Mortalidad en fibrosis quística: análisis retrospectivo de cinco años en institución de alta complejidad: Medellín - Colombia / Cystic fibrosis mortality: retrospective analysis of five years in a highly complex institution: Medellín - Colombia

INTRODUCTION: cystic fibrosis (CF) is a chronic, progressive disease with multisystem involvement and high mortality. An early diagnosis and a multidisciplinary approach lead to longer survival and better quality of life. OBJECTIVE: to characterize the patients who died with CF diagnosis in the period between 2011 and 2017 in the Hospital Universitario San Vicente in Colombia. METHODOLOGY: descriptive study of case series, with retrospective data collection. Results: 168 patients with CF were found in follow-up at the institution during the study period, of which 17 died (10,1%). Eleven deaths corresponded to children under 15 years old (64.7%), with equal distribution of gender (women 52.9% and men 47.1%). The median age at diagnosis was 3 years and median death 12 years. The most frequent clinical compromise was sinopulmonary and gastrointestinal. Pulmonary hypertension occurred in 29.4%. All the patients had a severe functional and nutritional compromise and 82.4% were colonized by Pseudomona aeruginosa. None had multidisciplinary management and most had a low socioeconomic level. BiPAP was used as a palliative measure in 6 patients and all died from ventilatory failure. CONCLUSION: CF mortality in our population continues to present at an early age. The diagnosis of the disease is still made late, compared to developed countries. There was a high proportion of bacterial colonization of the airway and the patients presented a severe clinical and functional status before dying.

INTRODUCCIÓN: la fibrosis quística (FQ) es una enfermedad crónica, progresiva, con compromiso multisistémico y de alta mortalidad. Un diagnóstico temprano y un manejo multidisciplinario llevan a una mayor sobrevida y mejor calidad de vida. OBJETIVO: caracterizar los pacientes que fallecieron con diagnóstico de FQ en el periodo comprendido entre 2011 y 2017 en el Hospital Universitario San Vicente Fundación (HUSVF) de Colombia. METODOLOGÍA: estudio descriptivo de series de casos, con recolección de la información de forma retrospectiva. RESULTADOS: Se encontraron 168 pacientes con FQ en seguimiento en la institución durante el periodo del estudio, de los cuales 17 fallecieron (10.1%). Once muertes correspondieron a menores de 15 años (64.7%), hubo una distribución de género equitativa (mujeres 52.9% y hombres 47.1%). La mediana de edad al momento del diagnóstico fue de 3 años y la de muerte 12 años. El compromiso clínico más frecuente fue sinopulmonar y gastrointestinal. La hipertensión pulmonar se presentó en el 29.4%. Todos los pacientes tenían un grave compromiso funcional, nutricional y el 82,4% estaban colonizados por Pseudomonas aeruginosa. Ninguno tuvo manejo multidisciplinario y la mayoría presentaban un bajo nivel socieconómico. En 6 pacientes se utilizó BiPAP como medida paliativa y todos murieron por falla ventilatoria. CONCLUSIÓN: la mortalidad por FQ en nuestra población se sigue presentando a edades tempranas. El diagnóstico de la enfermedad aún se realiza de forma tardía, comparado con países desarrollados. Hubo alta proporción de colonización bacteriana de la vía aérea y los pacientes presentaron un estado clínico y funcional grave antes de morir.

Burkholderia cepacia, cystic fibrosis and outcomes following lung transplantation: experiences from a single center in Brazil

OBJECTIVES: To evaluate the impact of Burkholderia cepacia complex colonization in cystic fibrosis patients undergoing lung transplantation. METHODS: We prospectively analyzed clinical data and respiratory tract samples (sputum and bronchoalveolar lavage) collected from suppurative lung disease patients between January 2008 and November 2013. We also subtyped different Burkholderia cepacia complex genotypes via DNA sequencing using primers against the recA gene in samples collected between January 2012 and November 2013. RESULTS: From 2008 to 2013, 34 lung transplants were performed on cystic fibrosis patients at our center. Burkholderia cepacia complex was detected in 13 of the 34 (38.2%) patients. Seven of the 13 (53%) strains were subjected to genotype analysis, from which three strains of B. metallica and four strains of B. cenocepacia were identified. The mortality rate was 1/13 (7.6%), and this death was not related to B. cepacia infection. CONCLUSION: The results of our study suggest that colonization by B. cepacia complex and even B. cenocepacia in patients with cystic fibrosis should not be considered an absolute contraindication to lung transplantation in Brazilian centers.

