A @entity1 with the inability to maintain in vivo levels of bound XXXX (Cbl) and manifestations of tissue deficiency of Cbl.

@entity1 @entity2187 @entity417 @entity23906 @entity73 @entity2186 @entity73146 @entity35160
A 39-year-old @entity1 presented with mild @entity417 , @entity35160 , an increased MCV, a low serum cobalamin ( @entity2187 ) ( @entity2186 ), mild tissue deficiency of Cbl, but with neither malabsorption of Cbl, @entity73 of or inactivity of @entity23906 ( @entity23906 ). Because of a persistently low holo- @entity23906 ( @entity23906 carrying Cbl as the circulating complex of @entity23906 -Cbl), much of the evaluation was focused on the @entity1 's @entity23906 . Her @entity23906 promoted the uptake of Cbl, reacted with anti- @entity23906 , and bound Cbl in vitro. A test dose of 200 micrograms of @entity2186 ( @entity73146 ) i.m. increased her holo @entity23906 to levels higher than those in healthy @entity1 , but with a much more abrupt fall to a subnormal level. Two milligrams of CN-Cbl i.m. followed by 100 micrograms i.m. monthly failed to maintain normal amounts of circulating @entity23906 -Cbl or to overcome the tissue deficiency of Cbl. One milligram i.m. weekly or daily p.o. corrected both. The low holo @entity23906 was considered to be responsible for the clinical expression and may have been primary to the reduced amounts of total and holo R binder of Cbl in the circulation. This study of a newly recognized defect points out the need for circulating holo @entity23906 , a rational use of pharmacologic amounts of Cbl, and a possible interrelationship between @entity23906 and the R binder of Cbl.
