Revue Du Praticien最新文献

EVOLUTIONS OF THE "FRAMEWORK OF BENEFITS" SYSTEM. In the field of compliance, the last thirty years have been marked by an evolution of the regulatory framework in order to adapt an anti-corruption system to the whole ecosystem of health professionals and industrialists. Although the various developments now make the "framework of benefits" a solid legislative construction, the latest news shows the need to move towards a reinforcement of illegal practices as well as a framework of canvassing for promotion between industrialists and health professionals. However, the construction of this legal framework has given rise to concerns and questions, particularly from physicians' associations, concerning their training prospects and their search for funding.

DIAGNOSIS AND STAGING OF HODGKIN LYMPHOMA. Hodgkin lymphoma (HL) is composed of two distinct disease entities: classical HL (cHL) and nodular lymphocyte predominant HL (NLPHL). NLPHL is a rare entity with a specific therapeutic management. The subgroups of cHL are nodular sclerosis, mixed cellularity, lymphocyte depletion and lymphocyte-rich HL. The initial diagnosis of HL is made by a biopsy to analyze with accuracy the architecture of the lymph node. Most HL patients present with supradiaphragmatic lymphadenopathy. Initial staging is determined by positron emission tomography (PET) scan and bone marrow biopsy is now avoided of initial staging. This staging allows the definition of prognostic groups to determine a risk-adapted initial therapy to maintain a high level of curability and reduce long-term therapeutic side effects. Before initial treatment, cardiac ultrasound and pulmonary function tests are needed. The question of fertility preservation is important to address before treatment and for older patients the requirement of geriatric assessment.

RELAPSING POLYCHONDRITIS. Relapsing polychondritis (RP) is a systemic disease which diagnosis relies on the existence of typical chondritis present at the beginning of the disease only in 1/3 of cases. Three phenotypes of RP have been described, each one characterized by specific manifestations and the need of a specific therapeutic management and follow-up. Screening for tracheo-bronchial manifestations must be systematic if RP is suspected, as it is responsible for most of the morbi-mortality of the disease. Screening for the presence of UBA1 mutations for VEXAS syndrome (Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) is key in male patients over 50 years-old presenting with macrocytic anemia, especially also in case of dermatologic or pulmonary manifestations or thrombo-embolic complications. Initial screening allow to rule-out the main differential diagnosis (ANCA-associates vasculitis) and to look for associated auto-immune or inflammatory diseases which are present in 30% of cases. Therapeutic management of RP is yet to be codified and depends on the severity of the disease.

Uterine transplantation: A TREATMENT FOR ABSOLUTE UTERINE INFERTILITY. Uterine transplantation (UT) is the first proposed treatment for absolute uterine infertility. It is also the first organ transplant of a transitory nature performed for a non-vital indication: the ability to carry a child and give birth. Today, with about one hundred transplants performed worldwide, uterine transplantation is at the crossroads between the experimental phase and current practice. The first uterine transplant was performed in France in 2019 at the Foch Hospital (Suresnes). It allowed the birth of two healthy little girls in 2021 and 2023. The second transplant was performed in September 2022. A state of the art allows to review the steps necessary for a successful transplantation from donor and recipient selection to surgery, immunosuppressive treatment and pregnancies. Potential future developments could make it possible to simplify this complex surgery, which is not without raising ethical questions.

DISEASE MODIFYING TREATMENTS FOR SICKLE CELL DISEASE. The two most widely available disease-modifying therapies, hydroxycarbamide and long-term redblood cells transfusions, are mostly introduced after the occurrence of complications. Hydroxycarbamide is mainly prescribed for the prevention of recurrent vaso-occlusive events (vaso-occlusive crisis and acute chest syndrome). Hydroxycarbamide efficacy and myelosuppressive effects are dependent on dose (usually 15 to 35 mg/kg/d) and patient compliance. Long-term transfusions are used for cerebral and end-organ damage protection or in second line after hydroxycarbamide for the prevention of recurrent vaso-occlusive events. The risks of each treatment should be weighed against the long-term risks and morbidity of the disease.