La Revue de médecine interne
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Review Comparative Study
[Genetic iron overloads and hepatic insulin-resistance iron overload syndrome: an update].
Hepcidin inhibits intestinal absorption of iron through internalisation of ferroportin. Its discovery helps to better understand the genetic iron overloads. The insulin resistance-hepatic iron overload (IR-HIO)--also coined as the dysmetabolic iron overload syndrome--is a common cause or iron overload. ⋯ Treatment of IR-HIO is focused on metabolic syndrome and phlebotomies are questionable because the overload is moderate and intestinal absorption of iron seems to be low. MRI (or other non invasive methods) is needed to truly assess iron overload because serum ferritin overestimates it in metabolic syndrome. Several points have to be elucidated: how HFE interferes with hepcidin in type 1 haemochromatosis; the causes of variability of iron overload; the benefits of populations screening; the advantage of phlebotomies in IR-HIO; the use of new oral iron chelators.
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Comparative Study
[Orofacial manifestations of systemic sclerosis: a study of 30 consecutive patients].
The face is frequently involved in systemic sclerosis. The main stomatologic manifestations include limited mouth opening, xerostomia, skin atrophy, trigeminal neuralgia. The objective of this study was to describe oral and facial manifestations observed in scleroderma patients from our cohort. ⋯ Oral and facial manifestations are frequently observed in scleroderma patients. These manifestations lead to major functional discomfort, mainly due to decreased mouth opening that seems to be frequently associated to oesophageal involvement. Xerostomia is also frequent and is commonly observed in anticentromere antibodies positive cutaneous limited forms of systemic sclerosis. Evolution of radiographic abnormalities like periodontal ligament space widening (33% of cases), or osteolytic lesions (7%) is poorly known.
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We report a second observation of autoimmune myelofibrosis associated with an inflammatory myositis in a 30-year-old female. The links between myelofibrosis and autoimmunity are discussed.
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Bilateral hilar lymphadenopathy, with or without lung parenchymal infiltrates, is the most common radiographic finding in patients with sarcoidosis. Atypical pulmonary findings have been uncommonly reported and include multiple large lung nodules, cavitation, lobar collapse, pleural effusions or pneumothorax. ⋯ In patients exhibiting unusual pulmonary manifestations, diagnosis of sarcoidosis relies on compatible clinical signs, evidence of non-caseating granulomas, and exclusion of underlying conditions including infections, malignancy and other granulomatous diseases (Wegener disease, pneumoconiosis).