Cystic fibrosis mortality trends in France.

BACKGROUND: In 1992 France set up a national cystic fibrosis observatory (Observatoire national de la mucoviscidose, ONM) to monitor the state of health of patients on an annual basis. Using the ONM data, this study estimates the main indicators for life expectancy and assesses the total number of cystic fibrosis patients. METHODS: The data for the years 1994 to 2003 are divided into 3-year periods. Life tables are drawn up for these periods, from which mean and median lengths of life are determined. Using the most recent life table, the number of births in 2003 and the incidence of the disease, the total population of patients can be estimated, assuming a stationary population. RESULTS: In 2001-2003, life expectancy at birth of patients registered with the ONM was 39.1 years and median length of life was 36.4 years. These results, substantially better than those of 1994-1996, are linked to improved conditions of patient inclusion in the ONM database, to improvements in their healthcare, but also to the limitations of the life tables. Based on the 2003 data, the total theoretical number of patients is 6490, and coverage by the ONM database is thus 63.2%. CONCLUSIONS: These provisional results demonstrate the need to convert the ONM observatory into a registry providing exhaustive coverage of all patients.     

BoneMorphing versus freehand localization of anatomical landmarks: consequences for the reproducibility of implant positioning in total knee arthroplasty.

OBJECTIVE: This study analyzed the influence of the acquisition method in image-free computer-assisted total knee arthroplasty (CAS-TKA), and the reproducibility of implant planning using BoneMorphing, a 3D morphometric model obtained by a 3D-to-3D elastic registration of statistical models to sparse point clouds acquired directly on the bone surface with a pointer. MATERIALS AND METHODS: Five surgeons (one expert, four trainees) each performed a CAS-TKA hybrid protocol based on morphometric models and landmarks on a cadaveric knee 10 times. In addition, several additional landmarks were digitized during each acquisition. The reproducibility of the implant positioning and sizing, as determined by an implant planning algorithm with morphometric models, was compared to direct digitization accuracy. RESULTS: Femoral and tibial implant positioning parameters with the hybrid protocol resulted in intra-surgeon standard deviations (SDs) of less than+/-1.4 degrees for rotation and 1.9 mm for translation for all surgeons in all directions except for tibial axial rotation (the only parameter determined by a digitized landmark and not recomputed in the 3D model). The variability in individual landmark digitization varied from 2 to 5 mm SD for certain landmarks, with ranges of 15-25 mm across all surgeons. The comparison study showed an improvement in femoral rotation reproducibility with the morphometric model when using the posterior condylar axis. Tibial implant reproducibility for each method was comparable, with the morphometric model giving better results in well-digitized areas such as the tibial plateau. CONCLUSION: A CAS-TKA protocol based on a deformed statistical model offers reproducible implant positioning. Some landmarks, such as distal condyles, show sufficient reproducibility in the direction of interest, while others, such as the anterior tibial tubercle, can lead to hazardous implant positioning. This should be taken into consideration when designing a CAS-TKA system with bony landmarks. In areas where a sufficient number of points have been digitized with good coverage, such as on the distal and posterior femoral condyles or the tibial plateau areas, the information derived from the 3D model is more accurate and reproducible than that derived from digitization. Good training and a guiding user interface are essential to guarantee coverage quality.     

Synthesis and antimalarial activity of new atovaquone derivatives

In this paper we describe the design and synthesis of 18 derivatives of the antimicrobial atovaquone which were substituted at the 3-hydroxy group by ester and ether functions. The compounds were evaluated in vitro for their activity against the growth of Plasmodium falciparum, the malaria causing parasite. All the compounds showed potent activity, with IC₅₀ values in the range of 1.25–50 nM, comparable to those of atovaquone and much higher than chloroquine or quinine.     

Histopathological Diagnosis of Partial and Complete Hydatidiform Mole in the First Trimester of Pregnancy

The diagnosis of molar pregnancy is a continuing diagnostic problem for many practicing histopathologists who are required to examine specimens of products of conception, particularly since changes in gynecological management in recent years have resulted in uterine evacuation at earlier gestations. The aim of this review is to provide practical, up-to-date, diagnostically useful information regarding the histological diagnosis of molar disease in early pregnancy. Pathophysiological issues relevant to molar pregnancies, such as genetic abnormalities, will be briefly summarized, but nonhistopathological aspects of molar disease will not be covered in detail in this review.     

Patch testing with beryllium alloy samples in guinea pigs

An experimental study was conducted in guinea pigs for the predictive assessment of the beryllium alloy hazard in occupational exposure of the skin to beryllium compounds. Guinea pigs were sensitized to beryllium sulfate according to the maximized Magnusson and Kligman test, and challenged with beryllium alloys and metallic copper, beryllium and aluminum samples. Results showed a delayed skin hypersensitivity reaction in 30 to 60% of pre-sensitized guinea pigs challenged with copper-beryllium alloys and aluminum-beryllium alloy. An inflammatory follicular reaction was induced by copper in both controls and pre-sensitized guinea pigs.     

Implications of a diagnosis of anorexia nervosa in a ballet school

Competitive pressures to achieve a slim body shape may be of importance in the etiology of eating disorders in ballet dancers. This study examines the presence of anorexia nervosa-like symptoms in a group of 49 female ballet students (mean age = 18.9 years, SD ± 1.9). All students were assessed for certain physical (weight and height) and psychological (Eating Attitude Test [EAT]) indices at the start of their academic training year. Thereafter, all subjects who presented with anorexia nervosa-like symptoms (EAT ≥ 30, and/or with current secondary amenorrhea or primary amenorrhea if aged 16 years or over) at the initial assessment, were invited for a semistructured interview (Morgan-Russel scales) to determine their diagnostic status. Another aim of the study was to assess the prognostic implications of a diagnosis of anorexia nervosa in this sample. All subjects previously interviewed were invited for a follow-up assessment at 10 months. Anorexia nervosa could be diagnosed in 2 students (4.1%), whilst another 4 students (8.2%) presented with “partial syndrome” anorexia nervosa. All diagnosed students managed to complete their academic training year. The development and implications of a diagnosis of anorexia nervosa in the ballet students are discussed.     

Laser interstitial thermotherapy in stereotactical neurosurgery

The authors present their first experience with laser interstitial thermotherapy, (ITT). Four patients with deep-seated non-infiltrative benign tumours (three astrocytomas, one oligodendrocytoma) underwent ITT. Previously, a stereotactic procedure had been performed to determine the exact location of the tumour, its spatial configuration and its histological diagnosis. The MRI controls confirmed the efficacy of hyperthermia on the tumour tissues. The authors discuss the possible ITT mechanisms. The indications of such laser ITT in neurosurgery should be enlarged to malignant tumours (gliomas, metastases) and to certain hypophyseal adenomas.