[Changes in the motor apparatus of the bronchial cilia. Comparative study of two types of myopathies: nemaline myopathy and the Werdnig-Hoffmann syndrome].
The Authors during an extensive applications of nasal brushing in infancy according to Rutland and Cole, remarked--first time in literature--the contemporary presence in a child of the nemaline myopathy syndrome and immotile cilia syndrome. Supposing the possibility that in other myopathy may be present the same ciliary immotile syndrome, they studied with nasal brushing three cases of Werdnig-Hoffman syndromes: but they presented normal ciliary conformation. The Authors suppose that in case in future it should be found in other cases of nemaline myopathy the contemporary presence of dinein arms lack, it should be demonstrated that this syndrome takes derivation from a genetic alteration both of the muscular apparatus and the ciliary mobility system of bronchial epithelius.
Calì
A
A
Istituto di Anatomia Patologica, IIa Facoltà dell'Università di Napoli, Italia.
Berni Canani
M
M
De Luca
L
L
Terraciano
L
L
Foggia
L
L
Corradino
G
G
Berni Canani
R
R
Chiummariello
S
S
D001980:Bronchi / Q000473:pathology*; D002648:Child; D002923:Cilia / Q000648:ultrastructure; D002925:Ciliary Motility Disorders / Q000473:pathology; D006801:Humans; D009135:Muscular Diseases / Q000473:pathology; D014897:Spinal Muscular Atrophies of Childhood / Q000473:pathology
L'alterazione dell'apparato motore delle ciglia bronchiali. Studio in rapporto a due diversi tipi di miopatia: la nemaline myopathy e la sindrome di Werdnig-Hoffmann.