Presse Med
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Primary Sjögren's syndrome (pSS) is an autoimmune inflammatory disorder characterized by lymphocytic infiltration of exocrine glands, mainly the lacrimal and salivary glands leading to a chronic sicca syndrome. However, extraglandular organ systems may frequently be involved, including both central and peripheral nervous systems. ⋯ The spectrum of pSS-related neuropathies is wide including sensory neuropathies, neuronopathies, sensory-motor neuropathies, mononeuritis multiplex related to vasculitis… Central nervous system involvement is composed by multiple sclerosis-like manifestations including acute and chronic myelopathies and by more diffuse manifestations (cognitive dysfunction, subacute aseptic meningitis, encephalopathy, psychiatric symptoms, chorea, seizures…). The diagnosis and treatment of such pSS-related manifestations must be optimized in order to avoid severe disability.
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Fraud in biomedical literature has been highlighted by recent cases referring to key opinion leaders responsible for "massive" fake due to the number of papers published with made-up data in peer-review journals with high impact factor. Editors have an increasing responsibility in preventing publication of culprit studies, leading to check not only the value and the interpretation of submitted data but the whole process of elaborating protocols and performing studies. Increased transparency in clinical trials performance is critical to prevent biomedical fraud.
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Ramadan fasting is accompanied by an increase of the gastric acidity over 24 hours especially in the daytime. Gastric acidity is maximal at the end of the fasting day. So, a patient with duodenal ulcer is exposed to a high risk of disease reactivation. ⋯ Helicobacter pylori eradication does not seem to play a role in the occurrence of these complications, particularly the perforation. The patient with duodenal ulcer can fast without risks while using a proton pump inhibitor if the ulcer is healed and H. pylori is eradicated. Ramadan fasting seems to us inadvisable when duodenal ulcer is active, but large prospective studies are needed.
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The treatment of familial amyloid polyneuropathies (FAP) is complex and requires a neurological and cardiological multidisciplinary coverage. It includes specific treatments to control the progression of the systemic amyloidogenesis, the symptomatic treatment of the peripheral and autonomic neuropathy (digestive, urinary, sexual, postural hypotension) and the treatment of organs severely involved by amyloidosis (heart, eyes, kidneys). First line specific treatment of met30 TTR-FAP is liver transplantation (LT) which allows to suppress the main source of mutant TTR, to stop the progression of the neuropathy in 70 % of cases at long-term (with an experience of 18 years) and to double the median survival. ⋯ In every case of FAP, a regular cardiological follow-up is required for life, because of the progress of the cardiac involvement, which is not always stopped by the liver transplantation. The symptomatic treatments are indispensable to improve the quality of life of the patients: neurogenic pains, urinary disorders, liqueurs, sexual impotence, postural hypotension. The familial screening of the carriers of the TTR gene mutation and their regular follow-up by appropriate clinical examination and complementary investigations are major to detect early the onset of the disease to start as soon as possible specific therapy.