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H : 소장처정보

T : 목차정보

La Revue de médecine interne 16건

  1. [해외논문]   Type 3 Gaucher disease, also an adult disease?  

    Leurs, A. (Service de médecine interne et d'immunologie clinique, centre national de références des maladies systémiques et auto-immunes rares, université) , Chepy, A. (de Lille, 2, hôpital Huriez, 59037 Lille cedex, France ) , Detonellaere, C. (Service de médecine interne et d'immunologie clinique, centre national de références des maladies systémiques et auto-immunes rares, université) , Pascal, L. (de Lille, 2, hôpital Huriez, 59037 Lille cedex, France ) , Gallois, P. (Service de médecine interne et d'immunologie clinique, centre national de références des maladies systémiques et auto-immunes rares, université) , Tran, T.-A.-C. (de Lille, 2, hôpital Huriez, 59037 Lille cedex, France ) , Caillaud, C. (Service d'onco-hématologie, centre de référence de Lille des maladies héréditaires du métabolisme (maladie de Gaucher adulte), groupement des hôpitaux de l'institut catholique de Lille, hôpital Saint-Vincent, u) , Hatron, P.-Y. , Rose, C.
    La Revue de médecine interne v.39 no.7 ,pp. 589 - 593 , 2018 , 0248-8663 ,

    초록

    Abstract Introduction Gaucher disease is a genetic lysosomal storage disorder due to a glucocerebrosidase deficiency. Type 3, including neurological impairment, may have a specific phenotype in the context of the D409H mutation. Observation We report the case of a 22-year-old woman who presented with Gaucher disease. Enzyme replacement therapy by imiglucerase was followed by rapid clinical and biological improvement. However, communication difficulties, which were initially attributed to the language barrier, revealed neurological impairment. After complementary assessment, the diagnosis of type 3 Gaucher disease was suspected. Gene analysis of the glucocerebrosidase showed a homozygous D409H mutation. Conclusion This mutation results in calcified heart valves, corneal opacities, alteration of oculomotricity and hydrocephalus. The mild manifestation at onset and the late neurological involvement in the medical history make the diagnosis more difficult. This particular clinical phenotype deserves to be known in adult medicine departments.

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    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

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  2. [해외논문]   Systemic sclerosis: Efficacy of intravenous immunoglobulins in severe cardiac involvement?  

    Cacciatore, C. (Service de médecine interne and inflammation (DHU i2B), université) , Riviere, S. (Paris 06, hôpital Saint-Antoine, AP–HP, 75012 Paris, France ) , Cohen, A. (Service de médecine interne and inflammation (DHU i2B), université) , Gatfosse, M. (Paris 06, hôpital Saint-Antoine, AP–HP, 75012 Paris, France ) , Ederhy, S. (Service de cardiologie, UPMC, hôpital Saint-Antoine, AP–HP, 75012 Paris, France ) , Fain, O. (Service de médecine interne and inflammation (DHU i2B), université) , Mekinian, A. (Paris 06, hôpital Saint-Antoine, AP–HP, 75012 Paris, France )
    La Revue de médecine interne v.39 no.7 ,pp. 594 - 596 , 2018 , 0248-8663 ,

    초록

    Abstract Background The heart involvement in systemic sclerosis is frequent and can touch various sites. The prognosis in the presence of heart disease is poor, but few data are available about its management. Case We report the case of 48 years old woman with systemic sclerosis which presented severe heart involvement. She has severe heart failure, supraventricular arrhythmias and symptomatic pericarditis, which required surgical intervention and immunosuppressive drugs (steroids with rituximab). Despite this treatment, she has persistent severe heart impaired function and intravenous immunoglobulins have been initiated. She experienced progressively the improvement of dyspnea, of heart systolic ejection fraction and decrease of Rodnan scale. Conclusion Our case illustrates a severe heart involvement in systemic sclerosis which have been improved by intravenous immunoglobulins.

    원문보기

    원문보기
    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

    이미지

    Fig. 1 이미지
  3. [해외논문]   Movement disorders in a 32-year-old woman  

    Diot, E. (Service de neurologie, hôpital Lucien-Hussel, montée du Dr-Chapuis, 38209 Vienne cedex 9, France ) , Brugirard, S. (Service de neurologie, hôpital Lucien-Hussel, montée du Dr-Chapuis, 38209 Vienne cedex 9, France ) , Hascar, T. (Service de neurologie, hôpital Lucien-Hussel, montée du Dr-Chapuis, 38209 Vienne cedex 9, France ) , Vallet, P. (Service de neurologie, hôpital Lucien-Hussel, montée du Dr-Chapuis, 38209 Vienne cedex 9, France ) , Vallet, A.-E. (Service de neurologie, hôpital Lucien-Hussel, montée du Dr-Chapuis, 38209 Vienne cedex 9, France ) , Badet, F. (Service de médecine interne, maladies infectieuses et tropicales, hôpital Lucien-Hussel, montée du Dr-Chapuis, 38209 Vienne cedex 9, France ) , Bouaziz, A. (Service de médecine interne, maladies infectieuses et tropicales, hôpital Lucien-Hussel, montée du Dr-Chapuis, 38209 Vienne cedex 9, France ) , Adelaide, L. (Service de médecine interne, maladies infectieuses et tropicales, hôpital Lucien-Hussel, montée) , Ré , gent, A.
    La Revue de médecine interne v.39 no.7 ,pp. 597 - 599 , 2018 , 0248-8663 ,

    초록

    Abstract Background The heart involvement in systemic sclerosis is frequent and can touch various sites. The prognosis in the presence of heart disease is poor, but few data are available about its management. Case We report the case of 48 years old woman with systemic sclerosis which presented severe heart involvement. She has severe heart failure, supraventricular arrhythmias and symptomatic pericarditis, which required surgical intervention and immunosuppressive drugs (steroids with rituximab). Despite this treatment, she has persistent severe heart impaired function and intravenous immunoglobulins have been initiated. She experienced progressively the improvement of dyspnea, of heart systolic ejection fraction and decrease of Rodnan scale. Conclusion Our case illustrates a severe heart involvement in systemic sclerosis which have been improved by intravenous immunoglobulins.

