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Introduction: The Doege-Potter syndrome is a rare paraneoplastic syndrome, first described by Doege and Potter in 1930, that usually involves solid fibrous pleural tumours. Hypoglycaemia is a rare complication of these tumours, due to secretion of insulin-like growth factors, and it can be the presenting symptom. The incidence of malignancy is low, about 12-15 %., Case Report: An 81-year-old Caucasian man, operated on 20 years previously for a benign pleural fibroma, presented with several episodes of loss of consciousness due to profound hypoglycaemia and a history of increasing dyspnea. The chest X-ray revealed an intrathoracic mass. Following excision histological examination confirmed recurrence with malignant transformation to a high-grade sarcoma., Conclusion: The Doege-Potter syndrome is a rare condition but it should be suspected in episodes of hypoglycaemia associated with a history of dyspnea. These patients should be followed up postoperatively because of the risk of tumour recurrence., (Copyright © 2014. Published by Elsevier Masson SAS.)

Human mesenchymal stem cells (MSCs) are a heterogeneous population of fibroblast-like cells, which are present in different locations, including bone marrow, adipose tissue, extra-foetal tissues, gingiva and dermis. MSCs, which present multipotency capacities, important expansive potential and immunotolerance properties, remain an attractive tool for tissue repair and regenerative medicine. Currently, several studies and clinical trials highlight the use of MSCs in cutaneous repair underlining that their effects are essentially due to the numerous factors that they release. MSCs are also used in skin substitute development. In this study, we will first discuss the different sources of MSCs actually available. We will then present results showing that bone marrow-derived MSCs prepared according to Good Manufacturing Practices and included in a dermal equivalent are able to promote appropriate epidermis growth and differentiation. These data demonstrate that bone marrow-derived MSCs represent a satisfactory alternative to dermal fibroblasts in order to develop skin substitute. In addition, MSCs could provide a useful alternative to sustain epidermis development and to promote wound healing., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Objective: The goal of this study was to evaluate whether the values of ADC in spondylarthritis axial active inflammatory lesions are different from ADC values in type 1 Modic changes., Subjects and Methods: 95 patients with recent lumbar pain, including 46 patients with diagnosed or suspected spondylarthritis and 49 patients with purely degenerative history, underwent spine MRI. T1w, STIR, and diffusion-weighted images (DWI) were obtained. Two musculoskeletal radiologists interpreted the images. Axial active inflammatory lesions from the SpA group and type 1 Modic changes from the degenerative group were identified on T1w and STIR sequences. ADC values from these lesions and from healthy subchondral bone were compared., Results: All axial active inflammatory lesions (n=27) and type 1 Modic changes (n=22) identified in T1w and STIR images were visible on DWI. ADC values were significantly higher (p<0.05) for axial active inflammatory lesions (median=0.788×10(-3)mm(2)/s, IQR 25-75 [0.7×10(-3)mm(2)/s; 0.9×10(-3)mm(2)/s]) than for type 1 Modic changes (median=0.585×10(-3)mm(2)/s, IQR 25-75 [0.55×10(-3)mm(2)/s; 0.60×10(-3)mm(2)/s]) and normal subchondral bone (median=0.443×10(-3)mm(2)/s, IQR 25-75 [0.40×10(-3)mm(2)/s; 0.50×10(-3)mm(2)/s]). Intra-class correlation coefficients for intra- and inter-reader ADC values comparison were excellent (0.89 and 0.98 respectively)., Conclusion: DWI is a sensitive and fast sequence that offer the possibility of quantifying diffusion coefficients of the lesions, which could help to discriminate between spondylarthritis axial active inflammatory and type 1 Modic changes., (Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.)

Objective: Urethral prolapse (UP) is a complete eversion of the distal urethral mucosa through the external meatus. UP must be distinguished by examination from trauma, prolapsed ureterocele, tumors or sexual abuse. Its management remains controversial. The aim of the study was to promote the benefits of primary surgical management for UP., Methods: A retrospective multicenter review of children who received surgery for UP between 1991 and 2011 was carried out. Non-complicated UP was primarily treated conservatively. A total of 19 patients were referred for complicated UP and underwent resection of the prolapsed urethral mucosa., Results: The mean delay in diagnosis was 2.2 days (range 1-6) and the most common symptoms were vaginal spotting and bleeding. No predisposing factor was found, but most patients had a mean weight, height and BMI greater than the 50th percentile. All patients underwent surgery successfully. One patient experienced a complication, i.e., dysuria. There was no case of recurrence after a mean 28 months of follow-up., Conclusion: Early detection is based on bedside examination. The first-line treatment strategy for uncomplicated UP should be conservative management. Surgical resection is safe and effective for patients with significant symptoms., (Copyright © 2013 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

