RESUMO
This paper presents the results of examination of children with primary ciliary dyskinesia. It has been found that ENT pathologies form an obligate characteristic of this lesion. Primary ciliary dyskinesia can be reliably diagnosed with the aid special examination methods. Adequate treatment by an ENT doctor and a pulmonologist yields good results.
Assuntos
Transtornos da Motilidade Ciliar/diagnóstico , Doenças Respiratórias/etiologia , Adolescente , Bronquite/etiologia , Bronquite/terapia , Criança , Pré-Escolar , Transtornos da Motilidade Ciliar/terapia , Diagnóstico Diferencial , Humanos , Doenças Respiratórias/diagnóstico , Doenças Respiratórias/terapiaRESUMO
Immunofluorescence was employed to study distribution of collagen type I, III, IV and V, and fibronectin in skin biopsy specimens from patients with Ehlers-Danlos syndrome (EDS) and cutis laxa. Abnormal distribution of collagen type V in the skin biopsies was found out in EDS type VII. A high level of dermal fibronectin occurred in EDS type III. Tissue fibronectin was absent in the skin extracellular matrix in EDS type X. Defective distribution of collagen, predominantly of type III and V and fibronectin absence were registered in the skin connective tissue in cutis laxa.