Pathological and biochemical study in the adenoid of mucopolysaccharidosis II.
In the patients with mucopolysaccharidosis II (MPS II, Hunter's syndrome), conductive and sensorineural hearing deficits are frequently observed. Two patients with MPS II underwent adenoidectomy and an ear douche, and their conductive hearing loss recovered after the surgery. Pathological examination of the adenoids revealed the infiltration of faintly PAS-positive plasmacytes with perinuclear vacuole and strongly Alcian-blue positive fibrotic area. Biochemical study was performed by chromatoscanning of electropholesis. The amount of glycosaminoglicans (GAG) in the specimens was increased 2.8-fold compared with the normal control. The electropholetic pattern showed an increase of dermatan sulfate (DS), heparan sulfate (HS) and hyaluronic acid (HA) in the adenoids of the patients. The ratio of DS to HS was 1.1-1.2. In the patients of MPS II, the accumulation of GAG occurs in the pharyngeal tonsil and causes conductive hearing impairment. We recommend adenoidectomy for such patients.
D000234:Adenoids / Q000378:metabolism / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D006314:Hearing Loss, Conductive / Q000209:etiology / Q000628:therapy; D006319:Hearing Loss, Sensorineural / Q000209:etiology / Q000628:therapy; D006651:Histocytochemistry; D006801:Humans; D008297:Male; D016532:Mucopolysaccharidosis II / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D010950:Plasma Cells / Q000473:pathology