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Disease Information
History of Gaucher Disease
In 1882, Phillipe Gaucher, a French
physician, first described the clinical disorder that now bears his name.
His astute medical thesis describing a 32-year-old woman with a remarkably
enlarged spleen ("splenomegaly") and the hallmark large, peculiar
cells present within it stimulated interest in the medical community.
The eponym "Gaucher Disease" was introduced by Nathan Brill,
the Mount Sinai pathologist who first suggested the familial nature of
the disorder and was the first to diagnose a case during life. Along with
his Mount Sinai colleagues, Drs. Mandelbaum and Libman, Nathan Brill extensively
described the anatomic and morphologic pathology of the disease. Throughout
the century following its discovery, the scientific and medical communities
have continued to advance our understanding of the clinical and pathologic
findings, metabolic disorder, familial transmission, and genetic defect
of the disease. Based on these studies, diagnosis and treatment of Gaucher
disease are possible and represent a cornerstone in the era of molecular
medicine.
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