French national diagnosis and care protocol (PNDS, protocole national de diagnostic et de soins): cystic lymphatic malformations.

Cystic lymphatic malformations (LMs) are rare chronic conditions which management differs according to the type (macrocystic LMs, microcystic LMs or both). Studies are lacking due to rarity of the pathology. We aimed to establish a French National Diagnosis and Care Protocol (PNDS: Protocole National de Diagnostic et de Soins), to provide health professionals with free open access synthesis on optimal management and care of patients with LMs ( https://www.has-sante.fr/upload/docs/application/pdf/2021-03/malformations_lymphatiques_kystiques_-_pnds.pdf ). The process included a critical review of the literature and multidisciplinary expert consensus. LMs are congenital but are not always discovered at birth. Nearly 75% of them are located in the head and neck because of the highly dense lymphatic system in this region. Physical examination (showing painless masses with normal skin color and depressible consistency, or cutaneous/mucosal lymphangiectasia) and color Doppler ultrasonography, usually allow for diagnosis. MRI (involving T2 sequences with fat saturation in at least two spatial planes) is the tool of choice for evaluating anatomical extension, characterizing lesions (microcystic and macrocystic), and before considering therapeutic management. A biopsy, coupled to a blood sample, can also be used for molecular biology analyses, to search for activating mutations of the PIK3CA gene, particularly with LM integrating in a syndromic form (CLOVES or Klippel-Trenaunay syndrome) but also in certain isolated (or common) LMs. The spontaneous evolution of LMs, in particular microcystic forms, is often toward progressive aggravation, with an increase in the number of vesicles, thickening, increased oozing and bleeding, while pure macrocystic LMs may regress due to "natural sclerosis", i.e. fibrosis secondary to an inflammatory reorganization after common infantile infections. In case of voluminous LMs or syndromic forms, functional and psychological repercussions can be major, deteriorating the patient's quality of life. LMs must be treated by physicians integrated in multidisciplinary teams, and be personalized. Management is a life-long process that involves one or several of these therapies: conservative management, physical therapy (compression), sclerotherapy, surgery, drugs such as mTOR inhibitors (sirolimus), that has shown efficacy in decreasing the volume of LMs, and, more recently, PI3K-inhibitors in syndromic forms. Psychological and social support is necessary, taking into account the patient and his family.

Home-based palliative care management: what are the useful resources for general practitioners? a qualitative study among GPs in France.

BACKGROUND: Most French people (71%) would like to die at home, but only one out of four actually do. While the difficulties inherent in the practice of home-based palliative care are well described, few studies highlight the resources currently used by general practitioners (GPs) in real life. We have therefore sought to highlight the resources actually used by GPs providing home-based palliative care. METHODS: Twenty-one GPs of different ages and practice patterns agreed to participate to this qualitative study based upon semi-structured interviews. They were recruited according to a purposive sampling. Transcripts analysis was based upon General Inductive Analysis. RESULTS: The resources highlighted have been classified into two main categories according to whether they were internal or external to the GPs. The internal resources raised included the doctor's practical experience and continuous medical education, personal history, work time organization and a tacit moral contract related to the referring GP's position. External resources included resource personnel, regional assistance platforms and health facilities, legislation. CONCLUSION: This study provides a simple list that is easy to share and pragmatic solutions for GPs and policymakers. Home-based palliative care practice can simultaneously be burdensome and yet a fulfilling, meaningful activity, depending on self-efficacy and professional exhaustion (burnout), perhaps to a greater extent than on medical knowledge. Home-based palliative care promotion is a matter of social responsibility. The availability of multidisciplinary teams such as regional assistance platforms and Hospitalization at Home is particularly important for the management of palliative care. Policymakers should consolidate these specific resources out of hospitals, in community settings where the patients wish to end their life.

Factors influencing Urate Lowering Therapies prescription for asymptomatic hyperuricemia by general practitioners: a qualitative study.

BACKGROUND: Urate Lowering Therapies (ULTs), mainly Xanthine Oxydase Inhibitors, are widely used by general practitioners (GPs) in asymptomatic hyperuricemia, although no guideline currently recommends to do so. The use of ULTs in asymptomatic hyperuricemia has been associated with an increased risk of ULTs-related adverse drug reactions. AIM: Our study aimed at exploring GPs' views and practices in relation to the prescription or non-prescription of ULT in asymptomatic hyperuricemia. METHODS: We conducted a qualitative study using individual semi-structured interviews with 14 French GPs. We built a purposeful sample searching for maximum variation on 8 GPs' personal and professional criteria such as age, years of installation, location of their practice. We conducted a thematic analysis of the transcripts, following Miles and Huberman three steps model: data reduction, data presentation, conclusion drawing and verifications. RESULTS: We identified two behaviors leading to inappropriate prescription of ULTs among interviewed GPs. Primary prescribers frequently used uric acid serum levels and had a positive representation of ULTs. Other GPs behaved in an ambivalent way: they did not initiate ULTs, but systematically renewed preexisting prescriptions. They had a negative perception of ULTs but considered them unimportant during drug reassessment. De-prescribing occurred mainly because of external input such as the need to lighten the prescription or the participation in an audit in general practice. CONCLUSIONS: Our results support several strategies of ULTs de-prescribing in asymptomatic hyperuricemia: the promotion of de-prescribing of serum acid uric lab test in daily practice (a), supporting the clinical reasoning in the case of asymptomatic hyperuricemia detection (b) but also during ULTs renewals (c) leading to a prioritization of the safest prescriptions (d) through shared medical decision (e). Additional studies are necessary to further develop and evaluate these de-prescribing strategies.

