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1.
Lancet Respir Med ; 2019 Sep 27.
Artigo em Inglês | MEDLINE | ID: mdl-31570318

RESUMO

The past six decades have seen remarkable improvements in health outcomes for people with cystic fibrosis, which was once a fatal disease of infants and young children. However, although life expectancy for people with cystic fibrosis has increased substantially, the disease continues to limit survival and quality of life, and results in a large burden of care for people with cystic fibrosis and their families. Furthermore, epidemiological studies in the past two decades have shown that cystic fibrosis occurs and is more frequent than was previously thought in populations of non-European descent, and the disease is now recognised in many regions of the world. The Lancet Respiratory Medicine Commission on the future of cystic fibrosis care was established at a time of great change in the clinical care of people with the disease, with a growing population of adult patients, widespread genetic testing supporting the diagnosis of cystic fibrosis, and the development of therapies targeting defects in the cystic fibrosis transmembrane conductance regulator (CFTR), which are likely to affect the natural trajectory of the disease. The aim of the Commission was to bring to the attention of patients, health-care professionals, researchers, funders, service providers, and policy makers the various challenges associated with the changing landscape of cystic fibrosis care and the opportunities available for progress, providing a blueprint for the future of cystic fibrosis care. The discovery of the CFTR gene in the late 1980s triggered a surge of basic research that enhanced understanding of the pathophysiology and the genotype-phenotype relationships of this clinically variable disease. Until recently, available treatments could only control symptoms and restrict the complications of cystic fibrosis, but advances in CFTR modulator therapies to address the basic defect of cystic fibrosis have been remarkable and the field is evolving rapidly. However, CFTR modulators approved for use to date are highly expensive, which has prompted questions about the affordability of new treatments and served to emphasise the considerable gap in health outcomes for patients with cystic fibrosis between high-income countries, and low-income and middle-income countries (LMICs). Advances in clinical care have been multifaceted and include earlier diagnosis through the implementation of newborn screening programmes, formalised airway clearance therapy, and reduced malnutrition through the use of effective pancreatic enzyme replacement and a high-energy, high-protein diet. Centre-based care has become the norm in high-income countries, allowing patients to benefit from the skills of expert members of multidisciplinary teams. Pharmacological interventions to address respiratory manifestations now include drugs that target airway mucus and airway surface liquid hydration, and antimicrobial therapies such as antibiotic eradication treatment in early-stage infections and protocols for maintenance therapy of chronic infections. Despite the recent breakthrough with CFTR modulators for cystic fibrosis, the development of novel mucolytic, anti-inflammatory, and anti-infective therapies is likely to remain important, especially for patients with more advanced stages of lung disease. As the median age of patients with cystic fibrosis increases, with a rapid increase in the population of adults living with the disease, complications of cystic fibrosis are becoming increasingly common. Steps need to be taken to ensure that enough highly qualified professionals are present in cystic fibrosis centres to meet the needs of ageing patients, and new technologies need to be adopted to support communication between patients and health-care providers. In considering the future of cystic fibrosis care, the Commission focused on five key areas, which are discussed in this report: the changing epidemiology of cystic fibrosis (section 1); future challenges of clinical care and its delivery (section 2); the building of cystic fibrosis care globally (section 3); novel therapeutics (section 4); and patient engagement (section 5). In panel 1, we summarise key messages of the Commission. The challenges faced by all stakeholders in building and developing cystic fibrosis care globally are substantial, but many opportunities exist for improved care and health outcomes for patients in countries with established cystic fibrosis care programmes, and in LMICs where integrated multidisciplinary care is not available and resources are lacking at present. A concerted effort is needed to ensure that all patients with cystic fibrosis have access to high-quality health care in the future.

