Dermatofibrosarcoma protuberans: a population-based cancer registry descriptive study of 66 consecutive cases diagnosed between 1982 and 2002

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare malignant tumour of the skin, with an estimated incidence of 0.8 to five cases per 1 million people per year. OBJECTIVE: To study epidemiological, immunohistochemical and clinical features, delay in diagnosis, type of treatment and outcome of DFSP from 1982 to 2002. METHODS: Using data from the population-based cancer registry, 66 patients with pathologically proved DFSP were included (fibrosarcomatous DFSP were excluded). Each patient lived in one of the four departments of Franche-Comté (overall population of 1 million people) at the time of diagnosis. The main data sources came from public and private pathology laboratories and medical records. The rules of the International Agency for Research on Cancer were applied. RESULTS: The estimated incidence of DFSP in Franche-Comté was about three new cases per 1 million people per year. Male patients were affected 1.2 times as often as female patients were. The trunk (45%) followed by the proximal extremities (38%) were the most frequent locations. DFSP occurred mainly in young adults between 20 and 39 years of age. Mean age at diagnosis was 43 years, and the mean delay in diagnosis was 10.08 years. Our 66 patients initially underwent a radical local excision. Among them, 27% experienced one or more local recurrences during 9.6 years of follow-up. There was one regional lymph node recurrence without visceral metastases. These recurrences were significantly related to the initial peripheral resection margins. We observed a local recurrence rate of 47% for margins less than 3 cm, vs. only 7% for margins ranging from 3 to 5 cm [P=0.004; OR=0.229 (95%, CI=0.103-0.510)]. The mean time to a first local recurrence was 2.65 years. Nevertheless, there was no death due to the DFSP course at the end of the follow-up, and the final outcome was favourable. CONCLUSION: Our study emphasizes the importance of wide local excision with margins of at least 3 cm in order to prevent local recurrence. However, the recent development of inhibitors of signal transduction by the PDGFB pathway should soon modify the surgical strategy, which is often too mutilating.     

Skin to skin contact in neonatal care: knowledge and expectations of health professionals in 2 neonatal intensive care units]

Skin to skin contact is a method derived from kangaroo care whose implementation in industrialized countries has rarely been assessed. OBJECTIVES: To evaluate the barriers, knowledge and expectations of health professionals regarding this care in 2 level III neonatal care units in the Nord-Pas-de-Calais. MATERIALS AND METHODS: Investigation was conducted by means of 2 questionnaires, one intended to physicians, the other to the nursing staff sharing some common questions. The 2 neonatal units differed by the level of implementation of skin to skin contact. Results were compared according to occupation and site of work. RESULTS: 80% of the physicians and 71.4% of the paramedical staff answered to the questionnaires. The difficulties were linked to technical or architectural constraints. Responses were not very different between the 2 teams. The majority (90%) considered this practice as a fully-fledged care. The positive effects on attachment (96% of the answers) were well-known but those on sleep (2,9%), breast-feeding (5%) and pain (0%) were only rarely mentioned. Barriers to implementation were centred on infant's safety. The majority of the team wished to benefit from an educational intervention. DISCUSSION: Although the perception of skin to skin contact was positive in these 2 teams, increased knowledge of the evidence supporting its practice could enable babies and parents to benefit from all its positive effects. The need for educational program was clearly mentioned to improve parents' information. CONCLUSION: This work emphasises the complexity of introducing new procedures in neonatal care particularly in the field of developmental care.     

Gastric schwannoma. A case diagnosed by puncture biopsy]

One case of schwannoma of the stomach diagnosed with ultrasound-guided biopsy puncture is reported. This diagnosis is usually not established with radiographic and endoscopic examinations. A preoperative diagnosis allows partly predicting the type of exeresis to be performed. On the other hand, guided biopsy puncture seldom allows establishing a prognosis, for which the criteria are based on the analysis of the whole tumor and the possible discovery of synchronous or metachronous metastases.     

