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Heart transplantation in two adolescents with Danon disease.
Oren, Daniel; Chau, Peter; Manning, Melanie; Kwong, Joann; Kaufman, Beth D; Maeda, Katsuhide; Rosenthal, David N; Hollander, Seth A.
Afiliação
  • Oren D; Heart and Vascular Center, Semmelweis University, Budapest, Hungary.
  • Chau P; Department of Pediatrics (Cardiology), Loma Linda Medical Center, Loma Linda, California.
  • Manning M; Department of Pediatrics (Medical Genetics) and Pathology, Stanford University Medical Center, Stanford University, Stanford, California.
  • Kwong J; Rehabilitation Services, Lucile Packard Children's Hospital, Stanford, California.
  • Kaufman BD; Department of Pediatrics (Cardiology), Stanford University Medical Center, Stanford University, Stanford, California.
  • Maeda K; Department of Cardiothoracic Surgery, Stanford University Medical Center, Stanford, California.
  • Rosenthal DN; Department of Pediatrics (Cardiology), Stanford University Medical Center, Stanford University, Stanford, California.
  • Hollander SA; Department of Pediatrics (Cardiology), Stanford University Medical Center, Stanford University, Stanford, California.
Pediatr Transplant ; 23(2): e13335, 2019 03.
Article em En | MEDLINE | ID: mdl-30536852
ABSTRACT
Danon disease (DD) is an X-linked dominant disorder caused by a mutation in the lysosomal-associated membrane protein-2 (LAMP-2) gene coding for the LAMP-2 protein. We report two cases of successful heart transplantation (HT) in adolescent brothers with DD, including one who was bridged to HT for 34 days with a HeartWare left ventricular assist device. In both patients, the post-transplant course was complicated by profound skeletal muscle weakness that resolved with corticosteroid withdrawal. These cases highlight that both HT and ventricular assist device support are feasible in patients with DD. Corticosteroid use may exacerbate skeletal myopathy, and therefore, steroid minimization may be warranted whenever possible.
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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Transplante de Coração / Doença de Depósito de Glicogênio Tipo IIb Idioma: En Ano de publicação: 2019 Tipo de documento: Article País de afiliação: Hungria
Texto completo
Imprimir
XML
PubMed Links
Buscar no Google

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Transplante de Coração / Doença de Depósito de Glicogênio Tipo IIb Idioma: En Ano de publicação: 2019 Tipo de documento: Article País de afiliação: Hungria