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This study aimed to: assess the mucosal alterations of the larynx and hypopharynx typical for mucopolysaccharidoses, in a standardised manner; compare the severity in different subtypes of mucopolysaccharidoses; and monitor the effect of an enzyme replacement therapy.
A classification for mucosal alterations of the larynx and hypopharynx was developed and utilised in 55 patients with mucopolysaccharidoses. Fifteen patients who started treatment with enzyme replacement therapy were followed longitudinally.
The most severe alterations were seen in the posterior region of the larynx and the arytenoids, and in the region of the false vocal folds. The alterations were most severe in patients with mucopolysaccharidosis II. No clear trend was observed in the patients who received enzyme replacement therapy.
Quantification of mucosal alterations of the hypopharynx and larynx in mucopolysaccharidoses patients can provide information about the disease's natural process and about the efficacy of enzyme replacement therapy.
Extremely narrow far-infrared lines  of OH-donors in ultrapure germanium are used to probe the fundamental dynamic processes of impurities. We examine the 1s-2p transition as a function of laser intensity, using photothermal ionization spectroscopy (PTIS) modified by Zeeman tuning with frequency-fixed lasers. We observe a change of the resonance line shape in the intensity region near 10-4 W/cm2. This effect can be quantitatively understood in a rate equation model which shows that at the critical intensity the ground state becomes depleted and the dependence of the recombination on the degree of ionization becomes important. Therefore the critical intensity depends also on compensation.