[Assessment of quality of life for home ventilated patients with Duchenne muscular dystrophy]. / Indices de satisfaction des patients atteints d'une dystrophie musculaire de Duchenne de Boulogne et ventilés à domicile.

A social and psychological survey was conducted for patients with DMD submitted to home mechanical ventilation for more than one year. Thirty six were tracheostomized and 16 were using non invasive ventilation. Fifty two patients were recruited: 36 were tracheostomized and 16 were using non invasive ventilation. Mean age was 25 +/- 5 years. In the two groups: 1- the main disagreement was air leaking and cutaneous erosions. 2- when present, headache, dyspnea, sleep troubles and general fatigue were improved by mechanical ventilation. 3- ventilation was considered as improving health but with an increasing of dependencies. 4- Majors disappointment are sexual life (70 percent) and physical status (40 percent) but patients spent more than half time with positive feeling (92 percent). There was no difference between satisfaction evaluation and type of ventilation. Home ventilated patients with DMD have positive assessment of satisfaction. Despite technical disagreement as air leaking, patients feel an improvement of their life and advise other DMD to use early home ventilation.

Specific abnormalities in a visual motor psychological test in short children with abnormal growth hormone secretion.

Forty short (< -2 SD) prepubertal children with no organic disease underwent two growth hormone (GH)-stimulation tests, a sleep test and a psychological assessment based on the Rey-Osterrieth figure that was scored for boundary-type errors (defined as non-developmental severe distortions or disrespect of boundaries) by a psychologist who had not met the child. Twenty-three children had normal endocrinological findings and 17 had abnormal GH secretion (4 with complete GH deficiency and 13 with normal GH response to pharmacological stimuli but abnormal nocturnal secretion). Seventeen children had normal results in the Rey-Osterrieth figure test and 23 made boundary-type errors. The biological and psychological analyses agreed in 32 cases, leading to a very significant (p < 0.0005) relation. This study reveals that short children with abnormal GH secretion, particularly low nocturnal secretion, show abnormalities independent of their developmental quotient in a visual motor psychological test.

Rorschach changes following brief and short-term therapy.

Two groups of 35 patients each, one treated with brief therapy, averaging 14.2 sessions on a once per week basis, and the other group, treated with a short-term type of therapy, averaging 47 sessions on a once per week basis were administered the Rorschach and the Katz Adjustment Scales-Form S three times. The first administration took place before entering therapy. The brief therapy group was tested a second time at termination of treatment and again 8 to 12 months following termination. The short-term therapy group was tested the second time at therapy termination and the third time 10 to 12 months after termination. Each group reported substantially fewer symptoms at termination and continued to report about the same level of symptom reduction at the second retest. The Rorschach data seem to provide an ample basis from which to argue that considerable improvement occurred in the psychological organization and/or operations of both groups, and this appears to have some confirmation in the self-report data.