[Respiratory function assessment in cooperative patients. Part II]. / Estudio de la función pulmonar en el paciente colaborador. Parte II.

Analysis of bronchial hyperresponsiveness using bronchial provocation tests are a key feature in the diagnosis of asthma, as well as a valid tool for monitoring disease severity, clinical course, and treatment response. We review non-specific bronchial challenge tests, including pharmacological stimuli (methacholine, adenosine) and physical stimuli (exercise, hypertonic saline, cold air hyperventilation). Although there is some correlation among responses to the distinct tests, individual responses are also observed. The indication for a single test will depend on whether the procedure will be used for diagnostic or epidemiologic purposes, and on experience of its use. Frequently, complementary information will be obtained. Indirect airway challenges tests such as physical stimuli and adenosine are more specific for asthma diagnosis.

[Nephrolitiasis in a patient with cystic fibrosis]. / Nefrolitiasis en un paciente con fibrosis quística.

Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. Defects in the CFTR gene cause abnormal chloride conductance across the apical membrane of epithelial cells, which results in progressive lung disease and also affects other organs. Because life expectancy has increased, other complications of CF have become more apparent. We present a patient with CF and symptomatic nephrolithiasis. Several stones were evident in both kidneys. A 24-hour urine sample showed hyperoxaluria (141 mg/24 h/ 1.73 m(2)) and hypocitraturia and (206 mg/24 h/1.73 m(2), 177 mg citrate/g creatinine). Nephrolithiasis should be included in the differential diagnosis of patients with CF and abdominal pain; urinary excretion of oxalate and citrate should be investigated.

[Effects of inhaled glucocorticoids effects on the body composition in children with moderate asthma]. / Efecto de los glucocorticoides inhalados en la composición corporal del niño con asma moderada.

BACKGROUND: To assess anthropometric variables and body composition in children with moderate asthma. SUBJECTS AND METHOD: Cross-sectional study of two homogeneous cohorts. Group 1 (study group): 84 children with moderate asthma treated with inhaled budesonide for al least 12 months; group 2 (control group): 89 healthy children. Body measurements were studied by bioelectrical impedance. RESULTS: Males with moderate asthma showed lower values for fat-free mass and total body water. Data corrected for weight rendered no statistically significant differences. CONCLUSIONS: Male children with moderate asthma show a lower fast-free mass.

[Horseshoe lung with normal pulmonary venous return]. / Pulmón en herradura con drenaje venoso pulmonar normal.

BACKGROUND: Horseshoe lung is a rare congenital anomaly characterised by a midline isthmus of pulmonary parenchyma connecting the posterior basal segments of the lungs behind the heart in conjunction with unilateral pulmonary hypoplasia. Of all cases, 80% are associated with scimitar syndrome, consisting of right anomalous pulmonary venous drainage, pulmonary hypoplasia of the right lung and systemic arterial perfusion to some lung areas. CASE: A six year old girl who had recurrent lower respiratory infections since birth. Chest Rx, angioCT and MR showed: hypoplasia of the right lung, dextrocardia and pulmonary isthmus bridging both lungs behind the pericardium. The right hypoplastic lung had little systemic supply coming from the abdominal aorta. The right pulmonary artery was hypoplastic. The right pulmonary venous drainage was normal. CONCLUSIONS: We present a case of horseshoe lung without abnormal venous drainage.

Sección de Neumología Infantil y Trastornos del Sueño: Características epidemiológicas y actividad de la Sección / Child Pneumology and Sleep Disorders Section. Epidemiological characteristics and activity of the Section

El desarrollo de la Unidad de Neumología Infantil tiene como objetivo la atención de las necesidades que tiene nuestra área asistencial. La patología respiratoria infantil tiene una alta prevalencia y genera altos costos sanitarios. Es el caso del asma bronquial, del cual se revisa su epidemiología y las formas más efectivas de tratamiento. Otra patología prevalente que atendemos es el síndrome de apnea-hipopnea del sueño. Más recientemente hemos puesto en marcha una consulta multidisciplinar para pacientes pediátricos con patología neuromuscular (AU)

The Respiratory Unit aims to satisfy the care needs of our reference population. The childhood respiratory diseases have a high prevalence and generates high health costs. It is the case of bronchial asthma. lts epidemiology and effective forms of treatment are reviewed. Another prevalent pathology cared in our section is children sleep apnea-hypopnea syndrome. More recently we have launched a multidisciplinary consultation for pediatric patients with neuromuscular disease (AU)

La implantación de guías clínicas: ¿cómo puede ayudarnos su informatización? / The introduction of clinical guidelines: could their computerisation help us?