2.
Pediatr Pulmonol ; 54(11): 1801-1810, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31402602

RESUMO

INTRODUCTION: Amikacin is commonly used in patients with pediatric cystic fibrosis (CF) for the treatment of pulmonary exacerbations. Amikacin efficacy is related to maximum plasma concentration/minimum inhibitory concentration (Cmax/MIC) ratio >8. Pharmacokinetic data in patients with pediatric CF are scarce. The aim of this study was to develop a population pharmacokinetic (PopPK) model describing amikacin disposition in patients with pediatric CF. METHODS: CF patients under 18 years of age with pulmonary exacerbation who received amikacin were enrolled. Patients received different amikacin regimens (30 mg-1 kg-1 day-1 every 8, 12, or 24 hours) depending on the patient's status and hospital protocols. Amikacin serum levels were obtained for therapeutic drug monitoring. PopPK model was developed using MONOLIX Suite-2018R1 (Lixoft). RESULTS: A total of 39 patients (114 amikacin concentrations) were included in this study. Population estimates for the elimination rate constant (k) and the volume of distribution (V) were 0.541 hours-1 and 0.451 L/kg, respectively. Between-subject and between-occasion variability were 53% and 16.5% for k and 31% and 22% for V, respectively. Bodyweight was a significant covariate associated with V. Based on simulations, almost 70% of the patients receiving 30 mg-1 kg-1 day-1 every 24 hours would achieve a Cmax/MIC ratio >8 which is an appropriate therapeutic goal while no patient in the other two groups (Q8 and Q12) would achieve that objective. CONCLUSIONS: The regimen of 30 mg-1 kg-1 day-1 every 24 hours more adequately fulfilled the therapeutic target for amikacin. Although all our patients had good clinical results and a good adverse-events profile, further studies are necessary to redefine the optimal treatment strategy.

3.
Arch. argent. pediatr ; 117(2): 115-120, abr. 2019. graf, tab
Artigo em Espanhol | LILACS-Express | ID: biblio-1001167

RESUMO

El anticuerpo monoclonal anti-IgE omalizumab está indicado para tratamiento del asma grave. Estudio longitudinal (pre-posintervención), observacional y analítico con el objetivo de evaluar la evolución clínica y funcional de pacientes con asma grave no controlada, 16 semanas antes y después del tratamiento. En los 17 casos, se controló el asma (p= 0,00001). Se redujeron en un 48,5 % las exacerbaciones (p= 0,009) y en un 100 % las crisis graves (p= 0,001). Dieciséis pacientes (el 94 %) tuvieron exacerbaciones pretratamiento y 10 (el 59 %), luego del omalizumab (p= 0,005). No hubo hospitalizaciones (p= 0,007). Se redujo en un 20 % la dosis de corticoides inhalados (0,002) y el número de casos con corticoides orales continuos (p= 0,01); disminuyó el uso de salbutamol (p= 0,001) y de corticoides orales (p= 0,003). No se modificó la función pulmonar. Dos casos presentaron reacciones adversas leves. El omalizumab permitió un adecuado control de la enfermedad en pacientes con asma grave.


Omalizumab, an anti-IgE monoclonal antibody, is indicated for the treatment of severe asthma. A longitudinal (pre-/post-intervention), observational, analytical study was conducted to assess the clinical and functional course of patients with uncontrolled severe asthma, 16 weeks before and after treatment. Asthma was controlled in 17 cases (p = 0.00001). Exacerbations were reduced by 48.5 % (p = 0.009) and severe crises, by 100 % (p = 0.001). Before omalizumab treatment, 16 patients (94 %) had exacerbations, whereas 10 (59 %) had them after treatment (p = 0.005). None of the patients was hospitalized (p = 0.007). The dose of inhaled corticosteroids was reduced by 20 % (0.002); the number of patients using continuous oral corticosteroids (p = 0.01), salbutamol (p = 0.001), and oral corticosteroids (p=0.003) also decreased. Pulmonary function was not affected. Two patients had mild adverse reactions. Omalizumab achieved an adequate asthma control in patients with severe asthma.

4.
Arch. argent. pediatr ; 117(2): 150-154, abr. 2019. ilus, tab
Artigo em Espanhol | LILACS-Express | ID: biblio-1001172

RESUMO

Elizabethkingia meningoseptica es un patógeno oportunista, con una elevada mortalidad y una incidencia en aumento en las terapias intensivas. Se presenta a una paciente de 4 años con antecedente de atresia de vías biliares y trasplante hepático a los 11 meses de vida, que se internó por infección respiratoria aguda baja con hipoxemia. Durante la internación, sufrió un empeoramiento clínico con requerimiento de asistencia respiratoria mecánica. Por fiebre e hipoxemia persistente, se realizó un minilavado broncoalveolar, con cultivo positivo para Elizabethkingia meningoseptica. Recibió vancomicina, trimetoprima/sulfametoxazol y ciprofloxacina durante 14 días, con buena respuesta. Una tomografía de tórax evidenció la presencia de hipoperfusión en mosaico, imágenes quísticas y bronquiectasias bilaterales. Durante los siguientes 2 años, presentó una buena evolución clínica, con escasas intercurrencias respiratorias, síntomas intercrisis aislados y buena tolerancia al ejercicio. En las imágenes de control, se evidenció la resolución de la mayoría de las lesiones iniciales a los 20 meses de su seguimiento.


