Transplantation of amniotic epithelial membranes in patients with mucopolysaccharidoses.
This paper reports the biochemical results of transplanting human amniotic epithelial cells in 3 children with Hunter's and 2 with Hurler's disease. A transient and modest increase of alpha-L-idurono-2-sulphate sulphatase or alpha-iduronidase was observed in the white cells collected from 1 patient with Hunter's and 1 with Hurler's disease. No variation in the excretion of glycosaminoglycans or oligosaccharides was detected in all 5 patients. There was no evidence of immune response towards the transplanted cells or the specifically deficient enzyme. Thus, in spite of the absence of the major histocompatibility antigens, HLA A, B, C and DR, on the surface of the amniotic epithelial cells, no long-term correction of lysosomal enzyme deficiencies was achieved by transplanting amniotic epithelial membranes collected at the end of the gestational period.
D000650:Amnion / Q000201:enzymology / Q000637:transplantation*; D002648:Child; D002675:Child, Preschool; D004848:Epithelium / Q000201:enzymology / Q000637:transplantation; D005260:Female; D006085:Graft Survival; D006801:Humans; D007066:Iduronate Sulfatase / Q000097:blood; D007068:Iduronidase / Q000097:blood / Q000172:deficiency; D007223:Infant; D007962:Leukocytes / Q000201:enzymology; D008059:Mucopolysaccharidosis I / Q000628:therapy*; D016532:Mucopolysaccharidosis II / Q000628:therapy*; D011247:Pregnancy
D007066:Iduronate Sulfatase; D007068:Iduronidase