We report four cases of acute pulmonary edema that occurred during treatment by intravenous tocolysis using nicardipine in pregnancy patients with no previous heart problems. Clinical severity justified hospitalization in intensive care unit (ICU) each time. Acute dyspnea has begun at an average of 63 hours after initiation of treatment. For all patients, the first diagnosis suspected was pulmonary embolism. The patients' condition improved rapidly with appropriate diuretic treatment and by modifying the tocolysis. The use of intravenous nicardipine is widely used for tocolysis in France even if its prescription does not have a marketing authorization. The pathophysiological mechanisms of this complication remain unclear. The main reported risk factors are spontaneous preterm labor, multiple pregnancy, concomitant obstetrical disease, association with beta-agonists, and fetal lung maturation corticotherapy. A better knowledge of this rare but serious adverse event should improve the management of patients. Nifedipine or atosiban, the efficiency of which tocolysis was also studied, could be an alternative.

Diagnosing Marfan syndrome in young children is difficult because of the great variability of expression of the disease and because the phenotype evolves over the life course. The goal of this retrospective study was to describe the first clinical symptoms in children under 10 years of age with Marfan syndrome and to evaluate the pertinence of the new 2010 Ghent criteria in comparison with the 1996 criteria. Seventeen patients under 10 by the time of the first medical examination were included. All children had an FBN1 gene mutation that was secondarily demonstrated. Clinical data including ophthalmological, cardiac, and orthopaedic examinations obtained during the first medical examination were analyzed. The most frequent abnormalities encountered were high arched palate (82%), arachnodactyly (71%), and flatfoot (59%). Aortic aneurysm (47%) and ectopic lens (35%) were also seen at the time of diagnosis. According to the 2010 Ghent criteria, the diagnosis of Marfan syndrome could be obtained in 71% of patients after identification of the mutation of the FBN1 gene, whereas only 59% of patients were diagnosed using the older criteria. All organs can be affected during childhood. An early diagnosis is essential in order to set up specific management., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Introduction: Kawasaki disease (KD) is the most common cause of acquired heart disease in developed countries. Ten percent of patients with KD develop coronary aneurism. Ten percent of patients treated with intravenous immunoglobulin (IgIV) have persistent coronary dilatations, which sustains the search for new therapies. We describe 2 cases of refractory KD and discuss the therapeutic options., Clinical Report: A 3-year-old child and a 3-month-old infant had refractory KD. Both were treated with IgIV and corticosteroids. They both had persistent fever and major coronary artery dilatation. The first patient received a treatment with acetyl-salicylic-acid (ACA) only. The second received an immuno-therapy with an anti-TNF-α agent. Fever and inflammatory symptoms disappeared within 12h in the second case. Coronary artery aneurisms worsened during the first month and then stabilized. The first child had fever and inflammatory symptoms for a longer duration, but coronary artery dilatations stabilized and disappeared with no additional treatment than ACA., Discussion and Conclusion: TNF-α is known to be one of the inflammatory factors involved in KD disease. Anti-TNF-α agents have been tested in treatment of refractory KD. In one of the cases reported herein, this therapy was not effective on coronary artery aneurism. More studies are needed to define the optimal treatment of refractory KD., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Desmoid tumor (DT) or aggressive fibromatosis is a histologically benign-appearing neoplasms of the soft tissues, arising from connective tissues, the fascial sheaths and musculoaponevrotic structures of muscles. DT is a non-metastasizing tumor but has a potential for local invasion and local recurrence. DT can be seen in all age groups including young children. The overall incidence is reported as 2-4 cases per million per year. The etiology of DT is unknown. However, a genetic predisposition, familial adenomatous polyposis and Gardner's syndrome, has been implicated in 2% of the cases. The histological diagnosis has to be confirmed by biopsy in order to eliminate other sarcomas. The clinical behavior of DT seems unpredictable with a high rate of local recurrence. Treatment depends on the aggressiveness of the disease. A "wait-and-see" strategy is, at the present time, preferred in case of asymptomatic or non-progressive disease. Complete resection after surgery is rare, that is why first-line surgery should be seriously questioned and only considered when a complete resection can be feasible without functional or cosmetic damage. If not, medical treatments (cytotoxic or not) are rather discussed. Radiation therapy is rarely an option in children in this benign tumor. Authors present accurate knowledge of this disease in children.

