eMedicine Specialties > Pulmonology > Congenital Disorders

Kartagener Syndrome: Treatment & Medication

Author: John P Bent lll, MD, Associate Professor, Director of Medical Student Education, Departments of Otolaryngology - Head and Neck Surgery and Pediatrics, Albert Einstein School of Medicine; Director, Airway Clinic, Children's Hospital at Montefiore
Coauthor(s): Esther X Vivas, MD, Staff Physician, Montefiore Medical Center, Bronx, New York
Contributor Information and Disclosures

Updated: Apr 2, 2009

Treatment

Medical Care

  • The most common infectious organisms affecting children with primary ciliary dyskinesia (PCD) are Haemophilus influenza and Staphylococcus aureus. All primary ciliary dyskinesia patients should receive comprehensive immunizations, including the influenza A and pneumococcal vaccines.
  • Antibiotics, intravenous or oral and continuous or intermittent, are used to treat upper and lower airway infections. Although prophylactic antibiotics should be used with great caution in this era of emerging antibiotic resistance, children with primary ciliary dyskinesia are especially good candidates for long-term low-dose preventative antibiotics.
  • Obstructive lung disease, if present, should be treated with inhaled bronchodilators and aggressive pulmonary toilet. Mucolytics may be helpful. Anecdotal reports indicate that inhaled antibiotics, oral and inhaled corticosteroids, and recombinant human DNAse have been used, but no large studies support the use of these agents.14

Surgical Care

  • Tympanostomy tubes are required to reduce conductive hearing loss and recurrent infections.
    • Many patients undergo repeated tympanostomy tube insertions, often complicated by chronic suppurative otitis media.
    • Chronic otorrhea may require special measures for aural hygiene, such as regular otomicroscopy, acetic acid irrigations, or culture-guided topical or systemic antibiotic therapy.
    • Because of anticipated long-term middle-ear disease, inserting tympanostomy tubes is the most sensible method of maintaining the myringotomy because the tube can be expected to stay in the tympanic membrane longer than routine grommets.
  • When sinus disease is refractory to medical management, functional endoscopic sinus surgery leads to transient improvement in upper and lower respiratory tract symptoms.15 The antiquated procedure of making a nasal antral window underneath the inferior turbinate may have a role in the management of primary ciliary dyskinesia because this procedure relies on gravitational rather than ciliary clearance of mucus.

Consultations

Consultations from an otolaryngologist, geneticist, pulmonologist, social services agent, or obstetrician/gynecologist (infertility) may be indicated.

Activity

Activities can be performed as tolerated; however, patients usually experience mild limitations in physical tolerance.

Medication

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.

Antibiotics

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.

Adult

160 mg TMP/800 mg SMZ PO q12h

Pediatric

<2 months: Do not administer
>2 months: 2-10 mg/kg/d, based on TMP, PO q12h

May increase PT when used with warfarin (perform coagulation tests and adjust dose accordingly); coadministration with dapsone may increase blood levels of both drugs; coadministration of diuretics increases incidence of thrombocytopenia purpura in elderly patients; phenytoin levels may increase with coadministration; may potentiate effects of methotrexate in bone marrow depression; hypoglycemic response to sulfonylureas may increase with coadministration; may increase levels of zidovudine

Documented hypersensitivity; megaloblastic anemia resulting from folate deficiency

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Discontinue at first appearance of skin rash or sign of adverse reaction; obtain CBC counts frequently; discontinue therapy if significant hematologic changes occur; goiter, diuresis, and hypoglycemia may occur with sulfonamides; prolonged IV infusions or high doses may cause bone marrow depression (if signs occur, give 5-15 mg/d leucovorin); caution in folate deficiency (eg, patients with chronic alcoholism, elderly patients, those receiving anticonvulsant therapy, or those with malabsorption syndrome); hemolysis may occur in G-6-PD deficient individuals; patients with AIDS may not tolerate or respond to TMP-SMZ; caution in renal or hepatic impairment (perform urinalyses and renal function tests during therapy); give fluids to prevent crystalluria and stone formation


Amoxicillin (Biomox, Trimox, Amoxil)

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

Adult

1-2 g/d PO q8-12h

Pediatric

20-80 mg/kg/d PO q12h

Reduces efficacy of oral contraceptives

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Adjust dose in renal impairment; may increase possibility of candidiasis


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.

