[Guidelines for the follow up of patients with bronchopulmonary dysplasia]. / Protocolo de seguimiento de los pacientes con displasia broncopulmonar.

Bronchopulmonary dysplasia (BPD) is the most common complication of preterm birth, and remains a major problem in pediatric pulmonology units. The decision of discharging from the Neonatal Unit should be based on a thorough assessment of the condition of the patient and compliance with certain requirements, including respiratory and nutritional stability, and caregiver education on disease management. For proper control of the disease, a schedule of visits and complementary tests should be established prior to discharge, and guidelines for prevention of exacerbations and appropriate treatment should be applied. In this paper, the Working Group in Perinatal Respiratory Diseases of the Spanish Society of Pediatric Pulmonology proposes a protocol to serve as a reference for the follow up of patients with BPD among different centers and health care settings. Key factors to consider when planning discharge from the Neonatal Unit and during follow up are reviewed. Recommendations on treatment and prevention of complications are then discussed. The final section of this guide aims to provide a specific schedule for follow-up and diagnostic interventions to be performed in patients with BPD.

Protocolo de diagnóstico y seguimiento de los pacientes con fibrosis quística / Protocol for the diagnosis and follow up of patients with cystic fibrosis

La fibrosis quística (FQ) es la enfermedad autosómica recesiva grave más frecuente de la raza caucásica. El aumento tan importante de la supervivencia de los pacientes con FQ en los últimos años se debe en gran parte a la disponibilidad de nuevos tratamientos de la enfermedad pulmonar, en especial de las infecciones respiratorias. Este protocolo revisa y actualiza los aspectos del diagnóstico y el manejo de la enfermedad respiratoria de la FQ. La prueba del sudor continúa siendo el estándar del diagnóstico de la FQ aunque, pese a realizarse de la forma adecuada, no siempre es concluyente. Se debe controlar a los pacientes con FQ en unidades especializadas por un equipo multidisciplinario y experto, mediante protocolos específicos de seguimiento clínico, estudios por imagen y pruebas funcionales respiratorias y microbiológicas que se actualizan en esta revisión. Se incluyen las recomendaciones sobre el tratamiento precoz y agresivo de la primoinfección y de la infección crónica por Pseudomonas aeruginosa, Staphylococcus aureus y otros microorganismos no habituales. Asimismo se describen los tratamientos de los diferentes aspectos de la enfermedad pulmonar y sus complicaciones y se revisan las indicaciones y el estado actual del trasplante pulmonar. Este documento de consenso ha sido elaborado por los miembros del Grupo de Trabajo de Fibrosis Quística de la Sociedad Española de Neumología Pediátrica con el objetivo de actualizar el previo, publicado en esta Revista en 1999 (AU)

Cystic fibrosis (CF) is the most common severe recessive genetic disease in Caucasians. During the last years, new therapies and aggressive management of the lung disease have contributed significantly to the increased life expectancy in CF patients. A review and update of CF diagnosis and management of lung disease are included. The sweat chloride test (SCT) remains the gold standard for CF diagnosis and should be performed properly. However, in a few patients SCT results may not be conclusive to clarify the CF diagnosis. Patients with CF should be followed up in specialist Units by an expert multidisciplinary expert applying standard clinical protocols and using lung function tests, and microbiological and imaging studies. An overview with the recommendations for treatment of early onset and chronic infections due to Pseudomonas aeruginosa, Staphylococcus aureus and other uncommon pathogens is included. Furthermore, the management of other aspects of CF lung disease and complications is provided, as well as the indications for lung transplantation. This document has been prepared by the members of the CF working group of the Spanish Paediatrics Pulmonary Society to provide an update to the earlier documents published in this Journal in 1999 (AU)

[Protocol for the diagnosis and follow up of patients with cystic fibrosis]. / Protocolo de diagnóstico y seguimiento de los pacientes con fibrosis quística.

Cystic fibrosis (CF) is the most common severe recessive genetic disease in Caucasians. During the last years, new therapies and aggressive management of the lung disease have contributed significantly to the increased life expectancy in CF patients. A review and update of CF diagnosis and management of lung disease are included. The sweat chloride test (SCT) remains the gold standard for CF diagnosis and should be performed properly. However, in a few patients SCT results may not be conclusive to clarify the CF diagnosis. Patients with CF should be followed up in specialist Units by an expert multidisciplinary expert applying standard clinical protocols and using lung function tests, and microbiological and imaging studies. An overview with the recommendations for treatment of early onset and chronic infections due to Pseudomonas aeruginosa, Staphylococcus aureus and other uncommon pathogens is included. Furthermore, the management of other aspects of CF lung disease and complications is provided, as well as the indications for lung transplantation. This document has been prepared by the members of the CF working group of the Spanish Paediatrics Pulmonary Society to provide an update to the earlier documents published in this Journal in 1999.