    원문보기

    원문보기
    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

    이미지

    Fig. 1 이미지
  4. [해외논문]   Renal abnormalities  

    Messika, J. (Service de réanimation médico-chirurgicale, hôpital Louis-Mourier, AP–HP, 178, rue des Renouillers, 92700 Colombes, France ) , Nahmani, Y. (Service de réanimation médico-chirurgicale, hôpital Louis-Mourier, AP–HP, 178, rue des Renouillers, 92700 Colombes, France ) , Aouad, P. (Service de radiologie, hôpital Louis-Mourier, AP–HP, 178, rue des Renouillers, 92700 Colombes, France ) , Berquier, G. (Service de réanimation médico-chirurgicale, hôpital Louis-Mourier, AP–HP, 178, rue des Renouillers, 92700 Colombes, France ) , Roux, D. (Service de réanimation médico-chirurgicale, hôpital Louis-Mourier, AP–HP, 178, rue des Renouillers, 92700 Colombes, France ) , Delahousse, M. (Service de néphrologie et transplantation rénale, hôpital Foch, 40, rue Worth, 92151 Suresnes, France)
    La Revue de médecine interne v.39 no.7 ,pp. 600 - 601 , 2018 , 0248-8663 ,

    초록

    Abstract Background The heart involvement in systemic sclerosis is frequent and can touch various sites. The prognosis in the presence of heart disease is poor, but few data are available about its management. Case We report the case of 48 years old woman with systemic sclerosis which presented severe heart involvement. She has severe heart failure, supraventricular arrhythmias and symptomatic pericarditis, which required surgical intervention and immunosuppressive drugs (steroids with rituximab). Despite this treatment, she has persistent severe heart impaired function and intravenous immunoglobulins have been initiated. She experienced progressively the improvement of dyspnea, of heart systolic ejection fraction and decrease of Rodnan scale. Conclusion Our case illustrates a severe heart involvement in systemic sclerosis which have been improved by intravenous immunoglobulins.

    원문보기

    원문보기
    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

    이미지

    Fig. 1 이미지
  5. [해외논문]   An aortic abnormality  

    Levasseur, T. (Service de cardiologie et maladies vasculaires, hôpital Robert-Debré, CHU de Reims, avenue Général-Koenig, 51100 Reims, France ) , N'Guyen, Y. (Service de médecine interne, maladies infectieuses et immunologie clinique, hôpital Robert-Debré, CHU de Reims, avenue Général-Koenig, 51100 Reims, France ) , Aludaat, C. (Service de chirurgie cardiothoracique, hôpital Robert-Debré, CHU de Reims, avenue Général-Koenig, 51100 Reims, France ) , Espinosa, M. (Service de cardiologie et maladies vasculaires, hôpital Robert-Debré, CHU de Reims, avenue Général-Koenig, 51100 Reims, France ) , Metz, D. (Service de cardiologie et maladies vasculaires, hôpital Robert-Debré, CHU de Reims, avenue Général-Koenig, 51100 Reims, France)
    La Revue de médecine interne v.39 no.7 ,pp. 602 - 603 , 2018 , 0248-8663 ,

    초록

    Abstract Background The heart involvement in systemic sclerosis is frequent and can touch various sites. The prognosis in the presence of heart disease is poor, but few data are available about its management. Case We report the case of 48 years old woman with systemic sclerosis which presented severe heart involvement. She has severe heart failure, supraventricular arrhythmias and symptomatic pericarditis, which required surgical intervention and immunosuppressive drugs (steroids with rituximab). Despite this treatment, she has persistent severe heart impaired function and intravenous immunoglobulins have been initiated. She experienced progressively the improvement of dyspnea, of heart systolic ejection fraction and decrease of Rodnan scale. Conclusion Our case illustrates a severe heart involvement in systemic sclerosis which have been improved by intravenous immunoglobulins.

    원문보기

    원문보기
    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

    이미지

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  6. [해외논문]   LittErature commentEe juillet 2018  


    La Revue de médecine interne v.39 no.7 ,pp. 604 - 607 , 2018 , 0248-8663 ,

    초록

    Abstract Background The heart involvement in systemic sclerosis is frequent and can touch various sites. The prognosis in the presence of heart disease is poor, but few data are available about its management. Case We report the case of 48 years old woman with systemic sclerosis which presented severe heart involvement. She has severe heart failure, supraventricular arrhythmias and symptomatic pericarditis, which required surgical intervention and immunosuppressive drugs (steroids with rituximab). Despite this treatment, she has persistent severe heart impaired function and intravenous immunoglobulins have been initiated. She experienced progressively the improvement of dyspnea, of heart systolic ejection fraction and decrease of Rodnan scale. Conclusion Our case illustrates a severe heart involvement in systemic sclerosis which have been improved by intravenous immunoglobulins.

    원문보기

    원문보기
    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

    이미지

    Fig. 1 이미지

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