Les personnes en situation de handicap complexe avec altérations des capacités de décision,d’action et de communication : retour sur la méthode

As a result of the collaborative national research on complex disability situations, which he conducted between 2010 and 2013, the author returns to method elements regarding the survey of people without access to a typical communication. The article discusses the conditions of a “semaphoric” approach (Delion, 2006) in meeting with a person with multiple disabilities with significant communicative limitations, and co-constructing possible “spaces” for discussion and observation of the shared experience of emotions.     

Five-year follow-up of clinical and radiological outcomes of LP-ESP elastomeric lumbar total disc replacement in active patients

Background context

The surgical treatment of degenerative disc disease at the lumbar spine may involve fusion. Total disc replacement (TDR) is an alternative treatment to avoid fusion-related adverse events, specifically adjacent segment disease. New generation of elastomeric non-articulating devices has been developed to more effectively replicate the shock absorption and flexural stiffness of native disc.

GDC 360° for the endovascular treatment of intracranial aneurysms: a matched-pair study analysing angiographic outcomes with GDC 3D Coils in 38 patients

Introduction  The purpose of this study was to determine whether coil embolisation with a new complex-shaped Guglielmi Detachable Coil (GDC 360°; Boston Scientific Neurovascular, Fremont, CA, USA) has any effect on the stability of aneurysm occlusion. Materials and methods  Fifty-one consecutive patients with intracranial aneurysms treated with GDC 360° were included. Angiographic results and adverse neurological events during the follow-up period were recorded. For 38 patients treated with GDC 360° with available follow-up data, a corresponding patient treated with GDC 3D was identified from our database. Matches were sought for rupture status, location, aneurysmal size, and neck size. The angiographic outcome of these matched controls at 6 months was compared to aneurysms treated with GDC 360°. Results  Initial angiographic controls for 38 patients treated with GDC 360° showed complete occlusion in 32 aneurysms, and a neck remnant in six. At 6-month follow-up, complete occlusion was found in 29, a neck remnant in eight, and a residual aneurysm in one. One patient treated with GDC 360° needed retreatment for a major recanalisation. In 38 matched patients treated with GDC 3D, initial angiographic controls found complete aneurysmal occlusion in 30 aneurysms and a residual neck in 8. At 6-month follow-up, 24 aneurysms were completely occluded, ten showed a neck remnant, and residual aneurysms were seen in four. Four patients, treated with GDC 3D, were retreated for major aneurysm recanalisations. Conclusion  Our data suggests that endovascular coil embolisation with GDC 360° might improve long-term stability of coiled aneurysms when compared to GDC 3D.     

Profound hypoxemia during treatment of low cardiac output after cardiopulmonary bypass

PURPOSE: To illustrate the multiple causes of hypoxemia to be considered following cardiopulmonary bypass and how therapy given to improve oxygen delivery may have contributed to a decrease in arterial oxygen saturation to life-threatening levels. CLINICAL FEATURES: A 61 yr old man with severe mitral regurgitation and chronic obstructive lung disease underwent surgery for mitral valve repair. A pulmonary artery catheter with the capacity to measure cardiac output and mixed venous oxygen saturation (SvO2) continuously was used. Two unsuccessful attempts were made to repair the valve which was finally replaced, requiring cardiopulmonary bypass of 317 min. Dobutamine 5 micrograms.kg-1.min-1 and sodium nitroprusside 1 microgram.kg-1.min-1 were used to increase cardiac output. Soon after, the SvO2 decreased progressively from 55 to 39%. The patient became cyanotic with a PaO2 of 39 mmHg. Sodium nitroprusside was stopped and amrinone 100 mg bolus followed by 10 micrograms.kg-1.min-1 was given in addition to adding PEEP to the ventilation. With these measures PaO2 could be maintained of safe levels but PEEP and high inspired oxygen concentrations were needed postoperatively until the trachea could be extubated on the third postoperative day. CONCLUSION: The profound hypoxemia in this case was likely due to a combination of intra- and extrapulmonary shunt, both augmented by sodium nitroprusside. The desaturation of mixed venous blood amplified the effect of these shunts in decreasing arterial oxygen saturation. The interaction of these factors are analyzed in this report.     

Embolization of Life-Threatening Arterial Rupture in Patients with Vascular Ehlers–Danlos Syndrome

Purpose

To evaluate the safety and efficacy of transarterial embolization of life-threatening arterial rupture in patients with vascular Ehlers–Danlos syndrome (vEDS) in a single tertiary referral center.

