@entity8351 ( @entity8351 ) is a @entity2315 characterized by accumulation of oligosaccharides in various tissues leading to symptoms such as coarse facial features, @entity19637 , @entity393 , @entity245 ( @entity19637 ). Without treatment, the severe infantile onset form of this @entity74 leads to progressive @entity179 and sometimes to early @entity204 . Stem cell transplantation has been shown to be an effective treatment. In the five @entity1 published so far, correction of @entity853 and improvement of neuropsychological capabilities have been observed. We report the first @entity1 who received a T-cell-depleted peripheral blood stem cell transplantation (PBSCT) for @entity8351 . The diagnosis of @entity8351 was made at the age of 14 months. At the age of 24 months, he underwent PBSCT with T-cell depletion by @entity2280 -positive selection from his HLA phenotypically identical mother. Conditioning was carried out with @entity1863 (20 mg/kg), @entity786 (200 mg/kg), OKT3 and @entity1667 . The @entity1 is alive and well 27 months after PBSCT and has made significant developmental progress. The pattern of urinary oligosaccharides has returned to almost normal. @entity2280 -positive-selected PBSCT is a feasible option to reduce risk for GVHD for these @entity1 .

T-cell-depleted peripheral blood stem cell transplantation for XXXX .

@entity2315 :: ('MESH:D016464', 'Disease') :: ['lysosomal storage disease'] @entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity1667 :: ('MESH:D008775', 'Chemical') :: ['methylprednisolone'] @entity19637 :: ('MESH:D004413', 'Disease') :: ['dysostosis multiplex'] @entity853 :: ('MESH:D009139', 'Disease') :: ['skeletal abnormalities'] @entity245 :: ('MESH:D002658', 'Disease') :: ['mental developmental delay and skeletal involvement'] @entity786 :: ('MESH:D003520', 'Chemical') :: ['cyclophosphamide'] @entity179 :: ('MESH:D019636', 'Disease') :: ['neurodegeneration'] @entity74 :: ('MESH:D030342', 'Disease') :: ['autosomal recessive disease'] @entity1863 :: ('MESH:D002066', 'Chemical') :: ['busulfan'] @entity2280 :: ('947', 'Gene') :: ['CD34'] @entity393 :: ('MESH:D034381', 'Disease') :: ['hearing disabilities'] @entity204 :: ('MESH:D003643', 'Disease') :: ['death'] @entity8351 :: ('MESH:D008363', 'Disease') :: ['alpha-mannosidosis', 'Alpha-mannosidosis']
@entity8351 :: (MESH:D008363,Disease) :: ['alpha-mannosidosis']