Fundamentos de la oxigenoterapia en situaciones agudas y crónicas: indicaciones, métodos, controles y seguimiento / Oxygen therapy in acute and chronic conditions: Indications, oxygen systems, assessement and follow-up

La oxigenoterapia es una herramienta fundamental para el tratamiento de la insuficiencia respiratoria, tanto aguda como crónica. Los objetivos principales que llevan a su empleo son tratar o prevenir la hipoxemia, tratar la hipertensión pulmonar y reducir el trabajo respiratorio y miocárdico. En situaciones agudas, su utilidad está ampliamente aceptada y en situaciones crónicas se ha extendido de forma importante. Sin embargo, sigue sin haber consenso en puntos fundamentales y son pocos los aspectos en los que la actuación entre los diferentes centros esté estandarizada. El Grupo de Trabajo de Técnicas de la Sociedad Española de Neumología Pediátrica acordó elaborar unas recomendaciones avaladas por esta Sociedad sobre el empleo de este tratamiento, e incorporó las diferentes formas de actuación recogidas en revisiones recientes para intentar establecer sus indicaciones, disminuir los efectos colaterales y procurar una correcta adecuación del gasto económico. Se han incluido aspectos generales del tratamiento con oxígeno, como los mecanismos fisiológicos, las indicaciones para su empleo, tanto en situaciones agudas como crónicas, y los medios disponibles para su correcta administración. Se aborda asimismo el tratamiento del paciente con oxigenoterapia en domicilio y las situaciones especiales que pueden producirse (AU)

Oxygen therapy has become a major tool for infants with acute and chronic respiratory failure. Appropriate goals when prescribing supplemental oxygen are reduction and prevention of hypoxemia, prevention and treatment of pulmonary hypertension and decrease in respiratory and cardiac overload. This is commonplace in the acute setting and is also becoming widespread in chronic pathologies. However, there is a lack of consensus on many fundamental issues, such as appropriate indications, desirable targets and outcome measures amongst centres, reflecting a variety of clinical practices. The Techniques Group of the Spanish Society of Pediatric Pneumology undertook to design recommendations for a rational approach to oxygen therapy, reviewing the existing literature in order to establish its indications, benefits and potential risks as well as its cost-effectivenes. General aspects of oxygen treatment are reviewed including physiological mechanisms, indications, delivery systems and assessment methods. Management of patients on home oxygen therapy is also addressed with discussion of benefits and potential risks of supplemental oxygen use (AU)

[Oxygen therapy in acute and chronic conditions: Indications, oxygen systems, assessement and follow-up]. / Fundamentos de la oxigenoterapia en situaciones agudas y crónicas: indicaciones, métodos, controles y seguimiento.

Oxygen therapy has become a major tool for infants with acute and chronic respiratory failure. Appropriate goals when prescribing supplemental oxygen are reduction and prevention of hypoxemia, prevention and treatment of pulmonary hypertension and decrease in respiratory and cardiac overload. This is commonplace in the acute setting and is also becoming widespread in chronic pathologies. However, there is a lack of consensus on many fundamental issues, such as appropriate indications, desirable targets and outcome measures amongst centres, reflecting a variety of clinical practices. The Techniques Group of the Spanish Society of Pediatric Pneumology undertook to design recommendations for a rational approach to oxygen therapy, reviewing the existing literature in order to establish its indications, benefits and potential risks as well as its cost-effectivenes. General aspects of oxygen treatment are reviewed including physiological mechanisms, indications, delivery systems and assessment methods. Management of patients on home oxygen therapy is also addressed with discussion of benefits and potential risks of supplemental oxygen use.

[Gastrointestinal malformations: a cause of chest pain]. / Malformaciones gastrointestinales: causa de dolor torácico.

Chest pain is an uncommon cause of consultation in childhood and is even less frequent if resulting from digestive causes. We present the cases of two patients with gastrointestinal tract malformations (diverticulum and esophageal duplication cyst) diagnosed after investigation of chest pain. A potential etiology of diverticulum could be a traction effect caused by fibrous adenopathy of tuberculous primary infection. Duplication cysts are inborn defects. Although these malformations are uncommon, clinicians should take them into account in patients with chest pain.