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Pulmón en herradura con drenaje venoso pulmonar normal / Horseshoe lung with normal pulmonary venous return

Antecedentes: El pulmón en herradura es una rara anomalía congénita caracterizada por un istmo medio de parénquima pulmonar que une los segmentos posterobasales de ambos pulmones por detrás del corazón, acompañándose de una hipoplasia pulmonar unilateral. En el 80% de los casos se asocia con un síndrome de la cimitarra, consistente en drenaje venoso pulmonar derecho anómalo, hipoplasia pulmonar derecha y perfusión arterial sistémica a alguna porción del pulmón. Caso clínico: Niña de 6 años que desde el nacimiento presenta infecciones respiratorias bajas de repetición y escoliosis. Rx de tórax, angio-TC y RM. Hipoplasia pulmonar derecha, dextrocardia e istmo pulmonar que une las bases de ambos pulmones por detrás del pericardio. El pulmón derecho hipoplásico tiene un pequeño suministro sistémico que proviene de la aorta abdominal. La arteria pulmonar derecha es hipoplásica. El drenaje venoso de las venas pulmonares derechas es normal. Conclusiones: Se presenta un pulmón en herradura sin drenaje venoso anómalo (AU)

Background: Horseshoe lung is a rare congenital anomaly characterised by a midline isthmus of pulmonary parenchyma connecting the posterior basal segments of the lungs behind the heart in conjunction with unilateral pulmonary hypoplasia. Of all cases, 80% are associated with scimitar syndrome, consisting of right anomalous pulmonary venous drainage, pulmonary hypoplasia of the right lung and systemic arterial perfusion to some lung areas. Case: A six year old girl who had recurrent lower respiratory infections since birth. Chest Rx, angioCT and MR showed: hypoplasia of the right lung, dextrocardia and pulmonary isthmus bridging both lungs behind the pericardium. The right hypoplastic lung had little systemic supply coming from the abdominal aorta. The right pulmonary artery was hypoplastic. The right pulmonary venous drainage was normal. Conclusions: We present a case of horseshoe lung without abnormal venous drainage (AU)

Estudio de la función pulmonar en el paciente colaborador. Parte II / Respiratory function assessment in cooperative patients. Part II

El análisis de la hiperrespuesta bronquial mediante pruebas de provocación bronquial es uno de los pilares fundamentales para el diagnóstico de asma, así como un instrumento válido para la monitorización de la enfermedad, valoración de su gravedad, su evolución y la respuesta al tratamiento. Revisamos las pruebas de provocación bronquial inespecíficas tanto por estímulos físicos (ejercicio físico, soluciones hiperosmolares, y la hiperventilación con aire frío) como por fármacos (metacolina y adenosina). Aunque hay una cierta correlación entre las respuestas a diferentes tipos de prueba, hay niños que responden de forma diferente. La elección de la prueba que hay que utilizar dependerá de los fines diagnósticos o epidemiológicos que persigamos, de la experiencia de su utilización, dándonos frecuentemente información complementaria. Las pruebas de provocación indirectas como los estímulos físicos y la adenosina son más específicas del asma

Analysis of bronchial hyperresponsiveness using bronchial provocation tests are a key feature in the diagnosis of asthma, as well as a valid tool for monitoring disease severity, clinical course, and treatment response. We review non-specific bronchial challenge tests, including pharmacological stimuli (methacholine, adenosine) and physical stimuli (exercise, hypertonic saline, cold air hyperventilation). Although there is some correlation among responses to the distinct tests, individual responses are also observed. The indication for a single test will depend on whether the procedure will be used for diagnostic or epidemiologic purposes, and on experience of its use. Frequently, complementary information will be obtained. Indirect airway challenges tests such as physical stimuli and adenosine are more specific for asthma diagnosis

Nefrolitiasis en un paciente con fibrosis quística / Nephrolitiasis in a patient with cystic fibrosis

La fibrosis quística está causada por mutaciones en el gen regulador de la conductividad transmembrana (CFTR). Defectos en el gen CFTR ocasionan una conductividad alterada del cloro a través de la membrana apical de las células epiteliales, dando lugar a una enfermedad pulmonar progresiva y afectando también a diferentes órganos. Con el aumento de las expectativas de vida otras complicaciones de la fibrosis quística se han hecho más frecuentes. Presentamos una paciente con fibrosis quística y nefrolitiasis sintomática. Varios cálculos eran visibles en ambos riñones. En la muestra de orina de 24 h se evidenció hiperoxaluria (141 mg/24 h/1,73 m 2) e hipocitraturia (206 mg/ 24 h/1,73 m 2, 177 mg citrato/g creatinina). La nefrolitiasis debería estar presente en el diagnóstico diferencial de un paciente con fibrosis quística que tiene dolor abdominal y la excreción de oxalato y citrato debería ser investigada

Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. Defects in the CFTR gene cause abnormal chloride conductance across the apical membrane of epithelial cells, which results in progressive lung disease and also affects other organs. Because life expectancy has increased, other complications of CF have become more apparent. We present a patient with CF and symptomatic nephrolithiasis. Several stones were evident in both kidneys. A 24-hour urine sample showed hyperoxaluria (141 mg/24 h/ 1.73 m 2) and hypocitraturia and (206 mg/24 h/1.73 m 2, 177 mg citrate/g creatinine). Nephrolithiasis should be included in the differential diagnosis of patients with CF and abdominal pain; urinary excretion of oxalate and citrate should be investigated

Guía de práctica clínica sobre asma (2ª parte) / Clinical practice guideline on asthma (part II)

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Guía de práctica clínica sobre asma (1ª parte) / Clinical practice guideline on asthma (part I)

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Inmunodeficiencias primarias / No disponible

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