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Primary Ciliary Dyskinesia Medication

  • Author: Girish D Sharma, MD, FCCP, FAAP; Chief Editor: Michael R Bye, MD  more...
 
Updated: Apr 14, 2016
 

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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Contributor Information and Disclosures
Author

Girish D Sharma, MD, FCCP, FAAP Professor of Pediatrics, Rush Medical College; Director, Section of Pediatric Pulmonology and Rush Cystic Fibrosis Center, Rush Children's Hospital, Rush University Medical Center

Girish D Sharma, MD, FCCP, FAAP is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Thoracic Society, Royal College of Physicians of Ireland

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Chief Editor

Michael R Bye, MD Professor of Clinical Pediatrics, State University of New York at Buffalo School of Medicine; Attending Physician, Pediatric Pulmonary Division, Women's and Children's Hospital of Buffalo

Michael R Bye, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Thoracic Society

Disclosure: Nothing to disclose.

Additional Contributors

Susanna A McColley, MD Professor of Pediatrics, Northwestern University, The Feinberg School of Medicine; Director of Cystic Fibrosis Center, Head, Division of Pulmonary Medicine, Children's Memorial Medical Center of Chicago

Susanna A McColley, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Sleep Disorders Association, American Thoracic Society

Disclosure: Received honoraria from Genentech for speaking and teaching; Received honoraria from Genentech for consulting; Partner received consulting fee from Boston Scientific for consulting; Received honoraria from Gilead for speaking and teaching; Received consulting fee from Caremark for consulting; Received honoraria from Vertex Pharmaceuticals for speaking and teaching.

Acknowledgements

Heidi Connolly, MD Associate Professor of Pediatrics and Psychiatry, University of Rochester School of Medicine and Dentistry; Director, Pediatric Sleep Medicine Services, Strong Sleep Disorders Center

Heidi Connolly, MD is a member of the following medical societies: American Academy of Pediatrics, American Thoracic Society, and Society of Critical Care Medicine

Disclosure: Nothing to disclose.

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Diagram showing the cross-section of normal cilia showing its ultrastructure. Important components are labeled.
Ciliary ultrastructure, Left, Normal cilium from a healthy individual in which both inner and outer dynein arms can clearly identified. Right, the absence of outer and inner dynein arms in a patient with primary ciliary dyskinesia. Image courtesy of J. Carson, PhD, University of North Carolina.
 
 
 
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