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Kartagener Syndrome Medication

  • Author: John P Bent, III, MD; Chief Editor: Ryland P Byrd, Jr, MD  more...
 
Updated: Feb 28, 2014
 

Medication Summary

Early intervention should be instituted with antibiotics directed at specific organisms identified by nasal secretions and/or expectorated sputum samples. Sensitivities of these samples should be obtained because resistant microorganisms can develop. Mucolytics may be helpful in specific individuals.

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Antibiotics

Class Summary

Used to treat acute or chronic infection or for prophylaxis against infection. Empiric antimicrobial therapy must be comprehensive and should cover all likely pathogens in the context of the clinical setting.

Trimethoprim and sulfamethoxazole (Bactrim DS, Septra)

 

Inhibits bacterial growth by inhibiting synthesis of dihydrofolic acid. Antibacterial activity of TMP-SMZ includes common urinary tract pathogens, except Pseudomonas aeruginosa.

Dose depends on whether treatment is prophylactic or for ongoing infection.

Amoxicillin (Biomox, Trimox, Amoxil)

 

Interferes with synthesis of cell wall mucopeptides during active multiplication resulting in bactericidal activity against susceptible bacteria.

Amoxicillin and clavulanate (Augmentin)

 

Drug combination treats bacteria resistant to beta-lactam antibiotics. Children older than 3 months, base dosing protocol on amoxicillin content. Due to different amoxicillin/clavulanic acid ratios in 250-mg tab (250/125) versus 250-mg chewable tab (250/62.5), do not use 250-mg tab until child weighs >40 kg.

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Expectorants

Class Summary

May thin mucous secretions.

Guaifenesin (Humibid LA)

 

Increases respiratory tract fluid secretions and helps loosen phlegm and bronchial secretions.

Large doses are necessary. Should be used in combination with adequate hydration.

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Contributor Information and Disclosures
Author

John P Bent, III, MD Professor, Director of Pediatric Otolaryngology, Departments of Otolaryngology-Head and Neck Surgery and Pediatrics, Albert Einstein School of Medicine; Director, Airway Clinic, Cochlear Implant Program, Children's Hospital at Montefiore

John P Bent, III, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, Society of University Otolaryngologists-Head and Neck Surgeons, American Society of Pediatric Otolaryngology, Society for Ear, Nose and Throat Advances in Children, Triological Society

Disclosure: Nothing to disclose.

Coauthor(s)

Elena B Willis, MD Resident Physician, Department of Otorhinolaryngology, Albert Einstein College of Medicine, Montefiore Medical Center

Elena B Willis, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Medical Student Association/Foundation, Wilderness Medical Society

Disclosure: Nothing to disclose.

Arvind K Badhey, MD Resident Physician, Department of Otolaryngology, Icahn School of Medicine at Mount Sinai

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Daniel R Ouellette, MD, FCCP Associate Professor of Medicine, Wayne State University School of Medicine; Chair of the Clinical Competency Committee, Pulmonary and Critical Care Fellowship Program, Senior Staff and Attending Physician, Division of Pulmonary and Critical Care Medicine, Henry Ford Health System; Chair, Guideline Oversight Committee, American College of Chest Physicians

Daniel R Ouellette, MD, FCCP is a member of the following medical societies: American College of Chest Physicians, Society of Critical Care Medicine, American Thoracic Society

Disclosure: Nothing to disclose.

Chief Editor

Ryland P Byrd, Jr, MD Professor of Medicine, Division of Pulmonary Disease and Critical Care Medicine, James H Quillen College of Medicine, East Tennessee State University

Ryland P Byrd, Jr, MD is a member of the following medical societies: American College of Chest Physicians, American Thoracic Society

Disclosure: Nothing to disclose.