Elizabethkingia meningoseptica is an opportunistic pathogen with a high mortality and an increasing incidence in the intensive care units. We present a 4-year-old patient with a history of atresia of the biliary tract and a liver transplant at 11 months of age, who was admitted for acute respiratory infection with hypoxemia. During the hospitalization, she required mechanical ventilation. Due to persistent fever and hypoxemia, mini bronchoalveolar lavage was performed with a positive culture for Elizabethkingia meningoseptica. She received vancomycin, trimethoprim/sulfamethoxazole and ciprofloxacin for 14 days with a good response. A chest tomography showed the presence of mosaic hypoperfusion, cystic images, and bilateral bronchiectasis. During the following 2 years, she presented good clinical progress, with scarce respiratory infections, isolated symptoms and good tolerance to exercise. The resolution of the majority of the initial lesions was evidenced at 20 months of follow-up.

5.
J Asthma ; : 1-10, 2019 Mar 27.
Artigo em Inglês | MEDLINE | ID: mdl-30915868

RESUMO

Objective: This study aimed to estimate asthma control at specialist treatment centers in four Latin American countries and assess factors influencing poor asthma control. Methods: Patients aged ≥12 years with an asthma diagnosis and asthma medication prescription, followed at outpatient specialist centers in Argentina, Chile, Colombia, and Mexico, were included. The study received all applicable ethical approvals. The Asthma Control Test (ACT) was used to classify patients as having controlled (ACT 20-25) or uncontrolled (ACT ≤19) asthma. Frequency and statistical tests were used to assess the association between hospital admissions/exacerbations/emergency department (ED) visits and uncontrolled asthma; multivariate logistic regression was used to assess the association of uncontrolled asthma with clinical/demographic variables. Results: A total of 594 patients were included. Overall controlled-asthma prevalence was 43.4% (95% confidence interval [CI]: 39.0, 47.4). Patients with uncontrolled asthma were more likely to be women (adjusted odds ratio [aOR]: 1.85; p = 0.003), non-white (aOR: 2.14; p < 0.001), obese (aOR: 1.71; p = 0.036), to have a low monthly family income (aOR: 1.75; p = 0.004), to have severe asthma (aOR:1.59; p = 0.26), and, compared with patients with controlled asthma, to have a higher likelihood of asthma exacerbations (34.5% vs. 15.9%; p < 0.001), hospital admissions (6.9% vs. 3.1%; p = 0.042), and ED visits (34.5% vs. 15.9%; p < 0.001) due to asthma. Conclusions: Even in specialist ambulatory services, fewer than half of patients were classified as having controlled asthma. The proportion of uncontrolled patients varied according to clinical and demographic variables.

6.
Arch Argent Pediatr ; 117(2): e115-e120, 2019 04 01.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-30869489

RESUMO

Omalizumab, an anti-IgE monoclonal antibody, is indicated for the treatment of severe asthma. A longitudinal (pre-/post-intervention), observational, analytical study was conducted to assess the clinical and functional course of patients with uncontrolled severe asthma, 16 weeks before and after treatment. Asthma was controlled in 17 cases (p = 0.00001). Exacerbations were reduced by 48.5 % (p = 0.009) and severe crises, by 100 % (p = 0.001). Before omalizumab treatment, 16 patients (94 %) had exacerbations, whereas 10 (59 %) had them after treatment (p = 0.005). None of the patients was hospitalized (p = 0.007). The dose of inhaled corticosteroids was reduced by 20 % (0.002); the number of patients using continuous oral corticosteroids (p = 0.01), salbutamol (p = 0.001), and oral corticosteroids (p=0.003) also decreased. Pulmonary function was not affected. Two patients had mild adverse reactions. Omalizumab achieved an adequate asthma control in patients with severe asthma.

7.
Arch Argent Pediatr ; 117(2): e150-e154, 2019 04 01.
Artigo em Espanhol | MEDLINE | ID: mdl-30869495

RESUMO

Elizabethkingia meningoseptica is an opportunistic pathogen with a high mortality and an increasing incidence in the intensive care units. We present a 4-year-old patient with a history of atresia of the biliary tract and a liver transplant at 11 months of age, who was admitted for acute respiratory infection with hypoxemia. During the hospitalization, she required mechanical ventilation. Due to persistent fever and hypoxemia, mini bronchoalveolar lavage was performed with a positive culture for Elizabethkingia meningoseptica. She received vancomycin, trimethoprim/sulfamethoxazole and ciprofloxacin for 14 days with a good response. A chest tomography showed the presence of mosaic hypoperfusion, cystic images, and bilateral bronchiectasis. During the following 2 years, she presented good clinical progress, with scarce respiratory infections, isolated symptoms and good tolerance to exercise. The resolution of the majority of the initial lesions was evidenced at 20 months of follow-up.