Effect of Diet in Chronic Spontaneous Urticaria: A Systematic Review.

Strategies for diets in chronic spontaneous urticaria (CSU) are controversial. This systematic review assessed the interest in diet for managing CSU. We searched for original reports in MEDLINE, EMBASE, CENTRAL and LILACS. Among the 278 reports screened, 20 were included, involving 1,734 patients. Reports described 3 types of systematic diet: pseudoallergen-free diet (n = 1,555 patients), low-histamine diet (n = 223) and diet without fish products (n = 47), which induced complete remission in 4.8%, 11.7% and 10.6% of patients, respectively, and partial remission in 37.0%, 43.9% and 4.3%. Eight reports described personalized exclusion diets (66 patients) adapted to symptoms/allergological test results and led to complete remission in 74.6% of patients, although the diagnosis of CSU was doubtful. No comparative randomized studies of diets were available. The only randomized studies were based on oral provocation tests with the suspected responsible diet. Population and outcomes were heterogeneous. In conclusion, there is evidence for the benefit of diets in CSU only in individual patients with clinical symptoms. However, the level of evidence is low for the benefit of systematic diets in CSU because systematic double-blind controlled trials of diet are lacking.

Chronic spontaneous urticaria in children - a systematic review on interventions and comorbidities.

BACKGROUND: Chronic spontaneous urticaria (CSU) is not frequent in children. Management guidelines have been developed for adults and randomized controlled trials (RCTs) included teenagers aged 12-18, but data for children under age 12 are limited. We performed a systematic review to assess comorbidities in children <12 years old with CSU and the efficacy and safety of treatments. METHODS: We searched for original articles of epidemiologic and treatment data in children <12 years old with CSU that were published from 2005 to July 2016 in MEDLINE, EMBASE, CENTRAL, and LILACS. Article selection and data extraction were performed in duplicate. RESULTS: Our systematic review included 9 reports on epidemiologic data (633 children). Five comorbidities and laboratory anomalies associated with CSU found were atopy (28.1%), positive autologous serum skin test (36.8%), thyroid biologic anomalies (6.4%) and detectable antinuclear antigen (10.4%), seroprevalence for Helicobacter pylori (21.1%), low vitamin D level (69.1%), and psychiatric disorders (70.4%). Only one study allowed for comparison with a control group. Our review included 10 studies (322 children), describing 5 different drug families, mostly H1-antihistamines (n = 297). One randomized controlled study compared single-dose rupatadine with single-dose desloratadine and placebo. Cyclosporine was effective and had no adverse effects in 18 children. Omalizumab, montelukast, and cefuroxime were reported in very small series (5, 1, and 1 patients). CONCLUSIONS: H1-antihistamines are effective for CSU in children <12 years old, with reassuring safety data at licensed doses. Cyclosporine seems effective, but the level of evidence is low.

[Factors associated with investment in an office medicine project by general practice residents]. / Déterminants du projet d'installation en ambulatoire des internes de médecine générale.

OBJECTIVE: To identify thefactors associated with investment in an office medicine project by French general practice (GP) residents. METHODS: We conducted a national survey using a web-based self-administered questionnaire and analyzed the data collected by multiple logistic regressions. The dependent variable was "an office medicine project" The explanatory variables were both individual (socio-demographic and linked to training trajectories) and contextual (related to the available training programmes and the regional medical demography). RESULTS: The response rate was 48.5%. Out of the 1,695 residents of the study sample, 315 (18.6%) already had a project to setup an office practice during their third cycle ofmedical studies. The main factors associated with this project were (p < 0.05): to receive strong academic support, to live in a rural or semi-rural area, to work as a GP locum, to perform residency training in the same city as the medical training and to perform residency training in a region with a high percentage of GPs 55years and older. CONCLUSIONS: This study showed that a project to setup an office practice was influenced by both individual and contextualfactors. Special attention should be paid to the means and content of training to ensure better supportfor residents, which could make office general practice more attractive.