en POLSKI
eISSN: 2300-8660
ISSN: 0031-3939
Pediatria Polska - Polish Journal of Paediatrics
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3/2020
vol. 95
 
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abstract:
Case report

Acute liver failure due to previously unrecognised dilated cardiomyopathy in a 17-year-old boy with Duchenne muscular dystrophy

Kamil Janowski
1
,
Lidia Ziółkowska
2
,
Agnieszka Boruc
2
,
Katarzyna Witulska
3
,
Maria Janowska
4
,
Marek Stefanowicz
4
,
Wioletta Jarmużek
5
,
Joanna Pawłowska
1

1.
Department of Gastroenterology, Hepatology, Nutritional Disorders, and Paediatrics, The Children’s Memorial Health Institute, Warsaw, Poland
2.
Department of Cardiology, The Children’s Memorial Health Institute, Warsaw, Poland
3.
Department of Anaesthesiology, The Children’s Memorial Health Institute, Warsaw, Poland
4.
Department of Paediatric Surgery and Organ Transplantation, The Children’s Memorial Health Institute, Warsaw, Poland
5.
Department of Nephrology, The Children’s Memorial Health Institute, Warsaw, Poland
Pediatr Pol 2020; 95 (3): 178–183
Online publish date: 2020/10/30
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We present a case of fatal liver failure secondary to previously unrecognised dilated cardiomyopathy in a 17-year-old boy with Duchenne muscular dystrophy (DMD). Acute liver failure in the clinical course of decompensated dilated cardiomyopathy in patients with DMD is rare and carries a high mortality rate. Severe congestive heart failure should always be considered as a possible cause of acute liver failure. In our article we summarise current recommendations including diagnostic and therapeutic standards regarding cardiac care in patients with DMD. Regular cardiac evaluation according to recommendations may detect the early stage of dilated cardiomyopathy and allow the implementation of preventive pharmacological treatment. Multispecialist management increases the survival and provides a better quality of life for children with DMD.
keywords:

dilated cardiomyopathy, muscular dystrophy, congestive heart failure, liver failure