Additional Contributors

Ryland P Byrd, Jr, MD Professor of Medicine, Division of Pulmonary Disease and Critical Care Medicine, James H Quillen College of Medicine, East Tennessee State University

Ryland P Byrd, Jr, MD is a member of the following medical societies: American College of Chest Physicians, American Thoracic Society

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors, Matthew Olearczyk, MD and Esther X Vivas, MD, to the development and writing of this article.

References
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Axial CT image showing dextrocardia and situs inversus in a patient with Kartagener syndrome. Image courtesy of Wikimedia Commons.
Axial CT image showing situs inversus (liver and inferior vena cava on the left, spleen and aorta on the right) in a patient with Kartagener syndrome. Image courtesy of Wikimedia Commons.
Normal cilia (A) compared with cilia in Kartagener syndrome with missing dynein arms (B). Image courtesy of Wikimedia Commons.
Table. Mutations in the Genes that Cause Human Primary Ciliary Dyskinesia [14]
Human Gene Human Chromosomal Location Chlamydomonas Ortholog Ciliary Ultrastructure in Subjects with Biallelic Mutations Presence of Laterality Defects Percentage of Individual with Biallelic Mutations MIM No.
DNAH5 5p15.2 DHC ? ODA defect Yes 15–21% of all PCD, 27–38% of PCD with ODA defects 608644
DNAI1 9p21-p13 IC78 ODA defect Yes 2–9% of all PCD, 4–13% of PCD with ODA defects 244400
DNAI2 17q25 IC69 ODA defect Yes 2% of all PCD, 4% of PCD with ODA defects 612444
DNAL1 14q24.3 LC1 ODA defect Yes na 614017
CCDC114 19q13.32 DC2 ODA defect Yes 6% of PCD with ODA defects 615038
TXNDC3 (NME8) 7p14-p13 LC5 Partial ODA defect (66% cilia defective) Yes na 610852
DNAAF1 (LRRC50) 16q24.1 ODA7 ODA + IDA defect Yes 17% of PCD with ODA + IDA defects 613193
DNAAF2 (KTU) 14q21.3 PF13 ODA + IDA defect Yes 12% of PCD with ODA + IDA defects 612517, 612518
DNAAF3 (C19ORF51) 19q13.42 PF22 ODA + IDA defect Yes na 606763
CCDC103 17q21.31 PR46b ODA + IDA defect Yes na 614679
HEATR2 7p22.3 Chlre4 gene model 525994 Phytozyme v8.0 gene ID Cre09.g39500.t1 ODA + IDA defect Yes na 614864
LRRC6 8q24 MOT47 ODA + IDA defect Yes 11% of PCD with ODA + IDA defects 614930
CCDC39 3q26.33 FAP59 IDA defect + axonemal disorganization Yes 36–65% of PCD with IDA defects + Axonemal disorganization 613798
CCDC40 17q25.3 FAP172 IDA defect + axonemal disorganization Yes 24–54% of PCD with IDA defects + Axonemal disorganization 613808
RSPH4A 6q22.1 RSP4, RSP6 Mostly normal, CA defects in small proportion of cilia No na 612649
RSPH9 6p21.1 RSP9 Mostly normal, CA defects in small proportion of cilia No na 612648
HYDIN 16q22.2 hydin Normal, very occasionally CA defects No na 610812
DNAH11 7p21 DHC ß Normal Yes 6% of all PCD, 22% of PCD with normal ultrastructure 603339
RPGR Xp21.1 na Mixed No PCD cosegregates with X-linked Retinitis pigmentosa 300170
OFD1 Xq22 OFD1 nd No PCD cosegregates with X-linked mental retardation 312610
CCDC164 (C2ORF39) 2p23.3 DRC1 Nexin (N-DRC) link missing; axonemal disorganization in small proportion of cilia No na 312610
CA = central apparatus; IDA = inner dynein arm; MIM = Mendelian Inheritance in Man; na = not available; N-DRC = nexin–dynein regulatory complex; ODA = outer dynein arm; PCD = primary ciliary dyskinesia.
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