Malformaciones gastrointestinales: causa de dolor torácico / Gastrointestinal malformations: a cause of chest pain

El dolor torácico es una causa poco común de consulta en la infancia; menos frecuente aún es que su origen sea de etiología digestiva. Presentamos 2 pacientes en los que se diagnostican malformaciones del tracto digestivo (divertículo y quiste de duplicación esofágico) a partir del estudio realizado por dolor torácico. El divertículo podría ser debido al efecto de tracción ejercido por una adenopatía fibrosada secundaria a una primoinfección tuberculosa. En caso del quiste de duplicación se trata de una malformación congénita. Aunque estas malformaciones son hallazgos poco frecuentes, deberían ser tenidas en cuenta al abordar el estudio de un paciente con dolor torácico

Chest pain is an uncommon cause of consultation in childhood and is even less frequent if resulting from digestive causes. We present the cases of two patients with gastrointestinal tract malformations (diverticulum and esophageal duplication cyst) diagnosed after investigation of chest pain. A potential etiology of diverticulum could be a traction effect caused by fibrous adenopathy of tuberculous primary infection. Duplication cysts are inborn defects. Although these malformations are uncommon, clinicians should take them into account in patients with chest pain

Broncoscopia flexible en el niño: indicaciones y aspectos generales / Flexible bronchoscopy in children: indications and general considerations

Dada la existencia de una marcada variabilidad en la ejecución de la broncoscopia flexible pediátrica por parte de los diferentes hospitales que la practican, los miembros componentes del Grupo de Técnicas de la Sociedad Española de Neumología Pediátrica, acordaron la realización de unas recomendaciones avaladas por dicha sociedad. Se han revisado sus indicaciones, contraindicaciones, recursos materiales y humanos, el cuidado y monitorización del paciente previo, durante y posterior al procedimiento, los fármacos empleados durante su realización, las complicaciones de la técnica, así como el cuidado y mantenimiento del instrumental. Se incluye, así mismo, un modelo de consentimiento informado. Estas recomendaciones pueden adoptarse, modificarse o rehusarse, según las necesidades clínicas de cada centro (AU)

[Flexible bronchoscopy in children: Indications and general considerations]. / Broncoscopia flexible en el niño: indicaciones y aspectos generales.

There is considerable interhospital variability in the practice of flexible bronchoscopy in children. The present report aims to provide some recommendations that are supported by the Spanish Society of Pediatric Pulmonologists. We review the indications, contraindications, equipment, setting and personnel involved in flexible bronchoscopy, as well as the pre-procedure preparation of the patient, medications, post-procedure monitoring, complications, care and maintenance of instruments, and informed consent. These recommendations may be adopted, modified or rejected according to clinical needs and constraints.

[Pulmonary function after liver transplantation in cystic fibrosis. Report of four cases]. / Evolución respiratoria de 4pacientes confibrosis quística receptores detrasplante hepático.

OBJECTIVES: The aim of this study was to evaluate pulmonary function in four patients with cystic fibrosis (CF) after liver transplantation. PATIENTS AND METHODS: From 1993 to 1997 three males and one female, aged 12 to 15 years, required liver transplantation for CF with cirrhosis and portal hypertension. Three had a history of esophageal variceal bleeding. In three patients, forced vital capacity (FVC) and forced expiratory volume in one second (FEV1) before liver transplantation were over 80 and 75% of predicted values, respectively; in the fourth patient FVC was 37% and FEV1was 26%. Two patients presented allergic bronchopulmonary aspergillosis before transplantation. Only one patient was chronically infected in sputum with multiresistant Pseudomonas aeruginosa and none had Burkholderia cepacea. RESULTS: After liver transplantation, only the patient with P. aeruginosa in sputum culture and the worst pulmonary function presented a complicated course requiring mechanical ventilation for 43 days followed by non-invasive nasal ventilation for 8 months. This patient died 19 months after transplantation. The remaining three patients, with better pulmonary function before transplantation, presented an uncomplicated course and currently lead normal lives. CONCLUSIONS: We conclude that liver transplantation can improve pulmonary function and is well tolerated in children with CF and mild or moderate pulmonary involvement. When pulmonary involvement is severe, combined lung and liver transplantation should be considered.

Evolución respiratoria de 4 pacientes con fibrosis quística receptores detrasplante hepático / Pulmonary function after liver transplantation in cystic fibrosis. Report of four cases

