and soon must die''
-European folklore

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