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Our work aimed to investigate the illnesses unrelated to systemic sclerosis (IUSS), diagnosed among patients with systemic sclerosis (SSc) throughout their follow-up in a referral tertiary care center.All the patients with SSc followed in the Internal Medicine Department of the University Hospital between October, 2014 and December, 2015, were included. We specifically reviewed the medical records of the patients who exhibited IUSS, defined as an illness that could not be considered as a typical clinical manifestation or as a usual complication of the disease.Two hundred patients were included, and 38 IUSS were diagnosed among 31 SSc patients, over a 4 years median follow-up period. These diagnoses included vascular diseases (26%), heart diseases (21%), neoplasia (8%), infectious diseases (6%), autoimmune diseases (5%), endocrinopathies (5%), and others (24%). The median follow-up time before IUSS diagnosis was two years. Seventeen (45%) of these diagnoses were considered in patients showing suggestive clinical signs. A specific therapy was delivered in 25 cases (66%). Group comparisons revealed that dyslipidemia was more frequent in patients with IUSS (OR = 2.6 [1.1-1.5]; p = 0.014), while no differences were found for the other characteristics. Especially, no association between auto-antibodies specificity and the occurrence of IUSS was found.This study focused on IUSS in SSc patients and highlights the need for a polyvalent clinical approach all along the follow up of SSc patients.

Sideroblastic anemia is a rare cause of microcytic anemia, which is characterized by ring sideroblasts on bone marrow aspirate. This anemia can be congenital or acquired.We report the case of an alcoholic 49-year-old man who presented with a severe microcytic sideroblastic anemia related to pyridoxine (B6 vitamin) deficiency. Acid folic deficiency was associated. The blood count normalized within one month after vitamin supplementation.Pyridoxine deficiency must be sought in sideroblastic anemia in patients at risk.

Though several assessment tools for resident professional skills based on workplace direct observation have been validated, they remain scarcely used in France. The objective of this study was to evaluate the reliability and the validity of a workbook including several assessment forms for different components of the professional competency.Three assessment forms have been tested over a period of 6 months in a multicentric study including 12 French internal medicine departments: the French version of the mini-CEX, an interpersonal skills assessment form (OD_CR) and the multisource feedback form (E_360). Reliability has been assess using the intra-class correlation coefficient (ICC) and the Cronbach alpha coefficient. Arguments for validity have been provided looking at the ability of the forms to detect an increase in the scores over time and according to the level of experience of the resident.Twenty-five residents have been included. The Cronbach alpha was of 0.90 (n=70) with the mini-CEX, 0.89 with the OD_CR (n=62) and 0.77 with the E_360 (n=86). ICC showed a wide variation according to the items of the mini-CEX and the OD-CR probably due to the poor number of observations performed by residents. The scores of most of the items of these two forms increased between M1 and M6. The scores of the E_360 were high: 7.3±0.8 to 8.3±2.4 (maximum 9) and did not vary according to the level of experience.This study suggest that it would be difficult to ensure a sufficient reliability for professional skills assessment using these tools given our available current human and material resources. However, these assessment forms could be added to the resident portfolio as supports for the debriefing in order to document their progression during their formation.

Uncertainty arises when information is not sufficient to predict the prognosis or the outcome following an intervention. It is omnipresent in medical daily practice, and will follow each practitioner all along his career. The communication or merely even the feeling of uncertainty is frequently perceived as a negative experience. Nevertheless, rather than a sign of weakness, feeling uncertain reflects a dynamic state of self-reassessment which should be experienced by each doctor involved in providing his patients with the best care. Furthermore, uncertainty may lead to disastrous consequences for practitioners who have not been properly prepared to it, involving the sphere of emotions (stress, anxiety, burn-out) as well as the behavioral field (misdiagnosis, excessive testing, impaired communication…). A growing number of observations, clinical or educational studies, supports the idea that learning uncertainty should be a critical prerequisite to a valuable medical practice. Through this literature review, we propose herein a conceptual glance on uncertainty. Then, we expose some sources of uncertainty in daily practice and teaching, its consequences, and the main factors that contribute to the wide variety of the individual relationship with uncertainty. We finally aim to prompt a global reflexion, falling within an uncertainty competency-based education approach, assuming that our obligation to professionalize the students necessitates making them skilled in dealing with uncertainty.

