PEDIATRICS Vol. 77 No. 1 January 1986, pp. 104-106
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Progression of Neurovisceral Storage Disease With Supranuclear Ophthalmoplegia Following Orthotopic Liver Transplantation

J. Carlton Gartner Jr MD1, Ira Bergman MD1, J. Jeffrey Malatack MD1, Basil J. Zitelli MD1, Ronald Jaffe MB, BCH1, John B. Watkins MD1, Byers W. Shaw MD1, Shunzaburo Iwatsuki MD1, and Thomas E. Starzl MD, PhD1

1 From the Departments of Pediatrics, Neurology, and Surgery, University of Pittsburgh, Pittsburgh, and the Department of Pediatrics, University of Pennsylvania, Philadelphia

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.

Key Words: lipidosis • sea-blue histiocyte • liver transplantation

Submitted on January 30, 1985
Accepted on April 1, 1985




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