Objetivos: El objetivo de este estudio es evaluar la función respiratoria de 4 pacientes con fibrosis quística sometidos a trasplante hepático. Pacientes y métodos: Entre 1993 y 1997, 4 pacientes con fibrosis quística, 3 varones y 1 mujer, de edades comprendidas entre los 12 y los 15 años, con cirrosis e hipertensión portal, fueron sometidos a un trasplante hepático. Tres tenían antecedentes de sangrado por varices gastroesofágicas. Antes del trasplante, 3 casos presentaban una capacidad vital forzada (FVC) y un volumen respiratorio máximo en el primer segundo (FEV1) superiores al 80 y 75%, respectivamente, con respecto a los valores teóricos; el cuarto caso presentaba una FVC del 37 por ciento y un FEV1 del 26%. Dos pacientes presentaron un brote de aspergilosis broncopulmonar alérgica en los meses previos al trasplante. Sólo un paciente estaba colonizado de forma crónica en esputo por Pseudomonas aeruginosa multirresistente y ninguno por Burkholderia cepacia. Resultados: Tras el trasplante hepático sólo el paciente colonizado por P. aeruginosa y peor función pulmonar presentó una evolución más tórpida precisando ventilación mecánica durante 43 días y ventilación nasal no invasiva durante 8 meses, y falleció a los 19 meses del trasplante. Los otros 3 casos, con mejor función pulmonar pretrasplante, presentaron una evolución sin complicaciones importantes y en la actualidad llevan una vida normal. Conclusiones: El trasplante hepático es bien tolerado en niños con fibrosis quística y afectación pulmonar leve-moderada, pudiendo incluso mejorar su función pulmonar. En los casos de afectación pulmonar grave debe considerarse la realización de un trasplante combinado hepatopulmonar (AU)

[Pulmonary granulomatosis caused by lentil aspiration]. / Granulomatosis pulmonar por aspiración de lentejas.

Pulmonary nodular granulomatosis caused by aspirated lentils is a rare entity. We report the case of a healthy 8 years-old girl who suffered a choking life-threatening choking event during a meal, with cardiorespiratory arrest. After a delay of one month delay without symptoms, she developed respiratory distress with radiologic changes. Lung biopsy disclosed foreing body granulomas. Steroids were not used because of their uncertain effectiveness in these cases. One year later the patient's progress was satisfactory.

[Primary Mycobacterium avium respiratory infection in nonimmunocompromised children]. / Primoinfección respiratoria por Mycobacterium avium en niños no inmunocomprometidos.

Mycobacterium avium is a common pathogen in barnyards, where it infects poultry and pigs. In human beings M. avium is most often found to cause disease in immunocompromised individuals, although it is also described fairly often as affecting patients with tuberculosis or chronic obstructive pulmonary disease; in recent years lung infections by M. avium have even been reported in elderly women with no underlying disease. Respiratory infection by this mycobacterium is unusual, however, in healthy children. We describe the case of a previously healthy 2-year-old boy with pneumonia whose course was complicated. After 6 months of treatment with various broad spectrum antibiotics there was no clinically or radiologically observable improvement. Other underlying diseases were ruled out, including infection by germs that cause atypical pneumonia. When all tests were negative, we investigated the possibility of primary infection by an atypical mycobacterium. A skin test was positive for M. avium. Acid-alcohol resistant bacilli were isolated from lung biopsy samples and the presence of tuberculoid granulomas were confirmed. Our patient then responded favorably after tuberculostatic treatment with 3 drugs (isoniazid, rifampicin and pyrazinamide).

[Clinical and laboratory indicators of etiology of diarrhea]. / Predicción de la etiología de la diarrea por criterios clínicos y de laboratorio.

Several clinical symptoms and laboratory findings from 352 pediatric patients, seen in the Emergency Room for acute diarrhea, were evaluated in order to develop a method to predict the bacterial etiology of the diarrhea. According to the microbiology findings, the patients were classified into two groups: proven bacterial diarrhea and diarrhea of another etiology. Among all clinical symptoms recorded, only the following were found to be useful for the prediction score: fever greater than 38 degrees C (8 points), fecal mucus (8 points), over fecal blood (6 points) and the presence of fecal leukocytes in a wet mount (7 points). An alternative score useful for outpatients was developed that does not include a score for the wet mount. When the fecal leukocyte score was included and a cutoff value of 20 points was assigned, a sensitivity of 74% and a specificity of 83% were obtained. When the score for the fecal leukocytes was excluded and a cutoff value of 13 points assigned a sensitivity of 84% and specificity of 59% were obtained. These rapid and simple scores may be useful methods for predicting acute bacterial diarrhea in children.

[Intestinal invagination: a childhood pathology amenable to conservative treatment]. / Invaginación intestinal: una patología infantil subsidiaria de tratamiento conservador.

Seventy five cases of intussusception during 1983 were studied in order to perform a statistical analysis of the relationship between clinical data an mode of treatment. Age (less than 3 months) and bloody stools upon finger rectal examination increased the percentage of surgical treatment. The reduction of intussusception by hydrostatic pressure under fluoroscopic guidance showed good therapeutical results in 89% of the cases in which it was performed.