We found that nebulised antibiotics, alone or in combination with oral antibiotics, were better than no treatment for early infection with Pseudomonas aeruginosa. Eradication may be sustained in the short term. Overall, there is insufficient evidence from this review to state which antibiotic strategy should be used for the eradication of early Pseudomonas aeruginosa infection in CF.

Analysis 3.2. Comparison 3 Oral ciprofloxacin and inhaled colistin versus inhaled tobramycin, Outcome 2 Adverse events......... Analysis 4.1. Comparison 4 Nebulised tobramycin 28 days versus 56 days, Outcome 1 Time to next isolation of P aeruginosa from BAL, sputum or oropharyngeal cultures.

Cerebro-Costo-Mandibular syndrome (CCMS) is a rare autosomal dominant condition comprising branchial archderivative malformations with striking rib-gaps. Affected patients often have respiratory difficulties, associated with upper airway obstruction, reduced thoracic capacity, and scoliosis. We describe a series of 12 sporadic and 4 familial patients including 13 infants/children and 3 adults. Severe micrognathia and reduced numbers of ribs with gaps are consistent findings. Cleft palate, feeding difficulties, respiratory distress, tracheostomy requirement, and scoliosis are common. Additional malformations such as horseshoe kidney, hypospadias, and septal heart defect may occur. Microcephaly and significant developmental delay are present in a small minority of patients. Key radiological findings are of a narrow thorax, multiple posterior rib gaps and abnormal costo-transverse articulation. A novel finding in 2 patients is bilateral accessory ossicles arising from the hyoid bone. Recently, specific mutations in SNRPB, which encodes components of the major spliceosome, have been found to cause CCMS. These mutations cluster in an alternatively spliced regulatory exon and result in altered SNRPB expression. DNA was available from 14 patients and SNRPB mutations were identified in 12 (4 previously reported). Eleven had recurrent mutations previously described in patients with CCMS and one had a novel mutation in the alternative exon. These results

In order to manage this complex group of patients, a combined cardiological, respiratory and surgical approach was required. A protocol with cardiac catheterisation, blood tests and respiratory assessment is suggested for the management of pulmonary hypertension in these children.

Aims: To gather data on the clinical presentation of parapneumonic effusion and empyema and to examine the effect of different management strategies on short term outcomes. Methods: Retrospective case note review of 48 children admitted to a tertiary unit between January 1998 and March 2001. Effusions were classified into three stages dependent on ultrasound findings. Results: The stage of effusion was not associated with duration of previous symptoms or length of previous admission. An interventional procedure was performed on median day 2 of admission in 46 children: eight (17%) had an intercostal drain alone, 14 (29%) had an intercostal drain followed by intrapleural fibrinolytic therapy, and 24 (50%) had a thoracotomy. Three children who had an initial intercostal drain alone returned to theatre for thoracotomy, and two children who had intrapleural fibrinolysis returned for thoracotomy. Median length of stay (interquartile range) for each initial procedure was 15 days (6-20) for intercostal drain alone, 8 days (6-12) for fibrinolytic therapy, and 6.5 days (5-9) for thoracotomy. Stay for intercostal drain alone was significantly longer than for thoracotomy. Conclusion: Early surgical management of empyema is associated with a favourable outcome.T he optimal management of parapneumonic effusions and empyema in children remains controversial and currently there is insufficient evidence to give clear guidance on therapy. Strategies include a conservative approach with antibiotic therapy, the use of chest drains, intrapleural fibrinolytic therapy, video assisted thoracoscopic therapy (VATS), and more aggressive surgical intervention with thoracotomy and decortication. To further complicate matters, parapneumonic effusion and empyema are unlikely to be a single entity, and more probably lie on an evolving continuum.1 2 Children therefore need to be treated differently depending on their clinical picture. Retrospective reviews have contributed to our knowledge of the disease processes and treatment, but these have often been over relatively long time periods with the use of evolving surgical strategies and imaging techniques.3-6 More recently there has been a trend for the use of fibrinolytic therapy and less invasive surgery, and a move away from thoracotomy. We reviewed our experience with a large number of children with parapneumonic effusion and empyema over a short time interval. Our aims were to gather data on clinical presentation and to examine the effect of different management strategies on short term outcomes.