[Acquired haemophilia: Update in 2024]. / Hémophilie acquise : quoi de neuf en 2024 ?

Acquired hemophilia A (AHA) is a rare autoimmune disorder due to autoantibodies against factor VIII, with a high mortality risk. It should be suspected in subjects with abnormal bleedings, especially subcutaneous bleed associated with prolonged activated partial thromboplastin time (aPTT). AHA is often idiopathic but is associated with autoimmune diseases, malignancies, pregnancy and postpartum period or drugs. Treatment is based on haemostatic agents as by-passants agents such as factor VIIa and activated prothrombine concentrate complex or recombinant porcine factor VIII for severe bleeding. Eradication of inhibitor should be established as soon as the diagnosis is confirmed with steroid alone often associated with cytotoxic agents or rituximab, depending on FVIII activity and inhibitor titer. The purpose of this review is to summarize the epidemiology, etiopathogenesis, diagnosis, treatment of AHA and discuss current recommendations.

[Adverse effects of the tezacaftor/ivacaftor/elexacaftor combination that may lead to discontinuation: About a series of 10 cases]. / Effets indésirables de l'association tezacaftor/ivacaftor/elexacaftor pouvant mener à un arrêt de traitement : à propos d'une série de 10 cas.

INTRODUCTION: Cystic fibrosis transmembrane regulator (CFTR) channel modulators (ivacaftor, lumacaftor, tezacaftor and elexacaftor) represent a major advance in the management of cystic fibrosis. However, few data are available on the real-life safety profile of these medications, in particular on adverse events that may lead to their discontinuation. The aim of this study is to describe the characteristics and evolution of adverse reactions to the tezacaftor/ivacaftor/elexacaftor combination that led to discontinuation and were reported to the Centre régional de pharmacovigilance (CRPV) in Rennes (France). MATERIALS AND METHODS: A retrospective study was conducted from December 2021 to May 2023, focusing on cases of discontinuation of the tezacaftor/ivacaftor/elexacaftor combination due to the occurrence of one or more adverse effects, and reported to the CRPV of Rennes, France. RESULTS: Ten cases of drug discontinuation were reported to the Rennes CRPV (6 women/4 men). Adverse effects mainly involved neuropsychiatric disorders (n=6), followed by liver disorders (n=2), ear, nose and throat disorders (n=1), and digestive disorders (n=1). The average duration of treatment at discontinuation was 339.8 [39-668] days. The drug was reintroduced in 7 patients on average 48.7 [7-123] days after discontinuation, with a dosage adjustments (n=4) consisting of changes in dosing times or a reduction in daily doses, with varying success in alleviating adverse symptoms depending on the case. CONCLUSION: This small case series suggests that neuropsychiatric adverse effects may occur more frequently than initially described after initiation of tezacaftor/ivacaftor/elexacaftor, and should be carefully screened and monitored. Dosage or administration schedule modifications may be considered for patients experiencing these adverse effects. Further pharmacovigilance studies are needed to better understand the adverse effect profiles of "caftors", their possible risk factors, and the impact of adjusting dosing modalities.

Sex differences in muscle-invasive bladder cancers: a study of a French regional population.

OBJECTIVES: Although men have a higher risk of developing a bladder cancer, women appear to have a poorer prognosis and a more advanced stage at diagnosis. We performed a retrospective population-based study on muscle invasive bladder cancer (MIBC) using data from a cancer registry in a French department to compare overall and specific survival data according to sex. MATERIAL AND METHODS: We included all patients living in the department of Hérault and diagnosed with MIBC between January 1, 2017 and December 12, 2019. Univariable and multivariable analyses were performed on all variables of interest. RESULTS: We included 124 women and 432 men. There was no significant difference in age or stage at diagnosis according to sex. Squamous cell carcinomas were more common in women (p<0.001). Cystectomy was more frequent in men than in women (50.7% vs 35.4%) (p=0.0039). By multivariable analysis, the independent factors for being treated by cystectomy were sex (p=0.004), age (p<0.001) and stage (p<0.001). Forty-seven percent of women received no treatment or palliative treatment. Overall mortality was 79% in women and 63.2% in men (p<0.001). The median specific survival was 10.8 months in women and 32.7 months in men (p<0.0001). By multivariable analysis, the independent risk factors for mortality were female sex (p=0.047), cT4 stage (p=0.005) and absence of cystectomy (p<0.001). CONCLUSIONS: Our study shows that women are less often treated with cystectomy and have worse prognosis than men. The reasons for this gender difference are multifactorial.

