RESUMEN
MPS encompasses a group of rare lysosomal storage disorders that are associated with the accumulation of glycosaminoglycans (GAG) in organs and tissues. This accumulation can lead to the progressive development of a variety of clinical manifestations. Ear, nose, throat (ENT) and respiratory problems are very common in patients with MPS and are often among the first symptoms to appear. Typical features of MPS include upper and lower airway obstruction and restrictive pulmonary disease, which can lead to chronic rhinosinusitis or chronic ear infections, recurrent upper and lower respiratory tract infections, obstructive sleep apnoea, impaired exercise tolerance, and respiratory failure. This review provides a detailed overview of the ENT and respiratory manifestations that can occur in patients with MPS and discusses the issues related to their evaluation and management.
Asunto(s)
Obstrucción de las Vías Aéreas/etiología , Mucopolisacaridosis/complicaciones , Mucopolisacaridosis/fisiopatología , Sistema Respiratorio/fisiopatología , Trastornos del Sueño-Vigilia/etiología , Obstrucción de las Vías Aéreas/fisiopatología , Humanos , Trastornos del Sueño-Vigilia/fisiopatologíaRESUMEN
A hydrogenated frustrated Lewis pair ([TMPH+][HB(C6F5)3 -]) promotes controlled living ionic polymerization of cyanoacrylates. Controlled growth of various homopolymeric CAs through sequential monomer addition has been achieved, in addition to CA block copolymers with controlled block sequences for the first time in the long history of these materials.