Progression of neurovisceral storage disease with supranuclear ophthalmoplegia following orthotopic liver transplantation

A 7-year-old girl with progressive ataxia, spasticity, supranuclear ophthalmoplegia, and sea-blue histiocytes in her bone marrow underwent orthotopic liver transplantation for hepatocellular carcinoma. After an initial period of stabilization, she has shown progression of neurologic symptoms with re...

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Veröffentlicht in:	Pediatrics (Evanston) 1986, Vol.77 (1), p.104-106
Hauptverfasser:	GARTNER, J. C. JR, BERGMAN, I, MALATACK, J. J, ZITELLI, B. J, JAFFE, R, WATKINS, J. B, SHAW, B. W, IWATSUKI, S, STARZL, T. E
Format:	Artikel
Sprache:	eng
Schlagworte:	Biological and medical sciences Carcinoma, Hepatocellular - complications Carcinoma, Hepatocellular - surgery Child Errors of metabolism Female Humans Lipidoses - complications Lipids (lysosomal enzyme disorders, storage diseases) Liver Neoplasms - complications Liver Neoplasms - surgery Liver Transplantation Medical sciences Metabolic diseases Ophthalmoplegia - complications Recurrence
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Zusammenfassung:	A 7-year-old girl with progressive ataxia, spasticity, supranuclear ophthalmoplegia, and sea-blue histiocytes in her bone marrow underwent orthotopic liver transplantation for hepatocellular carcinoma. After an initial period of stabilization, she has shown progression of neurologic symptoms with recurrence of storage material in the transplanted liver.
ISSN:	0031-4005 1098-4275
DOI:	10.1542/peds.77.1.104