Cilia Motility and Structure in Primary and Secondary Ciliary Dyskinesia

Armengot-Carceller, Miguel; Milara, Javier; Mata, Manuel; Cardá, Carmen; Cortijo, Julio

doi:10.2500/ajra.2010.24.3448

Cited by 46 publications

(45 citation statements)

References 21 publications

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“…For 6 of these the diagnosis of PCD was confirmed but one (14.3%) subject was diagnosed with secondary ciliary dyskinesia (SCD) [19,20]. In this patient the nNO levels at first evaluation were 333.3 ppb, and 324.0 ppb at the second nNO concentration test.…”

Section: Nno Levelsmentioning

confidence: 90%

“…The diagnosis of PCD is based on the confirmation of typical Ultra structural defects by transmission electron microscopy and abnormal ciliary motion analysis and is identified in ~90% of patients; it also involves the outer dynein arms, inner dynein arms, or both [2,3]. The majority of these patients have an early onset of symptoms [1,2], and neonatal respiratory distress is common [11,12]; but there are patients with PCD that have minimal or absence of transmission electron microscopy found defects (i.e., atypical PCD or SCD), and a wide spectrum of disease variability [1,[19][20][21][22]. The impaired mucociliary clearance is commonly associated with recurrent or chronic respiratory tract infections leading to sinusitis, serous otitis media, rhinitis, bronchitis and pneumonia.…”

Section: Discussionmentioning

confidence: 99%

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Different Levels of Exhaled Nasal Nitric Oxide in Patients Diagnosed with Primary Dyskinesia

Bodini¹,

Peroni²,

Tenero³

et al. 2015

Arch Pulmonol Respir Care

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Background: Primary ciliary dyskinesia (PCD) is a genetic disease characterized by abnormally beating cilia. In these patients levels of nasal nitric oxide (nNO) are lower than those observed in healthy subjects. Objectives:We recorded the nNO levels in PCD patients in order to use those nNO measurements in the screening and identification of patients with symptoms suggestive of disease PCD disease. Methods:We measured nasal NO in 36 PCD patients (3 uncooperative younger children and 33 cooperative adult patients) and did a nNO re-evaluation after 12 months in patients with higher levels of nNO.Results: Twenty-seven PCD patients showed very low nNO levels (29.1 ppb) and nine cooperative patients had high nNO levels (583.3 ppb, p<0.001) (T0); the PCD patients with high nNO levels were re-evaluated after 12 months (T1).The median T0 and T1 nNO values of the seven PCD patients were 360 ppb and 324 ppb (p=0.0180), respectively; in 6 patients with high levels of nNO the diagnosis of PCD was confirmed by electron microscopy, and in one subject the diagnosis was confirmed for secondary ciliary dyskinesia.Conclusions: Low levels of nNO remain indicative of PCD disease; high levels of nNO are supportive of PCD, but cannot be used to exclude diagnosis.These results suggest that repeated measures are warranted when nNO is occasionally high in patients with symptoms suggestive of PCD disease, and at present electron microscopy is still the only valid evaluation tool in unclear cases of PCD.

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Section: Nno Levelsmentioning

confidence: 90%

Section: Discussionmentioning

confidence: 99%

Different Levels of Exhaled Nasal Nitric Oxide in Patients Diagnosed with Primary Dyskinesia

Bodini¹,

Peroni²,

Tenero³

et al. 2015

Arch Pulmonol Respir Care

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“…Then, chronic stimulation/irritation of epithelial cells may lead to loss of already formed cilia and impairs the ciliogenesis. As the result, may appear atypical, de novo growing cilia, which reveal the abnormal movement pattern [12,17,19]. Obviously, the impaired cilia movement significantly increases the susceptibility to further infection [2].…”

Section: Secondary Ciliary Dyskinesiamentioning

confidence: 99%

“…On the other hand, the abnormal function of cilia, commonly known as the ciliary dyskinesia, may be secondary to various problems in cilia formation and elongation. Depending on the origin of these impairments, ciliary dyskinesia may be divided into a primary and secondary form [12,19].…”

Section: Ciliary Dyskinesiamentioning

confidence: 99%

Chronic inflammation in the respiratory tract and ciliary dyskinesia

Grzela¹,

Zagórska²,

Jankowska‐Steifer³

et al. 2013

cejoi

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“…On the contrary, several authors (3)(4)(5)(6) in Europe recommend the use of ciliary beat pattern and frequency analysis by high-speed video microscopy as a necessary first step in the diagnosis of PCD. High-speed video microscopy is an essential piece of the puzzle that leads to the definitive diagnosis, as well as the clinical picture, nasal NO measurements, and ultrastructural electron microscopy analysis.…”

mentioning

confidence: 99%