Deep learning applied to dose prediction in external radiation therapy: A narrative review.

Over the last decades, the use of artificial intelligence, machine learning and deep learning in medical fields has skyrocketed. Well known for their results in segmentation, motion management and posttreatment outcome tasks, investigations of machine learning and deep learning models as fast dose calculation or quality assurance tools have been present since 2000. The main motivation for this increasing research and interest in artificial intelligence, machine learning and deep learning is the enhancement of treatment workflows, specifically dosimetry and quality assurance accuracy and time points, which remain important time-consuming aspects of clinical patient management. Since 2014, the evolution of models and architectures for dose calculation has been related to innovations and interest in the theory of information research with pronounced improvements in architecture design. The use of knowledge-based approaches to patient-specific methods has also considerably improved the accuracy of dose predictions. This paper covers the state of all known deep learning architectures and models applied to external radiotherapy with a description of each architecture, followed by a discussion on the performance and future of deep learning predictive models in external radiotherapy.

[How I deal with this unusual paravalvular leak]. / Comment je gère cette fuite paravalvulaire inhabituelle.

Percutaneous treatment of para-prosthetic valve leaks (PVL) is an alternative to redo surgery. Based on the clinical case of an unusual aortic para-prosthetic leak closure (PVLc), are presented successively the diagnostic difficulties of PVL, the modalities of therapeutic choice, the main technical steps of PVLc followed by a review of results and complications.

French protocol for the diagnosis and management of systemic lupus erythematosus.

Because Systemic Lupus Erythematosus (SLE) is a rare disease, and due to the significant prognostic impact of early management, a diagnosis confirmed by a physician with experience in SLE is recommended, for example from an expert center. Once the diagnosis is confirmed, existing manifestations should be identified in particular, renal involvement by an assessment of proteinuria, disease activity and severity should be determined, potential complications anticipated, associated diseases searched for, and the patient's socioprofessional and family context noted. Therapeutic management of SLE includes patient education on recognizing symptoms, understanding disease progression as well as when they should seek medical advice. Patients are informed about routine checkups, treatment side effects, and the need for regular vaccinations, especially if they are receiving immunosuppressive treatment. They are also advised on lifestyle factors such as the risks of smoking, sun exposure, and dietary adjustments, especially when they are receiving corticosteroids. The importance of contraception, particularly when teratogenic medications are being used, and regular cancer screening are emphasized. Support networks can help relieve a patient's isolation. The first-line medical treatment of SLE is hydroxychloroquine (HCQ), possibly combined with an immunosuppressant and/or low-dose corticosteroid therapy. The treatment of flares depends on their severity, and typically involves HCQ and NSAIDs, but may be escalated to corticosteroid therapy with immunosuppressants or biologic therapies in moderate to severe cases. Because there is no curative treatment, the goals of therapy are patient comfort, preventing progression and flares, and preserving overall long-term health and fertility. The frequency of follow-up visits depends on disease severity and any new symptoms. Regular specialized assessments are necessary, especially when treatment changes, but a frequency of every 3 to 6 months is recommended during periods of remission and monthly during active or severe disease, especially in children. These assessments include both clinical and laboratory tests to monitor complications and disease activity, with specific attention to proteinuria.

Comparing efficacy and safety of in-house gemcitabine to mitomycin for bladder instillation in intermediate-risk NMIBC.

