Cystic fibrosis - Revision history

Danbot: Add Cefepime AntibioticDose entry

2026-03-20T10:58:29Z

Add Cefepime AntibioticDose entry

← Older revision		Revision as of 10:58, 20 March 2026
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	*Nebulised DNase		*Nebulised DNase
	*Lung transplant		*Lung transplant


			==Management==

			===Antibiotic Dosing===
			*{{AntibioticDose\|drug=Cefepime\|dose=>2 mo: Dose: 50mg/kg IV q8h x2wk; Max: 2 g/dose\|context=Respiratory Infections, cystic fibrosis patients\|disease=Cystic fibrosis\|population=Pediatric}}

	==Disposition==		==Disposition==

Rossdonaldson1: /* Short-Term Management */

2020-05-21T15:10:05Z

Short-Term Management

← Older revision		Revision as of 15:10, 21 May 2020
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	*See treatment for [[pancreatitis]]		*See treatment for [[pancreatitis]]
	*Rehydrate if volume depleted, replete electrolytes		*Rehydrate if volume depleted, replete electrolytes
	*Note potential for hypoalbuminemia when giving extensively protein-bound drugs		*Note potential for [[hypoalbuminemia]] when giving extensively protein-bound drugs

	==Long-Term Management==		==Long-Term Management==

SuperD: /* Background */

2019-12-30T12:01:12Z

Background

← Older revision		Revision as of 12:01, 30 December 2019
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	==Background==		==Background==
	*Autosomal recessive genetic disorder		*Autosomal recessive genetic disorder
	**Mutation in cystic fibrosis transmembrane conductance regulator protein (~~CTFR~~) leads to defect of sodium/chloride exchange channel		**Mutation in cystic fibrosis transmembrane conductance regulator protein (CFTR) leads to defect of sodium/chloride exchange channel
	**Defect in chloride transport leads to thick, viscous secretions in lungs, pancreas, liver, intestines, reproductive tract		**Defect in chloride transport leads to thick, viscous secretions in lungs, pancreas, liver, intestines, reproductive tract
	*Diagnosed by sweat chloride test		*Diagnosed by sweat chloride test

ClaireLewis at 15:23, 12 October 2019

2019-10-12T15:23:05Z

← Older revision		Revision as of 15:23, 12 October 2019
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	**[[Bronchitis]]		**[[Bronchitis]]
	**[[Sinusitis]] and nasal polyps		**[[Sinusitis]] and nasal polyps
	**Chronic inflammation and infection lead to [[bronchiectasis]] and angiogenesis (may have hemoptysis)		**Chronic inflammation and infection lead to [[bronchiectasis]] and angiogenesis (may have [[hemoptysis]])
	**Long-standing disease can lead to [[cor pulmonale]]		**Long-standing disease can lead to [[cor pulmonale]]
	*Gastrointestinal		*Gastrointestinal
	**Meconium ileus: failure to pass meconium within first 48 hours of life		**Meconium [[ileus]]: failure to pass meconium within first 48 hours of life
	***Earliest clinical manifestation of disease		***Earliest clinical manifestation of disease
	***Occurs in 10 - 20% of those diagnosed		***Occurs in 10 - 20% of those diagnosed
	***90% of babies with meconium ileus have cystic fibrosis		***90% of babies with meconium ileus have cystic fibrosis
	***Obstruction due to thick meconium		***[[SBO\|Obstruction]] due to thick meconium
	***Can lead to perforation if unrecognized		***Can lead to perforation if unrecognized
	***Diagnosed and treated with hyperosmolar contrast enema		***Diagnosed and treated with hyperosmolar contrast enema
	**Pancreatic insufficiency		**Pancreatic insufficiency
	***Often leads to failure to thrive in ~~infant~~		***Often leads to [[failure to thrive (peds)\|failure to thrive]] in infancy
	**[[Pancreatitis]]		**[[Pancreatitis]]
	**[[Diarrhea]] and malnutrition due to resultant malabsorption		**[[Diarrhea]] and malnutrition due to resultant malabsorption
	*Other		*Other
	**Electrolyte disturbances		**Electrolyte disturbances
	***Chloride wasting and diarrhea can lead to hypokalemic, hypochloremic [[metabolic alkalosis]]		***Chloride wasting and diarrhea can lead to [[hypokalemia\|hypokalemic]], hypochloremic [[metabolic alkalosis]]
	**[[Suppurative parotitis]]		**[[Suppurative parotitis]]
	***Rapid onset parotitis (warm, swollen, tender gland, fever, trismus)		***Rapid onset parotitis (warm, swollen, tender gland, fever, trismus)
Line 45:		Line 45:

