A quantitative and semi-automatic measurement of transverse sinus stenosis improves idiopathic intracranial hypertension diagnostic accuracy.

We propose a new reliable transverse sinus stenosis (TSS) index based on magnetic resonance venography (MRV) for the diagnosis of idiopathic intracranial hypertension (IIH). Our quantitative semi-automatic measurement analysis based on segmentation and cross-sectional TS diameter from 48 IIH patients and controls matched for age and sex, had a good inter-observer agreement (κ=0.729) compared to a visual examination (κ=0.467). A cut-off point≥2 discriminate IIH patients from controls, with a sensitivity and specificity of 100%.

[Changes in the incidence and mortality rates of malignant melanoma in the Seine-Maritime region over a 20-year period]. / Évolution de l'incidence et de la mortalité du mélanome en Seine-Maritime sur une période de 20ans.

BACKGROUND: The aim of this study was to assess changes in the incidence and mortality rates of malignant melanoma (MM) over a 20-year period in a region without a cancer registry. METHODS: All cases of MM studied were collected retrospectively from the databases of six private and three hospital-based histopathology laboratories in the Seine-Maritime region covering three 24-month periods each 10years apart: 1988-1989, 1998-1999 and 2008-2009, The incidence and mortality rates were estimated based on data provided by French National Institute for Statistics and Economic Studies (Insee) and French National Institute of Health and Medical Research (Inserm). RESULTS: Over the 20-year period, the incidence of MM increased from 8.6 to 21.2/100,000inhabitants per year (+147%, P<0.0001) while the mortality rate rose from 1.3 to 2.8/100,000inhabitants per year (+115%, P=0.0003). The incidence of invasive MM increased by +110%, while the incidence of MM in situ increased by +456%. The incidence and overall mortality rate of invasive MM increased particularly during the first 10-year period: +62% (P<0.0001) and +77% (P=0.01) respectively, and to a much lesser extent during the last 10-year period: +30% (P=0.0007) and +22% (P=0.22) respectively. This slowdown in the incidence of invasive MM and in overall mortality rates was even more pronounced in women over the last 10years (+17 and +9%), whereas these rates continued to increase in men (+49% and +35%, respectively). In contrast, the incidence of MM in situ increased above all during this same period (+257%). CONCLUSION: This study shows that while the incidence and mortality rate of invasive MM has increased little over the last 10years in the Seine-Maritime region, the incidence of MM in situ continues to rise sharply.

Simultaneous suprasellar and pineal germ cell tumors in five late stage adolescents: endocrinological studies and prolonged follow-up.

Primary germ cell tumors (PGCT) of the central nervous system usually develop in the third ventricle area, and most frequently in the pineal region. The suprasellar region is the second preferential site for development of these tumors which are rarely simultaneously present in these two sites. We report five new cases of PGCT with pineal and suprasellar localizations, which appeared in late puberty in four boys and one girl aged 17-19 years. The clinical picture associated signs of intracranial hypertension, convergence and verticality palsies, diabetes insipidus and pituitary deficiency. Encephalic MRI revealed a double localization. Endocrine tests revealed a particular pattern associating central diabetes insipidus and a hypothalamic-pituitary disconnection syndrome. Following identification of the pathological type of lesions via a neurosurgical approach, treatment was based on a combined method using chemotherapy, radiotherapy and hormone replacement. Based on this treatment, prolonged remissions were obtained with a good quality of life.

[Inpatient and outpatient follow-up of grade I malignant melanoma]. / Etude comparative du suivi des malades atteints de mélanome de stade Ien pratique libérale et hospitalière.