8.
Pediatr Transplant ; : e13311, 2018 Oct 25.
Artigo em Inglês | MEDLINE | ID: mdl-30362299

RESUMO

Cancer is a major adverse outcome of solid organ transplantation, and risks are especially high for malignancies caused by viral infections. HHV-8 is the etiologic agent of Kaposi´s sarcoma (KS). We report a case of visceral KS occurring in a 15-year-old patient after lung transplantation. The evolution was dramatically fast and interestingly, KS lesions were diffusely observed, but not in the skin. The autopsy showed the presence of numerous tumoral lesions in many organs. Microscopically, they all had very similar features, regardless of the organ affected. KS presented without cutaneous involvement. The girl was not tested for HHV-8 prior to transplantation as it was not part of our protocol. The donor was negative. The aim of the report is to alert other teams, especially those working in pediatrics, about this rare but potential complication in the setting of solid organ transplantation.

9.
Arch. argent. pediatr ; 116(2): 105-111, abr. 2018. ilus, tab
Artigo em Espanhol | LILACS-Express | ID: biblio-887455

RESUMO

Introducción. El asma grave representa 5-7% del total de asmáticos. La OMS propuso un protocolo de seguimiento para categorizarlos como asma grave resistente al tratamiento (AGRT) o asma grave de difícil control (AGDC). Objetivo. Analizar las características clínicas, funcionales y terapéuticas de pacientes con AGRT o AGDC. Métodos. Estudio transversal, observacional y analítico para evaluar el diagnóstico, grado de control (clínico y funcional), comorbilidades, adherencia al tratamiento, técnica inhalatoria y factores ambientales en pacientes con asma grave. Resultados. Se incluyeron 69 pacientes: AGRT (n= 33) y AGDC (n= 36). El 100% del grupo con AGRT fue hospitalizado previamente por asma vs. 87,8% del grupo con AGDC (p= 0,03). El 63% del grupo AGRT requirió cuidados intensivos (UCI)), 82%, asistencia ventilatoria y uno fue traqueostomizado. En el AGDC, 54% requirió internación en la UCI , y 33%, asistencia ventilatoria (p= 0,03). La espirometría basal fue normal en el AGDC; se observó incapacidad ventilatoria obstructiva leve en el AGRT (p= 0,009). En el AGDC, hubo menor cumplimiento del tratamiento (p= 0,01). Se requirieron dosis mayores de corticoides inhalados en AGRT (p= 0,0001). Omalizumab fue indicado en AGRT (p= 0,0001). A los 6 meses de seguimiento, más del 75% de los niños en ambos grupos presentó asma controlada. Conclusiones. Se observó significativa falta de adherencia al tratamiento en el grupo AGDC. Se redujeron las dosis de tratamiento en este grupo. Se logró controlar la enfermedad en un alto porcentaje de niños con AGRT y AGDC.


Introduction. Severe asthma accounts for 5-7% of all asthma cases. The World Health Organization proposed a follow-up protocol to classify cases into severe, treatment-resistant asthma (STRA) or severe, difficult-to-control asthma (SDCA). Objective. To analyze the clinical, functional, and therapeutic characteristics of patients with STRA or SDCA. Methods. Cross-sectional, observational, and analytical study to assess the diagnosis, the extent of control (clinical and functional), comorbidities, treatment adherence, inhalation technique, and environmental factors in patients with severe asthma. Results. A total of 69 patients were included: STRA (n= 33) and SDCA (n= 36). In the group with STRA, 100% of patients had been previously hospitalized due to asthma compared to 87.8% in the group with SDCA (p= 0.03). In the group with STRA, 63% required admission to the intensive care unit (ICU); 82%, ventilatory support; and 1 patient, tracheostomy. In the group with SDCA, 54% required admission to the ICU; and 33%, ventilatory support (p= 0.03). The baseline spirometry was normal in the SDCA group; a mild obstructive ventilatory defect was observed in the STRA group (p= 0.009). In the SDCA group, treatment adherence was lower (p= 0.01). Higher inhaled corticosteroid doses were required in the STRA group (p= 0.0001). Omalizumab was indicated in the case of STRA (p= 0.0001). After 6 months of follow-up, more than 75% of children in both groups achieved asthma control. Conclusions. A significant lack of treatment adherence was observed in the SDCA group. In this group, treatment doses were reduced. Asthma was controlled in a high percentage of children with STRA and SDCA.