Introduction: MacLeod syndrome, also known as Swyer-James syndrome, is a rare syndrome characterized by unilateral lung hyperlucency. It is a form of constrictive bronchiolitis which is caused by repeated acute bronchiolitis and/or pulmonary infections during infancy., Case Report: The patient was a 26-year-old man who had had a first left pneumothorax at the age of 21. He smokes tobacco. When he was 1-year-old he had experienced repeated episodes of acute bronchiolitis. At age 26, he suffered from a second left pneumothorax which was wrongly diagnosed as a recurrent drain-resistant pneumothorax. Misdiagnosed, he underwent a chemical pleurodesis during thoracoscopy. As a hyperlucency remained in the left upper part of the lung on chest X-ray, further investigations were undertaken and a diagnosis of MacLeod syndrome was made., Conclusions: MacLeod syndrome is rare. It can be associated with pneumothorax which can be a source of misdiagnosis and error in the management of these patients. Therefore, the diagnostic criteria and therapeutic indications are reiterated. The probable mechanism of association between pneumothorax and Macleod syndrome is also discussed., (Copyright © 2012 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

The increased prevalence of certain diseases, along with the development of new technologies and industrialization raised the possibility of the involvement of environmental factors, industrial products, nutritional factors, infections, drugs... and endocrine disruptors. These factors may interfere via signaling pathways specific to the organism. Endocrine Disrupting Chemicals (EDCs) have been redefined by the Endocrine Society in 2012 as "exogenous chemical, or mixture of chemicals, that can interfere with any aspect of hormone action". They have therefore potentially deleterious effects on development, growth, metabolism, reproduction, the nervous, immune and cardiovascular systems. Therefore, they constitute a real public health issue. Their long half-life may explain delayed effects and their often lipophilic character may promote maternofetal transmission. Except diethylstilbestrol (DES), few formal proofs have been made on the direct role of EDCs ; arguments are based on cross-sectional studies, in vitro models and animal models. Basic research puts insight into mechanisms of action of EDCs but many questions remain unanswered. Epidemiological data are difficult to interpret because of interindividual differences in susceptibility to EDCs and of nonlinear/nonmonotonique action (as opposed to toxic dose effect), multiple interactions between environmental agents (additive effects and/or synergistic and/or antagonists), the role of the window of exposure, latency, and the possibility of transgenerational effects., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Described by Reich and Johnson in 1992 [2], the Lady Windermere syndrome occurs exclusively in non-smoking women over the age of 60 years, without significant pre-existing pulmonary disease. It comprises bronchial dilatation, typically in the middle lobe and lingula, together with secondary infection by atypical mycobacteria (Mycobacterium avium in the first cases). Among the 17 cases of atypical mycobacterial infection that we have seen in the past 14 years, there were seven cases of this syndrome. It was associated with cough, sputum, sometimes haemoptysis, febrile episodes and deterioration of general health. The diagnostic criteria and treatment were defined by the American Thoracic Society. The pathophysiological hypothesis proposed by Reich and Johnson was that voluntary suppression of the cough led to congestion of the bronchi and secondary infection with atypical mycobacteria. Currently it is thought more likely that the following factors are involved: progressive increase in dilatation of small bronchi, delayed diagnosis, morphological abnormalities of the thorax, hormonal factors, immune deficiency, genetic neutrophil dysfunction, and even heterozygous forms of cystic fibrosis., (Copyright © 2012 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

Background. Only limited data are available concerning desmoid tumor in children. Methods. Fifty-nine children and adolescents with desmoid tumor treated in 2 French cancer centers with a very long followup were retrospectively reviewed. Results. Median age was 6 years (range, 0-15). Tumors mainly involved the limbs (42%). Five cases occurred in a context of genetic disorder. Surgery was first-line treatment in 80% of cases. Resection was microscopically complete in 3 patients (pts), with a microscopic residue in 19 pts and a macroscopic residue in 35 cases. Various adjuvant therapies were used. Overall response to all systemic therapies was 33%. Thirty-eight patients developed one or more recurrences or progressions. After a median followup of 8.5 years, 34 patients were alive in complete remission (CR), including 16 first CR. Seven patients died, 6 from refractory disease and 1 from colorectal carcinoma in a genetic context. Ten-year progression-free survival (PFS) and overall survival were 31% and 88%, respectively. In univariate analysis, age less than 10 years and head-neck site were favorable prognostic factors for PFS. Conclusions. When surgery is required, surgical margins must be negative. Low-dose chemotherapy can be proposed as adjuvant therapy. Prospective trials must be developed to evaluate long-term response and side effects.

