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

[Chronic liver disease associated with cystic fibrosis: energy expenditure at rest, risk factors, and impact on the course of the disease]. / Hepatopatía crónica asociada a fibrosis quística: gasto energético en reposo, factores de riesgo y repercusión en la evolución de la enfermedad.

INTRODUCTION AND GOALS: Cystic fibrosis is the most frequent recessive disease. There are various hepato-biliary alterations, of which the most important is the development of biliary obstruction and periportal fibrosis. The goal is to assess the influence of liver disease on the nutritional status, the progress of the disease and the associated risk factors. SCOPE: Nutrition Unit of the Gastroenterology Department at La Paz Children's Hospital. MATERIAL AND METHODS: Prospective longitudinal study with 53 patients assessed on three occasions over 3 years: at inclusion, after 1 year and after 3 years. Only 37 of them were followed-up for 3 years. A total of 111 measurements were taken, including analysis of body composition, energy expenditure, intake and energy losses as well as nitrogen balance. Simultaneously, respiratory function tests were performed and the presence of repeated acute respiratory problems was evaluated. RESULTS: 37 patients, 19 females and 18 males (mean age 13.04 years +/- 3.28). Twelve (32.43%) were diagnosed as having liver disease (mean age 12.16% +/- 3.86 SD, 11 males, 1 female), of whom 1 presented meconial ileum, 5 were homozygotic, 5 heterozygotic and the other two presented other mutations. Those with liver disease presented anthropometric parameters that were better than or similar to the patients without liver involvement (p NS). Mean Waterlow index in liver disease sufferers: 93.62% +/- 7.87 SD; without liver disease: 93.06% +/- 10.97 SD (p NS). Mean of LVEF in liver disease sufferers: 88.81 +/- 27.32 SD; without liver disease: 75.21 +/- 27.92 SD (p < 0.05). Mean FVC in liver disease sufferers: 95.38 +/- 22.92 SD; without liver disease: 83 +/- 23.7 SD (p < 0.05). Mean energy expenditure at rest/lean body mass in liver disease sufferers: 38.81 kcal +/- 7.26 SD; without liver disease: 42.36 kcal +/- f 6.91 SD (p < 0.05). CONCLUSIONS: The prevalence of chronic liver disease in patients with cystic fibrosis increases with age and is more frequent in males. Liver disease sufferers present a better evolution in pulmonary function and present less energy expenditure.

[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).

[Status asthmaticus; experience in a pediatric intensive care unit]. / Status asmático: experiencia en una unidad de cuidados intensivos pediátricos.

Thirty children presenting status asthmaticus in fifty episodes were studied during a period of three and a half years. All of them showed acute respiratory failure secondary to asthma. The purpose was to evaluate treatment results and complications compared with a previous similar study conducted in our unit. We have noticed that there is a lower number of admissions for SA in our ICU and a different seasonal tendency. Precious treatment of asthmatic crisis selective inhaled beta 2 bronchodilators can prevent the ICU admission of an asthmatic patient.

[Diabetic ketoacidosis and coma. Insulin perfusion (author's transl)]. / Cetoacidosis y coma diabético. Tratamiento con perfusión contínua de bajas dosis de insulina y actualización terapéutica.

Ten cases of diabetic ketoacidosis treated by low-dose insulin continuous perfusion are reported. The severe period therapeutic guide is exposed in two stages showing the form of correction of the most important ketoacidosis. Insulin doses have been, initially, 0.1 U./Kg. in a bolus, followed by 0.1-0.01 U./Kg./h. till acidosis and dehydration are corrected. Evolution of every case was good, without any important complication. Advantages of this therapeutic modality are appointed: an easy treatment management, lesser glycaemia oscillations and lesser complications than using the conventional method of intermittent high doses. In order to overcome, the acidosis and hyperglicaemia resistance found in some of the severest cases, authors suggest to increase the dose of perfused insulin as well as hydration, Finally, the importance of control and patient care is stressed, recommending their admission to a Paediatric Intensive Care unit.