10.
Arch Argent Pediatr ; 116(2): 105-111, 2018 Apr 01.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-29557596

RESUMO

INTRODUCTION: Severe asthma accounts for 5-7% of all asthma cases. The World Health Organization proposed a follow-up protocol to classify cases into severe, treatment-resistant asthma (STRA) or severe, difficult-to-control asthma (SDCA). OBJECTIVE: To analyze the clinical, functional, and therapeutic characteristics of patients with STRA or SDCA. METHODS: Cross-sectional, observational, and analytical study to assess the diagnosis, the extent of control (clinical and functional), comorbidities, treatment adherence, inhalation technique, and environmental factors in patients with severe asthma. RESULTS: A total of 69 patients were included: STRA (n= 33) and SDCA (n= 36). In the group with STRA, 100% of patients had been previously hospitalized due to asthma compared to 87.8% in the group with SDCA (p= 0.03). In the group with STRA, 63% required admission to the intensive care unit (ICU); 82%, ventilatory support; and 1 patient, tracheostomy. In the group with SDCA, 54% required admission to the ICU; and 33%, ventilatory support (p= 0.03).The baseline spirometry was normal in the SDCA group; a mild obstructive ventilatory defect was observed in the STRA group (p= 0.009).In the SDCA group, treatment adherence was lower (p= 0.01). Higher inhaled corticosteroid doses were required in the STRA group (p= 0.0001). Omalizumab was indicated in the case of STRA (p= 0.0001). After 6 months of follow-up, more than 75% of children in both groups achieved asthma control. CONCLUSIONS: A significant lack of treatment adherence was observed in the SDCA group. In this group, treatment doses were reduced. Asthma was controlled in a high percentage of children with STRA and SDCA.

11.
Buenos Aires; Médica Panamericana; 2018. 180 p. ilus.
Monografia em Espanhol | LILACS | ID: biblio-882735

RESUMO

La patología respiratoria presenta un gran desafío para las instituciones de salud, por su frecuencia, su complejidad diagnóstica y terapéutica y la carga que representa en costos económicos y vitales. Estos conceptos se extienden a todos los grupos etarios y sus características han ido cambiando a lo largo del tiempo ante los avances producidos en las inmunizaciones, los métodos diagnósticos y los tratamientos. Aún así, las infecciones respiratorias bajas son todavía la causa más frecuente de consulta, internación, morbilidad crónica, discapacidad y mortalidad en pediatría. Este nuevo volumen aborda esta temática especial y entre sus características se destacan: El estudio de las patologías más frecuentes en los diferentes ámbitos de atención pero en particular en el primer nivel, con una exposición centrada en los aspectos que facilitan el diagnóstico rápido y el tratamiento adecuado, con el menor uso de recursos y con pautas que fijan la derivación oportuna hacia el especialista o hacia una institución de mayor complejidad. El desarrollo de importantes temas, como la patología obstructiva de la vía aérea superior, su estudio diagnóstico y sus formas recurrentes; bronquiolitis; las intercurrencias respiratorias en pacientes con condiciones clínicas especiales; y la supuración pleuropulmonar. La inclusión, en todos los capítulos, de casos clínicos con su evolución y desenlace, textos destacados con los principales conceptos y puntos claves para recordar. Una obra sólida y práctica, que transmite la experiencia de los profesionales de una institución del prestigio internacional del Hospital dePediatría Prof. Dr. Juan P. Garrahan, dedicada a todos los pediatras, dondequiera que trabajen al servicio de la salud de los niños.


Assuntos
Humanos , Recém-Nascido , Lactente , Pré-Escolar , Criança , Adolescente , Obstrução das Vias Respiratórias , Argentina , Asma , Bronquiolite , Hemoptise , Laringe/anormalidades , Doenças Neuromusculares , Oxigenoterapia , Derrame Pleural , Doença Pulmonar Obstrutiva Crônica , Testes de Função Respiratória , Sons Respiratórios , Infecções Respiratórias , Traqueostomia
12.
Respirology ; 22(1): 187-191, 2017 01.
Artigo em Inglês | MEDLINE | ID: mdl-27511212