INTRODUCTION: Intermediate-risk (IR) Non-Muscle Invasive Bladder Cancer (NMIBC) is associated with a high rate of tumor recurrence. To improve patient outcomes, it is recommended to use adjuvant intravesical therapy, by mitomycin C (MMC) or Bacillus Calmette Guerin (BCG). Gemcitabine (GMC) is a known molecule used in urothelial cancer. We aimed to study the efficacy and safety profile of a gemcitabine solution, compared to mitomycin C, in the treatment of IR NMIBC. MATERIAL: In this retrospective study, patients with IR NMIBC treated between 2016 and 2020 were selected from two participating centers using either gemcitabine (center A) as the intravesical chemotherapy regimen or mitomycin C (center B). The primary endpoint was recurrence rate and secondary end points were treatment interruption and its causes. RESULTS: In our cohort of 102 IR NMIBC patients, 49 patients received GMC and 53 MMC with a median follow-up of 30 months. Overall recurrence rate was 42.1% with 22.4% in the GMC group and 60.3% in the MMC group (P<0.01). This difference was also found in the multifactorial analysis. Course interruption was observed in 14.7% of all patients, primarily attributed to adverse events (46.6%), without difference between groups. CONCLUSION: Adjuvant intravesical gemcitabine in patients with IR NMIBC seems to be an interesting option associated with a lower tumor recurrence rate and a favorable tolerance profile when compared to MMC. Larger scale prospective randomized trials are needed to validate our findings. LEVEL OF EVIDENCE: III.

[Treatment of lupus nephritis]. / Prise en charge de la néphropathie lupique en 2023.

Lupus nephritis remains the most frequent severe complication of systemic lupus erythematosus, leading to chronic renal impairment in 20 to 25% of cases. Current treatment is based on the combined use of immunosuppressive treatment and targeted biotherapies to optimize the chances of promptly obtaining and maintaining a complete renal response over the long term. The author discusses these recent advances.

Title: Prise en charge de la néphropathie lupique en 2023. Abstract: La néphropathie lupique reste la complication sévère la plus fréquente du lupus érythémateux disséminé. Elle évolue vers l'insuffisance rénale chronique dans 20 à 25 % des cas. Son traitement moderne repose sur l'utilisation combinée d'un traitement immunosuppresseur et de biothérapies ciblées pour optimiser les chances d'obtenir rapidement et de maintenir au long cours une réponse rénale complète. L'auteur discute ces progrès récents.

Incidence, initial management and survival of high-risk non-muscle invasive bladder cancer in Northern France.

OBJECTIVE: Information on bladder cancer (BC) according to the risk scoring for recurrence or progression in a general population is scarce despite its clinical relevance. The objective was to describe the characteristics of incident BC in a general population, with a focus on the initial management of high-risk non-muscle invasive BC (HR-NMIBC). MATERIALS: BC incident in 2011-2012 recorded in a population-based cancer registry were studied. Data was extracted from medical files. NMIBC were classified according to potential risk for recurrence/progression. Individual and tumor characteristics of incident BC were described. Incidence, initial management and survival (12/31/2021) of HR-NMIBC were assessed. RESULTS: Among 538 BC cases, 380 were NMIBC [119 low (22.1%), 163 intermediate (30.3%), 98 high (18.2%) risk] and 147 (27.3%) were MIBC. HR-NMIBC diagnostic and therapeutic management [imaging, re-TUR, multidisciplinary team meetings (MDT) assessment, specific treatment] revealed discrepancies with guidelines recommendations. Seventy-two out of 98 cases were assessed in an MDT with a median time from diagnosis of 18days [first quartile: 12-third quartile: 32]. Globally, treatment agreed with MDT decisions. Intravesical instillation was the most common treatment (n=56) but 27 HR-NMIBC did not receive specific treatment after TUR. Five and 10years overall survival was 52% [42-63] and 41% [31-51], respectively. Five years net survival was 63% [47-75]. CONCLUSIONS: Despite National cancer plans aiming to improve care giving and despite the severity of HR-NMIBC, guideline-recommended patterns of care were underused in this region. This may deserve attention to identify obstacles to guideline adoption to try to improve BC patient care and survival.

Survival and toxicity after breast-conserving surgery and external beam reirradiation for localized ipsilateral breast tumour recurrence: A population-based study.