BACKGROUND: In 1995, the French consensus conference on management of patients with grade I malignant melanoma recommended clinical examination for patient monitoring. To date, only one survey has been conducted to evaluate these recommendations and their consequences, providing no means of assessing follow-up practices. The aim of this study was to assess follow-up practices in patients with grade I malignant melanoma followed in an outpatient private practice setting and in a hospital setting with regular appointments. PATIENTS AND METHODS: This retrospective study was conducted in collaboration with private practice and hospital dermatologists, all members of an association of continuing medical education. Medical records of 584 patients with grade I malignant melanoma who had undergone surgery between January 1, 1991 and December 31, 1995 were reviewed. Three hundred twenty-nine patients were followed in an exclusively outpatient setting by their private dermatologist and 265 were followed in a hospital setting. Follow-up data were: age, sex, date of surgical excision of the melanoma, Breslow thickness, date of each follow-up visit, presence of possible metastases and mode of diagnosis. RESULTS: Patient features were different in the two groups: mainly greater Breslow thickness and more frequent metastatic course in patients followed in a hospital setting. Among all patients, 65 (11 p. 100) developed metastases. Diagnosis of metastasis was made clinically in 95 p. 100 whatever the mode of monitoring considered. The number of patients lost to follow-up was 11p. 100 among those followed in a hospital setting and 42 p. 100 in those followed in a private practice setting. Patients lost to follow-up had a higher risk of developing metastasis as their average Breslow thickness was 1.7 mm. CONCLUSION: This study shows that patients followed in a hospital setting have a more severe prognosis than patients followed in private practice. It confirms that systematic use of complementary tests is of little interest in detecting metastases since over the period considered, the diagnosis of metastasis was made clinically in most cases. It also discloses difficulties encountered in exclusively outpatient follow-up as a high number of patients were lost to follow-up in this setting. A systematic appointment fixed by the private dermatologist during the follow-up period appears to be needed to ensure good quality follow-up. Such an appointment system should help reduce the number of patients lost to follow-up.

[Evaluation of the diagnosis of pigmented tumors of the skin and factors leading to a decision to excise. Dermatologists of the Postgraduate Association of Haute-Normandie]. / Evaluation du diagnostic des tumeurs pigmentées de la peau et des éléments conduisant à une décision d'exérèse. Dermatologues de l'Association de FMC de Haute-Normandie.

INTRODUCTION: The necessity of excising melanomas characterized by a slight thickness at an early stage, leads dermatologists to remove pigmented lesions which do not correspond to melanomas. The aims of this study were: a) to prospectively assess the accuracy of melanoma diagnosis, b) to quantify the number of excisions performed according to the degree of melanoma suspicion, c) to determine the specific clinical sign or signs of relevant diagnostic value. PATIENTS AND METHODS: This study was conducted prospectively from January 1996 to August 1997 by dermatologists in private practice and dermatologists from a University Hospital staff. When it was decided to excise a pigmented lesion, a form was filled out choosing the most appropriate clinical diagnosis, the degree of melanoma suspicion, and clinical signs which lead to surgery. Based on histological findings as the reference, the sensitivity, specificity, accuracy of melanoma diagnosis and the kappa test that evaluates the concordance between clinical and histological diagnosis, were performed. The diagnostic value of clinical signs was assessed by variance analysis. RESULTS: Of the 353 excised lesions, 38 (10.7 p. 100) were identified as melanoma on histologic examination. The sensitivity, the specificity and diagnostic accuracy were: 79 p. 100, 94 p. 100 and 53 p. 100 respectively. The kappa test concordance between clinical and histological diagnosis was 0.66. Two hundred and two lesions (57 p. 100) were excised even though the clinical suspicion of melanoma was poorly considered. Only one of these 202 lesions was identified histologically as a true melanoma. Thirty seven (24.5 p. 100) of the 151 remaining excised lesions with an "average" or "strong" suspicion were true melanomas. The clinical signs considered, alone or associated, had a poor predictive positive value (< 38 p. 100). An analytical approach performed with a logistic model permitted the identification of two associated signs suggesting a best diagnostic value. DISCUSSION: This is the only study, to our knowledge, reported in the literature which prospectively assesses the sensitivity, specificity and concordance between clinical and histological diagnosis of melanoma. Results were considered from average to good. The originality of this study was to assess the number of pigmented lesions excised according to the degree of melanoma suspicion, suggesting the possibility of reducing the number of nevi removed when the melanoma risk was considered clinically poor. Finally, this study emphasizes the limits of clinical semiology and the need for future diagnostic methods in the assessment of melanoma.

Risk factors and outcomes for atherothrombotic disease in French patients: the RIVAGE study. RIsque VAsculaire Group d'Etude.

