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We report three children with an unusual radiological sign: "trachea with an air fluid level." We suggest this is related to paucity of cough leading to recurrent chest infections. Voluntary cough suppression as a cause of chronic lower respiratory tract infection is recorded in adults (The Lady Windermere Syndrome) but has not previously been reported in children. We propose that in these children impaired airway mucus clearance may be also be caused by voluntary cough suppression. However, the complex physiology of coughing means it is difficult to distinguish between true voluntary cough suppression and paucity of cough due to a subtle neurological deficit. In two patients, the cycle has led to permanent lung damage with bronchiectasis and reduced lung function. In the third, early diagnosis and multidisciplinary intervention has so far delayed progression to bronchiectasis. With greater awareness of this phenomenon in children, there is potential for effective early intervention with medical, physical, and psychological therapies.

We report a 6-year old girl with recurrent and non-resolving left-sided "pneumonia" over a 3-year period who was diagnosed with an endobronchial low-grade fibrosarcoma. This highlights the importance of considering underlying localized airway obstruction in any case of clinically or radiologically atypical pneumonia in a child and therefore the need for careful follow-up of all but the most typical cases.

Background The prevalence of idiopathic scoliosis in the general pediatric population is reported to be between 0.5% and 3.2%. Previous studies have reported an increased prevalence of scoliosis in children with cystic fibrosis (CF). Hypothesis The prevalence of scoliosis in CF children is greater than in the normal population. Methods Chest X-rays of 319 patients attending the Royal Brompton CF Centre were reviewed. The Cobb angle of any spinal lateral curvature was measured. Scoliosis was defined as a Cobb angle of more than 10°. Results Median age of the whole group of patients was 10.9 years (range 1.1–18 years), 53% were females. Seven patients (2.2%) had radiological signs of scoliosis, their mean age was 14.5 ± 2.3 years (range: 11.5–18 years), 5 were females. Age at onset of scoliosis was between 5 and 10 years for three patients and over 10 years for the others. All the curves were thoracic and with right convexity: apices between T7 and T9 for the single curves (n = 5) and between T4 and T5 (n = 2) for the double curves. Conclusion CF patients showed a similar prevalence of scoliosis as in the normal population (2.2% vs. 0.5–3.2%). There was the same gender (female) and side (right-sided) predilection as in normal population. Pediatr Pulmonol. 2013; 48:553–555. © 2012 Wiley Periodicals, Inc.

We report on a child with Mycoplasma pneumonia, who developed an unexplained new oxygen requirement. He was found to have an ileo-femoral thrombosis and an acute pulmonary embolus, with positive anti-phospholipid antibodies and acquired activated protein C resistance. These are both acquired risk factors for venous thrombosis. He was successfully anti-coagulated and well at follow-up with disappearance of the anti-phospholipid antibodies, and normalization of his activated protein C activity. Children who present with Mycoplasma infections who run an atypical course should be monitored closely for signs of thrombosis. Thrombosis formation should also be considered in those children with deteriorating respiratory status, but little change in radiographical findings. Children found to have developed thrombi should be investigated with a full thrombophilia screen to elicit both congenital and acquired risk factors, and should be anti-coagulated appropriately.

Until recently, the bronchial circulation has been relatively ignored in the research and clinical arenas, perhaps because of its small volume and seeming dispensability relative to the pulmonary circulation. Although the bronchial circulation only receives around 1% of the cardiac output in health, it serves functions that are critical to maintaining airway and lung function. The bronchial circulation also plays an important role in many lung and airway diseases; through its ability to increase in size, the bronchial circulation is able to provide lung parenchymal perfusion when the pulmonary circulation is compromised, and more recently the role of the bronchial circulation in the pathogenesis of inflammatory airway disease has been explored. Due to the anatomic variability and small volume of the bronchial circulation, much of the research to date has necessitated the use of animal models and invasive procedures. More recently, non-invasive techniques for measuring bronchial blood flow in the mucosal microvascular network have been developed and offer a new avenue for the study of this circulation in humans. In conjunction with molecular research, measurement of airway blood flow (Q(aw)) may help elucidate the role of the bronchial circulation in inflammatory airway disease and become a useful tool for monitoring therapy.