RESUMO

BACKGROUND AND OBJECTIVE: Pneumonectomy (PNE) is a procedure infrequently performed in children. A high morbidity/mortality rate associated with PNE has been described. Few series have been published in the last 15 years. Risk factors associated with morbidity/mortality after PNE were evaluated. Indications, course, survival and complications of PNE in children were also analized. METHODS: In a case series of 51 children who underwent PNE, death within 30 days of surgery, pneumonia, empyema, sepsis, adult respiratory distress syndrome, bronchopleural fistula, bleeding, pneumothorax and post-PNE syndrome were considered major morbidities. Scoliosis, wound infection and atelectasis were considered minor morbidities. RESULTS: Median age at PNE was 7.4 years; 45% were males. Indications of pneumonectomy were postinfectious bronchiectasis (61%), tumours (17%), pulmonary malformations (17%), aspiration syndrome (14%), cystic fibrosis (6%), immunodeficiency (4%) and trauma (2%). Mortality rate was 4% at 1 month. Major and minor morbidities were present in 23% and 27% of patients, respectively. Risk factors for development of morbidities after PNE were age ≤ 3 years (OR: 16.7; 95% CI: 2.4-117) and the need for mechanical ventilation for at least 4 days (OR: 8; 95% CI: 1.5-43.6). CONCLUSION: Children are at high risk of death, major and minor morbidities following PNE. Caution is recommended for this group of patients.


Assuntos
Pneumonectomia , Pneumonia , Pneumotórax , Complicações Pós-Operatórias , Respiração Artificial , Sepse , Argentina/epidemiologia , Criança , Feminino , Humanos , Masculino , Mortalidade , Avaliação de Processos e Resultados (Cuidados de Saúde) , Pneumonectomia/efeitos adversos , Pneumonectomia/métodos , Pneumonectomia/mortalidade , Pneumonia/diagnóstico , Pneumonia/epidemiologia , Pneumonia/etiologia , Pneumotórax/diagnóstico , Pneumotórax/epidemiologia , Pneumotórax/etiologia , Complicações Pós-Operatórias/classificação , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Respiração Artificial/efeitos adversos , Respiração Artificial/estatística & dados numéricos , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Sepse/diagnóstico , Sepse/epidemiologia , Sepse/etiologia , Resultado do Tratamento
13.
J Cyst Fibros ; 15(6): 791-793, 2016 11.
Artigo em Inglês | MEDLINE | ID: mdl-27262748

RESUMO

The burden of cystic fibrosis (CF) in Latin America is being increasingly recognized and is significant compared with other regions of the world. In this short communication, we assess the current situation in some Latin American countries and make suggestions for possible directions for future focus. We discuss the work that remains in deciphering how the various genetic, environmental and medical factors interact and influence outcomes in different ethnic groups. We also consider the need for consistency in both research and access to services across Latin America, including CF registries, neonatal screening programs, access to specialized CF healthcare practitioners, transition to adult clinics and treatment regimens. Progress in these areas is likely to build on the advances to date, and improve the lives of patients in Latin America who are affected by this debilitating and life-limiting disorder.


Assuntos
Fibrose Cística , Assistência à Saúde/estatística & dados numéricos , Triagem Neonatal/organização & administração , Efeitos Psicossociais da Doença , Fibrose Cística/diagnóstico , Fibrose Cística/epidemiologia , Fibrose Cística/genética , Fibrose Cística/terapia , Humanos , Recém-Nascido , América Latina/epidemiologia , Melhoria de Qualidade
14.
Arch. argent. pediatr ; 114(3): e138-e141, jun. 2016. ilus
Artigo em Espanhol | LILACS-Express | ID: biblio-838212

RESUMO

La proteinosis alveolar es una enfermedad pulmonar crónica poco frecuente, especialmente en pediatría, caracterizada por la acumulación anormal de lipoproteínas y derivados del surfactante en el espacio intraalveolar, que genera una grave reducción del intercambio gaseoso. La forma de presentación idiopática constituye más del 90% de los casos relacionados con un fenómeno de autoinmunidad, con producción de anticuerpos dirigidos contra el receptor del factor estimulante de colonias de granulocitos y macrófagos. Se presenta un caso clínico de una niña de 4 años de edad tratada por neumonía atípica con evolución desfavorable por hipoxemia persistente. El diagnóstico se obtuvo a través del estudio anatomopatológico de la biopsia pulmonar por toracotomía. Se llevaron a cabo 17 lavados broncopulmonares mediante endoscopía respiratoria y la paciente evidenció franca mejoría clínica.


Alveolar proteinosis is a rare chronic lung disease, especially in children, characterized by abnormal accumulation of lipoproteins and derived surfactant in the intra-alveolar space that generates a severe reduction of gas exchange. Idiopathic presentation form constitutes over 90% of cases, a phenomenon associated with production of autoimmune antibodies directed at the receptor for granulocyte-macrophage colony-stimulating factor. A case of a girl of 5 years of age treated because of atypical pneumonia with unfavorable evolution due to persistent hypoxemia is presented. The diagnosis is obtained through pathologic examination of lung biopsy by thoracotomy, as treatment is carried out by 17bronchopulmonary bronchoscopy lavages and the patient evidences marked clinical improvement.

