Yonel Zehra, Parma Sabrina, Chapple Iain L C
Dent Update. 2015 Sep;42(7):656-8, 660-2, 665-6. doi: 10.12968/denu.2015.42.7.656.
Juvenile hyaline fibromatosis (JHF) is a rare autosomal recessive inherited condition presenting early in life and characterized by the accumulation of hyaline-like tissue in the skin as well as various organs. Gingival overgrowth is a significant oral manifestation. This paper highlights how early and essential periodontal intervention may be necessary to improve mastication and subsequent weight gain, and to eliminate pain and improve the patient's quality of life. Here we highlight the key features of this condition and demonstrate how appropriate surgical management can have a significant impact on a patient's wellbeing. CPD/CLINICAL RELEVANCE: Juvenile hyaline fibromatosis has a significant impact on patient wellbeing and it is therefore important that clinicians are able to recognize the condition and ensure that patients receive appropriate care and management.
青少年透明纤维瘤病(JHF)是一种罕见的常染色体隐性遗传病,在生命早期发病,其特征是皮肤及各种器官中出现类透明样组织堆积。牙龈增生是一种重要的口腔表现。本文强调了早期且必要的牙周干预对于改善咀嚼功能及随后的体重增加、消除疼痛并提高患者生活质量可能是必要的。在此,我们突出该病症的关键特征,并展示适当的手术管理如何能对患者的健康产生重大影响。继续医学教育/临床相关性:青少年透明纤维瘤病对患者健康有重大影响,因此临床医生能够识别该病症并确保患者得到适当的护理和管理非常重要。