Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018133 (graft-versus-host disease)
18,032 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two girls had delayed umbilical cord detachment, recurrent bacterial infection, inability to form pus, and marked leucocytosis. Their phagocytes were defective in tests of adherence, random migration, chemotaxis, and oxidative burst. NK activity was virtually absent. This rare disorder, due to an inherited absence of a 180 kilodalton membrane glycoprotein on polymorphonuclear cells, is usually lethal within 2 years. Allogeneic HLA-matched bone-marrow transplantation done at ages 4 months in one patient and 2 years in the other after intensive conditioning was successful and resulted in nearly complete correction of phagocytic cell function and NK activity within two months. One patient died 9 months after transplantation from severe chronic graft-versus-host disease with obstructive bronchopneumopathy. The other is doing well 1 year after transplantation and showing stable chimerism and normal phagocytic function.
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PMID:Bone-marrow transplantation for inborn error of phagocytic cells associated with defective adherence, chemotaxis, and oxidative response during opsonised particle phagocytosis. 613 43

The HLA-DR molecule is a polymorphic membrane glycoprotein and one of the key molecules causing allograft rejection and graft-versus-host disease in organ transplantation. Serologic typing using DR specific alloantisera has long been used, but several problems have limited its application. The purpose of this study was to establish an efficient reverse-SSO typing system that detects DRB1 and DRB3/B4/B5 alleles on a single membrane. A DR typing membrane was prepared by immobilizing 21 dT-tailed sequence specific oligonucleotides (SSOs) on a nylon membrane and was used in a hybridization assay with digoxigenin-labeled PCR-amplified target DNA. The positive signals were detected on X-ray film using chemiluminescence. A comparison study with serology using DNAs from 105 unrelated individuals demonstrated that the reverse-SSO typing system was superior to serologic typing in terms of accuracy (100% vs 90.5%), simplicity, range of application, rapidity, and cost of the test. These data indicate that the reverse-SSO typing system can replace serology as a routine DR test, and will be useful in time-restricted solid organ transplantation and in selection of an unrelated marrow donor prior to bone marrow transplantation.
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PMID:Reverse-SSO hybridization provides an accurate and simple HLA-DR typing--a comparative study with HLA-DR serologic typing. 770 91