Adult-onset Still's Disease is a rare multisystemic inflammatory disease characterized by fever, maculo-papular erythematous rash and arthralgia. Adult-onset Still's disease is a diagnosis of exclusion.We report the case of a 33 years old man, hospitalized for fever, arthralgia and throat manifestations, leading to Adult-onset Stills's Disease diagnosis. Cardiac ultrasound revealed tricuspid vegetation. Once infectious causes were ruled out, the vegetation was related to Adult-onset Still's Disease according to Fautrel and Yamaguchi criteria. The patient was treated with systemic high doses corticosteroid and cardiac surgery. Histological examination excluded infection and neoplasia, and showed cruoric and fibrinous vegetation.Non-infectious endocarditis, with a vegetation made of cruoric and fibrinous material, is a rare complication of Adult-onset Still's disease.

Medication reconciliation (MR) is a systematic and comprehensive review of all medication a patient is taking. In this study, a discharge medication sheet (DMS) is given to patients upon discharge: it contains discharge prescription and any changes made to admission prescription with justifications. The aim of this study is to explore general practitioners' (GP) perceptions of this DMS in order to suggest improvements.In this prospective observational study, individual semi-directed interviews were conducted with GPs who received a DMS following the hospitalization of one of their patients. Answers were grouped by topic and subjected to descriptive analysis.Between October 2015 and July 2016, 33 DMS were completed. Among the 33 GPs, 16 had seen their patients with their DMS and agreed to be interviewed. The DMS was very appreciated and improved care pathway. However, this study highlights transmission difficulties for this sheet, attributed in particular to a lack of information of practitioners and patients and to the paper format, which appears to be inadequate. The main suggested improvement is real-time transmission of the DMS via email.Practitioners' opinion is in favor of the use of a DMS. Certain specific points need to be improved, such as better information of practitioners and patients, and transmission of the DMS via a secure email system.

National audience; Introduction L’atteinte œsophagienne est fréquente chez les patients présentant une sclérodermie systémique. Le diagnostic est confirmé par la manométrie œsophagienne. La sensibilité et la spécificité de la manométrie œsophagienne ont été améliorées par l’avènement de la manométrie de haute résolution. L’objectif principal de cette étude observationnelle est de caractériser les troubles moteurs œsophagiens par manométrie de haute résolution, à la lumière de la troisième version de la classification de Chicago, chez des patients présentant une sclérodermie systémique selon les critères de l’EULAR/ACR 2013. Patients et méthodes Trente-six patients répondant au diagnostic de sclérodermie systémique selon les critères EULAR/ACR 2013 et ayant bénéficié d’une manométrie de haute résolution dans un centre de compétence français ont été inclus de manière consécutive. Les résultats de la manométrie de haute résolution ont été interprétés selon la troisième version de la classification de Chicago. Les caractéristiques cliniques, biologiques et les atteintes viscérales des patients ont également été relevées et confrontées aux données manométriques. L’atteinte pulmonaire était évaluée selon la méthode de Goh et al. en aveugle des données manométriques. Résultats L’âge moyen à l’inclusion était de 58,4 ans (SD = 14,2 ans) ; 83,3 % des patients étaient de sexe féminin. 22,2 % des patients avaient une sclérodermie systémique avec atteinte cutanée diffuse selon la classification de LeRoy ; 67 % des patients avaient un pyrosis, 30,6 % une dysphagie, 33,3 % une dyspepsie. 11 % des patients avaient des épisodes de fausses routes. D’après les résultats de la manométrie de haute résolution, 55,6 % des patients avaient un apéristaltisme, 33,3 % une hypotonie du sphincter inférieur de l’œsophage et 19,4 % une hypotonie du sphincter supérieur de l’œsophage. En analyse univariée, il n’était pas retrouvé d’association significative entre la présence d’une hypotonie du sphincter inférieur de l’œsophage et les caractéristiques cliniques ou biologiques quelles qu’elles soient. La présence d’un apéristaltisme était associée avec une symptomatologie de pyrosis (p = 0,004). L’apéristaltisme était associé de manière significative avec la présence de télangiectasies (p = 0,007) et d’anticorps anti-centromères (p = 0,038). La présence de calcinose ou d’une sclérodactylie n’était pas spécifiquement associée à une atteinte sphinctérienne ou motrice œsophagienne. Vingt-neuf patients (80,6 %) avaient bénéficié d’une TDM thoracique dans l’année de la réalisation de la manométrie de haute résolution. Parmi eux, 7 patients étaient atteints d’une pneumopathie interstitielle diffuse sévère (atteinte interstitielle de plus de 20 % du parenchyme pulmonaire évaluée sur 5 coupes standardisées). Il existait une association significative entre la présence d’un apéristaltisme et la présence d’une dilatation œsophagienne en TDM (p = 0,002) et la présence d’une atteinte pulmonaire sévère (p = 0,005, OR = 6,85 IC95 % (1,12–41,82)). Discussion L’association retrouvée entre apéristaltisme en manométrie de haute résolution et sévérité de la PID est confirmée par ce travail. Néanmoins un lien de causalité n’est pas certain. Une étude prospective évaluant la mise en évidence d’une atteinte œsophagienne par manométrie de haute résolution au moment du diagnostic de sclérodermie systémique et l’atteinte pulmonaire par TDM à distance serait nécessaire. L’entité nosologique représentée par le syndrome CREST n’est pas retrouvée dans cette cohorte de patients atteints de sclérodermie systémique selon les critères EULAR/ACR 2013, où 2 des critères diagnostiques (présence de calcinose et sclérodactylie) ne sont pas associés de manière significative à l’atteinte œsophagienne d’après les résultats de la manométrie de haute résolution. Conclusions Dans cette série, chez plus de la moitié des patients atteints de sclérodermie systémique la manométrie de haute résolution permet de détecter un apéristaltisme. La présence de troubles moteurs œsophagiens est associée à une atteinte pulmonaire plus sévère en TDM

