Discinesia ciliar primária Primary ciliary dyskinesia

Discinesia ciliar primária é uma doença autossômica recessiva caracterizada pela história de infecções repetidas do trato respiratório superior e inferior, otite média, bronquite e rinossinusite, associada a situs inversus na metade dos casos. O diagnóstico é estabelecido pela análise ciliar ultra-estrutural de espécimes respiratórios, após a exclusão inicial de outras doenças, como fibrose cística, deficiência de alfa-1-antitripsina, imunodeficiências (IgG, neutrófilos e complemento) e síndrome de Young. O propósito deste artigo é revisar os achados clínicos, o diagnóstico e o manejo da discinesia ciliar primária, incluindo um fluxograma diagnóstico.<br>Primary ciliary dyskinesia is an autosomal recessive disorder characterized by a history of recurrent upper and lower respiratory tract infections with chronic otitis media, bronchitis and rhinosinusitis, associated with situs inversus in 50% of cases. The diagnosis is established by ciliary ultrastructural analysis of respiratory specimens, after ruling out some disorders as cystic fibrosis, alpha-1 anti-trypsin deficiency, immune deficiencies (IgG, neutrophils and complement) and Young's syndrome. The purpose of this paper is to review the clinical features, diagnosis and management of primary ciliary dyskinesia, including a diagnostic algorithm

A Streptococcus pneumoniae infection model in larvae of the wax moth Galleria mellonella

The bacterium Streptococcus pneumoniae is a leading human opportunistic pathogen. The limitations of the current vaccine have led to increased recognition of the need to understand bacterial behaviour and competitive dynamics using in vivo models of infection. Here, we investigate the potential application of the larvae of the wax moth Galleria mellonella as an informative infection model. Larvae were challenged with a range of doses of S. pneumoniae isolates differing in known virulence factors to determine the LD(50) values. Infection dynamics were determined by obtaining bacterial counts from larvae over a time course. Differences in virulence between serotypes could be distinguished in this host. Infection with strains differing in known virulence factors demonstrated predicted differences in virulence. Acapsulate and pneumolysin-negative strains were less virulent than their respective wild types. A large reduction in virulence was seen in strains lacking cell wall D-alanylation. The mortality of G. mellonella larvae is attributable to bacterial growth within larvae, while surviving larvae are able to clear infections by reducing bacterial numbers. These data demonstrate that G. mellonella larvae represent an in vivo infection model with applications for investigating aspects of bacterial-host interactions such as the role of antimicrobial peptide activity and resistance