Respiratory outcomes in children with congenital myotonic dystrophy
PURPOSE: Congenital myotonic dystrophy (CDM) results in hypotonia and acute respiratory distress at birth. Previous studies show that prolonged periods of intubation (>4 weeks) correlate with increased mortality rates. The objective is to describe the use and duration of respiratory support in newborns with CDM and how these relate to mortality.
METHODS: A retrospective chart review was performed at a tertiary pediatric hospital among children with confirmed diagnosis of CDM. The main outcome measures were: mortality, duration of invasive mechanical ventilation (IMV) and non-invasive partial pressure ventilation (NIPPV), along with long-term use of respiratory support and equipment.
RESULTS: A total of 18 subjects met inclusion criteria, 83%.f which had documented respiratory distress at birth, 39%.equired NIPPV, and 50%.equired intubation in the neonatal period. The earliest NIPPV was initiated at day one of life, and the latest extubation to NIPPV was at 17 days of life.
CONCLUSION: This cohort required IMV for shorter periods with earlier transitions to NIPPV which suggests a possible change in practice and earlier transition to NIPPV recently. Further data are needed to determine if there is a possible correlation between the need for NIPPV/IMV and mortality rates.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2022 |
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| Erschienen: |
2022 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:15 |
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| Enthalten in: |
Journal of pediatric rehabilitation medicine - 15(2022), 2 vom: 22., Seite 275-279 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Omura, Jaclyn [VerfasserIn] |
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| Links: |
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| Themen: |
Congenital myotonic dystrophy |
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| Anmerkungen: |
Date Completed 28.06.2022 Date Revised 17.09.2022 published: Print Citation Status MEDLINE |
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| doi: |
10.3233/PRM-200726 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM333439627 |
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| 245 | 1 | 0 | |a Respiratory outcomes in children with congenital myotonic dystrophy |
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| 500 | |a Date Revised 17.09.2022 | ||
| 500 | |a published: Print | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a PURPOSE: Congenital myotonic dystrophy (CDM) results in hypotonia and acute respiratory distress at birth. Previous studies show that prolonged periods of intubation (>4 weeks) correlate with increased mortality rates. The objective is to describe the use and duration of respiratory support in newborns with CDM and how these relate to mortality | ||
| 520 | |a METHODS: A retrospective chart review was performed at a tertiary pediatric hospital among children with confirmed diagnosis of CDM. The main outcome measures were: mortality, duration of invasive mechanical ventilation (IMV) and non-invasive partial pressure ventilation (NIPPV), along with long-term use of respiratory support and equipment | ||
| 520 | |a RESULTS: A total of 18 subjects met inclusion criteria, 83%.f which had documented respiratory distress at birth, 39%.equired NIPPV, and 50%.equired intubation in the neonatal period. The earliest NIPPV was initiated at day one of life, and the latest extubation to NIPPV was at 17 days of life | ||
| 520 | |a CONCLUSION: This cohort required IMV for shorter periods with earlier transitions to NIPPV which suggests a possible change in practice and earlier transition to NIPPV recently. Further data are needed to determine if there is a possible correlation between the need for NIPPV/IMV and mortality rates | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Congenital myotonic dystrophy | |
| 650 | 4 | |a intubation | |
| 650 | 4 | |a respiratory distress | |
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| 700 | 1 | |a Haviland, Miriam |e verfasserin |4 aut | |
| 700 | 1 | |a Apkon, Susan |e verfasserin |4 aut | |
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