Venous thromboembolic disease is a multifactorial, frequently recurrent pathology, whose treatment is based on anticoagulation. As part of the etiological investigation, screening for an inherited thrombophilia is framed by French guidelines published in 2009. The aim of the study is to assess the contribution of inherited thrombophilias testing in common practice.This is a retrospective single-center observational study. Over a period of a year, all records of patients who were screened for a hereditary thrombophilia were analyzed. The conformity of the indication of hereditary thrombophilia workup in balance with the guidelines, its completeness and therapeutic impact were studied.Of the 494 records analyzed, 225 were related to venous thromboembolism. Among them, there were 162 pulmonary embolisms or deep vein thrombosis of the lower limbs. In this subgroup, 57 % of records complied with guidelines and 69 % were complete. Thirty-four thrombophilias were highlighted: 4 protein S deficiencies, 1 protein C deficiency, 4 combined deficiencies, 17 factor V Leiden mutations and 8 factor II G20210A mutations. For one patient, hereditary thrombophilia diagnosis had profoundly changed the curative therapeutic approach.Adherence to French guidelines remains limited. In clinical practice, diagnosis of hereditary thrombophilia has little impact on the curative therapeutic approach in venous thromboembolic disease.

The yellow nail syndrome is a rare disorder described for the first time in 1964. The pathophysiology remains unclear. Its definition is based on a clinical triad of yellow nails, lymphoedema and chronic respiratory disorders including pleural effusions and bronchiectasis.We describe a retrospective series of five patients diagnosed with the yellow nail syndrome. All the patients were male, aged from 52 to 71 years (median=56). Three patients were diagnosed with the classic triad, whereas the other two had only yellow nails and bronchiectasis. Yellow nails and chronic sinusitis were present in all five patients. We also report atypical manifestations such as a transudative pleural effusion and facial oedema. The yellow nail syndrome was associated with cancer in two cases.More common alternative diagnoses must be excluded. The association with cancer should be explored. The treatment is only symptomatic.

Acquired hemophilia due to an inhibitor of factor VIII is a rare clinical situation.Rituximab is now used in the treatment of acquired hemophilia. We report two cases of acquired hemophilia treated by rituximab with efficiency.Rituximab appears to be a first line immunosuppressive therapy in acquired hemophilia, especially in post-partum hemophilia.

Cryptococcal infections are frequent in HIV-infected patients and are regularly looked after. This infection may occur in others immunosuppressives situations and, in those cases, diagnosis is often delayed.We report four cases of cryptococcal infections in patients whose immunosuppression isn't related with HIV infection but due to chronic lymphocytic leukemia, giant cell temporal arteritis, gastric neoplasm and lupus. Diagnosis, prognostic and treatment are detailed.Four patients aged from 25 to 76 presented a cryptococcal infection (three meningitis). A woman died at the admission. Another died seven years later. The two others are still alive under treatment. When infected, all patients were immunodeficiency.Cryptococcal infection may occur in patients non-HIV-infected patients. Early detection is needed to improve prognostic.

