Characterization of tracheobronchomalacia in infants with hypophosphatasia.

ABSTRACT:

Background

Perinatal and infantile hypophosphatasia (HPP) are associated with respiratory failure and respiratory complications. Effective management of such complications is of key clinical importance. In some infants with HPP, severe tracheobronchomalacia (TBM) contributes to respiratory difficulties. The objective of this study is to characterize the clinical features, investigations and management in these patients.

Methods

We report a case series of five infants with perinatal HPP, with confirmed TBM, who were treated with asfotase alfa and observed for 3-7 years. Additionally, we reviewed respiratory function data in a subgroup of patients with perinatal and infantile HPP included in the clinical trials of asfotase alfa, who required high-pressure respiratory support (

SUBMITTER: Padidela R 

PROVIDER: S-EPMC7407429 | biostudies-literature | 2020 Aug

REPOSITORIES: biostudies-literature