PURPOSE: Breast-conserving surgery followed by reirradiation for a localized ipsilateral breast tumour relapse may increase the radiation dose delivered to the heart and result in a greater risk of cardiac adverse events. This study aimed to compare the incidence of cardiac mortality in patients treated for a localized ipsilateral breast tumour relapse, either with breast-conserving surgery followed by reirradiation or with total mastectomy between 2000 and 2020. MATERIALS AND METHODS: All patients treated for a primary non-metastatic breast cancer with breast-conserving surgery and adjuvant radiotherapy were identified in the Surveillance, Epidemiology, and End Results (SEER) program database, and those who subsequently experienced a localized ipsilateral breast tumour relapse treated with breast-conserving surgery and reirradiation ("BCS+ReRT" group, n=239) or with total mastectomy ("TM" group, n=3127) were included. The primary objective was to compare the cardiac mortality rate between the patients who underwent breast-conserving surgery followed by reirradiation and total mastectomy. Secondary endpoints were overall survival and cancer specific survival. RESULTS: Cardiac mortality was significantly higher in patients treated with breast-conserving surgery followed by reirradiation (hazard ratio [HR]: 2.40, 95% confidence interval [95% CI]: 1.19-4.86, P=0.006) in univariate analysis; non-statistically significant differences were observed after adjusting for age, laterality and chemotherapy on multivariate analysis (HR: 1.96, 95% CI: 0.96-3.94, P=0.067), age being the only confounding factor. A non-statistically significant difference towards lower overall survival was observed in patients who had breast-conserving surgery followed by reirradiation compared with those who underwent total mastectomy (HR: 1.37, 95% CI: 0.98-1.90, P=0.066), and no differences were observed in terms of cancer specific survival (HR: 1.01, 95% CI: 0.56-1.82, P=0.965). CONCLUSION: In this study, the incidence of cardiac mortality was low, and breast-conserving surgery followed by reirradiation did not independently increased the risk of cardiac mortality for a localized ipsilateral breast tumour relapse.

Evaluation des Troubles Cognitifs Chez des Patients Tunisiens Atteints de Trouble Bipolaire en Rémission : Étude Cas-Témoins: Assessment of Cognitive Impairment in Tunisian Patients With Bipolar Disorder in Remission: A Case-Control Study.

OBJECTIVES: Our aims were to assess cognitive impairment in bipolar patients in remission compared with healthy controls, and to study its connection to clinical and therapeutic factors. METHODOLOGY: This was a case-control study of patients with bipolar disorder (BD) in remission and matched healthy controls. It was carried out at the Hédi Chaker University Hospital in Sfax, Tunisia. The Screen for Cognitive Impairment in Psychiatry (SCIP) scale was used to assess cognitive function in patients and controls. This scale comprises subtests for verbal learning with immediate (VLT-I) and delayed (VLT-D) recall, working memory (WMT), verbal fluency (VFT) and information processing speed (PST). RESULTS: We recruited 61 patients and 40 controls. Compared with controls, patients had significantly lower scores on the overall SCIP scale and on all SCIP subtests (p < 0.001 throughout) with moderate to high effects. In multivariate analysis, the presence of psychotic characteristics correlated with lower scores on the overall SCIP (p = 0.001), VLT-I (p = 0.001) and VLT-D (p = 0.007), WMT (p = 0.002) and PST (p = 0.008). Bipolar II correlated with lower LTV-I scores (p = 0.023). Age of onset and duration of the disorder were negatively correlated with PST scores (p < 10-3 and p = 0.007, respectively). Predominantly manic polarity correlated with lower VFT scores (p = 0.007). CONCLUSIONS: Our study showed that bipolar patients in remission presented significantly more marked cognitive impairments, affecting various cognitive domains, than the controls. These cognitive impairments appear to be linked to clinical and therapeutic factors that are themselves considered to be factors of poor prognosis in BD.