Methods

We retrospectively analyzed transarterial embolization for vEDS performed at our institution from 2000 to 2012. The indication of embolization was spontaneous arterial rupture or pseudoaneurysm with acute bleeding. All interventions used a percutaneous approach through a 5F or less introducer sheath. Embolic agents were microcoils and glue in 3 procedures, glue alone in 2, and microcoils alone in 2.

Results

Five consecutive vEDS patients were treated by 7 embolization procedures (4 women, mean age 29.8 years). All procedures were successfully performed. Two patients required a second procedure for newly arterial lesions at a different site from the first procedure. Four of the five patients were still alive after a mean follow-up of 19.4 (range 1–74.7) months. One patient died of multiple organ failure 2 days after procedure. Minor procedural complications were observed in 3 procedures (43 %), all directly managed during the same session. Remote arterial lesions occurred after 3 procedures (43 %); one underwent a second embolization, and the other 2 were observed conservatively. Puncture site complication was observed in only one procedure (14 %).

Conclusion

Embolization for vEDS is a safe and effective method to manage life-threatening arterial rupture.     

Juvenile-onset permanent weakness in muscle phosphofructokinase deficiency

We describe a 41-year-old Moroccan woman with phosphofructokinase (PFK) deficiency who presented slowly progressive muscular weakness since childhood, without rhabdomyolysis episode or hemolytic anemia. Deltoid biopsy revealed massive glycogen storage in the majority of muscle fibers and polysaccharide deposits. PFK activity in muscle was totally absent. A novel homozygous non-sense mutation was detected in PFKM gene. Our observation suggests that juvenile-onset fixed muscle weakness may be a predominant clinical feature of PFK deficiency. Vacuolar myopathy with polyglucosan deposits remains an important morphological hallmark of this rare muscle glycogenosis.     

Ten-year prospective follow-up study of the mortality by suicide in schizophrenic patients

This ten-year follow-up study examined the prevalence and the most relevant baseline predictors of suicide in schizophrenic patients. In 1993, 3470 patients meeting the ICD-10 criteria for schizophrenia were assessed. We used national death certificate data to identify patients that had died by suicide for each year included in the study. In this way, we calculated standardized mortality ratios, adjusting for age and sex relative to the general population. We used Cox's proportional hazards models to investigate potential sociodemographic and clinical risk factors. There were 141 suicides in the cohort during the follow-up period, corresponding to a risk of suicide that was approximately 16 times higher than that of the general population. Women had slightly higher standardized mortality ratios than men. Suicide was the cause of death in more than half (53.9%) of deaths occurring during the first year of follow-up and nearly one-third (31.8%) of those occurring in the ten-year period of the study. There were four significant baseline predictors of suicide remaining in the final logistic regression model: male gender, drug abuse, previous suicide attempts, and short duration of illness. Sex, age, history of suicide attempt should be particularly considered in the assessment of suicide risk in schizophrenic patients. Our findings also emphasize the need for detection and effective management of associated comorbid drug abuse.     

Contemporary issues and the potential uses of capecitabine in metastatic breast cancer

Since its first regulatory approval more than 10 years ago, oncologists have gained wide experience in using the oral fluoropyrimidine, capecitabine, as monotherapy or in combination with other agents and the body of evidence supporting these approaches continues to grow. Alongside this increasing experience has been the appearance of new challenges in patient management. We now recognise several different biological subtypes of breast cancer, such as HER2-positive disease. The standard of care in these tumours comprises anti-HER2 therapy, and phase III data show that capecitabine can be effectively combined with such agents. Another increasingly prominent and currently unresolved issue resulting from more effective treatment of metastatic disease is the management of patients with brain metastases. The introduction of new, well-tolerated, oral chemotherapies also provides the opportunity for longer duration of therapy. These new clinical scenarios are discussed in the current review.     

Danger of systemic cyclosporine for corneal graft

PURPOSE: To report a case of posttransplant lymphoproliferative disorder (PTLD) in a patient receiving oral cyclosporine (CS) for immunosuppression in a high-risk keratoplasty. METHODS: A systemic CS therapy was given to prevent graft rejection of a keratoplasty. Risk rejection was high in regard to a previous graft rejection and persistence of a corneal vascularization. One year after the keratoplasty, the patient developed a gastric Epstein-Barr virus (EBV)-induced B cell lymphoma. The outcome was favorable after chemotherapy. CONCLUSIONS: This unique case of lymphoma (PTLD) in the course of corneal graft management questions the indications and the follow-up of patients with CS therapy and raises the issue of topical CS treatment.