La Revue de médecine interne
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Shock states are the leading causes of intensive care admission and are nowadays associated with high morbidity and mortality. They are driven by a complex physiopathology and most frequently a multifactorial mechanism. They can be separated in whether a decrease of oxygen delivery (quantitative shock) or an abnormal cell distribution of cardiac output (distributive shock). ⋯ Vasoconstrictive drugs or inotropic support still often remain necessary. The primary goal of initial resuscitation should be not only to restore blood arterial pressure but also to improve clinical perfusion markers. On the biological side, decrease of lactate concentration is associated with better outcome.
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The diagnosis of bilateral papilledema implies emergency medical care to look for intracranial hypertension and arteritic ischemic neuropathy. However, other causes must also be mentioned, including drugs. Too often underrated because of their usual benignity, drug side ophthalmological effects can be severe and are typically bilateral. ⋯ Bilateral edematous optic neuropathy is a known side effect of amiodarone, uncommon but to be known because of the large number of patients benefiting from this treatment.
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Chronic inflammatory demyelinating polyradiculoneuropathies are acquired demyelinating neuropathies belonging to the group of autoimmune neuropathies. Since specific biological markers are present in less than 10% of cases, the diagnosis is based on the clinical and electrophysiological analysis of each patient. ⋯ A second line treatment may be proposed in case of no response, intolerance or inaccessibility to the three reference treatments. While some immunosuppressants or monoclonal antibodies can sometimes be very effective, there is currently no predictive marker or recommendation available to determine which treatment will be most appropriate for which patient.
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In the past ten years, the better understanding of the pathophysiological mechanisms underlying inflammatory and autoimmune diseases has led to the emergence of many targeted therapies. Among them, the Janus kinase inhibitors are acting upstream in the inflammatory cascade of several key cytokines in disorders such as rheumatoid arthritis, ulcerative colitis or psoriasis. ⋯ Preclinical data and therapeutic trials indicate their efficacy in other autoimmune or inflammatory conditions, such as lupus, dermatomyositis, ankylosing spondylitis, sarcoidosis and giant cell arteritis. This review provides a summary of current use and advancement of knowledge in the use of JAK inhibitors in pathologies faced by internists.
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Multicenter Study
[Diagnostic journey of type 1 Gaucher Disease patients: A survey including internists and hematologists].
Gaucher disease (GD) is a rare genetic lysosomal storage disorder caused by a beta-glucocerebrosidase deficiency and responsible for a lysosomal storage disorder. GD is characterized by haematological, visceral and bone involvements. The aim of this study was to describe the diagnostic journey of type 1 GD patients as well as the role of the internist. ⋯ GD seems to be better recognized and quickly diagnosed since 1990 in spite of the multiplicity of journeys. The role of the internist seems important.