We report on a case of true prenatal mosaic trisomy 13 on amniotic fluid associated with a normal phenotype at the age of 6 years. The amniocentesis was performed because of advanced maternal age and was controlled by a second sample. Morphological and cardiac ultrasonography did not reveal any fetal malformations. No trisomic cells were found in the fetal blood and a nuclear magnetic resonance imaging (IRM) of the brain was performed during the third trimester found no abnormality of the brain. Finally, at birth cytogenetic analysis was performed on two placental samples for chromosomal analysis: one in an area where the placenta seemed normal, and the other one in an area with infarcted and hemorrhagic aspect. We found a high rate of trisomic cells in the sample with abnormal aspect. Furthermore, no trisomic cell was observed by fluorescent in situ hybridation (FISH) on the buccal smears of the baby. We concluded to a confined placental mosaicism. The good outcome of the child aged 6 years confirms this diagnosis. So in the aim to predict a good development for the child in case of low rate mosaic trisomy 13 in amniotic fluid, we propose at birth: i) to take several samples from the placenta to confirm placental mosaicism; ii) to label by FISH buccal smears with a LSI 13 probe to prove that the baby is not a carrier of the trisomy., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Background: Lung cancer is a frequently occurring disease among elderly people. The objective of this study was to search for a relationship between multimodal geriatric assessment and a decision-making in the treatment of patients with this condition., Methods: Forty-nine elderly patients (aged 70 years and over) diagnosed with a primary lung cancer underwent a geriatric assessment before decision for treatment was made in a multidisciplinary meeting. We described the impact of the geriatric assessment on the management decisions made., Results: Almost half of treatment (44.9%) decisions were modified by the geriatric assessment. For patients with so called "pre-frailty" according to Balducci's criteria, 60% of treatment decisions were modified by the results of geriatric assessment (use of only one chemotherapeutic drug, dose disease or best supportive care). For this group of patients, Folstein MMSE and IADL were predictive of change in decision-making, with the threshold estimated to be 26 points. Only the MMSE was significantly associated with the medical decision in multivariate analysis., Conclusion: Geriatric assessment should be integrated into treatment decision-making for patients with primary lung cancer aged more than 70 years, particularly those where frailty is a concern. Folstein's MMSE was predictive in our study for changes in decision-making., (Copyright © 2011 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

Background and Aims: The aim of the study was to compare the incidence of parapneumonic pleural effusion in the Limousin region of France, based on the comparison of pre- and postvaccination periods., Methods: Subjects, 0-18-years-old, were retrospectively identified by searching in computerized databases of coded discharge diagnosis for patients with a diagnosis of pleural effusion and/or empyema and/or pulmonary infection in all the pediatric departments in Limousin hospitals. Medical records were reviewed by one of the authors and those with parapneumonic effusion and confirmed or suspected pneumococcal infection were included in the study. Data from the children hospitalized for parapneumonic pleural effusion were collected for two periods: period A, from July 2000 to July 2006, and period B, from July 2006 to July 2009 (before and after the generalization of the antipneumococcal vaccination). The main endpoint was the number of parapneumonic pleural effusion cases in each period in order to calculate the incidence within each period., Results: A total of 35 children were included: nine during period A and 26 during period B. The incidence was 1 per 100,000 children for period A and 5.8 per 100,000 for period B. Bacteriological tests allowed us to serotype eight S. pneumoniae over the two periods. All serotypes were non-vaccine serotypes (1, 3, and 19A)., Conclusion: This study demonstrates the increase in parapneumonic pleural effusion in the Limousin region., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Introduction: Distal humerus condyle fracture in children is rare. These fractures often mislead the emergency physician or surgeon. However, treatment adapted to the degree of displacement achieves excellent clinical and radiographic results. The objective of this study was to clarify indications for conservative treatment of lateral humeral condyle fracture in children., Materials and Methods: Twenty-two children who had sustained lateral humeral condyle fracture between January 2007 and January 2010 were reviewed in April 2010. At this consultation, the children underwent clinical and radiological examination. The Hardacre functional score was used to determine objective clinical outcome., Results: Conservative treatment was exclusive to cases of lateral condyle displacement equal to or less than 1mm. All other fractures were managed by surgical open reduction and fixation using cross-pinning. There was no statistically significant difference in clinical or radiological outcome between conservative and surgical management., Discussion: Lateral humeral condyle fracture is difficult to diagnose in children. The majority of poor results reported in literature relate to inadequate initial treatment. Given a radiological aspect of hemarthrosis of the elbow, the emergency physician prescribes multiple X-ray views of the affected elbow (anteroposterior, lateral and internal oblique). The clinical aspect of lateral humeral condyle fracture is often characteristic (ecchymosis facing the head of the radius). Nondisplaced or minimally displaced lateral humeral condyle fracture can be managed conservatively under close survey. However, secondary displacement under the cast is often difficult or impossible to detect, and outpatient surgery is therefore being increasingly indicated in our department., (Copyright © 2011. Published by Elsevier Masson SAS.)

We report a case of placenta percreta with bladder invasion in a multiparous woman with five previous cesarean sections. At 25 weeks of gestation, because of severe hematuria, antenatal diagnosis was easy. A multidisciplinary management was carried out with conservative treatment. Three repeated artery embolizations were necessary because of placenta volume and collateral vessels. None surgical treatment was realized to avoid hemorrhage and morbidity secondary to hysterectomy and partial cystectomy., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

Plant intoxications account for 5% of all intoxication cases according to French anti-poison centers. We report an uncommon case of intoxication with deadly nightshade (Atropa belladonna) in a 2-year-old child. The child presented at the ER with an atropinic syndrome, both central and peripheral, after ingestion of wild berries a few hours before. The fruit and leaves brought in by the mother allowed the anti-poison center to identify belladonna, in agreement with clinical findings. The child was kept in the intensive care unit for 48 h and progression was favorable with symptomatic treatment., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