15.
Arch. argent. pediatr ; 114(3): e175-e178, jun. 2016. ilus
Artigo em Espanhol | LILACS-Express | ID: biblio-838221

RESUMO

En pediatría, la enfermedad vascular pulmonar es multifactorial y heterogénea. Si bien comparte algunas características con la hipertensión pulmonar en los adultos, hay diferencias en las comorbilidades y condiciones asociadas, la coexistencia de enfermedades genéticas o del desarrollo. Las enfermedades pulmonares intersticiales pueden ser causantes de esta entidad. Una de ellas es la displasia alvéolo-capilar con mal alineamiento de las venas pulmonares, una patología infrecuente pero con 100% de mortalidad, caracterizada por la falla en la formación del tejido pulmonar que da por resultado final la alteración en la difusión de gases. Se describe un caso clínico de una paciente de 5 meses de edad estudiada a partir de sospechar una cardiopatía congénita con hipoxemia persistente, a cuyo diagnóstico se llega por la biopsia pulmonar.


Pulmonary vascular disease in children is multifactorial and heterogeneous. While it shares some features with pulmonary hypertension in adults, there are differences in the associated comorbidities and conditions, the coexistence of genetic or developmental diseases. Interstitial lung diseases may be responsible for this entity. One is alveolar capillary dysplasia with misalignment of pulmonary veins, a rare pathology but with a mortality rate of 100%, characterized by a failure in the formation of lung tissue that eventually results in impaired gas diffusion. We present a 5-month-old patient studied due to suspected congenital heart disease with persistent hypoxemia; diagnosis was made through lung biopsy.

16.
Arch Argent Pediatr ; 114(3): e175-8, 2016 Jun 01.
Artigo em Espanhol | MEDLINE | ID: mdl-27164352

RESUMO

Pulmonary vascular disease in children is multifactorial and heterogeneous. While it shares some features with pulmonary hypertension in adults, there are differences in the associated comorbidities and conditions, the coexistence of genetic or developmental diseases. Interstitial lung diseases may be responsible for this entity. One is alveolar capillary dysplasia with misalignment of pulmonary veins, a rare pathology but with a mortality rate of 100%, characterized by a failure in the formation of lung tissue that eventually results in impaired gas diffusion. We present a 5-month-old patient studied due to suspected congenital heart disease with persistent hypoxemia; diagnosis was made through lung biopsy.


Assuntos
Hipertensão Pulmonar/etiologia , Doenças Pulmonares Intersticiais/complicações , Feminino , Humanos , Lactente
17.
Respirology ; 20(6): 982-6, 2015 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-25939617

RESUMO

BACKGROUND AND OBJECTIVE: Post-infectious bronchiolitis obliterans (PIBO) is a severe disorder following acute lower pulmonary infection in young children, especially caused by adenovirus. Mannose-binding lectin (MBL) deficiency arising from polymorphisms in the coding and non-coding region on the MBL2 gene has been associated with more frequent and severe respiratory infections. Our aim was to evaluate the influence of MBL variants in the susceptibility and evolution of children with PIBO. METHODS: One hundred eleven children with PIBO diagnosis were studied. The coding A, B, D and X promoter variants of MBL2 gene were assessed by PCR-RFLP. B and D alleles were pooled as O. The combined genotypes A/A and YA/O were grouped as sufficient MBL (sMBL), and O/O and XA/O as insufficient MBL (iMBL) groups. To evaluate the frequency of MBL2 polymorphisms in the general population, we studied DNA samples from 127 healthy donors from the blood bank of the hospital (control group). RESULTS: iMBL variants were significantly more frequent in PIBO children compared with controls (21.6% vs 10.2%, P = 0.01). PIBO patients with iMBL required intensive care unit (P = 0.001) and mechanical assistance at the moment of viral injury (P = 0.001) more frequently than those with sMBL. CONCLUSIONS: Insufficiency of MBL was more common in PIBO children than in healthy controls. This genetic condition was significantly associated with more severe initial disease, illustrating the relevance of innate immune defence factors prior to the maturation of the adaptative immune system.