This prospective observational study was designed to delineate the course of atherosclerotic disease in a representative group of French patients receiving standard medical care and to look for clinical and laboratory factors predictive of recurrent cardiovascular events. The 2416 study patients (75.2% men and 24.8% women) had diagnoses of peripheral arterial disease (stage II or III), ischemic heart disease (stable angina or myocardial infarction), or cerebrovascular disease (transient ischemic attack or stroke); 2004 patients (82.9%) had only one of these diagnoses, and 412 (17.1%) had more than one. Among patients with a given stage of peripheral arterial disease, mean age was older in the women than in the men. Coronary disease and cerebrovascular disease were more severe in the men. During the 18-month follow-up, 408 cardiovascular events were recorded in 380 patients (15.7% of the overall study group). In patients who had a single clinical event at inclusion, subsequent clinical events usually occurred in the same vascular bed. The incidences of coronary and cerebral events were correlated with age and the incidence of peripheral events with smoking status. Fatal events were correlated with age but not with the baseline diagnosis, except for a weak relationship with peripheral arterial disease. In a subset of 411 patients who had laboratory tests, plasma fibrinogen level was the only independent predictor of recurrence for all cardiovascular events; this parameter was more closely correlated with fatal events than with all events.

[Infant feeding until the age of 4 months old in Seine-Maritime]. / L'alimentation du nourrisson jusqu'à 4 mois en Seine-Maritime.

This study reports infant feeding practices during the first 4 months of life. A representative sample of 10% of the births in the Seine-Maritime department formed the subject of a questionnaire cohort study carried on at home by public health institution for infants specialized nurses. Among 1,407 interviewed mothers, 52% had been thinking about their future child feeding before birth, essentially with their circles, especially their husbands. The type of feeding had been chosen before pregnancy in 64.3% of cases. Earlier choices resulted more often in breast-feeding. 46.2% of mothers tried to breast-feed their children, however 17% among them discontinued when back home. Carrying out breast-feeding (median duration 10 weeks) delayed the feeding diversification and was associated with a lower frequency of health difficulties in children.

[A case of primary osteosarcoma of the liver]. / Un cas d'ostéosarcome primitif du foie.

The authors report a case of primary osteosarcoma of the liver in a 75 year-old man with post hepatitic B cirrhosis. The hepatic tumor was discovered on ultrasound and computed tomography. Angiography showed a hypervascularized tumor. The patient died rapidly. Diagnosis of primary osteosarcoma of the liver was established through histopathological examination of post mortem specimens and was confirmed by immunohistochemical study. From this and two other previously reported cases the authors describe the different primary hepatic tumors mimicking osteosarcoma.

[Parameters of hemostasis in Behçet's disease]. / Etude de paramétres de l'hémostase dans la maladie de Behçet.

Venous thrombosis occurred frequently in Behçet's disease. In 12 patients with Behçet's disease of whom 6 have had leg venous thrombosis, some parameters of hemostasis or fibrinolysis have been studied. Platelet count, fibrinogen level, factor VIII coagulant activity, factor VIII antigen, plasminogen and antithrombin III level were in the normal range. Fibrinolytic capacity in response to venous occlusion of the arms was the same in patients and in healthy subjects, in the patients, there was no difference in regard to the occurrence of venous thrombosis. These parameters seemed therefore not able to detect the thrombotic tendency in this disease. These results are discussed with the other reports of the literature.

[Molecular abnormalities in recurrent thromboembolic disease]. / Anomalies moléculaires des maladies thrombo-emboliques récidivantes.

The diagnosis and treatment of apparently idiopathic recurrent thromboembolic disease (RTED) still raise difficult problems. However, recent data suggest that abnormalities of coagulation and fibrinolysis factors are important. Hereditary antithrombin III (AT III) deficiency or abnormal AT III, and hereditary protein C deficiency with autosomal dominant transmission have been associated with severe familiar RTED. More recently, we described a dysfibrinogenemia characterized by abnormal fibrin polymerization and abnormal plasminogen binding to the fibrin clot, responsible for familial RTED. Disorders of fibrinolytic activity in RTED are represented, in 70% of the cases, by reduced release or lack of production by endothelial cells of a vascular plasminogen activator. Hereditary plasminogen deficiency or abnormal plasminogen, although rare, are regularly responsible for RTED.