Background: Cepacia syndrome (CS) is an acute, necrotizing pneumonia with a high mortality rate, occurring in patients with cystic fibrosis (CF) infected with Burkholderia cepacia complex (BCC). Due to its low incidence, data on this condition are limited. Methods: We conducted a systematic review of the reported cases of CS by searching MEDLINE, Embase and the Cochrane Library to improve knowledge of this rare but potentially lethal condition. Results: We included 15 eligible articles, describing 18 cases (9 females) of CS. Median age at onset was 22 years (range: 10–60 years); median time to CS after first infection by BCC was 5 years (range: 1–26 years). Burkholderia cenocepacia was the most frequently reported causative agent. All patients received intravenous antibiotic treatment (most frequently including cotrimoxazole), while inhaled antibiotics were used in five patients (27.8%). Immunosuppressant agents were the most commonly prescribed supportive treatment (n = 7, 38.9%). Half of the patients died (9/18, 50%). Conclusions: This study describes epidemiological, clinical characteristics, and prognosis of CS cases reported over the last 24 years. CS is a rare yet severe complication of BCC infection in patients with CF, which occurs several years after BCC colonization and has a negative outcome in 50% of the patients. Data are too scanty to identify the most effective therapeutic approach.

Cepacia syndrome in cystic fibrosis: A systematic review of the literature and possible new perspectives in treatment / V. Dacco, G. Alicandro, A. Consales, C. Rosazza, C.S. Sciarrabba, L. Cariani, C. Colombo. - In: PEDIATRIC PULMONOLOGY. - ISSN 8755-6863. - 58:5(2023 May), pp. 1337-1343. [10.1002/ppul.26359]

Cepacia syndrome in cystic fibrosis: A systematic review of the literature and possible new perspectives in treatment

G. Alicandro
Secondo
;
A. Consales;C. Rosazza;C.S. Sciarrabba;L. Cariani
Penultimo
;
C. Colombo
Ultimo
2023

Abstract

Background: Cepacia syndrome (CS) is an acute, necrotizing pneumonia with a high mortality rate, occurring in patients with cystic fibrosis (CF) infected with Burkholderia cepacia complex (BCC). Due to its low incidence, data on this condition are limited. Methods: We conducted a systematic review of the reported cases of CS by searching MEDLINE, Embase and the Cochrane Library to improve knowledge of this rare but potentially lethal condition. Results: We included 15 eligible articles, describing 18 cases (9 females) of CS. Median age at onset was 22 years (range: 10–60 years); median time to CS after first infection by BCC was 5 years (range: 1–26 years). Burkholderia cenocepacia was the most frequently reported causative agent. All patients received intravenous antibiotic treatment (most frequently including cotrimoxazole), while inhaled antibiotics were used in five patients (27.8%). Immunosuppressant agents were the most commonly prescribed supportive treatment (n = 7, 38.9%). Half of the patients died (9/18, 50%). Conclusions: This study describes epidemiological, clinical characteristics, and prognosis of CS cases reported over the last 24 years. CS is a rare yet severe complication of BCC infection in patients with CF, which occurs several years after BCC colonization and has a negative outcome in 50% of the patients. Data are too scanty to identify the most effective therapeutic approach.
Burkholderia cepacia; cepacia syndrome; cystic fibrosis; cytokine storm syndrome;
Settore MED/10 - Malattie dell'Apparato Respiratorio
mag-2023
mar-2023
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/976628
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