Sternal cleft is a rare congenital abnormality. We report on a neonatal case of sternal cleft with median raphe with a follow-up of 3 years. We review the advantages of surgical repair in the neonatal period because the flexibility of the chest wall is maximal and compression of underlying structures is minimal., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

We report here on a familial case of centromeric heteromorphism of chromosome 18 detected by prenatal interphase fluorescence in situ hybridization (FISH) analysis transmitted by the mother to her fetus, and resulting in complete loss of one 18 signal. The prenatal diagnosis was performed by interphase FISH (AneuVysion probe set, and LSI DiGeorge 22q11.2 kit) because of the presence of an isolated fetal cardiac abnormality, and was first difficult to interpret: only one centromeric 18 signal was detectable on prenatal interphase nuclei, along with one signal for the Y and one for the X chromosome. The LSI DiGeorge 22q11.2 kit also showed the absence of one TUPLE 1 signal on all examined nuclei. In fact, the FISH performed on maternal buccal smear displayed the same absence of one chromosome 18 centromeric signal, combined with the presence of two TUPLE1 signals. All these results led to the diagnosis of an isolated 22q11.2 fetal microdeletion that was confirmed on metaphases spreads. This case illustrates once again that the locus specific (LSI) probes are more effective than the alpha centromeric probes for interphase analysis. The development of high-quality LSI probes for chromosomes 18, X and Y could avoid the misinterpretation of prenatal interphase FISH leading to numerous additional and expensive investigations., (Copyright 2010 Elsevier Masson SAS. All rights reserved.)

Agressive chemotherapy can lead to premature ovarian failure and loss of fertility in women and children. Embryo cryopreservation is an established clinical procedure of fertility preservation but with several limitations. Others options are available. Cryopreservation ovarian cortex tissu have to be suggested in case of high gonadotoxic treatment. It doesn't require puberty and delay in initiation of chemotherapy. The first birth in France after orthotopic graft of ovarian tissu thawed have been recently described with a promising process. Oocyte cryopreservation is available for women without partner but the experience is limited. Gonadotrophin-releasing hormone (GnRH) agonist therapy as ovarian protectants seem interesting. Follicular growth and maturation in vitro are still experimental., (Copyright 2010 Elsevier Masson SAS. All rights reserved.)

Inherited bleeding disorders contraindicate epidural analgesia because of the risk of epidural haematoma. However, there is no consensus about a minimal rate of factor XI required for its performance. We report here a case of epidural analgesia in a pregnant woman with a moderate and symptomatic factor XI deficiency, despite this theoretical contraindication. Indeed, the factor XI deficiency results in a haemorrhagic risk which is variable and not easily foreseeable. Nevertheless, no neurological after-effect or excessive bleeding occurred although no specific prophylaxis had been performed.

Many cases of not life saving transplanted organs were described with the aim of improving quality of life. Uterus graft could be an alternative solution to adoption or surrogacy for women who have uterine factor infertility. Different animals' studies with mouse, sheep or monkey showed feasibility of the surgical technique with large vessels patch. One case of human uterine transplant has been reported but failed. Cold storage of the uterus in protective solution has been explored with mouse, sheep and human. Only pregnancy after uterus graft by syngenic mouse has been published. Results about pregnancy after allograft with sheep or monkey are necessary before pregnancy after human uterus graft becomes a reality.

Currently, the role of dopaminergic and somatostatinergic agonists in the treatment of pituitary adenomas is quite well established. Nevertheless, a clearer understanding of the expression of dopaminergic and somatostatinergic receptors at the cellular level of pituitary adenomas as well as the development of newer analogues compounds may drastically change current therapeutic modalities. In particular, the emphasis on the co-expression of different receptors types or subtypes in adenomatous cells highlights functional interactions between receptors leading to an increase in their activity. Newer molecules are also in the process of development : new somatostatin analogues with more universal binding properties to different receptors subtypes, as well as chimeric molecules capable of binding to somatostatinergic and dopaminergic receptors. In the midst of GH-secreting pituitary adenomas, a positive correlation exists between the expression of Sst2 mRNA and the inhibition of GH release by somatostatin analogues. The involvement of Sst5 subtype in adenomas resistant to preferential Sst2 agonists has recently been proved. Another recently developed compound has a more universal Sst binding profile. This compound, named SOM-230, has a 25, 5 and 40 times higher binding affinity to Sst1, Sst3 and Sst5 receptors respectively, and 2,5 times lower affinity to Sst2, when compared to octreotide. SOM-230 could therefore allow for much more effective methods in treating patients suffering from acromegaly. Besides, the use of a chimeric molecule presenting a binding affinity to both Sst2 and D2 subtypes (BIM-23A287) inhibits the secretion of GH in ways similar to the Sst2 or D2 agonists used alone or concurrently but however in a concentration 50 times lower than that of the latter. The discovery of Sst5 and D2 subtypes at the level of corticotropic adenomas reveals newer therapeutic perspectives with promising preliminary results with the use of SOM-230 ; these finding lead to a rise in interest in cabergoline. In the midst of non-functioning pituitary adenomas, the expression of Sst2, Sst3 and D2 receptors will perhaps allow the use of combined therapies associating the new somatostatin analogues to the dopaminergic agonists or even use dopastatin (BIM-23A760, chimeric molecule Sst2-Sst5-D2). The preliminary results obtained in vitro with this molecule are actually encouraging since they show a dose dependent inhibition of the cellular replication mechanisms in 60 % of the cases. Finally, concerning prolactinomas the discovery of Sst5 receptors lead to consider the use of somatostatinergic agonists specific to the Sst5 receptor, SOM-230 in particular. Nevertheless, it seems that adenomas resistant to dopaminergic agonist due to a lack of expression of D2 receptor fail to express Sst5 receptors as well. On the other hand, dopastatin appears to be more efficient than cabergoline in the management of this type of adenomas. Therefore, the growing awareness concerning the mechanisms involved in the control of pituitary secretions as well as cellular proliferation will perhaps allow physicians to treat the pathology of pituitary adenomas, macroadenomas in particular, using solely pharmacological means instead of invasive surgical procedures and/or radiotherapy.

