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Any disease marked by a defective metabolism of sphingolipids. These genetically determined errors of metabolism include Sandhoffs disease, Fabry’s disease, Tay-Sachs disease, Kufs’ disease, Gaucher’s disease, Krabbe’s leukodystrophy, Niemann-Pick disease, Batten disease, and Spielmeyer-Vogt disease. They are marked by neurological deterioration, usually beginning a few months after birth and eventually leading to death except in…

A lipid containing one of several long-chain bases such as sphingosine or dihydrosphingosine or bases of similar chemical structure but containing longer chains.  

A genus of gram-negative bacilli formerly identified as Flavobacterium. It is commonly present in soil and may occasionally cause skin infections and sepsis.  

A surgical instrument for cutting a sphincter.  

Inspection of the internal anal sphincter. The internal anal sphincter is covered by anodermal and mucous membrane tissues that cannot be seen unless exposed surgically.  

An instrument for inspection of the anal sphincter.  

Freeing of the iris from the cornea in anterior synechia affecting only the pupillary border.  

A spasm of the sphincter ani muscles.  

The smooth muscle encircling the pancreatic duct just before it joins the ampulla.