Assuntos
Bronquiolite Obliterante/epidemiologia , Lectina de Ligação a Manose/deficiência , Erros Inatos do Metabolismo/epidemiologia , Adolescente , Argentina/epidemiologia , Bronquiolite Obliterante/etiologia , Criança , Pré-Escolar , Suscetibilidade a Doenças , Feminino , Frequência do Gene , Variação Genética , Genótipo , Humanos , Lactente , Masculino , Lectina de Ligação a Manose/genética , Erros Inatos do Metabolismo/genética , Polimorfismo Genético , Infecções Respiratórias/complicações
18.
J Cyst Fibros ; 14(1): 78-83, 2015 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-25178872

RESUMO

BACKGROUND: There is a considerable variation in the phenotype and course of the disease in cystic fibrosis (CF) even in patients with the same CFTR genotype, suggesting that other factors are important for prognosis. Mannose-binding lectin (MBL) has been proposed as one of these factors. We therefore investigated the influence of MBL2 gene variants on disease severity, age at acquisition of Pseudomonas aeruginosa, and survival in CF patients. METHODS: MBL2 variants were studied in 106 Argentinean pediatric CF patients carrying two severe CFTR mutations. Clinical phenotype was defined according to the Shwachman score and lung function tests. Age at infection with P. aeruginosa and age at death were also recorded. RESULTS: MBL insufficiency was associated with a 3.5-fold risk of having a severe phenotype (CI 95%: 1.2-10.3, p=0.03). It was also associated with an earlier onset of infection with P. aeruginosa (p=0.035). No statistically significant differences were found in FEV1 and survival. CONCLUSIONS: MBL insufficiency was associated with detrimental progression of the disease. These results together with previous findings suggest that the effect of MBL2 expression may be a major determinant of the severity of the clinical phenotype in patients with CF.


Assuntos
Fibrose Cística/genética , Predisposição Genética para Doença/epidemiologia , Lectina de Ligação a Manose/genética , Polimorfismo Genético , Argentina/epidemiologia , Criança , Pré-Escolar , Estudos de Coortes , Fibrose Cística/mortalidade , Fibrose Cística/fisiopatologia , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Progressão da Doença , Feminino , Genótipo , Humanos , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Masculino , Fenótipo , Testes de Função Respiratória , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Taxa de Sobrevida
19.
Acta Paediatr ; 103(9): 913-21, 2014 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-24832610

RESUMO

UNLABELLED: Postinfectious bronchiolitis obliterans (PIBO) is an infrequent chronic lung that causes irreversible obstruction and, or, obliteration of the smaller airways. This review particularly focuses on more than 30 studies from South America. CONCLUSION: The initial PIBO event occurs in the early years of life and is strongly associated with adenovirus infection and the need for mechanical ventilator support. Treatment requires a multidisciplinary strategy. Multicentre studies are needed to determine progression, optimal management and long-term follow-up.


Assuntos
Bronquiolite Obliterante/microbiologia , Bronquiolite Obliterante/diagnóstico , Bronquiolite Obliterante/epidemiologia , Bronquiolite Obliterante/terapia , Humanos , Testes de Função Respiratória , Fatores de Risco , América do Sul
20.
Arch Argent Pediatr ; 111(4): 315-21, 2013 Jul-Aug.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-23912289

RESUMO

INTRODUCTION: Pediatricians are in a strategic position to prevent smoking. OBJECTIVES: To estimate the prevalence of smoking among pediatric residents, analyze associated factors, describe preventive actions, and assess the differences observed over the past decade. METHODS: Cross-sectional study with a self-administered, anonymous survey conducted among pediatric residents from eight Argentine hospitals. Smoking habit, associated factors, and attitude towards patients' or their parents' smoking were evaluated; results were compared to those obtained in 2002. RESULTS: Out of 448 surveyed physicians, 20.1% smoked. There were no significant differences between smokers and non-smokers in terms of gender, having children, number of on-call shifts, and having a supervisor who smokes. Having a parent who smoked was a risk factor for tobacco use only among women (OR: 1.98; 95% CI: 1.09-3.61; p = 0.01). Surveyed residents living with a couple had a lower smoking rate (OR: 0.57; 95% CI: 0.34-0.96; p = 0.03). Only 18.1% referred having an active behavior towards smoking patients, no differences were observed between smokers and non-smokers. In addition, there were no differences in smoking prevalence from 2002, but there was a higher rate of residents who advised their patients (32.4% versus 26.1%; p < 0.01) and warned them against smoking (37.7% versus 18.6%; p < 0.01), and of those who received information on this topic during their training (63.6% versus 39.8%; p < 0.01). CONCLUSIONS: Among resident pediatricians, 20.1% were smokers, with a higher prevalence among women with a father or mother who is a smoker. The rate of active behavior regarding patients or parents who smoke was very low. In spite of official policies, tobacco use in this group has not changed over the past decade, but there was an increase in the rate of those who received information during their training and of those who advised their patients of smoking risks.


Assuntos
Internato e Residência , Pediatria , Fumar/epidemiologia , Adulto , Argentina , Estudos Transversais , Feminino , Humanos , Masculino , Pais , Prevalência , Fatores de Risco , Fatores de Tempo
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