PDF(Pubmed)
Abstract：
Hunter syndrome (MPS II), an X-linked recessive lysosomal storage disorder, is a result of deficiency of the iduronate 2-sulfatase enzyme (IDS), leading to cognitive impairment, systemic organ involvement, and increased dental problems. This case report describes the management of a child with Hunter syndrome who was referred to the Department of Paediatric and Preventive Dentistry for pain in the upper front teeth. Intraoral examination revealed severe early childhood caries, prompting planning for full-mouth rehabilitation under general anaesthesia due to the child\'s uncooperative behaviour. In response to recommendations from the Department of Otolaryngology and the Department of Paediatric Surgery, a comprehensive treatment plan consolidated full-mouth rehabilitation in addition to adenoidectomy and inguinal and umbilical herniotomy procedures during a single session of general anaesthesia. Successful interventions were reflected in the uneventful one-month follow-up of the patient, highlighting the efficacy of the interdisciplinary approach. The key takeaway underscores the importance of collaborative interventions, emphasising singular intubation for patients requiring recurrent hospitalisations, providing both monetary relief and reducing post operative healing time. Designed to address global developmental delay in the child, a personalised home care plan was also implemented. Evaluation of plaque and gingival indices before and after the home care regimen demonstrated a notable improvement, indicating an enhanced oral quality of life.
摘要：
亨特综合征（MPSII），X连锁隐性溶酶体贮积症,是艾杜糖醛酸2-硫酸酯酶（IDS）缺乏的结果，导致认知障碍，全身器官受累，增加牙齿问题。此病例报告描述了一名患有亨特综合征的儿童的管理，该儿童因上门牙疼痛而被转诊至儿科和预防牙科部门。口内检查显示严重的儿童早期龋齿，由于儿童的不合作行为，促使计划在全身麻醉下进行全口康复。针对耳鼻咽喉科和小儿外科的建议,在一次全身麻醉期间，除了腺样体切除术以及腹股沟和脐疝切开术外，全面的治疗计划还巩固了全口康复。成功的干预措施反映在患者一个月的平稳随访中，强调跨学科方法的功效。关键要点强调了协作干预的重要性，强调需要反复住院的患者的单一插管，提供货币救济和减少术后愈合时间。旨在解决全球儿童发育迟缓的问题，还实施了个性化的家庭护理计划。在家庭护理方案之前和之后，对牙菌斑和牙龈指数的评估显示出显着的改善，表明口腔生活质量提高。