Microorganismos involucrados en las infecciones respiratorias de pacientes con fibrosis quísitca / Microorganisms involved in respiratory infections in cystic fibrosis patients

La fibrosis quística es la enfermedad autosómica recesiva letal más frecuente en la infancia. Se caracteriza por presentar una evolución crónica, progresiva y compromiso multisistémico. El objetivo de este trabajo fue conocer la frecuencia de los microorganismos implicados en las infecciones respiratorias de pacientes fibroquísticos atendidos en el Hospital de Pediatría Prof. Dr. Juan P. Garrahan durante el año 2012 y su resistencia a los antimicrobianos. Para la identificación bacteriana se utilizaron pruebas bioquímicas convencionales, sistemas automatizados y semiautomatizados. En la identificación de miembros del complejo Burkholderia cepacia se utilizaron además métodos moleculares. De esta manera se pudo lograr la clasificación genética de las especies incluidas dentro de ese complejo presentes en los pacientes fibroquísticos de nuestro medio. Este trabajo nos permitió conocer la situación microbiológica actual de las infecciones respiratorias en los pacientes fibroquísticos. Tanto el estudio minucioso de los cultivos convencionales como la caracterización molecular de las especies de B. cepacia deben seguirse en los pacientes colonizados por microorganismos multirresistentes y son imprescindibles en el control postratamiento después del aislamiento de estos patógenos (AU)

Cystic fibrosis is the most common lethal autosomal recessive disease in childhood. It is characterized by a chronic, progressive evolution and multisystemic involvement. The aim of this study was to assess the incidence of the microorganisms involved in respiratory infections of patients with cystic fibrosis seen at the Pediatric Hospital Prof. Dr. Juan P. Garrahan in 2012 and their resistance to antimicrobial agents. To identify the microorganisms conventional biochemical tests with automatized and semiautomatized systems were used. For the identification of members of the Burkholderia cepacia complex molecular studies were additionally used. Species of this complex found in cystic fibrosis patients in our setting were genetically classified allowing for the definition of the current microbiological situation of respiratory infections in cystic fibrosis patients. Careful study of conventional cultures as well as molecular typing of the B. cepacia species should be routinely performed in patients colonized by multiresistant microorganisms and is fundamental in the post-treatment monitoring after the isolation of these pathogens (AU)

Infecção bacteriana crônica e indicadores ecocardiográficos de hipertensão pulmonar em pacientes com fibrose cística / Chronic bacterial infection and echocardiographic parameters indicative of pulmonary hypertension in patients with cystic fibrosis

