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GINGIVAL FIBROMATOSIS, SHORT STATURE, BORDER-LINE IQ, FACIAL DYSMORPHISM AND HEPATOMEGALY

机译：牙龈纤维化，身材矮小，边界线智商，面部肌营养不良和肝癌

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摘要

Gingival fibromatosis, short stature, border-line IQ, facial dysmorphism and hepatomegaly: Gingi-val flbromatosis is a rare and benign disorder. The enlarged gingivae are firm and may interfere with speech, closure of the lips, and mastication. We report a thirteen years old girl, with gingival fibromatosis referred to the periodontics clinics. Full mouth gingivectomy and gingivoplasty were performed. Medical investigation showed short stature, low-borderline IQ, facial dysmorphism, and hepatomegaly. Histological analysis revealed hyperplasia in the epithelial area and fibrotic appearance of gingival connective tissue with dense collagen fibre clusters. Pedigree analysis confirmed that mode of inheritance is autosomal recessive. According to the combination of clinical findings, this case report may represent a previously unreported syndrome.
机译：牙龈纤维瘤病，身材矮小，边界智商，面部畸形和肝肿大：牙龈纤维瘤病是一种罕见的良性疾病。增大的牙龈变硬，可能会干扰言语，嘴唇闭合和咀嚼。我们报告了一个十三岁的女孩，患有牙龈纤维瘤病，被转到牙周病诊所。进行了全口龈切除和牙龈成形术。医学调查显示身材矮小，低边界智商，面部畸形和肝肿大。组织学分析显示上皮区增生和具有密集胶原纤维簇的牙龈结缔组织的纤维化外观。家谱分析证实遗传方式是常染色体隐性遗传。根据临床发现的组合，该病例报告可能代表以前未报告的综合征。

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