The clinical course of Burkholderia cepacia complex bacteria respiratory infection in cystic fibrosis patients

Susana Correia 1

Catarina Nascimento 1

Luísa Pereira 2

Mónica V Cunha 3

Isabel Sá-Correia 4

Celeste Barreto 5

Resumo

O complexo Burkholderia cepacia (Bcc) é um grupo constituído por nove espécies de bactérias patogénicas oportunistas na fibrose quística (FQ), associadas a prognóstico mais reservado e a infecção cruzada entre os doentes. Existe grande heterogeneidade na deterioração pulmonar dos doentes colonizados/infectados com Bcc, evoluindo, por vezes, de forma fulminante – síndroma da cepacia.

Com o objectivo de avaliar a relação entre a colonização/infecção com as diferentes espécies do Bcc e a evolução clínica, os autores analisaram, retrospectivamente, 31 doentes com FQ acompanhados no Hospital de Santa Maria com isolamentos entre Janeiro de 1995 e Março de 2006. Os doentes foram divididos nos grupos: Grupo I – isolamento intermitente (15 doentes) e Grupo II – isolamento crónico (16 doentes). A prevalência das espécies do Bcc foi: B. cepacia 57%, B. cenocepacia 43%, B. multivorans 7%, B. stabilis 13%. Três doentes faleceram com síndroma da cepacia. As espécies B. cepacia e B. stabilis, pouco frequentes nas populações de FQ caracterizadas na Europa e na América do Norte, foram isoladas de uma percentagem importante dos doentes estudados, não tendo sido possível estabelecer uma correlação entre a espécie e a evolução clínica.

Nos doentes deteriorados, mas não nos estáveis, do grupo II, em quem foi possível analisar retrospectivamente a função respiratória (FEV1) e os períodos de internamento por exacerbação pulmonar, encontraram-se algumas diferenças relevantes antes e após o isolamento de Bcc.

Perante a incapacidade actual de orientar as medidas de profilaxia através da caracterização molecular dos isolados de Bcc, há que manter as medidas de controlo recomendadas.

 Palavras-chave: Complexo Burkholderia cepacia (Bcc), fibrose quística (FQ), B. cepacia, B. cenocepacia, B. multivorans, B. stabilis

Bacteria of the Burkholderia cepacia complex (Bcc), a group of nine related species, are opportunistic pathogens in cystic fibrosis (CF) patients, associated with a poor prognosis and patient-to-patient transmissibility.

The pulmonary deterioration in Bcc-colonised/infected patients has a heterogeneous pattern leading, sometimes, to a fulminant development – the cepaciasyndrome.

To evaluate the relationship between colonisation/infection by the different Bcc species and the clinical course, the authors carried out a retrospective study of 31 CF patients with Bcc bacteria isolations followed at Hospital de Santa Maria from January 1995 to March 2006. Patients were categorised into two groups: Group I, with intermittent isolations and Group II with chronic isolations. The prevalence of Bcc species was as follows: B. cepacia 57%, B. cenocepacia 43%, B. multivorans 7%, B. stabilis 13%.

Three of the patients died of cepacia syndrome. The species B. cepacia and B. stabilis, usually less frequent in CF populations of Europe and America, were isolated in a considerable percentage of the patients examined. No correlation could be established between the species and the clinical outcome.

Deteriorated but not stable patients from group II, whose lung function and pulmonary exacerbationcaused hospitalisation could be retrospectively analysed, exhibited significant differences in the number of hospitalisations and pulmonary function (FEV1) in the year prior to and the years following Bcc isolation.

Based on the available data, it is not currently possible to outline preventive measures through the molecular characterisation of Bcc isolates, reinforcing the notion that the recommended control measures must be followed.

Key-words: Burkholderia cepacia (Bcc) complex, cystic fibrosis (CF), B. cepacia, B. cenocepacia, B. multivorans, B. stabilis

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1 Interna do Internato Complementar de Pediatria / Resident, Specialist Training, Paediatrics

2 Assistente Hospitalar / Consultant

Centro Especializado em fibrose quística (Coordenadora: Dr.ª Celeste Barreto) / Cystic Fibrosis Centre (Coordinator: Dr Celeste Barreto)

Unidade de Pneumologia (Coordenadora: Dr.ª Celeste Barreto) / Pulmonology Unit (Coordinator: Dr Celeste Barreto)

Departamento da Criança e da Família (Director: Prof. J. Gomes Pedro) / Child and Family Department (Director: Prof. J. Gomes Pedro)

Hospital de Santa Maria, Av. Prof. Egas Moniz, 1699 Lisboa

3 Investigadora Doutorada / PhD Researcher

4 Professora Catedrática / Cathedratic Professor

IBB – Institute for Biotechnology and Bioengineering, Centro de Engenharia Biológica e Química, Instituto Superior Técnico.

Av. Rovisco Pais, 1049-001 Lisboa, Portugal

Correspondência / Correspondence to:

Susana Correia

Departamento da Criança e da Família, Hospital de Santa Maria

Av. Prof. Egas Moniz, 1699 Lisboa, Portugal

E-mail: susanaamcorreia@hotmail.com

Recebido para publicação/received for publication: 07.07.18

Aceite para publicação/accepted for publication: 07.10.15