OBJETIVO: Determinar as relações entre infecção bacteriana crônica e hipertensão pulmonar, avaliada por ecocardiografia Doppler, em pacientes com fibrose cística (FC). MÉTODOS: Estudo transversal e prospectivo em pacientes com FC (idade > 16 anos) atendidos por um programa para adultos com a doença. O estudo incluiu 40 pacientes com média de idade de 23,7 ± 6,3 anos. Os pacientes foram submetidos a avaliação clínica, ecocardiografia Doppler, testes de função pulmonar, exame radiológico do tórax e exames culturais do escarro de Pseudomonas aeruginosa e Burkholderia cepacia. RESULTADOS: Não foram observadas diferenças entre os casos positivos para P. aeruginosa e os negativos para P. aeruginosa quanto às seguintes variáveis: escore clínico (p = 0,472); volume expiratório forçado no primeiro segundo (VEF1; p = 0,693); escore radiológico (p = 0,760); velocidade de regurgitação tricúspide (VRT, p = 0,330); diâmetro do ventrículo direito (DVD, p = 0,191); e tempo de aceleração sistólica (TAS) do ventrículo direito/artéria pulmonar (VD/AP, p = 0,330). O VEF1 foi significativamente menor nos casos positivos para B. cepacia do que nos casos negativos para B. cepacia (p = 0,011). Não foram observadas diferenças entre os casos positivos para B. cepacia e os casos negativos para B. cepacia quanto às seguintes variáveis: escore clínico (p = 0,080); escore radiológico (p = 0,760); VRT (p = 0,613); DVD (p = 0,429); e TAS do VD/AP (p = 0,149). CONCLUSÕES: Não foi observada relação entre infecção crônica por P. aeruginosa e por B. cepacia com hipertensão pulmonar em pacientes adultos com FC. A função pulmonar foi pior nos pacientes positivos para B. cepacia do que nos pacientes positivos para P. aeruginosa.

OBJECTIVES: To examine the relationship between chronic bacterial infection and pulmonary hypertension, using Doppler echocardiography, in patients with cystic fibrosis (CF). METHODS: A prospective cross-sectional study involving CF patients (>16 years of age) admitted to a program for adults with the disease. The study included 40 patients with a mean age of 23.7 ± 6.3 years. Patients were submitted to clinical evaluation, Doppler echocardiography, pulmonary function tests, chest X-rays and sputum cultures of Pseudomonas aeruginosa and Burkholderia cepacia. RESULTS: In terms of the following variables, no significant differences were found between P. aeruginosa-positive patients and P. aeruginosa-negative patients: clinical score (p = 0.472); forced expiratory volume in one second (FEV1; p = 0.693), radiological score (p = 0.760); tricuspid regurgitant jet velocity (TRV, p = 0.330); diameter of the right ventricle (DRV, p = 0.191); and right ventricular/pulmonary artery (RV/PA) systolic acceleration time (SAT, p = 0.330). B. cepacia-positive patients presented significantly lower FEV1 than did B. cepacia-negative patients (p = 0.011). No significant differences were found between B. cepacia-positive patients and B. cepacia-negative patients regarding the following variables: clinical score (p = 0.080); radiological score (p = 0.760); TRV (p = 0.613); DRV (p = 0.429); and RV/PA SAT (p = 0.149). CONCLUSIONS: Chronic infection with P. aeruginosa or B. cepacia presented no association with pulmonary hypertension in adult CF patients. Pulmonary function was worse in B. cepacia-positive patients than in P. aeruginosa-positive patients.

Solubilization of insoluble inorganic phosphate by Burkholderia cepacia DA23 isolated from cultivated soil

A mineral phosphate solubilizing bacterium, Burkholderia cepacia DA23 has been isolated from cultivated soils. Phosphate-solubilizing activities of the strain against three types of insoluble phosphate were quantitatively determined. When 3 percent of glucose concentration was used for carbon source, the strain had a marked mineral phosphate-solubilizing activity. Mineral phosphate solubilization was directly related to the pH drop by the strain. Analysis of the culture medium by high pressure liquid chromatography identified gluconic acid as the main organic acid released by Burkholderia cepacia DA23. Gluconic acid production was apparently the result of the glucose dehydrogenase activity and glucose dehydrogenase was affected by phosphate regulation.

Uma bactéria capaz de solubilizar fosfato mineral, Burkholderia cepacea DA23, foi isolada de solo cultivado. A capacidade dessa bactéria solubilizar o fosfato de três tipos de fosfato insolúvel foi quantificada. Quando foi utilizada glicose a 3 por cento como fonte de carbono, a bactéria apresentou uma intensa atividade solubilizante de fosfato, sendo a solubilização diretamente relacionada com a queda de pH causada pela bactéria. A análise do meio de cultura por cromatografia líquida de alta pressão indicou o ácido glicônico como principal ácido produzido por Burkholderia cepacea DA23. Aparentemente, a produção de ácido glicônico foi causada pela atividade da glicose desidrogenase. A enzima foi afetada pela regulação do fosfato.

