Resultados del primer registro nacional de fibrosis pulmonar idiopática en Chile / Results of the first national register of idiopathic pulmonary fibrosis in Chile

INTRODUCCIÓN: La FPI es la neumonía intersticial idiopática más común, con cifras de incidencia y prevalencia que varían en el mundo por la poco uniforme manera de recolectar casos en los diferentes estudios. No hay cifras publicadas sobre la epidemiología de la FPI en Chile ni Latinoamérica. Se hace relevante conocerlas por la carga sanitaria que representan los pacientes con FPI y por la aprobación reciente de drogas antifibróticas de alto costo. El objetivo de este estudio fue realizar un registro de pacientes con FPI atendidos por neumólogos chilenos de diversos regiones del país, con los medios diagnósticos que habitualmente utilizan en la vida real. PACIENTES Y MÉTODOS: Se utilizó una encuesta electrónica en línea diseñada por el grupo de enfermedades pulmonares difusas del Instituto Nacional del Tórax para registro de pacientes con diagnóstico de FPI según criterios ATS/ERS/JRS/ALAT desde junio de 2015 a junio de 2017. RESULTADOS: 40 de los 200 neumólogos invitados enviaron casos de FPI de las 13 regiones del país, completando un total de 700 pacientes. 2/3 eran casos antiguos, un número similar de hombres y mujeres, 73% tienen patrón definitivo de UIP (Usual Interstitial Pneumonia) en tomografía axial computarizada, la mayoría eran pacientes sobre 60 años y en solo 16% se solicitó biopsia para diagnóstico. CONCLUSIONES: Un registro de 700 casos representa un número muy importante de pacientes con FPI en Chile que nos permite acercarnos a la caracterización de la cohorte y a fortalecer una red de especialistas dedicados al cuidado de estos pacientes y sus familias.

INTRODUCTION: Idiopathic pulmonary fibrosis is the most common idiopathic interstitial pneumonia, with incidence and prevalence figures varying worldwide because of the inconsistent way of collecting cases in different studies. There are no published figures on the epidemiology of IPF in Chile or Latin America. It is relevant to know them because of the health burden of patients with IPF and the recent approval for treatment purposes of high cost antifibrotic drugs. The objective of this study was to develop a clinical registry of patients with IPF treated by Chilean pulmonologists from different regions of the country, using the diagnostic means they usually use in real life. PATIENTS AND METHODS: An online electronic survey was designed by the group of diffuse pulmonary diseases of the "Instituto Nacional del Tórax" to register patients with diagnosis of IPF from June 2015 to June 2017 according to ATS/ERS/JRS/ALAT criteria. RESULTS: 40 of the 200 invited pulmonologists sent IPF cases from the country's 13 regions, completing a total of 700 patients. 2/3 were old cases, a similar number of men and women, 73% had definitive UIP pattern in CT, the majority were patients over 60 years old and in only 16% biopsy was requested for diagnosis. CONCLUSIONS: A register of 700 cases represents a very important number of patients with IPF in Chile that allows us to approach the characterization of the cohort and to strengthen a network of specialists dedicated to the care of these patients and their families.

Hospitalización diurna como modelo de atención de salud en pacientes adultos inmunocompetentes con neumonía adquirida en la comunidad / Respiratory day hospital care for immunocompetent adult patients with community-acquired pneumonia

Background: Day hospitals can reduce health care costs without increasing the risks of patients with lower respiratory tract infection. Aim: To report the experience of a respiratory day hospital care delivered to adult patients with community-acquired pneumonia (CAP) in a public hospital. Material and Methods: During the fall and winter of 2011 and 2012, adult patients with CAP of intermediate risk categories were assessed in the emergency room, their severity was stratified according to confusion, respiratory rate, blood pressure, 65 years of age or older (CRB-65) score and the Chilean CAP Clinical Guidelines, and were admitted to the respiratory day hospital. Results: One hundred seventeen patients aged 67 ± 16 years, (62% females) with CAP were attended in the respiratory day hospital. Ninety percent had comorbidities, especially chronic obstructive pulmonary disease in 58%, heart disease in 32%, diabetes in 16% and asthma in 13%. Their most important risk factors were age over 65 years in 60%, comorbidities in 88%, failure of antibiotic treatment in 17%, loss of autonomy in 21%, vital sign abnormalities in 60%, mental confusion in 5%, multilobar CAP in 23%, pleural effusion in 15%, hypoxemia in 41% and a serum urea nitrogen over 30 mg/dL in 16%. Patients stayed an average of seven days in the day hospital with oxygen, hydration, chest physiotherapy and third-generation cephalosporins (89%) associated with quinolones (52%) or macrolides (4%). Thirteen patients required noninvasive ventilation, eight patients were hospitalized because of clinical deterioration and three died in hospital. Conclusions: Day hospital care reduced hospital admission rates of patients with lower respiratory tract infections.

Safety and tolerability of targeted therapies for pulmonary hypertension in children.

