Intérêt de ZAP-70 dans la leucémie lymphoïde chronique (mise au point de la mesure de l'expression de ZAP-70 par cytométrie en flux au C.H.U. de Nice)

NICE-BU Médecine Odontologie (060882102) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Correlations entre zap-70, auto-immunité et hypogammaglobulinémie dans la leucémie lymphoïde chronique

NICE-BU Médecine Odontologie (060882102) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Late clinical failure associated with cytochrome b codon 268 mutation during treatment of falciparum malaria with atovaquone–proguanil in traveller returning from Congo

International audienc

Emergence of Lyme Disease on the French Riviera, a Retrospective Survey

International audienceBackground The French Riviera has been declared free of Lyme Borreliosis (LB) for years. Many patients are referred for presumed LB, sometimes with atypical clinical signs and/or doubtful serology, calling the diagnosis into question. Methods Patients were assessed for LB diagnosis, depending on clinical presentation, laboratory findings, and further examination by other medical professionals. Results Among 255 patients, 45 (18%) were classified as confirmed LB cases [including 28 ongoing LB (10%) and 17 past LB (8%)], and for 210 (82%) a Lyme borreliosis diagnosis was ruled out. Among ongoing LB, 56% had been exposed to or bitten by ticks, exclusively in rural locations of the Alpes-Maritimes. As a result of the diagnostic procedure, 132 (52%) patients had been treated. An alternative diagnosis was established for 134 (52%) patients, covering a wide range of conditions, including mainly psychological (28%) and neurological conditions (25%) or inflammatory and systemic diseases (22%). Conclusions Our results strongly suggest the endemicity of LB in the Alpes-Maritimes region. Confirmed LB accounted for 18% of patients while 52% were diagnosed with other conditions

Knowledge and application of the HAS recommendations concerning the diagnosis of undernutrition in people aged 70 and above: an international online survey among members of the "Société Française de Gériatrie et de Gérontologie".

editorial reviewed[en] OBJECTIVES: To assess the knowledge and the use in clinical practice of the "French National Authority for Health" (HAS) 2021 recommendations on the diagnosis of malnutrition in older adults aged 70 years and above. METHODS: An online survey was developed by the working Group on Geriatrics and Nutrition (GEGN) of the "Société Française de Gériatrie et de Gérontologie (SFGG)" and conducted among members of the SFGG. A self-administered questionnaire composed of 21 closed questions was used. RESULTS: A total of 132 (10.2 %) members of the SFGG responded to the survey. The respondents were aged 43.4 ± 9.94 years old and 97 (73.5 %) were women. Almost half of them were medical doctors (n = 53 ; 40.2 %). Of the respondents, 81 % had already heard of the HAS 2021 recommendations. This percentage is higher for doctors (96 %) than for other professions (74 %). French respondents were more likely to have heard of these -recommendations (84 %) than respondents from other countries (42.9 %). However, only 41.6 % of the respondents believe they know these recommendations in detail. On a visual analogue scale from 0 (never) to 100 (always), respondents use these recommendations in their professional practice at 76.8 ± 20.2 %. However, on a scale from 0 (never difficult) to 100 (always difficult), some barriers to their use in clinical practice were highlighted. These mainly concerned the measurement of muscle mass (84.4 ± 17.2 %) and muscle strength (79.5 ± 19.9 %) in patients. CONCLUSION: Although the majority of respondents use the HAS 2021 recommendations in their professional practice, they often have difficulties in measuring muscle strength and muscle mass of their patients. The assessment of sarcopenia still needs to be implemented in clinical routine. Until then, better communication about simple alternatives that do not require special equipment is needed.Objectifs : Évaluer la connaissance et l’utilisation en pratique clinique des recommandations de la Haute Autorité de Santé (HAS) 2021 pour le diagnostic de la malnutrition chez la personne âgée de 70 ans et plus. Méthodologie : Enquête en ligne réalisée auprès des membres de la Société française de gériatrie et gérontologie (SFGG) entre juin et septembre 2022. Résultats : 132 (10,2 %) membres de la SFGG ont répondu à l’enquête. Parmi ces derniers, 81 % avaient connaissance des recommandations HAS 2021. Sur une échelle visuelle analogique de 0 (jamais) à 100 (toujours), les répondants à l’enquête utilisent ces recommandations dans leur pratique professionnelle à 76,8 ± 20,2 / 100. Toutefois, certaines barrières liées à leur application ont été mises en lumière sur une échelle allant de 0 (jamais difficile) à 100 (toujours difficile). Celles-ci concernent principalement l’obtention des informations relatives à la masse (84,4 ± 17,2 / 100) et à la force (79,5 ± 19,9 / 100) des patients. Conclusion : Bien que la majorité des répondants utilisent ces recommandations dans leur pratique professionnelle, ils éprouvent souvent des difficultés à recueillir la force et la masse musculaires des patients. Pour remédier à ces difficultés, il conviendrait de mieux communiquer sur l’utilisation d’alternatives simples et rapides

Platelet count threshold for hemorrhage in patients with immune thrombocytopenia treated with antiplatelet agents.

