Latinone

10485893

In humans, mutations in the genes encoding components of the dystrophin-glycoprotein complex cause muscular dystrophy. Specifically, primary mutations in the genes encoding α-, β-, γ-, and δ-sarcoglycan have been identified in humans with limb-girdle muscular dystrophy. Mice lacking γ-sarcoglycan develop progressive muscular dystrophy similar to human muscular dystrophy. Without γ-sarcoglycan, β- and δ-sarcoglycan are unstable at the muscle membrane and α-sarcoglycan is severely reduced. The expression and localization of dystrophin, dystroglycan, and laminin-α2, a mechanical link between the actin cytoskeleton and the extracellular matrix, appears unaffected by the loss of sarcoglycan. We assessed the functional integrity of this mechanical link and found that isolated muscles lacking γ-sarcoglycan showed normal resistance to mechanical strain induced by eccentric muscle contraction. Sarcoglycan-deficient muscles also showed normal peak isometric and tetanic force generation. Furthermore, there was no evidence for contraction-induced injury in mice lacking γ-sarcoglycan that were subjected to an extended, rigorous exercise regimen. These data demonstrate that mechanical weakness and contraction-induced muscle injury are not required for muscle degeneration and the dystrophic process. Thus, a nonmechanical mechanism, perhaps involving some unknown signaling function, likely is responsible for muscular dystrophy where sarcoglycan is deficient.

Muscle degeneration without mechanical injury in sarcoglycan deficiency

A. A. Hack,*† L. Cordier,‡ D. I. Shoturma,‡ M. Y. Lam,† H. L. Sweeney,‡ and E. M. McNally†§

1999 Sep 14;

26768890

Background
Providers of psychological therapies are encouraged to offer patients choice about their treatment, but there is very little information about what preferences people have or the impact that meeting these has on treatment outcomes.

Method
Cross-sectional survey of people receiving psychological treatment from 184 NHS services in England and Wales. 14,587 respondents were asked about treatment preferences and the extent to which these were met by their service. They were also asked to rate the extent to which therapy helped them cope with their difficulties.

Results
Most patients (12,549-86.0 %, 95 % CI: 85.5-86.6) expressed a preference for at least one aspect of their treatment. Of these, 4,600 (36.7 %, 95 % CI: 35.8-37.5) had at least one preference that was not met. While most patients reported that their preference for appointment times, venue and type of treatment were met, only 1,769 (40.5 %) of the 4,253 that had a preference for gender had it met. People who expressed a preference that was not met reported poorer outcomes than those with a preference that was met (Odds Ratios: appointment times = 0.29, venue = 0.32, treatment type = 0.16, therapist gender = 0.32, language in which treatment was delivered = 0.40).

Conclusions
Most patients who took part in this survey had preferences about their treatment. People who reported preferences that were not met were less likely to state that treatment had helped them with their problems. Routinely assessing and meeting patient preferences may improve the outcomes of psychological treatment

Psychological treatment, Choice behaviour, Patient preference, Psychotherapy, Treatment outcome

Patient preference in psychological treatment and associations with self-reported outcome: national cross-sectional survey in England and Wales

Ryan Williams, Lorna Farquharson, Lucy Palmer, Paul Bassett, Jeremy Clarke, David M. Clark, and Mike J. Crawfordcorresponding author

2016