OBJECTIFS: Nos objectifs étaient d'évaluer les troubles cognitifs chez des patients bipolaires en rémission comparativement à des témoins sains et d'étudier leur rapport avec les facteurs cliniques et thérapeutiques. MÉTHODES: Il s'agissait d'une étude cas-témoins, menée auprès de patients atteints de trouble bipolaire (TBP) en rémission et de témoins sains appariés. Elle a été réalisée au centre hospitalo-universitaire (CHU) Hédi Chaker de Sfax (Tunisie). L'échelle the Screen for cognitive impairment in psychiatry (SCIP) a été utilisée pour l'évaluation des fonctions cognitives chez les patients et les témoins. Cette échelle se compose des sous-échelles d'apprentissage verbal avec rappel immédiat (VLT-I) et différé (VLT-D), de la mémoire de travail (WMT), de la fluence verbale (VFT) et de la vitesse de traitement de l'information (PST). RÉSULTATS: Nous avons recruté 61 patients et 40 témoins. Comparés aux témoins, les cas avaient des scores totaux du SCIP et de toutes les sous-échelles du SCIP significativement plus bas (p < 0,001 partout) avec des tailles d'effet modérées à élevées. Dans l'analyse multivariée, la présence de caractéristiques psychotiques était corrélée à l'abaissement des scores du SCIP total (p = 0,001), du VLT-I (p = 0,001) et VLT-D (p = 0,007), du WMT (p = 0,002), et du PST (p = 0,008). Le TBP de type 2 était corrélé à l'abaissement du score de VLT-I (p = 0,023). L'âge de début et la durée d'évolution du trouble étaient corrélés négativement au score PST (p < 10−3 et p = 0,007 respectivement). La polarité maniaque prédominante était corrélée à l'abaissement du score VFT (p = 0,007). CONCLUSIONS: Notre étude a montré que les patients bipolaires en rémission présentaient des troubles cognitifs touchant différents domaines cognitifs, significativement plus marqués que chez les témoins. Ces troubles cognitifs semblent être liés à des facteurs cliniques et thérapeutiques considérés eux-mêmes comme des facteurs de mauvais pronostic de la maladie bipolaire.

[Knowledge and practices of the steps prior to the sterilization of heat-resistant medical devices: Study at University Hospital Centre of Sahloul, Sousse-Tunisia - 2022]. / Connaissances et pratiques des étapes préalables à la stérilisation des dispositifs médicaux thermorésistants : étude au centre hospitalo-universitaire Sahloul, Sousse-Tunisie ­ 2022.

OBJECTIVES: The standard process of central sterilization is crucial for the optimal functioning of the operating room. The outcome of this process is closely linked to the steps preceding the steps prior to the sterilization step itself. These steps include pre-disinfection carried out in the operating rooms and other stages, namely washing, drying and packaging, which must be performed in the central sterilization unit. In this context, this study aimed to describe the knowledge of the staff in the operating rooms and the central sterilization unit at Sahloul University Hospital in Sousse (Tunisia) in 2022, regarding the steps prior to the sterilization of reusable thermoresistant medical devices and to describe their practices in terms of compliance with these steps. METHOD: A descriptive study was conducted from January 2022 to June 2022 with the aforementioned staff, using a self-administered questionnaire to assess their knowledge of the pre-sterilization steps and a direct observation audit of their practices with regard to these steps. Both measurement instruments were pre-tested. RESULTS: Out of 102 self-administered questionnaires (knowledge assessment) distributed to the staff concerned, only 80 were returned and correctly filled out, giving a response rate of 78.4%. Participants' responses regarding the order of steps prior to the sterilization were incorrect in 64% of cases. With regard to the evaluation of professional practices, 224 observations were made in the study area (practice audit). In 82% of these observations, the pre-disinfection step was confused with the washing step. The use of Betadine brushes and scrubbing pads for device washing was noted in 89.3%, along with the absence of swabbing of the canals and hollow parts in 9.4% of cases and the absence of drying of the canals with compressed air. CONCLUSION: Mastery of the steps prior to sterilisation of reusable thermoresistant medical devices was insufficient in our institution, suggesting the importance of reinforcing the implementation of the procedure through a continuous training program followed by action plans.

Programme de soins psychiatriques : Etude de l'expérience de jeunes patients par une méthode qualitative: Psychiatric care program: A qualitative study of the experience of young patients.

