Rapidly Progressive Type III Gaucher Disease: Deterioration Following Partial Splenectomy
作者:
M. KYLLERMAN,
N. CONRADI,
J. ‐E. MÅNSSON,
A. K. PERCY,
L. SVENNERHOLM,
期刊:
Acta Pædiatrica
(WILEY Available online 1990)
卷期:
Volume 79,
issue 4
页码: 448-453
ISSN:0803-5253
年代: 1990
DOI:10.1111/j.1651-2227.1990.tb11492.x
出版商: Blackwell Publishing Ltd
关键词: type III Gaucher disease;partial splenectomy;glucosylceramide
数据来源: WILEY
摘要:
ABSTRACT.Total splenectomy has been found to accelerate disease progression in Type III Gaucher disease, therefore partial splenic resection was performed in a 28 month old girl with rapidly progressive (non‐Norrbottnian) Type III disease to alleviate the effects of hypersplenism. Surgery failed to arrest the disease process and splenomegaly recurred within three months. The erythrocyte glucosylceramide level increased post‐operatively. Post‐mortem studies revealed dense accumulation of Gaucher cells in spleen, liver, lungs, bone marrow and within the central nervous system. The glucosylceramide content of brain and liver was elevated to the range previously noted in splenectomised Norrbottnian patients. Thus, in rapidly progressive Type III Gaucher disease, neither total nor partial splenectomy alone provides a favourable ou
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