Several case reports suggest that rituximab (RTX) could be effective in steroid-dependent nephrotic syndrome, but RTX efficacy has not yet been studied in a series of patients. Safety and efficacy of RTX were assessed in a multicenter series of 22 patients aged 6.3-22 years with severe steroid-dependent nephrotic syndrome or steroid-resistant but cyclosporin-sensitive idiopathic nephrotic syndrome. Patients were treated with two to four infusions of RTX. Seven patients were nephrotic at the time of RTX treatment. Peripheral B cells were depleted in all subjects. Remission was induced in three of the seven proteinuric patients. One or more immunosuppressive (IS) treatments could be withdrawn in 19 patients (85%), with no relapse of proteinuria and without increasing other IS drugs. RTX was effective in all patients when administered during a proteinuria-free period in association with other IS agents. When relapses occurred, they were always associated with an increase in CD19 cell count. Adverse effects were observed in 45% of cases, but most of them were mild and transient. This study suggests that RTX could be an effective treatment for severe steroid-dependent nephrotic syndrome.

On one hand the etiology of Alzheimer's disease (AD) always remains unknown, on the other hand, the associated lesions are described in a precise way. Two types of lesions are visible on patients' brains affected by AD, associated with a neuronal and synaptic loss: amyloid plaques and the neurofibrillar degeneration. Alzheimer's disease is a pathology putting even at the moment multiple questioning as for its etiopathogeny, which results credibly from several processes.

Torticollis is an abnormal head posture. Clinical presentation includes ipsilateral tilt and controlateral rotation, and translation. Aetiologies are various including muscular entities, traumatic, inflammatory, infectious, tumour and non-muscular entities ocular, ORL or digestive. Majorities are minor and generally, a complete physical examination is sufficient to eliminate serious entities. Concretely, it is important to separate acute and chronic form. Traumatic torticollis justifies a systematic standard radiograph and then 15 days after dynamic evaluation. Septic torticollis evokes otolaryngological infection or spondylodiscitis. Chronic torticollis is congenital, postural or malformative. In case of recurrent form or torticollis with neurological abnormalities, TDM or RMI are necessary to eliminate tumoral diseases.

Introduction: Rhabdomyolysis with fibrate have been reported when fibrate are associated with statin or during renal insufficiency or hypothyroidism., Case Record: We describe one patient with diabetes mellitus treated by fenofibrate monotherapy since several years; 48 h after gliclazide therapy was introduced, rhabdomyolysis occurred., Discussion: Responsibilities of deshydratation and / or drug interaction with gliclazide. are discussed.

Sclerosing epithelioid fibrosarcoma is a rare tumor recently described. The histological presentation can be confused with certain soft tissue benign tumors and certain sarcomas. Metastatic spread is usually late in the natural course of the disease. We report a case of recurrent sclerosing epithelioid fibrosarcoma with pleural metastases which developed ten years after surgical resection of the primary tumor. The tumor was formed by small uniform round epithelioid cells with a clear cytoplasm. The tumor cells were strongly positive for vimentin. This clinical case is discussed in light of other cases reported in the literature.