International audienc

Splenectomy for primary immune thrombocytopenia revisited in the era of thrombopoietin receptor agonists: New insights for an old treatment

International audienceAlthough splenectomy is still considered the most effective curative treatment for immune thrombocytopenia (ITP), its use has significantly declined in the last decade, especially since the approval of thrombopoietin receptor agonists (TPO-RAs). The main objective of the study was to determine whether splenectomy was still as effective nowadays, particularly for patients with failure to respond to TPO-RAs. Our secondary objective was to assess, among patients who relapsed after splenectomy, the pattern of response to treatments used before splenectomy. This multicenter retrospective study involved adults who underwent splenectomy for ITP in France from 2011 to 2020. Response status was defined according to international criteria. We included 185 patients, 100 (54.1%) and 135 (73.0%) patients had received TPO-RAs and/or rituximab before the splenectomy. The median follow-up after splenectomy was 39.2 months [16.5-63.0]. Overall, 144 (77.8%) patients had an initial response and 23 (12.4%) experienced relapse during follow-up, for an overall sustained response of 65.4%, similar to that observed in the pre-TPO-RA era. Among patients who received at least one TPO-RA or rituximab before splenectomy, 92/151 (60.9%) had a sustained response. Six of 13 (46%) patients with previous lack of response to both TPO-RAs and rituximab had a sustained response to splenectomy. Among patients with relapse after splenectomy, 13/21 (61.2%) patients responded to one TPO-RAs that failed before splenectomy. In conclusion, splenectomy is still a relevant option for treating adult primary ITP not responding to TPO-RAs and rituximab. Patients with lack of response or with relapse after splenectomy should be re-challenged with TPO-RAs

Chromogranin A preferential interaction with Golgi phosphatidic acid induces membrane deformation and contributes to secretory granule biogenesis

International audienceChromogranin A (CgA) is a key luminal actor of secretory granule biogenesis at the trans‐Golgi network (TGN) level but the molecular mechanisms involved remain obscure. Here, we investigated the possibility that CgA acts synergistically with specific membrane lipids to trigger secretory granule formation. We show that CgA preferentially interacts with the anionic glycerophospholipid phosphatidic acid (PA). In accordance, bioinformatic analysis predicted a PA‐binding domain (PABD) in CgA sequence that effectively bound PA (36:1) or PA (40:6) in membrane models. We identified PA (36:1) and PA (40:6) as predominant species in Golgi and granule membranes of secretory cells, and we found that CgA interaction with these PA species promotes artificial membrane deformation and remodeling. Furthermore, we demonstrated that disruption of either CgA PABD or phospholipase D (PLD) activity significantly alters secretory granule formation in secretory cells. Our findings show for the first time the ability of CgA to interact with PLD‐generated PA, which allows membrane remodeling and curvature, key processes necessary to initiate secretory granule budding

Abnormal T-cell phenotype in episodic angioedema with hypereosinophilia (Gleich’s syndrome): frequency, clinical implication and prognosis

International audienceBackground: Episodic Angioedema with eosinophilia (EAE, Gleich's syndrome) is a rare disorder consisting of recurrent episodes of angioedema, hypereosinophilia and frequent elevated serum Immunoglobin M.Methods: We conducted a retrospective multicenter nationwide study regarding the clinical spectrum and therapeutic management of patients with EAE in France.Results: Thirty patients were included with a median age at diagnosis of 41 years [5-84]. The median duration of each crisis was 5.5 days [1-90] with swelling affecting mainly the face and the upper limbs. Total serum IgM levels were increased in 20 patients (67%). Abnormal T-cell immunophenotypes were detected in 12 patients (40%) among which 5 (17%) showed evidence of clonal TCR γ gene rearrangement. Median follow-up duration was 53 months [31-99]. The presence of an abnormal T-cell population was the sole factor associated with a shorter time to flare (hazard ratio 4.15 [CI 95% 1.18-14.66; p=0.02). At last follow-up, 3 patients (10%) were able to withdraw all treatments and 11 (37%) were in clinical and biological remission with less than 10 mg of daily prednisone.Conclusion: EAE is a heterogeneous condition that encompasses several disease forms. Although patients usually respond well to glucocorticoids, those with evidence of abnormal T-cell phenotype have a shorter time to flare