Prevalência de bactérias potencialmente patogênicas em espécimes respiratórios de fibrocísticos do Recife / Prevalence of potentially pathogenic bacteria in respiratory specimens of cystic fibrosis patients from Recife

Entre 147 espécimes respiratórios (114 escarros e 33 swabs faríngeos) coletados de 36 portadores de fibrose cística durante consultas de rotina ou na exacerbação de seus sintomas respiratórios, no período de dezembro de 2000 a dezembro de 2002, isolaram-se: Pseudomonas aeruginosa (65,3%), Staphylococcus aureus (29,9%), Burkholderia cepacia (29,2%) e Haemophilus influenzae (20,4%). Entre os isolados de S. aureus e H. influenzae, 6,8% foram resistentes à oxacilina e 6,7% foram produtores de beta-lactamase, respectivamente. Das 96 linhagens de P. aeruginosa encontradas, 59,4% foram do fenótipo mucóide. Em 12 espécimes, ambos os biótipos, mucóide e não-mucóide, estiveram presentes. Bactérias gram-negativas emergentes, tais como Stenotrophomonas maltophilia e Achromobacter xylosoxidans, foram isoladas em pequeno número. Com exceção do H. influenzae, mais freqüente nas crianças entre seis e 12 anos, não se encontrou diferença entre espécie bacteriana isolada e grupo etário.

Of 147 respiratory specimens (114 sputum and 33 pharyngeal swabs) collected from 36 cystic fibrosis patients during routine visits or exacerbation of their respiratory symptoms, from December 2000 to December 2002, the following bacterial species were recovered: Pseudomonas aeruginosa (65,3%), Staphylococcus aureus (29,9%), Burkholderia capacia (29,2%), and Haemophilus influenzae (20,4%). Among the S. aureus and H. influenzae isolates, 6,8% were oxacillin resistant and 6,7% were b-lactamase producers, respectively. Of 96 isolates of P. aeruginosa, 59.4% belonged to the mucoid phenotype. Both mucoid and non-mucoid morphotypes were simultaneously found in 12 specimens. Emerging gram-negative bacteria, such as Stenotrophomonas maltophilia and Achromobacter xylosoxidans, were present at a low number. H. influenzae was more prevalent in the cystic fibrosis children between six and 12 years old. Concerning the other bacterial species there was not preference for age groups.

Control of burkholderia [pseudomonas] bacteraemia in the intensive care and paediatric units

Investigation and control of nosocomial bacteraemia caused by Burkholderia [Pseudomonas] cepacia in the Intensive Care and Paediatric Units of a general care hospital. Design: A cross-sectional analytical study. Place and Duration of Study: Departments of Pathology, Intensive Care and Paediatrics, Kahota Research Laboratory Hospital, Islamabad from January 1998 to June 2002. Subjects and Blood cultures from patients admitted to Intensive Care Unit and Paediatric Ward were inoculated into brain heart infusion broth and incubated for upto 10 days. Any Gram-negative rods isolated were characterized by API-20E. Environmental samples were inoculated on blood and MacConkey's agars and isolates, if any, were identified as above. Intensive intervention in the form of hand washing, strict adherence to aseptic practices and standard sterilization techniques were adopted and then cultures were again carried out with similar methodology. Cultures yielded 58 strains of Burkholderia cepacia, 52 from blood cultures and 6 from hospital environment, including 1 from the washbasin of the ICU. Thirty- four of these were isolated before intervention measures were adopted, mainly during 1998. Findings suggested a strong probability of nosocomial transmission, with washbasin as the common source. After a lapse of about a year, B. cepacia infection re-emerged in a sporadic form but remaining confined to paediatric unit. Only 18 isolates were yielded over the next two and-a-half years. The intervention measures for Burkholderia bacteraemia within the hospital, proved effective in stopping the nosocomial transmission leading to disappearance of B. cepacia from blood cultures. We emphasize the crucial role of hand hygienic practices in the hospital setting, especially in critical care units

