• Medicine
  • Published in
    La Pediatria medica e…
    1994

[Heart involvement in progressive spinal muscular atrophy. A review of the literature and case histories in childhood].

@article{Distefano1994HeartII,
  title={[Heart involvement in progressive spinal muscular atrophy. A review of the literature and case histories in childhood].},
  author={Giuseppe Distefano and P Sciacca and M. Gabriela Parisi and Enrico Parano and Pierluigi Smilari and M Marletta and Agata Fiumara},
  journal={La Pediatria medica e chirurgica : Medical and surgical pediatrics},
  year={1994},
  volume={16 2},
  pages={
          125-8
        }
}
There are few cardiological studies in progressive spinal muscular atrophy and mainly concern subjects affected by the juvenile form (Kugelberg-Welander disease). The presence of a cardiomyopathy has been reported in these patients but the cardiac involvement is often secondary to the chronic respiratory insufficiency typical of the disease. We performed a retrospective study in our Institute on 43 patients, age range 3 months to 3 years, 37 of which presented type I (Werdnig-Hoffmann disease… CONTINUE READING