RESPIRATORY COMPLICATIONS IN CHILDREN WITH SPONDYLOEPIPHYSEAL DYSPLASIA-CONGENITA

RESPIRATORY COMPLICATIONS IN CHILDREN WITH SPONDYLOEPIPHYSEAL DYSPLASIA-CONGENITA
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DOI:
10.1002/ppul.1950090112
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发表时间:
1990-01-01
影响因子:
3.1
通讯作者:
PAULI, RM
PAULI, RM
中科院分区:
医学3区
文献类型:
--
作者:
HARDING, CO;GREEN, CG;PAULI, RM

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出生时轻度呼吸窘迫常见于先天性脊柱骨骺发育不良(SEDC)婴儿,而严重呼吸功能不全和死亡仅罕见报道。我们描述了三名患有SEDC的婴儿,他们经历了严重的呼吸并发症并需要气管切开术。其中两名婴儿还需要长期持续气道正压通气来维持足够的通气。本文描述了这些儿童的特点和临床过程,总结了以往关于SEDC呼吸并发症的报道,假设了SEDC导致呼吸问题的机制,并与其他骨骼发育不良引起呼吸风险的机制进行了比较。儿科肺科杂志1990;9:49-54。
Mild respiratory distress at birth is a common occurrence in infants with spondyloepiphyseal dysplasia congenita (SEDC) while severe repiratory insufficency and death have been reported only rarely. We describe three infants with SEDC who experienced severe respiratory complications and required tracheostomies. Two of these infants have also needed long‐term continuous positive airway pressure to maintain adequate ventilation. The features and clinical course of these children are described, previous reports of respiratory complications in SEDC are summarized, mechanisms resulting in respiratory problems in SEDC are postulated, and comparison is made with mechanisms giving rise to respiratory risks in other skeletal dysplasias.Pediatr Pulmonol 1990; 9:49–54.