Bone marrow transplantation (BMT) was performed in 10 patients with Hunter disease (mucopolysaccharidosis type II, iduronate-2-sulphatase deficiency). The donor was an HLA-identical sibling in 2 cases, an HLA-nonidentical relative in 6 cases, a volunteer unrelated donor in 1 case, and details were not available in 1 case. Only three patients have survived for more than 7 years post BMT; however, this high mortality probably resulted from poor donor selection. In two, there has been a steady progression of physical disability and mental handicap. One patient has maintained normal intellectual development, with only mild physical disability. It is possible that BMT may be useful in selected patients with MPS II.
We describe the results of bone marrow transplantation (BMT) in four patients with mucopolysaccharidosis type VI (MPS VI, McKusick 253200)--Maroteaux-Lamy disease. The indications for transplantation were cardiomyopathy in three patients and severe obstructive sleep apnoea in one. The follow-up period ranges between 1 and 9 years, and three of the patients are at mainstream schools. In all of the patients the facial features have become less coarse and the cardiac manifestations have improved or remained stable. However, skeletal changes have persisted or even progressed, although posture and joint mobility have improved and all the patients have remained ambulatory and active. BMT appears to prolong survival and improve the quality of life in MPS VI patients, but careful selection of patients is essential.
This is a reflection on cross-cultural therapy from the perspective of a practitioner and trainer who is an outsider to the culture in which she is working. The context is work in Bolivia. The therapist's own position, the process of interaction and the influences on the family of its context are considered. Cultural notes are provided to highlight what an outsider may represent, and the legitimacy of his involvement and intervention in family situations. Themes frequently encountered in therapy are reviewed including jealousy, violence and the changing societal relationship between men and women. The usefulness of getting to know the cultural background of such themes, including the possibility of exploring them anew in therapy, is examined. Finally, a case study is presented related to the themes which also illustrates the adaptation of process to fit patient's culturally related expectations.
Perspectives in cross-cultural therapyWhen I first came to Bolivia I thought it would be an opportunity to put into practice the ideas I had read about in the literature on cross-cultural systemic practice.Systemic therapy was well into second-order cybernetics when I began my training. At the time, second-order practice, also referred to as postmodern, was described as being less hierarchical, less instrumental and non-pejorative (Pocock, 1995;Hayward, 1996). In contrast to modern or first-order practices, the client is respected, and the therapist is seen as part of the therapeutic system, thus stressing his observer-dependent position and undermining the possibility of 'objective' knowledge about families (Mason, 1993). For me, the most significant theme brought to us by the change in paradigm from first to second order or modern to postmodern was
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