Primary Ciliary Dyskinesia Medication

Updated: Oct 17, 2017
  • Author: Girish D Sharma, MD, FCCP, FAAP; Chief Editor: Denise Serebrisky, MD  more...
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Medication

Medication Summary

Antimicrobial therapy is indicated for the treatment of pulmonary infections, otitis media, and sinusitis. Starting with the usual antibiotics, including amoxicillin or amoxicillin-clavulanate, is reasonable. In the absence of response, the choice of a different antibiotic depends on the results of bacterial cultures. Some of the drugs commonly used are listed below.

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Antimicrobial agents

Class Summary

Empiric antimicrobial therapy must be comprehensive and should cover all likely pathogens in the context of the clinical setting.

Amoxicillin (Trimox, Amoxil)

A penicillin antibiotic with activity against gram-positive and some gram-negative bacteria. Binds to PBPs, inhibiting bacterial cell wall growth.

Amoxicillin and clavulanic acid (Augmentin)

Combination product that extends the antibiotic spectrum of this penicillin to include bacteria normally resistant to beta-lactam antibiotics.

Different amoxicillin/clavulanic acid ratios are recognized. (eg, 250-mg tab [250/125] vs 250-mg chewable tab [250/62.5]). Do not use products containing 125 mg of clavulanate until child weighs >40 kg. Note different product ratios for bid and tid dosing schedules.

Sulfamethoxazole and trimethoprim (Bactrim, Septra)

Inhibits bacterial growth by inhibiting synthesis of dihydrofolic acid.

Erythromycin and sulfisoxazole (Pediazole)

Erythromycin is a macrolide antibiotic with a large spectrum of activity. Erythromycin binds to the 50S ribosomal subunit of the bacteria, which inhibits protein synthesis.

Sulfisoxazole expands erythromycin's coverage to include gram-negative bacteria. Sulfisoxazole inhibits bacterial synthesis of dihydrofolic acid by competing with para-aminobenzoic acid.

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Bronchodilators

Class Summary

Inhaled bronchodilators are used to treat associated bronchospastic symptoms or before chest physical therapy to help airway clearance.

Albuterol (Proventil, Ventolin)

May be administered as either metered dose inhaler or nebulized form. Beta-agonist for bronchospasm refractory to epinephrine. Relaxes bronchial smooth muscle by action on beta2-receptors with little effect on cardiac muscle contractility.

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Glucocorticoids

Class Summary

Anti-inflammatory agents are used to treat inflammation associated with chronic and recurrent pulmonary infections. Various inhaled corticosteroids are used.

Inhaled corticosteroids are the most commonly used anti-inflammatory agents. Various preparations are available in metered dose inhaler form. Recently, a nebulized form of budesonide was approved and made available.

Budesonide inhaled (Beclovent, Vanceril)

Inhibits bronchoconstriction mechanisms. Produces direct smooth muscle relaxation. May decrease number and activity of inflammatory cells, in turn decreasing airway hyperresponsiveness.

Fluticasone inhaled (Flovent)

Inhibits bronchoconstriction mechanisms. Produces direct smooth muscle relaxation. May decrease number and activity of inflammatory cells, in turn decreasing airway hyperresponsiveness.

Budesonide (Pulmicort)

The nebulized form (ie, Respules) is now approved by the FDA, allowing younger children the benefit of administration. Alters level of inflammation in airways by inhibiting multiple types of inflammatory cells and decreasing production of cytokines and other mediators involved in the asthmatic response. Available as dry inhaled powder (Flexhaler - 90 mcg/actuation [delivers 80 mcg]; Turbuhaler – 200 mcg/actuation [delivers 160 mcg]) or suspension for nebulization (Respules).

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