	==Evaluation==		==Evaluation==
	*Sweat chloride test to make diagnosis		*Sweat chloride test to make diagnosis, may also be diagnosed by amniocentesis if high suspicion antenatally (outside ED scope)
	*May also be diagnosed by amniocentesis if high suspicion antenatally
	*CBC (signs of infection)		*CBC (signs of infection)
	*Electrolytes		*Electrolytes
	*~~Liver function tests~~ and lipase (if concern for pancreatitis)		*[[LFTs]] and lipase (if concern for [[pancreatitis]])
	*Consider blood and sputum cultures (may have resistant organisms)		*Consider blood and sputum cultures (may have resistant organisms)
	*[[CXR]]		*[[CXR]]
	**Advanced disease: peribronchial thickening, mucous plugs, cystic/bullous lesions, atelectasis, hilar adenopathy, air trapping		**Advanced disease: peribronchial thickening, mucous plugs, cystic/bullous lesions, atelectasis, hilar adenopathy, air trapping
	**Infiltrates if pneumonia		**Infiltrates if [[pneumonia]]
	**Pneumothorax		**[[Pneumothorax]]

	==Short-Term Management==		==Short-Term Management==
	*Infections		*Infections
	**Many patients already on maintenance [[azithromycin]] or tobramycin		**Many patients already on maintenance [[azithromycin]] or [[tobramycin]]
	**Antibiotics for acute pneumonia must be broad and include pseudomonal coverage		**Antibiotics for acute pneumonia must be broad and include pseudomonal coverage
	***e.g. [[~~Cefipime~~]], [[Imipenem]], '''OR''' [[Piperacillin/Tazobactam]] + IV [[fluoroquinolone]], +/- [[vancomycin]] for MRSA		***e.g. [[Cefepime]], [[Imipenem]], '''OR''' [[Piperacillin/Tazobactam]] + IV [[fluoroquinolone]], +/- [[vancomycin]] for MRSA
	*Other respiratory adjuncts		*Other respiratory adjuncts
	**Albuterol or other short-acting [[Beta-2 agonist]]		**[[Albuterol]] or other short-acting [[Beta-2 agonist]]
	**Dornase alfa (inhaled recombinant deoxyribonuclease I, hydrolizes DNA in sputum to decrease viscosity)		**Dornase alfa (inhaled recombinant deoxyribonuclease I, hydrolizes DNA in sputum to decrease viscosity)
	**Nebulized ~~Hypertonic~~ saline (reduce sputum viscosity)		**Nebulized [[hypertonic saline]] (reduce sputum viscosity)
	**Inhaled nitric oxide		**Inhaled nitric oxide
	**Chest physical therapy		**Chest physical therapy

ClaireLewis at 20:25, 24 August 2019

2019-08-24T20:25:34Z

← Older revision		Revision as of 20:25, 24 August 2019
Line 19:		Line 19:
	**[[Bronchitis]]		**[[Bronchitis]]
	**[[Sinusitis]] and nasal polyps		**[[Sinusitis]] and nasal polyps
	**Chronic inflammation and infection lead to bronchiectasis and angiogenesis (may have hemoptysis)		**Chronic inflammation and infection lead to [[bronchiectasis]] and angiogenesis (may have hemoptysis)
	**Long-standing disease can lead to [[cor pulmonale]]		**Long-standing disease can lead to [[cor pulmonale]]
	*Gastrointestinal		*Gastrointestinal

SLuckettG: /* Management */

2019-03-19T15:58:50Z

Management

← Older revision		Revision as of 15:58, 19 March 2019
Line 56:		Line 56:
	**Pneumothorax		**Pneumothorax

	==Management==		==Short-Term Management==
	*Infections		*Infections
	**Many patients already on maintenance [[azithromycin]] or tobramycin		**Many patients already on maintenance [[azithromycin]] or tobramycin
Line 70:		Line 70:
	*Rehydrate if volume depleted, replete electrolytes		*Rehydrate if volume depleted, replete electrolytes
	*Note potential for hypoalbuminemia when giving extensively protein-bound drugs		*Note potential for hypoalbuminemia when giving extensively protein-bound drugs

			==Long-Term Management==
			*Chest physiotherapy
			*Exercise therapy
			*Pancreatic enzymes
			*Fat-soluble vitamin supplementation
			*Nebulised DNase
			*Lung transplant

	==Disposition==		==Disposition==

SLuckettG: /* Evaluation */

2019-03-19T15:56:43Z

Evaluation

← Older revision		Revision as of 15:56, 19 March 2019
Line 45:		Line 45:

	==Evaluation==		==Evaluation==
	*CBC (signs of infection)~~, electrolytes, LFTs/~~lipase if concern for pancreatitis		*Sweat chloride test to make diagnosis
			*May also be diagnosed by amniocentesis if high suspicion antenatally
			*CBC (signs of infection)
			*Electrolytes
			*Liver function tests and lipase (if concern for pancreatitis)
	*Consider blood and sputum cultures (may have resistant organisms)		*Consider blood and sputum cultures (may have resistant organisms)
	*[[CXR]]		*[[CXR]]

