Primary ciliary dyskinesia: evaluation using cilia beat frequency assessment via spectral analysis of digital microscopy images


Autoria(s): OLM, Mary A. K.; KOEGLER JR., Joao E.; MACCHIONE, Mariangela; SHOEMARK, Amelia; SALDIVA, Paulo H. N.; RODRIGUES, Joaquim C.
Contribuinte(s)

UNIVERSIDADE DE SÃO PAULO

Data(s)

19/10/2012

19/10/2012

2011

Resumo

Olm MA, Kogler JE Jr, Macchione M, Shoemark A, Saldiva PH, Rodrigues JC. Primary ciliary dyskinesia: evaluation using cilia beat frequency assessment via spectral analysis of digital microscopy images. J Appl Physiol 111: 295-302, 2011. First published May 5, 2011; doi:10.1152/japplphysiol.00629.2010.-Ciliary beat frequency (CBF) measurements provide valuable information for diagnosing of primary ciliary dyskinesia (PCD). We developed a system for measuring CBF, used it in association with electron microscopy to diagnose PCD, and then analyzed characteristics of PCD patients. 1 The CBF measurement system was based on power spectra measured through digital imaging. Twenty-four patients suspected of having PCD (age 1-19 yr) were selected from a group of 75 children and adolescents with pneumopathies of unknown causes. Ten healthy, nonsmoking volunteers (age >= 17 yr) served as a control group. Nasal brush samples were collected, and CBF and electron microscopy were performed. PCD was diagnosed in 12 patients: 5 had radial spoke defects, 3 showed absent central microtubule pairs with transposition, 2 had outer dynein arm defects, 1 had a shortened outer dynein arm, and 1 had a normal ultrastructure. Previous studies have reported that the most common cilia defects are in the dynein arm. As expected, the mean CBF was higher in the control group (P < 0.001) and patients with normal ultrastructure (P < 0.002), than in those diagnosed with cilia ultrastructural defects (i.e., PCD patients). An obstructive ventilatory pattern was observed in 70% of the PCD patients who underwent pulmonary function tests. All PCD patients presented bronchial wall thickening on chest computed tomography scans. The protocol and diagnostic techniques employed allowed us to diagnose PCD in 16% of patients in this study.

FAPESP Sao Paulo State Research Foundation[06/50063-5]

Identificador

JOURNAL OF APPLIED PHYSIOLOGY, v.111, n.1, p.295-302, 2011

8750-7587

http://producao.usp.br/handle/BDPI/22624

10.1152/japplphysiol.00629.2010

http://dx.doi.org/10.1152/japplphysiol.00629.2010

Idioma(s)

eng

Publicador

AMER PHYSIOLOGICAL SOC

Relação

Journal of Applied Physiology

Direitos

restrictedAccess

Copyright AMER PHYSIOLOGICAL SOC

Palavras-Chave #Kartagener`s syndrome #ciliary motility disorders #LUNG-FUNCTION #NITRIC-OXIDE #MUCOCILIARY CLEARANCE #FUNCTION TESTS #DIAGNOSIS #CHILDREN #ULTRASTRUCTURE #DISEASE #COHORT #ABNORMALITIES #Physiology #Sport Sciences
Tipo

article

original article

publishedVersion