The frequency of amyloidosis is not well known in France. We compiled a register of amyloidosis diagnosed from 1995 to 1999 in the University Hospital of Rennes.This retrospective study was performed between 01 January 1995 and 31 december 1999. Diagnosis was assessed on positivity of red Congo by anatomopathology. Immunohistochemistry allowed the definition the type of amyloidosis. Clinical data, staging and outcome of patients were analysed.Forty-three amyloidosis were diagnosed (27 women, 16 men) with an incidence of 8,6 new cases per year. Mean age was 63.7 years. Five diagnosis were realised in 1995, six in 1996, six in 1997, 12 in 1998, 14 in 1999. Twenty amyloidosis were AL type (46.5%), seven AA (16.3%), 1 beta2 microglobulin type, 15 (35%) remained of undetermined type. Thirty-three amyloidosis (77%) were systemic, 10 were localized to one organ (23%). When diagnosis was made, biopsies concerned affected organs in 86% of the cases, accessory sites (labial salivary glands, bone marrow) in only 14% of the cases. Twenty-five patients died (58%). Two deaths were treatment-related, 16 to amyloidois, seven patients died of another complaint.Increased incidence of amyloidosis needs to be confirmed. We emphasize the importance of immunohistochemical typing on frozen samples, the value of accessory biopsies and the need for complete extension staging.

Monoclonal gammopathy of undetermined significance (MGUS) are very frequent in the elderly. Its incidence is about 3% after 70 years. We have performed a retrospective study of patients aged of more than 70 years who were examined until 1995 for a MGUS.The study included 51 patients: 24 men and 27 women. Information about evolution was obtained by medical file or by asking medical practitioner. We know the evolution of all patients until January 1st 2000. The median follow-up is 5.8 years (70 months).The MGUS remained stable for 34 patients (67%) with a median follow-up of 83 months (12 to 180 months). Nine patients (17%) developed malignant transformation of MGUS (6 multiple myeloma, 2 Waldenström macroglobulinemia, 1 malignant lymphoma). Eight (15%) developed a cancer. Twelve died without evidence of multiple myeloma or related disorder. The actuarial probability of malignant transformation at 5 years was 12%.The risk of malignant transformation of MGUS doesn't decrease in the elderly. A regular and prolonged follow-up is necessary.

Monoclonal gammopathy of undetermined significance is an asymptomatic disorder associated with serum monoclonal immunoglobulin spike. Its incidence is about 1% in patients of 50 years of age, and rapidly increases in elderly patients.Within the 20 years following diagnosis, about 25% of patients will evolve towards either multiple myeloma (for patients with IgG or IgA) or malignant lymphoproliferative disorder (for patients with IgM). Definition, circumstances associated with a transient monoclonal spike, and currently available parameters used for differential diagnosis with either multiple myeloma or malignant lymphoproliferative disorder are successively discussed. One part of the most usual biological parameters is of prognostic value, and is reviewed in more detail. Recent data concerning immunophenotype, cytogenetics and molecular biology of plasma cells reinforce the link between the asymptomatic condition and multiple myeloma. In monoclonal gammopathy of undetermined significance, some plasma cells resemble normal or reactive plasma cells, whereas others mimic those found in multiple myeloma.The most recent biological data are also discussed in order to evaluate whether some would help to discriminate those patients who will remain asymptomatic lifelong from those who will evolve towards multiple myeloma.

In hematologic malignancies, respiratory syncytial viral infections can be explained by neutropenia, and cellular and humoral immunodepression, and may cause severe respiratory infections.Four patients with hematologic malignancies developed a severe respiratory syncytial virus infection. Three of them had previously received autologous bone marrow transplantation (ABMT). Progress was favorable for three patients. One patient died of acute respiratory failure.When such patients present with respiratory symptoms, especially during the winter months, they should be screened for RSV. Bronchoalveolar lavage allowed quick and accurate diagnosis by immunofluorescence. Treatment with nebulized ribavirin is controversial. Its use may be interesting in patients with high-risk factors (intensive chemotherapy, ABMT, diffuse pneumonia with hypoxemia).

This review is aimed at defining the frequency, anatomical and clinical presentation, pathogenesis, predictive factors and treatment of malignant lymphoproliferative diseases occurring in the course of Sjögren's syndrome.The frequency of non-Hodgkin's lymphoma (NHL) is estimated to be about 7%. Other malignant lymphoproliferative diseases (Waldenstrom's macroglobulinemia, multiple myeloma, Hodgkin's disease) are rarely observed. NHL is most frequently extranodal (affecting the salivary glands, stomach, lung, etc) in low grade malignancy (MALT lymphoma [mucosa associated lymphoid tissue]). The pathogenesis of NHL in Sjögren's syndrome is a multi-step process, including B cell monoclonal proliferation, oncogenic and/or infectious agents, and/or cytokines. Various predictive factors such as persistent enlargment of parotid glands, adenopathy, splenomegaly, mixed cryoglobulinemia, monoclonal gammopathy, suggest potential lymphoma evolution. The treatment of Sjögren's syndrome-associated NHL depends on the type of lymphoma. Moreover, in patients with low-grade lymphoma therapeutical strategies varies according to the stage and evolution of the disease.Future prospective longitudinal studies should permit to define the best criteria for malignant transformation and to improve therapeutical strategies.

