Managing upper respiratory tract complications of primary ciliary dyskinesia in children

Campbell, Raewyn

doi:10.1097/aci.0b013e32834eccc6

Cited by 44 publications

(41 citation statements)

References 94 publications

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“…Advocates of tympanostomy (ventilation) tubes suggest that hearing may be improved (long term) in some patients, and otorrhea can be controlled (97). In contrast, the European Respiratory Society Consensus Statement recommends against placement of tubes for chronic otitis media in patients with PCD, because resultant otorrhea is a problem, and spontaneous resolution of chronic otitis media may occur in the teenage years (3,98).…”

Section: Management Of Otolaryngologic Manifestations Middle Ear Diseasementioning

confidence: 99%

“…Management of otitis media with effusion is controversial, even though it affects almost all children with PCD, and has implications for conductive hearing loss, delayed speech and language development, and cholesteatoma formation (18,97). Advocates of tympanostomy (ventilation) tubes suggest that hearing may be improved (long term) in some patients, and otorrhea can be controlled (97).…”

Section: Management Of Otolaryngologic Manifestations Middle Ear Diseasementioning

confidence: 99%

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Primary Ciliary Dyskinesia. Recent Advances in Diagnostics, Genetics, and Characterization of Clinical Disease

Knowles¹,

Daniels²,

Davis

et al. 2013

Am J Respir Crit Care Med

440

460

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Primary ciliary dyskinesia (PCD) is a genetically heterogeneous recessive disorder of motile cilia that leads to oto-sino-pulmonary diseases and organ laterality defects in approximately 50% of cases. The estimated incidence of PCD is approximately 1 per 15,000 births, but the prevalence of PCD is difficult to determine, primarily because of limitations in diagnostic methods that focus on testing ciliary ultrastructure and function. Diagnostic capabilities have recently benefitted from (1) documentation of low nasal nitric oxide production in PCD and (2) discovery of biallelic mutations in multiple PCD-causing genes. The use of these complementary diagnostic approaches shows that at least 30% of patients with PCD have normal ciliary ultrastructure. More accurate identification of patients with PCD has also allowed definition of a strong clinical phenotype, which includes neonatal respiratory distress in .80% of cases, daily nasal congestion and wet cough starting soon after birth, and early development of recurrent/chronic middle-ear and sinus disease. Recent studies, using advanced imaging and pulmonary physiologic assessments, clearly demonstrate early onset of lung disease in PCD, with abnormal air flow mechanics by age 6-8 years that is similar to cystic fibrosis, and age-dependent onset of bronchiectasis. The treatment of PCD is not standardized, and there are no validated PCD-specific therapies. Most patients with PCD receive suboptimal management, which should include airway clearance, regular surveillance of pulmonary function and respiratory microbiology, and use of antibiotics targeted to pathogens. The PCD Foundation is developing a network of clinical centers, which should improve diagnosis and management of PCD.

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Section: Management Of Otolaryngologic Manifestations Middle Ear Diseasementioning

confidence: 99%

Section: Management Of Otolaryngologic Manifestations Middle Ear Diseasementioning

confidence: 99%

Primary Ciliary Dyskinesia. Recent Advances in Diagnostics, Genetics, and Characterization of Clinical Disease

Knowles¹,

Daniels²,

Davis

et al. 2013

Am J Respir Crit Care Med

440

460

View full text Add to dashboard Cite

show abstract

“…Evidence-based treatment strategies are unavailable because of limited data (9). The current therapeutic strategy for KS includes medication, surgery, and some adjuvant therapies.…”

Section: Discussionmentioning

confidence: 99%

Endoscopic Sinus Surgery for Treatment of Kartagener Syndrome: A Case Report

Tang¹,

Zou²,

Liu³

2013

Balkan Med J

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Background: Kartagener syndrome (KS) is a rare congenital disease characterised by a clinical triad of symptoms: situs inversus, chronic rhinosinusitis, and bronchiectasis. Although congenital ciliary defect is recognised as the main cause of this syndrome, it remains difficult to treat the associated airway infection.Case Report: A 17-year-old female patient presented with repeated refractory airway infection. She also had bronchiectasis and situs inversus. Electron microscopic evaluation of her nasal mucosa revealed ciliary defect and confirmed the diagnosis of KS. She underwent functional endoscopic sinus surgery (FESS) followed by long-term postoperative debridement of the sinonasal cavity. This treatment reduced chronic rhinosinusitis and protected against subsequent airway infection in a 7-year follow-up.Conclusion: FESS is effective for relieving both chronic rhinosinusitis and lung infection of KS in the long term.

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“…Sino-nasal symptoms vary but it is estimated that chronic rhinosinusitis (CRS) affects over 50% of patients with PCD, with 15%-40% suffering from nasal polyposis [7,8]. Symptoms of CRS in PCD may be debilitating, as patients suffer from purulent nasal secretions and pansinusitis.…”

Section: Introductionmentioning

confidence: 99%

A Systematic Review of the Treatment of Chronic Rhinosinusitis in Adults with Primary Ciliary Dyskinesia

2017

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Abstract:Background: Primary ciliary dyskinesia (PCD) may be an underlying factor in some cases of refractory chronic rhinosinusitis (CRS). However, clinical management of this condition is not well defined. This systematic review examines the available evidence for the diagnosis and management of CRS in adults with PCD. Methods: A systematic review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Pubmed, EMBASE, and Cochrane database were queried for studies pertinent to treatment of PCD in adults. Two investigators performed eligibility assessment for inclusion or exclusion in a standardized manner. Results: Of the 278 articles identified, six studies met the criteria for analysis. These studies had a predominately low level of evidence. Medical therapy included oral antibiotics and nasal saline rinses. Endoscopic sinus surgery (ESS) was described in three of six studies. Outcomes measures were limited and included non-validated questionnaires, subjective reporting of CRS symptoms, and decreased preciptins against pseudomonas following ESS. Recommendation for a standardized therapeutic strategy was not possible with the available literature. Conclusion: A paucity of evidence is available to guide the treatment of PCD in the adult population. Further prospective studies are needed to determine the optimal diagnostic and management strategy for this condition.

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Managing upper respiratory tract complications of primary ciliary dyskinesia in children

Cited by 44 publications

References 94 publications

Primary Ciliary Dyskinesia. Recent Advances in Diagnostics, Genetics, and Characterization of Clinical Disease

Primary Ciliary Dyskinesia. Recent Advances in Diagnostics, Genetics, and Characterization of Clinical Disease

Endoscopic Sinus Surgery for Treatment of Kartagener Syndrome: A Case Report

A Systematic Review of the Treatment of Chronic Rhinosinusitis in Adults with Primary Ciliary Dyskinesia

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