Adult-onset pulmonary involvement in Niemann-Pick disease type B.

Niemann-Pick disease type B is caused by a deficiency in acid sphingomyelinase activity; among the six variants of Niemann-Pick disease known to date, it is the most frequently associated with lung involvement, a major cause of morbidity and mortality in this subtype in patients of all ages. Nevertheless, the vast majority of reports in the literature concern infantile forms, while less reported is, for several reasons, the onset in adults being consequently still poorly understood and characterized its clinical, radiographic and functional manifestations. We report a case of a 37 years-old female patient affected by subtype B since she was an infant, operated for aortic valve replacement two years before and came to our attention for the onset of a worsening exertional dyspnoea which proved, through a series of functional tests and radiological exams, to be a consequence of the diffuse lung involvement by the metabolic disorder; we performed a review on this topic through a Medline search of all the available "adult-onset" case reports published since the first description in 1964, also considering the possible association between NPDB and, more generally lysosomal storage disorders, and the valvular disease, already suggested by several Authors in previous works.

Mold allergy: a three year investigation (1980-1982) of the airborne fungal spores in Naples, Italy.

The purpose of the study was to examine the seasonal changes of the most important anemophilous species of molds, mainly those of allergenic interest. A spore-mold calendar for Naples, Italy was developed by continuous sampling over a 3-year period (January 1980-December 1982). The results reveal the climatic conditions are favorable for the growth of some fungi with dry spores in Naples and very high atmospheric concentrations of mold spores are to be found in summer months, particularly those of Cladosporium. Allergic sensitization with skin test reactivity and clinical signs of sensitivity to inhaled fungal spores is nevertheless found infrequently and generally not correlated with the seasonal patterns of presence of the spore molds in the atmosphere.