Department of Pediatrics, Nemours, Alfred I. DuPont Hospital for Children, Wilmington, DE, United States; Center for Pediatric Lung Research, Nemours, Alfred I. DuPont Hospital for Children, Wilmington, DE, United States; Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, United States.
Center for Pediatric Lung Research, Nemours, Alfred I. DuPont Hospital for Children, Wilmington, DE, United States; Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, United States; Temple University School of Medicine, Philadelphia, PA, United States.
Respir Med. 2017 Oct;131:18-26. doi: 10.1016/j.rmed.2017.07.063. Epub 2017 Aug 1.
Skeletal dysplasia encompasses a variety of developmental disorders of the bone and cartilage that manifest as disproportionate shortening of limbs and trunk in the neonate. Many types of skeletal dysplasia are complicated by respiratory failure at or soon after birth and require intensive care and prolonged hospitalization. Respiratory complications in these infants are complex and are characterized by airway anomalies, restrictive lung disease due to a narrow and abnormally compliant chest wall, pulmonary hypoplasia, and central apnea. Appropriate management of these unique patients requires a clear understanding of the pathophysiology and use of pulmonary function tests for early recognition and management of complications.
This review provides an overview of the underlying respiratory pathology and a practical guide to the newborn care provider for the diagnosis and management of respiratory complications in infants with skeletal dysplasia.
骨骼发育不良包括多种骨骼和软骨的发育障碍，表现为新生儿四肢和躯干不成比例地缩短。许多类型的骨骼发育不良在出生时或出生后不久会并发呼吸衰竭，需要重症监护和长时间住院治疗。这些婴儿的呼吸并发症较为复杂，其特点是气道异常、由于胸壁狭窄和异常顺应性导致的限制性肺疾病、肺发育不全和中枢性呼吸暂停。要妥善处理这些特殊患者，就需要清楚了解病理生理学，并使用肺功能检查来早期识别和处理并发症。
本综述概述了骨骼发育不良患儿的基础呼吸病理学，并为新生儿护理人员提供了诊断和处理骨骼发育不良患儿呼吸并发症的实用指南。
