Mucolipidosis type IV is a genetic lysosomal storage disease associated with degenerative processes in the brain, eye, and other tissues. Mucolipidosis type IV results from mutations in the gene MCOLN1, which codes for the TRP family ion channel, mucolipin 1. The connection between lysosomal dysfunction and degenerative processes in mucolipidosis type IV is unclear. Here we report that mucolipidosis type IV and several unrelated lysosomal storage diseases are associated with significant mitochondrial fragmentation and decreased mitochondrial Ca

Forty-two comminuted intraarticular fractures of the humeral condyles treated between 1975 and 1981 were analyzed in order to compare the results of operative versus nonoperative management. Twenty-nine patients were treated by open reduction and internal fixation; the remainder were treated by nonoperative techniques. Functional results were evaluated by the method of Bickel and Perry. Of the patients treated by open reduction and internal fixation, 76% had an excellent or good result. Of the nonsurgically-treated patients, only eight percent had a satisfactory result. Anatomic restoration by open reduction and rigid internal fixation in conjunction with early motion was associated with satisfactory results. The preferred surgical exposure was a posterior "U" incision with extraarticular olecranon osteotomy. This provided good visualization and facilitated anatomic restoration in these complex fractures.Comminuted intraarticular fractures of the distal humerus have received relatively little

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