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MicroNAPS分类法在罗宾序列中的应用。

Application of the MicroNAPS Classification for Robin Sequence.

作者信息

Resnick Cory M, Katz Eliot, Varidel Alistair

机构信息

Associate Professor of Oral and Maxillofacial Surgery, Harvard Medical School, Boston, MA, USA.

Department of Plastic and Oral Surgery, Boston Children's Hospital, Boston, MA, USA.

出版信息

Cleft Palate Craniofac J. 2025 Apr;62(4):710-714. doi: 10.1177/10556656241229892. Epub 2024 Feb 1.

Abstract

ObjectiveThe only findings consistent among infants with Robin sequence (RS) are the presence of micrognathia, glossoptosis, and upper airway obstruction (UAO). Feeding and growth dysfunction are typical. The etiopathogenesis of these findings, however, is highly variable, ranging from sporadic to syndromic causes, with widely disparate levels of severity. This heterogeneity has created inconsistency within RS literature and debate about appropriate workup and treatment. Despite several attempts at stratification, no system has been broadly adopted.DesignWe recently presented a novel classification that is summarized by the acronym . Each of 5 elements is scored: gnathia, utrition, irway, alate, yndrome/comorbidities, and element scores are summarized into a "stage".ResultsTesting of this system in a sample of 100 infants from our center found it to be clinically relevant and to predict important management decisions and outcomes.ConclusionsWe herein present an interactive website (www.prscalculator.com) and printable reference card for simple application of MicroNAPS, and we advocate for this classification system to be adopted for clinical care and research.

摘要

目的

患有罗宾序列征(RS)的婴儿中唯一一致的发现是小颌畸形、舌后坠和上呼吸道梗阻(UAO)。喂养和生长功能障碍很典型。然而,这些发现的病因发病机制高度可变,从散发性到综合征性病因不等,严重程度差异很大。这种异质性导致了RS文献中的不一致以及关于适当检查和治疗的争论。尽管有几次分层尝试,但没有一个系统被广泛采用。

设计

我们最近提出了一种新颖的分类方法,用首字母缩写来概括。对5个要素中的每一个进行评分:颌骨、营养、气道、腭部、综合征/合并症,要素评分汇总为一个“阶段”。

结果

在我们中心的100名婴儿样本中对该系统进行测试,发现它具有临床相关性,并能预测重要的管理决策和结果。

结论

我们在此提供一个交互式网站(www.prscalculator.com)和可打印的参考卡片,以便于简单应用微型罗宾序列征评估系统(MicroNAPS),并且我们提倡采用这种分类系统用于临床护理和研究。

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