Objective: Among obstetricians and midwives, estimate the percentage of smokers, appraise the proportion of medical professionals who mention the subject of nicotine addiction to their pregnant patients, and assess the quality of the information delivered to patients as well as their own experience in the field of tobacology., Patients and Methods: Between February and March 2002, 188 postal questionnaires were sent to obstetricians and gynecologists, midwives and midwife students of public hospitals in Limousin and private clinics in Limoges., Results: The response rate was 75,5% (142 answers) and the median age was 37 years old (20-59). 43,6% of the respondents were current smokers. Patients' smoking habits would be asked for and the risks of tobacco smoking would be explained by the majority of praticians and midwives. Minimal counsel to be given was known and given by 34,4% of gynecologists an 26,5% of midwives. Smoking cessation counselling was never proposed by 62% of gynecologists and 79% of midwives. Seventy per cent of them did not have any specific training in the field of tobacology during their studies., Discussion and Conclusion: There seems to be a deficiency in smoking cessation help for pregnant women in Limousin. Specifics programs and trainings for obstetricians, gynecologists, as well as midwives should be organized.

Background: The aim of this study was to validate a new method of quality control of medical records of the "Medicalisation of Information System Program" (P.M.S.I.) in Limoges university hospital., Methods: Lot quality assurance sampling (L.Q.A.S.), developed to meet industrial quality control needs, has been applied. This method allows to give an opinion on the quality of a lot from a sample randomised in this lot. Ten hospital units have been randomised. During three months, these medical units have recoded ten medical units abstracts (R.U.M.) which were randomised each month within their stays. If five or more R.U.M. had been modified on Principal Diagnosis (D.P.), Associated Significant Diagnosis (D.A.S.) or Diagnosis Related Group (D.R.G.), the lot was rejected. The hospital unit had then less 70% R.U.M. of good quality (alpha=5%). The global errors rates were compared to the results of usually performed quality control (1998)., Results: Nine hospital units were included. Hospital units which already had a low quality rate during the 1998 quality control had also an insufficient quality by L.Q.A.S. method. Among these, 33.7 [CI(95%): 21.6-45.8] R.U.M. have been modified. Medical units had a poorer data quality than surgical ones. No statistically significant difference was shown between 1998 errors rates and L.Q.A.S. rates, except for D.P. (p=0.002)., Conclusion: L.Q.A.S. is easy to apply, could be repeated more frequently than usual controls, and allows to detect hospital units which require a faster action. This process could be applied to P.M.S.I., in which the search of quality is necessary because of the new cost model which includes secondary D.R.G.

The economic assessment of treatments or medical strategies has been the subject of an increasing number of publications. The elevated costs and relative lack of efficacy of many treatments of chronic diseases such as lung cancer are an added impetus to such analyses. In the first part of this review, we summarise the theoretical basis of economic assessment, the tools available and the limitations of these methods. In the second part, we examine their application to the treatment of lung cancer. We discuss the evaluation of the cost of these cancers to society, economic assessment of new chemotherapeutic drugs, and the place of cost-effectiveness analysis in randomized trials. In the last part, we outline the interest and future of such considerations for clinicians. These methods, which provide complementary data for clinicians when making decisions on therapeutic options, will be adopted more widely in coming years.

Objectives: To review own cases of laparoscopic adrenalectomy in order to better ascertain limits therapeutic management and indications, compared with the literature data., Patients-Methods: Medical files of 15 patients after 17 laparoscopic adrenalectomies in the Dupuytren hospital of Limoges from February 1994 to November 1996, were analyzed 10 women and 5 men mean aged 59.4 years (22-77) were operated. Transperitoneal laparoscopic adrenal resection indications were: Conn adenoma (3), Cushing discase (2 bilateral resection), adrenal incidentaloma (9) and adrenal metastasis (1)., Results: Mean (range) of adrenal tumor size was 4 cm (0.8-7.5 cm). Operating times mean (range) was: 3 hours 40 minutes (1 h 50-6 h). Mean (range) of hospital stay was 3.4 day (3-6), marquedly reduced than open traditional adrenal surgery. Neither complication nor mortality per- or post-operative were present. But the laparoscopic operation required conversion to an open adrenal resection in 3 cases (18%)., Comments: Laparoscopic adrenalectomy offer real advantages: effectiveness and safety with a reduced time hospital discharge. Indications and adrenal size limits need to be precised on largest studies.

In 1983, the risk of developing severe shock during anaesthesia was estimated to be 1 case in 4600 procedures with an associated mortality rate of 6 per cent. The incidence of severe accidents has further increased since this time. This review focuses on the aspects of anaphylactoid shock (AS) in the context of iatrogenic responsibility involving drugs or substances used during anaesthesia or placed in contact with viscera. The elements predictive of anaphylactoid shock are described together with the arguments involved in a biochemical conception and a physiological conception. The authors emphasize the importance of the operative report and anaesthetic sheet to retrospectively establish the chronology of the events and to facilitate the allergologic work-up. The treatment of AS is an emergency. The therapeutic protocol is relatively well defined and, in addition to IV fluids, adrenalin is the drug of choice during the acute phase of AS. The therapeutic approach to certain particular cases is discussed and future therapeutic concepts are proposed. The aetiological agents of AS are discussed together with their respective frequencies in the specific case of the anaphylaxis reaction. Muscle relaxants are the leading cause of anaphylaxis (70 per cent of cases), especially suxamethonium, vecuronium and atracurium (43 per cent, 33 per cent and 6.8 per cent of cases, respectively). Latex present in various materials, especially surgical gloves, has become the most disturbing substance, responsible for 0.5 per cent of AS in 1989 vs 12.5 per cent of cases in 1991. Hypnotic drugs, although very widely used, are much more rarely incriminated.

