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Titolo:
Primary ciliary dyskinesia: Diagnosis in children with inconclusive ultrastructural evaluation
Autore:
Pifferi, M; Cangiotti, AM; Ragazzo, V; Baldini, G; Cinti, S; Boner, AL;
Indirizzi:
Univ Pisa, Dept Pediat, I-56100 Pisa, Italy Univ Pisa Pisa Italy I-56100Univ Pisa, Dept Pediat, I-56100 Pisa, Italy Univ Ancona, Umberto I Hosp, Electron Microscopy Unit, Inst Normal Human Morphol, Ancona, Italy Univ Ancona Ancona Italy Unit, Inst Normal Human Morphol, Ancona, Italy Univ Verona, Dept Pediat, Misurina, Italy Univ Verona Misurina ItalyUniv Verona, Dept Pediat, Misurina, Italy Ist Pio XII, Misurina, Italy Ist Pio XII Misurina ItalyIst Pio XII, Misurina, Italy
Titolo Testata:
PEDIATRIC ALLERGY AND IMMUNOLOGY
fascicolo: 5, volume: 12, anno: 2001,
pagine: 274 - 282
SICI:
0905-6157(200110)12:5<274:PCDDIC>2.0.ZU;2-V
Fonte:
ISI
Lingua:
ENG
Soggetto:
MUCOCILIARY CLEARANCE; IMMOTILE-CILIA; BEAT FREQUENCY; KARTAGENERS SYNDROME; RESPIRATORY-TRACT; NASAL CILIA; MOTILITY; ABNORMALITIES; DISEASE; BRONCHIECTASIS;
Keywords:
primary ciliary dyskinesia; ciliary motion; ciliary ultrastructure; chronic respiratory disease; children;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
63
Recensione:
Indirizzi per estratti:
Indirizzo: Pifferi, M Univ Pisa, Dept Pediat, Via Roma 67, I-56100 Pisa, Italy Univ Pisa Via Roma 67 Pisa Italy I-56100 , I-56100 Pisa, Italy
Citazione:
M. Pifferi et al., "Primary ciliary dyskinesia: Diagnosis in children with inconclusive ultrastructural evaluation", PEDIAT A IM, 12(5), 2001, pp. 274-282

Abstract

The purpose of this study was to distinguish between acquired and genetically determined ciliary abnormalities in children with severe chronic respiratory diseases. Samples of nasal ciliated epithelium from 50 subjects (25 male. 25 female: age-range 2-19 years) with severe chronic respiratory diseases were examined using transmission electron microscopy (TEM). Based on TEM findings, patients were divided into two groups: A and B. Group A comprised 39 children with ciliary alterations compatible with a condition probably occuring secondary to chronic inflammation (alterations of peripheral pairs, swollen cilia, and compound cilia). The other 11 patients, Group B, exhibited a greater number of alterations of the central pair and dynein arms (p <0.001), which were qualitatively similar to, but less numerous than. those observed in primary ciliary dyskinesia (PCD). In both groups, analysis of ciliary beat frequency and waveform was performed by phase contrast microscopy (PCM). All the children with a ciliary beat frequency of <7 Hz were treated with daily physiotherapy and with antibiotics, as recommended for PCD, for a 6-month period. After this treatment. the children were reexamined by PCM. Almost 50% of the children from Group B (i.e. those with a small proportion of specific ultrastructural defects) showed permanence of low ciliary beat frequency. This was also observed in two children of Group A. These children were considered to be affected by PCD. Our study describes a method for the diagnosis of PCD in the absence of specific ultrastructural defects or when these defects are present in only a small proportion of the cilia.

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Documento generato il 01/04/20 alle ore 20:59:37