About: Characterization of tracheobronchomalacia in infants with hypophosphatasia

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About: Characterization of tracheobronchomalacia in infants with hypophosphatasia Goto Sponge NotDistinct Permalink

An Entity of Type : schema:ScholarlyArticle, within Data Space : wasabi.inria.fr associated with source document(s)

Attributes	Values
type	Academic Article research paper schema:ScholarlyArticle
isDefinedBy	Covid-on-the-Web dataset
title	Characterization of tracheobronchomalacia in infants with hypophosphatasia
Creator	Chan, Elaine Benscoter, Dan Mcphail, Gary Myer Iv, Charles Narayan, Omendra Nichani, Jaya Nissenbaum, Claire Padidela, Raja Saal, Howard Wilkinson, Stuart Yates, Robert Zhou, Shanggen Zulf Mughal, M
source	PMC
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 (positive end-expiratory pressure [PEEP] ≥6 cm H(2)O and/or peak inspiratory pressure ≥18 cm H(2)O) during the studies. RESULTS: The case series showed that TBM contributed significantly to respiratory morbidity, and prolonged respiratory support with high PEEP was required. However, TBM improved over time, allowing weaning of all patients from ventilator use. The review of clinical trial data included 20 patients and found a high degree of heterogeneity in PEEP requirements across the cohort; median PEEP was 8 cm H(2)O at any time and some patients presented with high PEEP (≥8 cm H(2)O) over periods of more than 6 months. CONCLUSION: In infants with HPP presenting with persistent respiratory complications, it is important to screen for TBM and initiate appropriate respiratory support and treatment with asfotase alfa at an early stage. TRIAL REGISTRATION: ClinicalTrials.gov numbers: NCT00744042, registered 27 August 2008; NCT01205152, registered 17 September 2010; NCT01176266, registered 29 July 2010.
has issue date	2020-08-06(xsd:dateTime)
bibo:doi	10.1186/s13023-020-01483-9
has license	cc-by
sha1sum (hex)	c937b1ef6e9949487c8b94ea7d9e9e3d90ce5ab9
schema:url	https://doi.org/10.1186/s13023-020-01483-9
resource representing a document's title	Characterization of tracheobronchomalacia in infants with hypophosphatasia
has PubMed Central identifier	PMC7407429
schema:publication	Orphanet J Rare Dis
resource representing a document's body	covid:c937b1ef6e9949487c8b94ea7d9e9e3d90ce5ab9#body_text
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