Syringomyelia in mucopolysaccharidosis type VI (Maroteaux-Lamy syndrome): imaging findings following bone marrow transplantation

1997 ◽  
Vol 27 (9) ◽  
pp. 736-738 ◽  
Author(s):  
S. H. Hite ◽  
William Krivit ◽  
Stephen J. Haines ◽  
Chester B. Whitley
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Transplantation ◽  
Marrow Transplantation ◽  
Mucopolysaccharidosis Type ◽  
Imaging Findings ◽  
Mucopolysaccharidosis Type Vi

Bone-Marrow Transplantation in the Maroteaux–Lamy Syndrome (Mucopolysaccharidosis Type VI)

1984 ◽  
Vol 311 (25) ◽  
pp. 1606-1611 ◽  
Author(s):  
William Krivit ◽  
Mary Ella Pierpont ◽  
Kathleen Ayaz ◽  
Michael Tsai ◽  
Norma K.C. Ramsay ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Transplantation ◽  
Marrow Transplantation ◽  
Mucopolysaccharidosis Type ◽  
Mucopolysaccharidosis Type Vi

BONE MARROW TRANSPLANTATION IN NEWBORN RATS WITH MUCOPOLYSACCHARIDOSIS TYPE VI

1997 ◽  
Vol 63 (10) ◽  
pp. 1386-1393 ◽  
Author(s):  
Calogera M. Simonaro ◽  
Mark E. Haskins ◽  
Tetsuo Kunieda ◽  
Sydney M. Evans ◽  
Jan W.M. Visser ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Transplantation ◽  
Marrow Transplantation ◽  
Mucopolysaccharidosis Type ◽  
Newborn Rats ◽  
Mucopolysaccharidosis Type Vi

scholarly journals Increased life span and correction of metabolic defects in murine mucopolysaccharidosis type VII after syngeneic bone marrow transplantation

Blood ◽  
1991 ◽  
Vol 78 (11) ◽  
pp. 3081-3092 ◽  
Author(s):  
EH Birkenmeier ◽  
JE Barker ◽  
CA Vogler ◽  
JW Kyle ◽  
WS Sly ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Transplantation ◽  
Life Span ◽  
Marrow Transplantation ◽  
Mesangial Cells ◽  
Radiation Doses ◽  
Mucopolysaccharidosis Type ◽  
Glomerular Mesangial Cells ◽  
Glucuronidase Activity ◽  
Mps Vii

Abstract The gusmps/gusmps mouse has no beta-glucuronidase activity and develops murine mucopolysaccharidosis type VII (MPS VII). The clinical and pathologic abnormalities are similar to those found in humans with severe MPS VII. Mutant mice are dysmorphic, dwarfed, and have a shortened life span. Pathologic findings include widespread lysosomal storage. To determine whether bone marrow transplantation (BMT) corrects these abnormalities, genetically identical mutant animals were given syngeneic bone marrow transplants using cells from +/+ mice. Initial experiments showed that levels of beta-glucuronidase activity in recipient tissues correlated with the amount of radiation administered before BMT. Two groups of mice given BMT therapy were observed for periods of 1 and 2 years, respectively. These mice were evaluated using a combination of clinical, biochemical, histochemical, and pathologic analyses. Spleen, liver, cornea, and glomerular mesangial cells showed essentially complete correction at all radiation doses. Storage was partially corrected in meninges and perivascular cells in brain, and in renal tubular epithelial cells at the higher radiation doses. Life span in BMT-treated animals was increased approximately three-fold, approaching that seen in normal mice after BMT. These results support the position that BMT has a place in the therapeutic regimen for MPS VII.

scholarly journals Bone marrow transplantation for mucopolysaccharidosis type I: experience of two British centres

1997 ◽  
Vol 76 (2) ◽  
pp. 92-99 ◽  
Author(s):  
A Vellodi ◽  
E P Young ◽  
A Cooper ◽  
J E Wraith ◽  
B Winchester ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Transplantation ◽  
Marrow Transplantation ◽  
Type I ◽  
Mucopolysaccharidosis Type ◽  
Mucopolysaccharidosis Type I
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