Adult

1-2 g/d PO q8-12h

Pediatric

20-60 mg/kg/d PO q12h

Coadministration with warfarin or heparin increases risk of bleeding

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Give for a minimum of 10 d to eliminate organism and prevent sequelae (eg, endocarditis, rheumatic fever); following treatment, perform cultures to confirm eradication of streptococci

Expectorants

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.

Adult

500-1000 mg/d PO in divided doses

Pediatric

<6 years: 10-20 mg/kg/d PO in divided doses
6-12 years: 500 mg/d PO in divided doses
>12 years: Administer as in adults

May increase renal clearance of urate and lower serum uric acid levels; may interfere with urine laboratory tests for 5-hydroxyindoleacetic acid and urine testing for catecholamines

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

When prescribing medication that may suppress cough, important to identify cause of cough and ensure suppression will not increase risk of clinical or physiologic complications

More on Kartagener Syndrome

Overview: Kartagener Syndrome
Differential Diagnoses & Workup: Kartagener Syndrome
Treatment & Medication: Kartagener Syndrome
Follow-up: Kartagener Syndrome
References
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References

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Further Reading

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Keywords

Kartagener syndrome, KS, immotile cilia syndrome, primary ciliary dyskinesia, PCD, situs inversus, chronic sinusitis, bronchiectasis

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Contributor Information and Disclosures

Author

John P Bent lll, MD, Associate Professor, Director of Medical Student Education, Departments of Otolaryngology - Head and Neck Surgery and Pediatrics, Albert Einstein School of Medicine; Director, Airway Clinic, Children's Hospital at Montefiore
John P Bent lll, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Medical Association, American Rhinologic Society, American Society of Pediatric Otolaryngology, Society for Ear, Nose and Throat Advances in Children, and Society of University Otolaryngologists-Head and Neck Surgeons
Disclosure: Nothing to disclose.

Coauthor(s)

Esther X Vivas, MD, Staff Physician, Montefiore Medical Center, Bronx, New York
Esther X Vivas, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery and American Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Ryland P Byrd Jr, MD, Professor, Department of Internal Medicine, Division of Pulmonary Medicine and Critical Care Medicine, James H Quillen College of Medicine, East Tennessee State University; Chief of Pulmonary Medicine, Medical Director of Respiratory Therapy, Intensive Care Unit, Program Director of Pulmonary Diseases and Critical Care Medicine Fellowship, James H Quillen Veterans Affairs Medical Center
Ryland P Byrd Jr, MD is a member of the following medical societies: American College of Chest Physicians, American Thoracic Society, and Southern Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Daniel R Ouellette, MD, FCCP, Associate Professor of Medicine, Wayne State University School of Medicine; Consulting Staff, Pulmonary Disease and Critical Care Medicine Service, Henry Ford Health System
Daniel R Ouellette, MD, FCCP is a member of the following medical societies: American College of Chest Physicians and American Thoracic Society
Disclosure: Boehringer Ingleheim Honoraria Speaking and teaching; Pfizer Honoraria Speaking and teaching

CME Editor

Timothy D Rice, MD, Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, Saint Louis University School of Medicine
Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians
Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD, Director, Division of Pulmonary and Critical Care Medicine, Director, Women's Guild Pulmonary Disease Institute, Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center; Professor of Medicine, David Geffen School of Medicine at UCLA
Zab Mosenifar, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, and American Thoracic Society
Disclosure: Nothing to disclose.

 
 
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