Etomidate modifies hemodynamic response to fentanyl in patients with impaired left ventricular function]

The haemodynamic effects and the side-effects of anaesthesia using high doses of fentanyl were compared in two groups of 12 patients each. All the patients had poor left ventricular function and were scheduled for elective coronary artery bypass graft surgery or valvular replacement. Patients were randomly assigned to either group. In group EF, patients were given 5 micrograms.kg-1 of fentanyl, followed by 0.3 mg.kg-1 of etomidate. Once they had lost consciousness, they were given 15 mg of pancuronium and 25 micrograms.kg-1 of fentanyl over a 5 min period. Patients in group F received the full 30 micrograms.kg-1 dose of fentanyl over a 5 min period, followed by 15 mg of pancuronium. The patients were intubated 2 min after the end of the fentanyl infusion. They were mechanically ventilated with 100% oxygen. Anaesthesia was maintained with a continuous infusion of fentanyl (total dose 100 micrograms.kg-1). The usual haemodynamic parameters were monitored and calculated, as well as pain during injection of the drugs, myoclonia, chest wall rigidity and the time to loss of consciousness. The two groups were comparable with respect to age, weight, height and surgery. One third of the patients in group EF complained of pain during etomidate injection. The time required to loose consciousness was shorter in group EF (55 +/- 16 sec) than in group F (177 +/- 56 sec) (p < 0.001). The cardiac index decrease in group EF (2.0 +/- 0.4 l.min-1.m-2 vs. 1.9 +/- 0.4 l.min-1.m-2) (p < 0.05), respectively between the time just before tracheal intubation (T1), and 10 min after tracheal intubation (T3).(ABSTRACT TRUNCATED AT 250 WORDS)     

Extending the capability paradigm to address the complexity of disability

Amartya Sen looks at people's well-being in terms of functioning and freedom, rather than in terms of the amount of goods or services consumed. The capability approach, developed by Sen, deals with what people are able to achieve by using these commodities. Concerning disability, he wrote: “We must take note that a disabled person may not be able to do the many things that an able-bodied individual can, with the same bundle of commodities” [Sen, A. K. (1985). Commodities and capabilities. Oxford: Oxford University Press [1999], p. 7]. The capability approach makes it possible to analyse the economic situation of people with disabilities in a different way. What becomes important is their functioning, i.e. what they are able to achieve within a given context. In fact, the capability set includes not only what a person is effectively able to achieve, but also the potential functionings that he/she can choose. This expresses the degree of freedom that a person with disability benefits from in a given environment. The challenge is therefore to reduce the constraints that the environment adds to a person's impairment in order to expand their capability set and to allow them to live a life which they value. This paper reviews the paradigms that address disability and the ways of assessing a person's capability set within this framework. Achieved functionings are easily measured through cross-sectional surveys, using counterfactual analysis to compare the situations of disabled and non-disabled people. This was done in 2005 in Afghanistan when a national disability survey was carried out on a random sample of households. Measuring detailed capabilities, especially their freedom dimension, is quite complex and requires identifying people's potential choices in an ever-changing environment. This implies a need to extend the philosophical framework and to adopt appropriate statistical methodologies.     

Movement Disorder Society-sponsored revision of the Unified Parkinson's Disease Rating Scale (MDS-UPDRS): Process, format, and clinimetric testing plan.

This article presents the revision process, major innovations, and clinimetric testing program for the Movement Disorder Society (MDS)-sponsored revision of the Unified Parkinson's Disease Rating Scale (UPDRS), known as the MDS-UPDRS. The UPDRS is the most widely used scale for the clinical study of Parkinson's disease (PD). The MDS previously organized a critique of the UPDRS, which cited many strengths, but recommended revision of the scale to accommodate new advances and to resolve problematic areas. An MDS-UPDRS committee prepared the revision using the recommendations of the published critique of the scale. Subcommittees developed new material that was reviewed by the entire committee. A 1-day face-to-face committee meeting was organized to resolve areas of debate and to arrive at a working draft ready for clinimetric testing. The MDS-UPDRS retains the UPDRS structure of four parts with a total summed score, but the parts have been modified to provide a section that integrates nonmotor elements of PD: I, Nonmotor Experiences of Daily Living; II, Motor Experiences of Daily Living; III, Motor Examination; and IV, Motor Complications. All items have five response options with uniform anchors of 0 = normal, 1 = slight, 2 = mild, 3 = moderate, and 4 = severe. Several questions in Part I and all of Part II are written as a patient/caregiver questionnaire, so that the total rater time should remain approximately 30 minutes. Detailed instructions for testing and data acquisition accompany the MDS-UPDRS in order to increase uniform usage. Multiple language editions are planned. A three-part clinimetric program will provide testing of reliability, validity, and responsiveness to interventions. Although the MDS-UPDRS will not be published until it has successfully passed clinimetric testing, explanation of the process, key changes, and clinimetric programs allow clinicians and researchers to understand and participate in the revision process.     

