Literature DB >> 2883444

Beneficial effect of pre-transplant splenectomy on displacement bone marrow transplantation for Gaucher's syndrome.

J R Hobbs, K H Jones, P J Shaw, I Lindsay, M Hancock.   

Abstract

2 patients with fast and 4 with medium varieties of Gaucher's syndrome were treated by displacement bone marrow transplantation to install a donor enzyme factory for life. Immunoprophylaxis was given to prevent host impairment of normal enzymes. 5 patients showed rapid and remarkable improvement and were leading fully active lives at 310-1207 days postgraft. Hypersplenism caused the death of 1 patient and a stormy time for another. Pre-transplant elective splenectomy hastened recovery.

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Year:  1987        PMID： 2883444     DOI： 10.1016/s0140-6736(87)91673-4

Source DB:  PubMed          Journal:  Lancet        ISSN： 0140-6736            Impact factor:   79.321


  16 in total

Review 1.  Remaining problems in the management of patients with Gaucher disease.

Authors:  A Erikson
Journal:  J Inherit Metab Dis       Date:  2001       Impact factor: 4.982

2.  Long-term in vivo expression of the human glucocerebrosidase gene in nonhuman primates after CD34+ hematopoietic cell transduction with cell-free retroviral vector preparations.

Authors:  L C Xu; S Karlsson; E R Byrne; S Kluepfel-Stahl; S W Kessler; B A Agricola; S Sellers; M Kirby; C E Dunbar; R O Brady
Journal:  Proc Natl Acad Sci U S A       Date:  1995-05-09       Impact factor: 11.205

Review 3.  Bone marrow transplantation. Part I--Allogeneic.

Authors:  N J Chao; K G Blume
Journal:  West J Med       Date:  1989-12

Review 4.  Alglucerase. A pharmacoeconomic appraisal of its use in the treatment of Gaucher's disease.

Authors:  R Whittington; K L Goa
Journal:  Pharmacoeconomics       Date:  1995-01       Impact factor: 4.981

Review 5.  Gaucher disease as a paradigm of current issues regarding single gene mutations of humans.

Authors:  E Beutler
Journal:  Proc Natl Acad Sci U S A       Date:  1993-06-15       Impact factor: 11.205

Review 6.  Hematopoietic stem cell transplantation for Gaucher disease.

Authors:  Usha R Somaraju; Krishna Tadepalli
Journal:  Cochrane Database Syst Rev       Date:  2017-10-18

Review 7.  Displacement bone marrow transplantation for some inborn errors.

Authors:  J R Hobbs
Journal:  J Inherit Metab Dis       Date:  1990       Impact factor: 4.982

8.  Phenotypic and genotypic heterogeneity in gaucher disease: Implications for genetic counseling.

Authors:  E Sidransky; E I Ginns
Journal:  J Genet Couns       Date:  1994-03       Impact factor: 2.537

9.  Efficient transfer and sustained high expression of the human glucocerebrosidase gene in mice and their functional macrophages following transplantation of bone marrow transduced by a retroviral vector.

Authors:  T Ohashi; S Boggs; P Robbins; A Bahnson; K Patrene; F S Wei; J F Wei; J Li; L Lucht; Y Fei
Journal:  Proc Natl Acad Sci U S A       Date:  1992-12-01       Impact factor: 11.205

10.  Bone marrow transplantation for Sanfilippo disease type B.

Authors:  A Vellodi; E Young; M New; C Pot-Mees; K Hugh-Jones
Journal:  J Inherit Metab Dis       Date:  1992       Impact factor: 4.982
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