Scott John, Adams Chad, Beetstra Stephen, Zarate Yuri A
Arkansas Children's Hospital, Little Rock, Arkansas, USA.
Spec Care Dentist. 2019 Mar;39(2):220-224. doi: 10.1111/scd.12340. Epub 2019 Jan 16.
Identify, diagnose, and document oral clinical and radiographic evidence associated with the genetic condition known as special AT-rich sequence-binding protein 2 (SATB2)-associated syndrome. Through identifying and publishing these common dental and behavioral findings, we hope to educate oral and medical healthcare providers to identify this condition in an attempt to develop meaningful comprehensive care to this patient population.
A total of 37 patients (19 female), ranging from ages 2 to 20 were evaluated at Arkansas Children's Hospital in Little Rock, Arkansas. Patient geographic distribution included: the United States, Canada, Portugal, Spain, and the Netherlands. Patients were clinically and radiographically examined for oral findings. Panoramic radiographs were obtained when patient's behavior allowed. Patient's parents or guardians were also interviewed concerning dental, medical, and behavioral histories.
Clinical findings included delayed tooth eruption, bruxism, sialorrhea, larger than normal teeth with an increased propensity for maxillary anterior tooth trauma due to unsteady ambulation. Radiographic findings included delayed permanent root formation, significantly delayed or missing second bicuspids, malformed teeth, and taurodontism. Medical and behavioral issues included: insomnia, hyperphagia, cognitive delays, and an extremely high pain threshold.
Patients with SATB2-associated syndrome have shown to have a consistent and unique set of dental findings both clinically and radiographically. A thorough health and dental history along with the aforementioned results of the study may facilitate a diagnosis of this syndrome. Due to the complexity of the patient's dental needs and behavior, a health practitioner with special needs care experience on a comprehensive craniofacial team would be optimal.
识别、诊断并记录与特殊富含AT序列结合蛋白2（SATB2）相关综合征这一遗传疾病相关的口腔临床和影像学证据。通过识别并公布这些常见的牙齿和行为表现，我们希望教育口腔和医疗保健提供者识别这种疾病，以便为这一患者群体制定有意义的综合护理方案。
在阿肯色州小石城的阿肯色儿童医院对37例患者（19名女性）进行了评估，年龄范围为2至20岁。患者的地理分布包括：美国、加拿大、葡萄牙、西班牙和荷兰。对患者进行了口腔检查的临床和影像学检查。在患者行为允许时拍摄全景X线片。还就患者的牙齿、医疗和行为病史对其父母或监护人进行了访谈。
临床发现包括牙齿萌出延迟、磨牙症、流涎、牙齿比正常大，由于行走不稳而上颌前牙外伤的倾向增加。影像学发现包括恒牙牙根形成延迟、第二双尖牙显著延迟或缺失、牙齿畸形和牛牙症。医疗和行为问题包括：失眠、食欲亢进、认知延迟和极高的疼痛阈值。
SATB2相关综合征患者在临床和影像学上均表现出一系列一致且独特的牙齿表现。全面的健康和牙齿病史以及上述研究结果可能有助于诊断该综合征。由于患者牙齿需求和行为的复杂性，在综合颅面治疗团队中具有特殊需求护理经验的健康从业者将是最佳选择。
