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
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container_end_page 106
container_issue 1
container_start_page 104
container_title Pediatrics (Evanston)
container_volume 77
creator 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
description 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.
doi_str_mv 10.1542/peds.77.1.104
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subjects 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
title Progression of neurovisceral storage disease with supranuclear ophthalmoplegia following orthotopic liver transplantation
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