Automated three-dimensional analysis of histological and autoradiographic rat brain sections: application to an activation study.

Besides the newly developed positron emission tomography scanners (microPET) dedicated to the in vivo functional study of small animals, autoradiography remains the reference technique widely used for functional brain imaging and the gold standard for the validation of in vivo results. The analysis of autoradiographic data is classically achieved in two dimensions (2D) using a section-by-section approach, is often limited to few sections and the delineation of the regions of interest to be analysed is directly performed on autoradiographic sections. In addition, such approach of analysis does not accommodate the possible anatomical shifts linked to dissymmetry associated with the sectioning process. This classic analysis is time-consuming, operator-dependent and can therefore lead to non-objective and non-reproducible results. In this paper, we have developed an automated and generic toolbox for processing of autoradiographic and corresponding histological rat brain sections based on a three-step approach, which involves: (1) an optimized digitization dealing with hundreds of autoradiographic and histological sections; (2) a robust reconstruction of the volumes based on a reliable registration method; and (3) an original 3D-geometry-based approach to analysis of anatomical and functional post-mortem data. The integration of the toolbox under a unified environment (in-house software BrainVISA, http://brainvisa.info) with a graphic interface enabled a robust and operator-independent exploitation of the overall anatomical and functional information. We illustrated the substantial qualitative and quantitative benefits obtained by applying our methodology to an activation study (rats, n=5, under unilateral visual stimulation).     

Can we understand partnerships in general practice? A pilot study

BACKGROUND: Partnerships have been investigated in different professions,but other than identifying problems, little work has been carriedout on general practice. OBJECTIVE: The aim of this present study was to develop methods for studyingpartnerships in general practice. METHOD: A tripartite methodological approach was used, with questionnairesadapted from other instruments in use in other professions,followed by an individual interview with each partner, and non-participantobservation at a partnership meeting. Results for one case-studypartnership are given. RESULTS: There were no major differences between the partners on alldimensions measured; the minor differences indicated by theresults of the questionnaires were corroborated by the partnerinterviews and observations. CONCLUSIONS: We conclude that the use of such techniques could provide supportto partnerships going through significant periods of change. Keywords. General practice, interview, observation, partnerships, questionnaire.     

Anatomic variations of the spermatic vein and endovascular treatment of left varicoceles: a pediatric series]

OBJECTIVE: To report on the high incidence of anatomical variants of the origin and course of the internal spermatic vein (ISV) discovered at the time of percutaneous embolization of left varicoceles in a pediatric population. METHODS: We reviewed retrospectively the 65 cases of left varicocele treated by percutaneous embolization (grade II and III) in our institution between 1990 and 2000. The course of the left renal vein (LRV), the origin of the ISV, and the number of ISVs and their pathway were recorded in all cases, according to the B?hren classification. RESULTS: In 37/65 (57%), the ISV was single and arose from a normal LRV (type I). The following variants were encountered: type V--circumaortic LRV 9/65 (14%); type IVb--intrarenal origin of ISV 8/65 (12%); type II--multiple ISV 5/65 (8%); and pelvic collaterals 6/65 (9%). CONCLUSION: Venous anatomical variants are frequently encountered (43%) at the time of left varicocele embolization in children. Such variants often impose some adjustments to the technique of embolization and, at times, hamper the procedure.