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Table 1 Main mucolytic agents used in Cystic Fibrosis, with indication of the methods of preparation and storage, devices recommended and indications for use

From: Hypertonic saline in people with cystic fibrosis: review of comparative studies and clinical practice

Type of mucolytic

Indication for age (years)

Formulation, preparation and dosage

Conservation

Inhalation device

Hypertonic saline solution 7% of NaCl + hyaluronic acid

>  6

Ready-made 5 ml vial

2vv/die

Room temperature

- Jet nebulizer

- Mesh nebulizer

Hypertonic saline solution 7% of NaCl + sodium bicarbonate

>  6

Ready-made 5 ml vial

2vv/die

Temperature 5 °C - 25 °C

- Jet nebulizer

- Mesh nebulizer

Hypertonic saline solution 7% of NaCl

>  6

Ready-made 4 ml vial

2vv/die

Temperature 4 °C–25 °C.

- Jet nebulizer

- Mesh nebulizer

Hypertonic saline solution 6% of NaCl

>  6

Ready-made 4 ml vial

2vv/die

Room temperature

- Jet nebulizer

- Mesh nebulizer

- Ultrasonic nebulizers

Hypertonic saline solution 3% of NaCl

Every age

Ready-made 3–5 ml vial

Room temperature

- Jet nebulizer

- Mesh nebulizer

- Ultrasonic nebulizers

Mannitol 40 mg

>  18

10 capsules to be inhaled with specific device

400 mg × 2 times/die

Temperature < 30 °C

In absence of humid environment

Specific inhaler

Dornase alfa

>  5

Ready-made 2.5 ml vial

1 time/die

>  21 years

2 times/die (for severe patients)

Temperature: 2 °C - 8 °C

max 30 °C for 24 h

- Jet nebulizer

- Mesh nebulizer

- Adaptive aerosol delivery system.

No ultrasonic nebulizers