Langerhans Cell Histiocytosis (LCH) is an orphan disease of clonal dendritic cells which may affect any organ of the body. Most of the knowledge about the diagnosis and therapy is based on pedriatic studies. Adult LCH patients are often evaluated by physicians who focus on only the most obviously affected organ without sufficient evaluation of other systems, resulting in patients being underdiagnosed and/or incompletely staged. Furthermore they may be treated with pediatric-based therapies which are less effective and sometimes more toxic for adults. The published literature on adult LCH cases lacks a comprehensive discussion on the differences between pediatric and adult patients and there are no recommendations for evaluation and comparative therapies. In order to fill this void, a number of experts in this field cooperated to develop the first recommendations for management of adult patients with LCH. Key questions were selected according to the clinical relevance focusing on diagnostic work up, therapy, and follow up. Based on the available literature up to December 2012, recommendations were established, drafts were commented by the entire group, and redrafted by the executive editor. The quality of evidence of the recommendations is predominantly attributed to the level of expert opinion. Final agreement was by consensus.

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The prevalence and clinical significance of sleep-related breathing disorders (SRBDs) in patients with cardiac disease and a history of life-threatening ventricular tachyarrhythmias is unclear. Forty consecutive recipients of implantable cardioverter defibrillators (ICDs) with cardiac disease and a documented history of spontaneous, life-threatening, ventricular tachyarrhythmias underwent full night polysomnography. SRBDs were diagnosed if the apnea/hypopnea index was > 10. SRBD were diagnosed in 16 of 40 patients (40%): central sleep apnea (CSA) was present in 9 of these 16 patients (56%), 8 of whom had associated Cheyne-Stoke respiration. Seven of the 16 patients with SRBD (44%) had obstructive sleep apnea (OSA). Patients with and without SRBDs were comparable with respect to left ventricular ejection fraction, NYHA classification, underlying heart disease, ICD indications, and concomitant antiarrhythmic drug and beta-blocker therapy. Patients were followed prospectively for 2 years. ICD-treated ventricular tachyarrhythmias occurred in 10 of 24 patients (42%) without SRBD, in 4 of 9 patients (44%) with CSA, and in 3 of 7 patients (44%) with OSA (NS). The numbers and circadian distributions of episodes recorded during follow-up in patients without SRBD versus with CSA or OSA were not significantly different (14 +/- 25, median = 4 vs 4 +/- 5, median = 2.5 vs 15 +/- 15, median = 7, respectively). The 2-year mortality, which was entirely attributable to nonsudden cardiac events, was highest in patients with CSA (4/9 [44%], vs 0/7 [0%] with OSA, vs 3/24 patients (12.5%) without SRBD; P < 0.05).

In 6 normal and 7 obstructed excised human lungs the interrupter resistance (APTA, Jaeger Company) with an airway occlusion period of 100 ms was determined, by measuring the equivalent of the alveolar pressure at the end of the occlusion period. To check the pressure equilibration between the tracheal pressure and the alveolar space, catheters were put in the most peripheral layer of the lung. The lungs were ventilated in an artificial thorax. The airway resistance determined from the transbronchial pressure difference by the catheters was taken as a reference. Compared with the reference method, an overestimation of the airways resistance by the interrupter technique in normal lungs was found which was caused by an overshoot of the pressure equilibration during the occlusion period. In contrast, in severely obstructed lungs the pressure equilibration was not complete which led to an underestimation of the airways resistance by the interrupter technique. The best approximation of the airways resistance by the interrupter method was found in lungs with a low degree of obstruction.

protocol 1 Ci had impaired VO2max and reduced AT (P < 0.05). Basal plasma concentrations of insulin, glucagon, growth hormone and adrenaline were increased in Ci (P < 0.05); cortisol was normal. During exercise, only glucagon remained different between groups. In protocol 2 Ci had decreased resting respiratory exchange ratio (RQ: p < 0.05) associated with increased plasma concentrations of free fatty acids and glycerol. They had disproportionately enhanced lipolysis and RQ. heart rate (+24%), ventilation (+28%), thermal effects of exercise (+31%) and intrapulmonary shunt volume (+76%), which accounted for 11.7 (SD 3.0) or 7.4 (SD 0.9%) of cardiac output during exercise in Ci and Co, respectively (P < 0.05 for all the differences reported). The metabolic effects of Ci were independent of the clinical and nutritional state of the patients. In protocol 3 muscle glycogen content was highly variable in Ci, but mean values were normal [16.9 (SD 8.9) mumol.g-1 wet mass]. Glycogen content positively correlated with resting and exercise-induced RQ, but negatively correlated with the exercise-induced alterations in plasma glucose concentration. From these results we concluded that with reduced liver function VO2max and AT are reduced, but metabolic, pulmonary and haemodynamic responses per unit power output are enhanced. Muscle glycogen content would seem to contribute to the metabolic response, but its mobilization to be limited in individuals with reduced liver function.