[New nosological and therapeutic perspectives in syndromic vascular malformations with a vein-lymphatic component]. / Nouvelles perspectives nosologiques et thérapeutiques pour les malformations vasculaires syndromiques à composante veino-lymphatique.

Vascular malformations are poorly recognized constitutional anomalies which arises during early childhood. Several classifications tried to draw a distinction across the different entities. Recent advances in molecular biology have contributed to the update of their nosology. Syndromic vascular malformations are an example: while Klippel-Trenaunay syndrome, Proteus or CLOVES syndrome share many common features, understanding of pathological mechanism and specially the role of the PIK3/AKT/mTOR pathway enables us to rethink their classification. Then, some syndromes associated with overgrowth and vascular malformation have been grouped under a single term: "PIK3CA-related overgrowth spectrum" (PROS), and this group continues to grow. This new approach suggests new treatment options. Rapamycin, a PIK3/AKT/mTOR pathway inhibitor, demonstrated its efficiency for some forms of PROS. Targeted therapies such as PIK3 or mTOR selective inhibitor are still in a developmental phase and results are encouraging. This is an example of personalized medicine with significant therapeutic benefit for some patients. However, genotype relation with therapeutic efficiency must be clarified and physicians should pay attention to possible negative effects of these drugs, especially for young patients.

Interest of an auto-adjustable nighttime compression sleeve (MOBIDERM® Autofit) in maintenance phase of upper limb lymphedema: the MARILYN pilot RCT.

PURPOSE: Breast cancer-related lymphedema (BCRL) is a debilitating condition. The recommended treatment is based on decongestive lymphedema therapy (DLT) with two separate phases: a short-term intensive phase to reduce lymphedema volume and a long-term maintenance phase to stabilize it. Optimizing compression therapy and compliance during maintenance phase are key factors for long-term control of lymphedema. The primary objective of this pilot prospective open-label randomized study was to assess the benefit of a new auto-adjustable nighttime arm sleeve (MOBIDERM® Autofit) on lymphedema volume during the maintenance phase after the intensive phase. METHODS: Forty women with BRCL were consecutively enrolled and randomized (D0) for 1 month in 1:1 ratio either in night-use group: with MOBIDERM® Autofit (on top of a daytime compression hosiery), or in no night-use group: without MOBIDERM® Autofit (daytime hosiery alone). From Day 31 to Day 90, all patients were fitted with MOBIDERM® Autofit. Primary endpoint was lymphedema volume variation between Day 0 and Day 30. Secondary endpoints were compliance, quality of life (LYMQOL arm questionnaire), functional symptoms (heaviness, limb use limitation, pain), sleep quality, and safety. RESULTS: In ITT population, between Day 0 and Day 30, mean lymphedema volume increase was higher in no night-use group with 92.9 mL (i.e., 3.2%) than in night-use group with 46.7 mL (i.e., 1.80%), p = 0.757. Between Day 30 and Day 90, all patients fitted with MOBIDERM® Autofit, lymphedema volume remained stable in both groups. The device improved functional symptoms and function domain of the LYMQOL arm questionnaire. MOBIDERM® Autofit was worn overnight almost 85% of the nights. It was well accepted by the patients and no adverse reaction leading to permanent device discontinuation occurred. CONCLUSIONS: Our results suggest that MOBIDERM® Autofit offers clinical benefits during maintenance phase of lymphedema treatment and enhances patient's self-management.

[Venous thrombo-embolic disease in cancer. Low molecular weight heparin indications]. / Maladie thrombo-embolique veineuse au cours du cancer. Indication des héparines de bas poids moléculaires.

Cancer and venous thrombo-embolic disease (VTE) are closely related. Indeed, cancer can reveal VTE and VTE can be the first sign of cancer. Low molecular weight heparin (LWMH) is now the first line treatment in cancer patients. Compliance with marketing authorizations and guidelines are crucial for patient-centered decision-making. This work deals with the prescription of LWMH in patients who develop VTE during cancer in order to better recognize what should or should not be done. The patient's wishes must be taken into consideration when making the final therapeutic decision. The other treatments are discussed: vitamin K antagonists and direct oral anticoagulants (DOACs) may be useful.

[Deep venous thrombosis complications during infections in pediatric patients: analysis of a series of 24 cases]. / Infections et thromboses veineuses en pédiatrie : analyse d'une série de 24 cas.

Venous thromboembolism disease (VTE) is rare in children (5.3 of 10,000 hospitalized children). However, morbidity and mortality are high, especially when the child is already suffering from severe sepsis. We report an analytical study of 24 cases of deep venous thrombosis occurring in children during infection, recorded at the Montpellier University Hospital between 1999 and 2009. Many parameters were studied in each population (age, sex, familial and personal history of thrombosis, history of thrombophilia, the presence of a venous catheter, a causative organism, time to onset of thrombus, topography of lesions, acquired abnormalities of hemostasis, and thrombosis prophylaxis). The children were aged from 1 day of life to 16 years. Thromboses occurred in two clinical contexts: "contact" thrombosis (which appeared near the infection) and disseminated thrombosis. This is an early complication because in most of the cases, it appeared in the first 10 days of sepsis. Infection and coagulation appear to be closely related and the states of latent or decompensated disseminated intravascular coagulation are common. Nevertheless, it is not possible to predict the occurence of a thrombotic event. The presence of risk factors (venous catheters, acquired thrombophilia, or constitutional thrombophilia) may increase the thrombogenic potential of the infection. VTE should always be suspected and sought in case of an unfavorable clinical course, and routine prophylaxis of thrombosis during sepsis should be discussed.