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Hypertrophic cardiomyopathy in mucopolysaccharidoses: Regression after bone marrow transplantation
Authors:Xavier Viñallonga  Nuria Sanz  Alberto Balaguer  Luis Miro  Juan J. Ortega  Dr. Jaume Casaldaliga
Affiliation:(1) Unidad de Cardiologia Pediátrica, Universidad Autónoma de Barcelona, Barcelona, Spain;(2) Unidad de Hematologia Pediátrica, Universidad Autónoma de Barcelona, Barcelona, Spain;(3) Servicio de Pediatria, Hospital Materno-Infantil Valle Hebrón, Barcelona, Spain;(4) Sección de Cirugía, Cardiovascular Pediatrica, Hospital Infantil Valle Hebrón, Barcelona, Spain;(5) Unidad de Cardiología, Hospital Infantil Valle Hebrón, Paseo Valle Hebrón s/n, Tln 4272000, 08035 Barcelona, Spain
Abstract:
Summary Mucopolysaccharide storage disease (MPS) presents clinically with a broad spectrum of abnormalities, among which cardiovascular involvement has been described.The echocardiographic findings have recently been reported for the various types of MPS. Among these, asymmetric septal hypertrophy (ASH) has been documented.We present a case of a 9-year-old girl suffering from type I MPS, atypical variant, with echocardiographic signs of ASH. She was given a bone marrow transplant after which the hypertrophic cardiomyopathy regressed.
Keywords:Mucopolysaccharidoses  Hypertrophic cardiomyopathy  Bone marrow transplantation
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