The objective of this study is to evaluate the safety and tolerability of the pharmacological treatment of pulmonary hypertension in pediatric patients. It is a retrospective, longitudinal, observational study on pediatric patients undergoing treatment with pulmonary targeted therapies. 63 patients were included (51% male), with a median age of 3.4 years (IQR, 3.6 months-10 years) and a median weight 13 kg (IQR, 6-30 kg). Congenital heart disease was the etiology of pulmonary hypertension in the majority of cases (n = 33) and 28 patients were in NYHA functional class III-IV. The most commonly used drug was sildenafil (n = 79, 56%), followed by bosentan (n = 27, 23%), and a combination of both (n = 14, 41%). 34 patients had adverse reactions (54%) with an incidence rate of 1.02 per patient per year. The most commonly reported reactions were gastrointestinal symptoms (22%) and spontaneous erections (22%) in males. Nine severe adverse reactions (10%) occurred, requiring eight treatment withdrawal and one hospital admission. Treatment with targeted therapies for pulmonary hypertension is safe in the pediatric population. Severe ADRs were uncommon both in monotherapy and in combination therapy. Combination therapy was associated with a higher rate of ADRs. We observed similar survival rates in children receiving sildenafil doses according to the European Medicines Agency (EMA) recommendations or higher.

Manejo de la estenosis traqueal / Management of tracheal stenosis

Objetivo del estudio: Proponer una nueva clasificación para la estenosis traqueal para estandarizar el manejo. Diseño: Estudio prospectivo realizado entre Octubre del 2002 y septiembre del 2005 en el Hospital San José. Pacientes: Se incluyeron 32 pacientes, 9 mujeres y 23 varones, con estenosis traqueal de etiología benigna (20 casos) y maligna (12 casos), de los cuales 4 tenían fístula traqueoesofágica. Método: Los pacientes fueron divididos en tres grupos de acuerdo a la morfología, longitud, número y localización de la o las lesiones que causaban estenosis. Según lo anterior Tipo I (7 casos; 22 por ciento), Tipo II (5 casos; 16 por ciento). Tipo III (20 casos; 62 por ciento). Intervenciones: Las estenosis Tipo I fueron tratadas mediante electrocoagulación. Las Tipo II con cirugía (resección y anastomosis traqueal termino-terminal primaria). Las Tipo III utilizando un stent dinámico. Resultados: La evolución posterior a la realización del procedimiento correspondiente a cada grupo, fue favorable, mejorando la capacidad funcional, demostrado por la realización de curva flujo-volumen y la desaparición de manifestaciones clínicas (según escala de Borg modificada) en el 69 por ciento de los pacientes, con una calidad de vida que mejora en el 100 por ciento de los casos, objetivado mediante el cuestionario de Saint-George. La mortalidad global es de 25 por ciento (8/32), no atribuible al tratamiento otorgado. Conclusiones: Consideramos que la clasificación propuesta es un método operacional, de fácil utilización, que permite planificar un tratamiento independientemente de la etiología de la estenosis traqueal, permitiendo asignar un pronóstico y mejorar calidad de vida.

Background: Tracheal stenosis is a common complication of intubation or tracheostomy. Aim: To report the experience with tracheal stenosis and propose a new classification to standardize its management. Material and Methods: Prospective study of 32 patients aged 17 to 79 years, 23 males. Twenty had a benign and 12 a malignant stenosis, four of which had tracheoesophageal fistula. Seven patients had a Type I stenosis, defined as a lesion of less than 1 cm in length, five had a type II lesion, defined as a diffuse lesion of less than 3 cm in length and 20 cases had a type III lesion defined as a stenosis of more than 3 cm, in length or multiple lesions or tracheomalacia. Patients with type I stenosis were treated with electro coagulation, those with type II lesions were subjected to a primary resection and tracheal end-to-end anastomosis and type III patients were treated using a dynamic stent. Results: Evolution after treatment was favorable with disappearance of clinical manifestations, improvement in functional capacity and modified Borg Scale score in 69 percent of the patients. Life quality and flow-volume curves improved in 100 percent of patients. Seven patients died due to progression of cancer and one committed suicide. Conclusions: The classification of tracheal stenosis in three types, depending on the morphology and extent of the lesion allows a better planning of treatment.

Neumonía por Pneumocistis carinii en pacientes con SIDA en El Hospital Santo Tomas / Pneumocistis carinii pneumonia in patients with AIDS at the Saint Thomas Hospital

The authors examined material from the respiratory tract obtained from 55 patients, with silver Methenamine and Giemsa stains to detect Pneumocystis carinii. Twenty five patients were positive. All had fever and fatigue, 80% had dyspnea, 72% had productive cough, and significant weight loss occurred in 48%. None of the patients had signs of pulmonary consolidation. Chest X-Ray showed diffuse infiltrates in 55.2%. Nodular lesions in both lung fields were present in 6.9%. A local infiltrate was seen in 13.6% and no infiltrates were seen in 10.3%. LDH was elevated in all (ranging from 885 4500 UI), 84% of the patients had a PO2 of 70 mmHg or less. Most of the patients, 80%, responded to therapy with Trimethoprim-Sulfa

[Pneumocistis carinii pneumonia in patients with AIDS at the Saint Thomas Hospital]. / Neumonía por Pneumocistis carinii en pacientes con SIDA en El Hospital Santo Tomas.

The authors examined material from the respiratory tract obtained from 55 patients, with silver Methenamine and Giemsa stains to detect Pneumocystis carinii. Twenty five patients were positive. All had fever and fatigue, 80% had dyspnea, 72% had productive cough, and significant weight loss occurred in 48%. None of the patients had signs of pulmonary consolidation. Chest X-Ray showed diffuse infiltrates in 55.2%. Nodular lesions in both lung fields were present in 6.9%. A local infiltrate was seen in 13.6% and no infiltrates were seen in 10.3%. LDH was elevated in all (ranging from 885 4500 UI), 84% of the patients had a PO2 of 70 mmHg or less. Most of the patients, 80%, responded to therapy with Trimethoprim-Sulfa.