Identificación y caracterización de bacilos gramnegativos no fermentados aislados en el medio hospitalario / Identification and characterization of nonfermenting gramnegative bacili isolated from a hospital environment

Se estudio un total de 251 cepas aisladas en el medio hospitalario con diagnóstico presuntivo de bacilos gramnegativos no fermentadores, enviadas por los Laboratorios de Infecciones Nosocomiales de los Centros Provinciales de Higiene y Epidemiología de Ciudad de La Habana, Santiago de Cuba, Holguín, Guantánamo, Camagüey e Isla de la Juventud desde septiembre de 1992 hasta junio de 1993. Se empleó un esquema inicial para corroborar el diagnóstico primario de estas cepas, los medios de producción de pigmentos King A y King B para la identificación de Pseudomonas aeruginosas y pruebas bioquímicas claves para el diagnóstico microbiológico de otros no fermentadores, se utilizó la piocinotipia y la serotipia para la caracterización posterior de la especie aeruginosa. Resultaron confirmadas 238 cepas y de ellas, el 88,23 por ciento correspondió a la especie aeruginosa cuyos piocinotipo y serotipo predominantes fueron al 10a y 011, respectivamente. Otros bacilos encontrados con frecuencia fueron Pseudomonas cepacia (25 por ciento); Pseudomonas fluorescens (17,8 por ciento) y Acinetobacter calcoaceticus var. anitratus 17,8 por ciento

Infecçöes do catéter venoso central / Infections of central venous catheter

O catéter venoso central é considerado a linha vital de sutentaçäo dos pacientes dependentes de nutriçäo venosa prolongada, e suas complicaçöes infecciosas constituem freqüente causa de internaçäo e morbidade. Os modernos catéteres de Silastic aumentaram a durabilidade e eficiência do acesso venoso, mas sua substituiçäo é também mais complexa e dispendiosa. Numa pequena série de pacientes submetidos a nutriçäo parenteral domiciliar por síndrome do intestino curto, e portadores de catéteres de Silastic, a infecçäo da cânula foi tratada mediante a oferta de antibióticos sistêmicos combinados com instilaçäo local de antimicrobianos. Houve integral regressäo do processo infeccioso sem necessidade de remoçäo do catéter, e com excelente tolerância à medicaçäo. Conclui-se que a esterilizaçäo in-situ de catéteres venosos de longa duraçäo merece ser tentada em casos selecionados

Formulation of culture medium for growth of microbes from the sugar cane industry and use of cetyltrimethylammonium bromide as antimicrobial agent

Foi desenvolvido um meio adequado para cultivo de microrganismos importantes na indústria de acúcar e alcool. A eficácia do meio foi determinada medindo-se os parâmetros de crescimento dos principais microrganismos contaminantes do caldo de cana que säo exigentes quanto aos fatores de crescimento. Esse meio teve base no caldo de cana desde que a sua finalidade era avaliaçäo dos agentes antimicrobianos comumente usados no controle de contaminaçäo da indústria sucro-alcooleira. Como exemplo de sua utilizaçäo, o comportamento do brometo de cetiltrimetilamônio (CTAB) no meio de cana 11 foi analisado quanto à sua atividade sobre os microrganismos selecionados. Bactérias do ácido acético e do ácido láctico foram os mais susceptíveis seguidas de leveduras, Pseudomonas acidovorans e depois pelas enterobactérias. Algumas Pseudomonas (Pseudomonas aeruginosa, Ps. cepacia e Ps. mendocina) foram extremamente resistentes no CTAB, provavelmente em virtude da impermeabilidade do invólucro celular. A eficácia do CTAB em concentraçöes muito baixas sobre os microrganismos de interesse à indústria sucro-alcooleira pode ser medida por essa técnica