SLuckettG: /* Clinical Features */

2019-03-19T15:55:14Z

Clinical Features

← Older revision		Revision as of 15:55, 19 March 2019
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	**Acute exacerbations lead to increase in baseline cough and sputum production		**Acute exacerbations lead to increase in baseline cough and sputum production
	**[[Pneumonia]]		**[[Pneumonia]]
	***Chronic ~~infection~~ with multiple organisms		***Chronic colonisation with multiple organisms
	***[[Staph aureus]] and [[H. influenzae]] common in childhood		***[[Staph aureus]] and [[H. influenzae]] common in childhood
	***Most ~~eventually~~ chronically ~~infected~~ with [[pseudomonas aeruginosa]] and/or virulent [[gram-negative]]		***Most become chronically colonised with [[pseudomonas aeruginosa]] and/or virulent [[gram-negative]] bacteria
			****Colonisation with [[pseudomonas aeruginosa]] usually occurs by late adolescence
	***Pseudomonas features include: severe pneumonia, cyanosis, confusion, often bilateral, occasionally empyema		***Pseudomonas features include: severe pneumonia, cyanosis, confusion, often bilateral, occasionally empyema
	***Higher risk for [[aspergillosis]]		***Higher risk for [[aspergillosis]]
	**Increased risk of [[pneumothorax]] (8%–20% will develop one in lifetime<ref>Tintanelli's</ref>)		**Increased risk of [[pneumothorax]] (8%–20% will develop one in lifetime<ref>Tintanelli's</ref>)
	**[[Bronchitis]]		**[[Bronchitis]]
	**[[Sinusitis]], nasal polyps		**[[Sinusitis]] and nasal polyps
	**Chronic ~~inflamation/~~infection ~~leads~~ to bronchiectasis and angiogenesis~~, which~~ may ~~lead to~~ hemoptysis		**Chronic inflammation and infection lead to bronchiectasis and angiogenesis (may have hemoptysis)
	**Long-standing disease can ~~eventually~~ lead to [[cor pulmonale]]		**Long-standing disease can lead to [[cor pulmonale]]
	*Gastrointestinal		*Gastrointestinal
			**Meconium ileus: failure to pass meconium within first 48 hours of life
			***Earliest clinical manifestation of disease
			***Occurs in 10 - 20% of those diagnosed
			***90% of babies with meconium ileus have cystic fibrosis
			***Obstruction due to thick meconium
			***Can lead to perforation if unrecognized
			***Diagnosed and treated with hyperosmolar contrast enema
	**Pancreatic insufficiency		**Pancreatic insufficiency
			***Often leads to failure to thrive in infant
	**[[Pancreatitis]]		**[[Pancreatitis]]
	**[[Diarrhea]] and malnutrition due to resultant malabsorption		**[[Diarrhea]] and malnutrition due to resultant malabsorption
	**Meconium ileus: failure to pass meconium within first 48 hours of life
	***Obstruction due to thick meconium
	***Can lead to perforation if unrecognized
	*Other		*Other
	**Electrolyte disturbances		**Electrolyte disturbances

SLuckettG: /* Background */

2019-03-19T15:50:29Z

Background

← Older revision		Revision as of 15:50, 19 March 2019
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	==Background==		==Background==
	*Autosomal recessive genetic disorder		*Autosomal recessive genetic disorder
	**~~Due to a mutation~~ in cystic fibrosis transmembrane conductance regulator protein (CTFR)		**Mutation in cystic fibrosis transmembrane conductance regulator protein (CTFR) leads to defect of sodium/chloride exchange channel
	**~~CTFR=~~chloride ~~channel important~~ in ~~function of mucus~~, ~~sweat~~, ~~and digestive fluids.~~		**Defect in chloride transport leads to thick, viscous secretions in lungs, pancreas, liver, intestines, reproductive tract
	*Causes thick, ~~viscous mucus leading to obstruction~~, ~~inflammation, and damage to respiratory~~ tract ~~and exocrine organs~~		*Diagnosed by sweat chloride test
	*Diagnosed by "sweat test~~" and/or genetic test~~		*Predicted life expectancy less than 40 years

	==Clinical Features==		==Clinical Features==

Rossdonaldson1: /* Management */

2017-10-12T19:35:15Z

Management

← Older revision		Revision as of 19:35, 12 October 2017
Line 50:		Line 50:
	**Many patients already on maintenance [[azithromycin]] or tobramycin		**Many patients already on maintenance [[azithromycin]] or tobramycin
	**Antibiotics for acute pneumonia must be broad and include pseudomonal coverage		**Antibiotics for acute pneumonia must be broad and include pseudomonal coverage
	***e.g. [[Cefipime]], [[Imipenem]] '''OR''' [[Piperacillin/Tazobactam]] + IV [[fluoroquinolone]], +/- [[vancomycin]] for MRSA		***e.g. [[Cefipime]], [[Imipenem]], '''OR''' [[Piperacillin/Tazobactam]] + IV [[fluoroquinolone]], +/- [[vancomycin]] for MRSA
	*Other respiratory adjuncts		*Other respiratory adjuncts
	**Albuterol or other short-acting [[Beta-2 agonist]]		**Albuterol or other short-acting [[Beta-2 agonist]]