Les désétayages psychiques dans les agirs adolescents

The approach of the adolescent agirs can get along according to three principal registers: specific degrees of disintegration of the mediation symbolic system, narcissistic engluements and modes of elision of phallic feature meaning. These agirs are frequently in bond with traumatisms. Those carry out a loss of the internal and psychosocial stays. We will try to specify the psychic impact of those by stressing several processes concerned: failures of the mechanisms of identification and affiliation, the prevalence of the relations of influence, embrittlements of the structuring of ego and dead ends of the figuration.     

Cutaneous Manifestations in Costello and Cardiofaciocutaneous Syndrome: Report of 18 Cases and Literature Review

Costello syndrome (CS) and cardiofaciocutaneous syndrome (CFCS) are congenital disorders involving the Ras‐MAPK pathway with phenotypic overlap. These two entities are thought to share common cutaneous findings, although so far they have been poorly studied. The objective of this prospective observational study was to describe the spectrum of skin findings in CS and CFCS and to highlight those specific to each of these two diseases. Patients with a confirmed diagnosis of CFCS or CS underwent a systematic skin examination during the annual workshop organized by the French CS association in 2007 and 2009 in Bordeaux, France. Eighteen patients were included in the study. Specific skin abnormalities, including cutis laxa, curly hair, pruritus, and hyperhidrosis, are shared by CFCS and CS, whereas others may help to differentiate between these two syndromes. Acanthosis nigricans, papillomas, and loose thick skin of the dorsum of the hands are characteristic of CS, whereas sparse eyebrows and dry hyperkeratotic skin are suggestive of CFCS. Our results highlight that a systematic cutaneous examination, in addition to dysmorphologic and noncutaneous anomalies, may be helpful in establishing the diagnosis of CFCS and CS. The physiopathologic link between constitutional Ras‐MAPK pathway activation and the observed ectodermal findings remains to be investigated.     

How far are people with learning disabilities involved in funeral rites?

Many referrals to specialist learning disability services follow bereavement and loss experiences. Research and clinical experience both suggest that people who have not been fully involved in funeral rituals have delayed and/or prolonged grief reactions. This study in a multicultural inner London borough examined local religious and cultural practices following bereavement of people with learning disabilities. Interviews were sought with funeral directors and representatives from six religious groups to build up a picture of local practice, resources and knowledge, particularly with regard to how people with learning disabilities are involved in the process. It was found that most of the local funeral directors had little direct experience of people with learning disabilities. The religious group representatives who were interviewed also spoke of a lack of involvement of people with learning disabilities. A pamphlet has been designed for funeral directors and religious groups to give to families or friends of people with learning disabilities who have been bereaved.     

MRI aspects of left ventricular non compaction (LVNC): About 3 cases from Sub-Saharan Africa and review of the literature

Left ventricular non compaction (LVNC) is a relatively rare variety of cardiomyopathy of genetic origin.We report three cases of LVNC diagnosed on cardiac magnetic resonance imaging (MRI) in Abidjan in patients aged 42, 46 and 60 years, referred for suspected LVNC on echocardiography.We used a 1.5 T MRI and performed the following sequences: black blood and white blood, LV minor axis, LV major axis, 4 cavities, and T1 SPIR Gadolinium (early and late enhancement at 10 minutes).MRI made the diagnosis of LVNC based on a double-layered myocardium, the inner (endocardium) non compacted, fibrillar thickened and the outer (epicardium) compacted thin with a non compacted to compacted myocardium ratio greater than 2.3, making the formal diagnosis.Cardiac MRI is an excellent diagnostic tool for LVNC. Its recent use in Africa should be common in the management of this cardiomyopathy.