Kalsi Jagdip S, Auplish Gita, Johnson Adele R, Darbar Ulpee R
Department of Restorative Dentistry, Croydon University Hospital, London, UK.
Pediatr Dent. 2012 Sep-Oct;34(5):140-3.
Alpha-mannosidosis is a rare genetic lysosomal storage disorder that is inherited in an autosomal recessive pattern. Severe periodontal breakdown in alpha-mannosidosis patients has not previously been reported in the literature. The purposes of this paper are to: present the cases of 2 siblings diagnosed with alpha-mannosidosis, each of whom had varying severity of periodontal destruction; and provide an overview of alpha-mannosidosis, the possible reasons for the periodontal destruction, and the periodontal management in the 2 affected siblings. Both had preventive and nonsurgical periodontal therapy followed by a 5-year period of supportive therapy. Their pattern of bone loss was consistent with those with periodontitis as a manifestation of systemic diseases, with the extent of periodontal destruction being related to the severity of the alpha-mannosidosis. Alpha-mannosidosis patients present with social disfigurements and, to prevent tooth loss that can add to this, early periodontal diagnosis is important to optimize management and intervention.
α-甘露糖苷贮积症是一种罕见的遗传性溶酶体贮积病,呈常染色体隐性遗传模式。此前文献中未报道过α-甘露糖苷贮积症患者出现严重的牙周破坏情况。本文的目的是:介绍2例被诊断为α-甘露糖苷贮积症的同胞病例,他们各自患有不同严重程度的牙周破坏;并概述α-甘露糖苷贮积症、牙周破坏的可能原因以及对这2例患病同胞的牙周治疗情况。两人均接受了预防性和非手术性牙周治疗,随后进行了为期5年的支持性治疗。他们的骨质流失模式与那些以牙周炎作为全身性疾病表现的患者一致,牙周破坏的程度与α-甘露糖苷贮积症的严重程度相关。α-甘露糖苷贮积症患者存在社会形象受损问题,为防止牙齿脱落加剧这一情况,早期牙周诊断对于优化治疗和干预非常重要。