Our study exemplifies detailed long term management of severe molecularly defined alveolar proteinosis from childhood into young adolescence. It is of interest that a dedicated specialized team may be advantageous to maintain the appropriate expertise of complex procedures such as e.g. whole lung lavages in small children [8, 15, 24, 25] . Therefore a centralized approach, as it has been employed for rare lung diseases and PAP in particular on a national basis in France [24] , may be warranted. A web-based system to collect these rare cases, follow them and also to receive support is available at the kids lung register (http://www.kids-lung-register.eu). The novel whole lung lavages technique using an inflatable balloon catheter was feasible in very small sized airways. Whereas empirical immunosuppressive therapy and inhaled and subcutaneous GMCSF were without significant benefit, a very intense treatment with WLL resulted in complete resolution of respiratory insufficiency, and a normalisation of lung physiology and overall somato-psychosocial condition of the child.