An intranasal ethmoidectomy prospective study was carried out from 1985 to 1991 on 152 patients and 290 ethmoidectomies. This surgery is intended for patients who have been treated often for nasal polyps, chronic ethmoiditis and recurrent sinus barotrauma. A discrepancy was observed between patients satisfaction (82%) and clinical symptoms, given that results remain good in 75% of the cases three years afterwards but only in 60 cases after five years. There is a recurrence of nasal polyps in 36% of the cases.

Between 1985 and 1989, 395 patients with primary lung cancer were treated at the Percy military teaching hospital, Paris. Among them were 16 patients (4%) aged between 29 and 40 years (mean: 34 years) and smokers (mean cumulative tobacco consumption 24 P.A.). Six of these (37%) had lung parenchyma dystrophy with bilateral apical bullae of emphysema, 1 had a clean cavity left by a previous lung abscess and 1 had microcytic fibrosis resulting from histiocytosis X of the lungs and bones. In all cases cancer had developed in contact with bullous lesions or sequelae. Cancer was discovered during radiological mass screening in 6 cases (37.5%), on the finding of systemic or thoracic symptoms in 5 cases and because of a distant metastasis in 5 other cases. Pathological examination revealed an adenocarcinoma in 8 out of 16 patients and only one small-cell carcinoma. Nearly two-thirds of the patients had reached an advanced stage: 3 were in stage III A, 1 in stage III B and 6 in stage IV. Eight patients underwent curative surgery (7 lobectomies, 1 pneumonectomy). Survival was known with precision in 14 patients: 8 died after a mean follow-up of 15 months (range: 3 and 31 months); 3 were alive with an active cancer and 4 are still alive in complete remission after curative surgery. Six published studies totalling 387 cases are concordant in demonstrating that primary lung cancer is severe in adults below 40 years of age and that surgery is useful in such cases, even with N2 lymph node involvement.

Atherosclerosis is a multi-factor disease representing a real plague for developed countries, and spreading at an increasing rate in third world nations. In industrialized countries, coronary pathology occupied the first place in terms of morbidity and cardiovascular mortality, affecting more and more frequently younger people, thus constituting a serious problem of public health. Therefore, it seems absolutely necessary to carry out systematic screening for atherosclerosis amongst all high cardiovascular risk families groups, from infancy onwards. Lacking knowledge about the exact cause of atheroma, we had to determine what are commonly called the factors of cardiovascular risk. Among these, some are transmissible. Transmission may be direct: genetic and hereditary factors (sex, race, antecedents, dyslipemia, high blood pressure, diabetes) but it may also concern the transmission of a way of life. To prevent ischaemic heart disease, it is necessary to bring out change in behaviour from infancy onwards to detect high risk subjects and to correct every factor of cardiovascular risk upon which an action is possible.

The authors analysed the prevalence of atrioventricular conduction defects and sinus node dysfunction by Holter monitoring in three population groups with similar clinical characteristics: 50 controls (Group I), 17 physically fit subjects with an atrioventricular conduction defect on the resting ECG which was usually asymptomatic (Group II), and 48 professional pilots who were also physically fit but who had no conduction defects on resting ECG (Group III). The 24 hour recordings showed: --a similar incidence of sinus dysrhythmias and atrioventricular conduction defects in the three groups although the frequency of low degree block was a little higher in sportsmen; --a preponderance of the association of first degree block and nocturnal Luciani-Wenckebach periods, sometimes with episodes of Mobitz II block; --the absence of progression to high-degree atrioventricular block. Holter monitoring would therefore seem to be a good method of detecting and following up abnormalities of sinus node function and atrioventricular conduction defects in asymptomatic patients. In addition, it gives a qualitative and quantitative assessment of the recorded changes and provides a means of monitoring their evolution.

Rhythm disorders observed in space have always been minor but they are not unfrequent. They include: ventricular or supra-ventricular extrasystoles, nodal arrhythmias, auriculo-ventricular conduction disorders. There are several etiopathogenetic hypotheses: a strict selection must permit its elimination of an underlying heart disease; the potassium deficiency is often advanced but its role is not certain; the role of catecholamines is also discussed; the role of hypervagotony is extensively analysed as great clinical and electro-cardiographic evidence speaks for it. It can induce disorders which are more serious than those observed so far, particularly fibrillation or intermittent atrial flutter; weightlessness itself could partly condition the vagotonic state; and the effects of fluid shifts are also discussed from this point of view. The possible therapies for various atrial, nodal, ventricular disorders are reviewed, with greater detail for vagal atrial arrhythmias.