@article{c502c96fa05c451aa5d15ef78fb522c4,
title = "Gaucher disease: Fate of the splenic remnant after partial splenectomy-A case of rapid enlargement",
abstract = "In Gaucher disease, partial splenectomy has been suggested for alleviating the complications of splenomegaly as well as for avoiding the immunologic compromise and potential acceleration of bony and hepatic involvement that may follow total splenic resection. However, the fate of the splenic remnant has been reported rarely. A subtotal splenectomy (85\%) was performed in a 19-month-old girl with rapidly progressing Gaucher disease and massive splenomegaly (12\% of body weight). Within 3 months, the splenic remnant had increased four-fold in size. Previous reports indicated only three Gaucher patients had significant enlargement of the splenic remnant after partial splenectomy. These findings indicate that splenomegaly may recur rapidly in Gaucher disease following partial splenectomy.",
keywords = "Gaucher disease, Lysosomal storage disease, partial splenectomy",
author = "Fleshner, \{Phillip R.\} and Astion, \{Donna J.\} and Ludman, \{Mark D.\} and Aufses, \{Arthur H.\} and Grabowski, \{Gregory A.\} and Dolgin, \{Stephen E.\}",
note = "Funding Information: From the Department of Surgery and Division of Medical Genetics, The Mount Sinai Hospital and The Mount Sinai School of Medicine, New York City. Supported by grants from the National Institutes of Health (DK 36729, G.A.G.), the March of Dimes Birth Defects Foundation (1-857, G.A.G.), the National Gaucher Foundation (NGF 19, G.A.G.), the Division of Research Resources General Clinical Research Centers (RR-71), and Florence and Theodore Baumritter to the Mount Sinai Center for Jewish Genetic Diseases. Dr Grabowski is the recipient of an NIH Research Career Development Award (DK 01351), and Dr Ludman is the recipient of a GCRC Clinical Associate Physician Award. Address reprint requests to Stephen E. Dolgin, MD, Department of Surgery, Box 1259, The Mount Sinai Hospital, One Gustave L. Levy PI, New York, NY 10029. 9 1989 by Grune \& Stratton, Inc. 0022-3468/89/2406-0026503.00/0",
year = "1989",
month = jun,
doi = "10.1016/S0022-3468(89)80520-2",
language = "English",
volume = "24",
pages = "610--612",
journal = "Journal of Pediatric Surgery",
issn = "0022-3468",
publisher = "W.B. Saunders",
number = "6",
}