Primary neuraminidase deficiency with prenatal disclosure

Tabardel, Y.; Soyeur, D.; Vivario, E.; Senterre, J.

Archives Francaises de Pediatrie 46(10): 737-740

1989


ISSN/ISBN: 0003-9764
PMID: 2697196
Document Number: 336719
The authors report a case of infantile sialidosis with hydrops fetalis and heart failure. At birth the baby presented a dysmorphic syndrome with histological anomalies. A storage disease with deficiency of neuraminidase activity, sialidosis type II, was confirmed. Amniocentesis with sialic-acid dosage or thin-layed chromatography seems necessary in hydrops fetalis with heart failure of unknown origin.

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