Two large epidermoid cysts of the spleen have been accidentally discovered in two young men. These cysts are uncommon and represent about 10% of the non parasitic benign cysts of the spleen. From these cases, the authors reviewed the literature. These cysts, which are caused by an abnormal development during the seventh week of the intra-uterine life, are often asymptomatic but the occurrence of a complication may enable the diagnosis. This diagnosis relies on the surgical ablation of the cyst and the histopathologic examination. The surgery should be as conservative as possible.

The authors report the case of a 24-year-old woman with paradoxical embolism of the right arm subsequent to childbirth. Patent foramen ovale was diagnosed by right cardiac catheterization and contrast echocardiography. On the basis of clinical, immunoscintigraphic and radiologic data, the pelvic region was the only possible origin of the embolic process. The main interest of this case is that arterial ischemic signs were indicative of an embolic process of pelvic origin diagnosed by scintigraphy using radiolabeled antifibrin antibody.

The authors report the case of a 30-year-old woman with Takayasu's disease revealed by acute leg ischemia and complicated by abortion during the fourth month of pregnancy. Clinically suspect lesions were confirmed by angiography showing a severe stenosis at the aorto-iliac bifurcation complicated by overlying mild ectasic lesions and a large thrombus. A total occlusion of embolic origin was noted in the right popliteal artery. An attempt to open up the thrombosis at the bifurcation led unfortunately to massive rethrombosis requiring implantation of an aorto-iliac prosthesis. Histopathological examination of the aortic surgical specimen indicated Takayasu's disease; the placenta showed multiple infarcts. The complete angiographic study revealed no arterial involvement in any other region. Five months after the initial operation, rapid development of claudication in both lower limbs suggested stenosis at the prosthesis implantation sites which was confirmed by angiography. Initiation of general corticosteroid therapy led to partial remission in less than two weeks. The unusual feature in this case was the revelation of the affection by distal arterial embolic manifestations, which have been reported only exceptionally in the literature. Moreover, isolated involvement of the aorto-iliac bifurcation constitutes a rare form of Takayasu's disease. With respect to therapy, the implantation of a prosthesis was complicated by severe stenoses of the junction zones, which is unfortunately an event in the course of this affection. The normality of biologic inflammation studies did not suggest an initial need for corticosteroid therapy, especially in the case of an isolated arterial manifestation.(ABSTRACT TRUNCATED AT 250 WORDS)

We have compared the properties of the deoxythymidine kinases extracted from the oviducts of newts which have been stimulated by estradiol or from control ones. The estradiol treatment affects the apparent Km which decreased from 10(-5) M (control) to 5.10(-6) M (stimulated). Experiments in which we mixed extracts of control and stimulated oviducts indicate that the increase of the affinity cannot be interpreted as the result of an hormonal action upon an enzymatic effector. The study of heat denaturation (45 degrees C) indicates that the oviduct of stimulated animals and control ones contains two thymidine kinases (one is heat resistant: ES; the other is heat denaturated: EL). The estradiol increases the activity of both enzymes (EL and ES) but this action is differential: the EL activity is twice or five time more increased than ES activity after 6 or 12 days of hormonal treatment.

The jelly envelopes, surrounding amphibian eggs and playing a major part in the initial steps of fertilization, are deposited around the eggs as they traverse the different parts of the oviduct. These envelopes form 5 to 8 morphologically distinct layers according to the species, i.e. in Anura and Caudata they are organized differently, and are composed of about ten different macromolecular components (glycoproteins). The innermost layer is rich in sulfate and poor in fucose, a carbohydrate abundant in the other layers. This is due to a fucosyltransferase deficit in the cranial part of the oviduct secreting this layer. The cranial and the medial oviduct secretory products show a lectin carbohydrate-like reaction which is pH-dependent and can be inhibited by carbohydrates like glucuronolactone. This reaction has made it possible to isolate, by affinity chromatography, the main secretory component of the cranial oviduct (Spop). This paper discusses the main functions of the jelly layers of the egg, with particular reference to fertilization. The role of their macromolecular components in sperm capacitation is clear in Anura; experimental investigation in Urodeles is more difficult. We propose several hypotheses to explain the molecular interactions leading to capacitation. Block to polyspermy in amphibians is discussed.

"Antigen-antibody" like reactions between products of secretion of different parts of the Pleurodeles waltlii Michah's oviduct have been observed using the usual immunodiffusion methods. The possible physiological importance of such reactions in egg-jelly formation and in the phenomena associated with the fecondation (particularly capacitation) is discussed.