E. Kattner et al., HYDROPS-FETALIS - MANIFESTATION IN LYSOSOMAL STORAGE DISEASES INCLUDING FARBER-DISEASE, European journal of pediatrics, 156(4), 1997, pp. 292-295
The authors describe a case of disseminated lipogranulomatosis (Farber
disease) presenting as nonimmune hydrops fetalis. This is the tenth l
ysosomal storage disease which can show this clinical manifestation. T
he literature is reviewed for all hydrops cases associated with lysoso
mal storage diseases. Conclusion Although rare, the lysosomal storage
diseases collectively are significant causes of nonimmune hydrops and
appropriate investigations are required in all cases of unexplained hy
drops fetalis.