Higher oxygen saturation with hydroxyurea in paediatric sickle cell disease.

Introduction: Sickle cell disease (SCD) is one of the most common inherited diseases worldwide. It is associated with lifelong morbidity and reduced life expectancy. Hydroxyurea (HU) has been shown to reduce the frequency and severity of vaso-occlusive episodes in SCD.

Lung clearance index at 4 years predicts subsequent lung function in children with cystic fibrosis.

Rationale: The markedly improved life expectancy of children with cystic fibrosis (CF) has created a new challenge, as traditional markers of lung disease are frequently normal in young children. This prevents identification of individuals who may benefit from more aggressive therapy and also obliges large study numbers and prolonged duration for intervention studies. There is an urgent need for alternative surrogates that detect early lung disease and track through early childhood.

Lung function from infancy to the preschool years after clinical diagnosis of cystic fibrosis.

Rationale: After recent standardization of forced expiratory maneuvers for both infants and preschool children, longitudinal measurements are now possible from birth.

Objectives: The aim of this study was to investigate the evolution of lung function during the first 6 years of life after a clinical diagnosis of cystic fibrosis (CF) in infancy in children with CF and in healthy control subjects.

Methods: The raised volume technique was used during infancy and incentive spirometry during the preschool years.

Randomised controlled trial of nasal continuous positive airways pressure (CPAP) in bronchiolitis.

Aims: To compare continuous positive airways pressure (CPAP) with standard treatment (ST) in the management of bronchiolitis.

Methods: Children <1 year of age with bronchiolitis and capillary PCO2 >6 kPa were recruited and randomised to CPAP or ST and then crossed over to the alternative treatment after 12 h. ST was intravenous fluids and supplemental oxygen by nasal prongs or face mask.

Multiple breath washout with a sidestream ultrasonic flow sensor and mass spectrometry: a comparative study.

Over recent years, there has been renewed interest in the multiple breath wash-out (MBW) technique for assessing ventilation inhomogeneity (VI) as a measure of early lung disease in children. While currently considered the gold standard, use of mass spectrometry (MS) to measure MBW is not commercially available, thereby limiting widespread application of this technique. A mainstream ultrasonic flow sensor was marketed for MBW a few years ago, but its use was limited to infants.

Spirometry in the pre-school age group.

Spirometry is a widely used lung-function test in school-aged children and adults. It can also be used to test lung function in the pre-school population, provided that they are tested in a child-friendly setting, by experienced paediatric-orientated personnel and in a way that is congruent with their development. Accurate results can be obtained provided care and attention are paid to applying quality-control criteria suitable for this age group.

Gas mixing efficiency from birth to adulthood measured by multiple-breath washout.

Efficient mixing of inspired gas with the resident gas of the lung is an essential requirement of effective respiration. This review focuses on one method for quantifying ventilation inhomogeneity: the multiple-breath inert gas washout (MBW). MBW has been employed as a research tool in adults and school age children for more than 50 years.