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[胸廓发育不全综合征]

[Thoracic insufficiency syndrome].

作者信息

Ramírez N, Devaris A, Arroyo S, Restrepo R L, Cuneo A, Fernández C A, Marrero-Ortiz P

机构信息

Hospital de la Concepción. San German, Puerto Rico.

Universidad Central del Caribe Escuela de Medicina Bayamon Puerto Rico.

出版信息

Acta Ortop Mex. 2020 Jul-Aug;34(4):254-260.

Abstract

The compendium of disorders that affect the normal growth or function of the thorax will cause Thoracic Insufficiency Syndrome (TIS). TIS is defined as the inability of the chest to sustain normal breathing and/or lung growth. The etiology of the syndrome may be secondary to spinal deformities, global deformities of the chest, neuromuscular dysfunction or the combination of any these. Its manifestation is based on a history that highlights respiratory symptoms, a physical examination that demonstrates chest deformity, abnormal radiographic findings and/or computed tomography of the chest, accompanied by alterations in lung function or other studies of respiratory function. This syndrome must be treated with haste since it progressively worsens with the aggravation of the underlying condition(s) which is unfavorable to the irreversible physiological changes of the lung that occur during development, and are directly related to the respiratory insufficiencies. The vertical expandable prosthetic titanium rib (VEPTR) was developed as a treatment procedure that aims to restore the volume and function of the thorax with the purpose of enabling thoracic growth during the development of the child or adolescent. The treatment targets the components of the rib cage as a unit, in order to prevent or treat respiratory insufficiencies. Its indications include children with early development scoliosis who are prone to develop SIT. The proposed procedure entails a high incidence of complications and conflicting results that limit its efficacy as a treatment, which is why it is a subject of great controversy in the medical literature.

摘要

影响胸廓正常生长或功能的疾病汇总将导致胸廓发育不全综合征(TIS)。TIS的定义为胸部无法维持正常呼吸和/或肺生长。该综合征的病因可能继发于脊柱畸形、胸廓整体畸形、神经肌肉功能障碍或这些情况的任意组合。其表现基于突出呼吸症状的病史、显示胸廓畸形的体格检查、胸部X线异常表现和/或胸部计算机断层扫描,以及伴有肺功能改变或其他呼吸功能检查结果。该综合征必须尽快治疗,因为随着潜在病情加重,它会逐渐恶化,这不利于发育过程中发生的、与呼吸功能不全直接相关的肺不可逆生理变化。垂直可扩展人工钛肋骨(VEPTR)作为一种治疗方法而被研发出来,其目的是恢复胸廓的容积和功能,以便在儿童或青少年发育期间促进胸廓生长。该治疗将胸廓作为一个整体来针对其组成部分,以预防或治疗呼吸功能不全。其适应症包括易患胸廓发育不全综合征的早期发育性脊柱侧弯儿童。所提议的手术并发症发生率高且结果相互矛盾,限制了其作为一种治疗方法的疗效,这就是它在医学文献中备受争议的原因。

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