Adherence to treatment, a challenge even in treatable metabolic rare diseases: A cross sectional study of Wilson's disease.

Wilson's disease (WD), a rare genetic disorder responsible for copper accumulation in the body, is fatal if left untreated. Although there are effective treatments, adherence to treatment tends to be low. We evaluated the medication adherence of 139 patients using the Morisky scale. Adherence was correlated with age at diagnosis and at inclusion in the study, the form of the disease, the treatment, the duration of treatment, delivery and storage problems, depression, anxiety, the level of education, and the biological data. 32.4% of the patients had low adherence; their levels of exchangeable copper were significantly higher than those of the patients with high or medium adherence (P = .049). The average age of the patients at the time of the study was significantly higher in those with high adherence than in those with medium or low adherence (P = .043). 75.9% of the patients with high adherence had a neurological form and 26.7% of the patients with low adherence were asymptomatic (P = .0090). The duration of treatment was significantly longer in the patients with high adherence than in those with medium or low adherence (P = .0192). The type of treatment (chelators or zinc) had no impact on the level of adherence. Forty-four percent of the patients experienced problems dispensing and storing medications. Despite the availability of effective treatments for this rare disease, adherence problems occur with Wilson's disease in particular in asymptomatic patients. Although different factors are involved, sustained multidisciplinary management on a case-by-case basis is necessary.

Parricide et violence psychique dans la famille: le rapport d'une mère / Parricide and psychic violence in the family: a mother tells / Parricidio y violencias psíquicas en la familia: una madre le dice

Sur la base du discours d'une mère venue nous consulter, nous reprenons les faits saillants de son récit sur le parricide commis par son fils adolescent sur son ex-mari. À la suite de son discours, nous explorons la psychopathologie du lien parent-enfant pour mettre en évidence la violence psychologique qui aurait pu mener à l'action du fils. Les hypothèses dynamiques qui se dégagent de ce parricide touchent à la complicité mère-fils, au deuil impossible des figures parentales et à la nature incestueuse des liens familiaux.

Based on the discourse of a mother who came to us for consultation, we take up the highlights of her narrative on the parricide committed by her adolescent son on her exhusband. Following her discourse, we explore the psychopathology of the parent-child bond to highlight the psychological violence which could have led to the son's action. The dynamic hypotheses that emerge with regard to this parricide touch on mother-son complicity, the impossible mourning of the parental figures and the incestuous nature of familial ties.

A partir de las declaraciones de una madre recibida en consulta, reanudamos los puntos salientes de este relato sobre el parricida actuado por el hijo adolescente de ella sobre su ex marido. Siguiendo el hilo de su discurso, exploramos la psicopatología de los lazos padres-niños para destacar las violencias psicológicas que pudieron contribuir este paso al acto. Las hipótesis dinámicas que emergen concerniendo a este parricidio tocan la complicidad madre-hijo, el luto imposible de las figuras paternas o todavía el carácter incestuoso de los lazos familiares.