Abstract |
Barth syndrome is an X-linked recessive disorder that is characterized by cardiomyopathy, variable neutropenia, skeletal myopathy, growth delay, and organic aciduria. The cardiac involvement typically results in a high risk of severe heart failure in infancy or early childhood. While Berlin Heart EXCOR is widely accepted as ventricular assistance in pediatric patients with end-stage cardiac failure, infections remain a frequent and potentially severe complication. Therefore, the extended use of the device in the setting of intermittent or severe neutropenia is challenging. We present the case of a three-yr child with Barth syndrome who was bridged successfully to transplant with a Berlin Heart EXCOR assist device for eight months (251 days) without major infectious complication, despite several episodes of severe neutropenia. This case demonstrates that prolonged mechanical circulatory support for a patient with neutropenia is feasible without important morbidity, with careful monitoring and a multidisciplinary approach. G-CSF provides an excellent support in managing neutropenia.
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Authors | Nathalie Dedieu, Alessandro Giardini, Colin G Steward, Matthew Fenton, Ann Karimova, T Y Hsia, Michael Burch |
Journal | Pediatric transplantation
(Pediatr Transplant)
Vol. 17
Issue 2
Pg. E46-9
(Mar 2013)
ISSN: 1399-3046 [Electronic] Denmark |
PMID | 23190323
(Publication Type: Case Reports, Journal Article)
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Copyright | © 2012 John Wiley & Sons A/S. |
Topics |
- Barth Syndrome
(complications, surgery)
- Child, Preschool
- Heart-Assist Devices
- Humans
- Male
- Neutropenia
(etiology)
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