OBJECTIVE: Ambulatory care without consent is widely used, but it is controversial, and its effectiveness has not yet been proven. The patient experience remains largely unstudied in France, particularly that of young patients, yet their adherence to care in the early stages of the disease is complex and has an impact on their prognoses. The aim of this study is to investigate the experience of young patients undergoing a program of care (POC) in order to suggest ways of optimizing their care and to enrich the debate on the use of POCs. METHOD: Semi-structured interviews were conducted with 11 patients between ages 20 and 32, who were either undergoing a POC or had done so in the previous 5 years, followed by a semi-pragmatic phenomenological analysis of the resulting transcripts. RESULTS: (1) The outpatient experience with restraints is contradictory, ranging from deprivation and intrusion to support and protection. (2) The POC is portrayed as a framework for developing awareness of disorders and acceptance of care. (3) Patients report a lack of information about care with restraints, even to the point of being unaware of its existence. Patient-psychiatrist interactions within the POC are complicated by the restraints, but remain perceived as a care relationship. (4) They report constraints in their daily lives in connection to the POC, which can complicate professional involvement. CONCLUSION: The results support the importance of nurturing the therapeutic relationship within the POC and involving patients more in their care, starting with better information. They support the use of POCs as a temporary tool to be combined with work on adherence to treatment and support for social and professional reintegration. SPECIALTY: Psychiatry.

OBJECTIF: Les dispositifs de soins ambulatoires sans consentement sont largement utilisés, mais ils sont controversés et leur efficacité n'est pas prouvée à ce jour. L'expérience des patients concernés reste très peu étudiée en France, et notamment celui des jeunes patients, or leur adhésion aux soins en début de maladie est complexe et a un impact sur le pronostic futur. L'objectif est d'étudier l'expérience de jeunes patients suivis en programme de soins (PDS) afin de proposer des perspectives pour optimiser leur prise en charge et d'enrichir la réflexion sur l'utilisation des PDS. MÉTHODE: Des entretiens semi-directifs ont été réalisés auprès de 11 patients de 20 à 32 ans suivis en PDS ou l'ayant été dans les 5 dernières années, puis une analyse phénoménologique de type sémio-pragmatique a été réalisée sur les verbatims obtenus. RÉSULTATS: (1) L'expérience de la contrainte en ambulatoire est contrastée avec un vécu de privation de libertés et d'intrusion, mais aussi de soutien et de protection. (2) Le PDS est représenté comme un cadre permettant l'évolution de la conscience des troubles et de l'acceptation des soins. (3) Les patients rapportent un manque d'information sur les modalités de soins sous contrainte, pouvant aller jusqu'à la méconnaissance de l'existence de cette mesure. Les interactions patient-psychiatre au sein du PDS sont complexifiées par la contrainte mais restent perçues comme une relation de soin. (4) Ils rapportent des contraintes dans la vie quotidienne liées au PDS et qui peuvent compliquer l'insertion professionnelle. CONCLUSION: Les résultats soutiennent l'importance de soigner la relation thérapeutique au sein du PDS et d'impliquer davantage le patient dans sa prise en charge, en commençant par une meilleure information. Ils soutiennent une utilisation du PDS comme un outil temporaire à associer à un travail sur l'adhésion aux soins et à un accompagnement à la réinsertion sociale et professionnelle. SPÉCIALITÉ: Psychiatrie.

[An update on total neoadjuvant treatment of adenocarcinoma of the rectum]. / Actualisation des données sur le traitement néoadjuvant total de l'adénocarcinome du rectum.

A major advance has been made in the management of rectal cancer, with the emergence in 2021 of total neoadjuvant treatment. The main publications from the RAPIDO and PRODIGE-23 trials reported a significant improvement in progression-free survival and the pathological complete response rate. The aim of this review is to synthesize recent data on neoadjuvant treatment of rectal cancer, to explain the long-term results of the RAPIDO and PRODIGE-23 trials, and to put them into perspective, considering current advances in de-escalation strategies. The update of the 5-year survival data from the RAPIDO trial highlights an increased risk of loco-regional relapse, with 11.7% of relapses in the experimental group and 8.1% in the control group, while the update of the PRODIGE-23 trial confirms the benefits of this treatment regimen, with a significant improvement in overall survival. In addition, the results of the OPRA and PROPSPECT trials confirm the benefit of total neoadjuvant treatment with induction chemotherapy, as well as the possibility of surgical de-escalation in the OPRA trial and radiotherapy in the PROSPECT trial. The challenge for the future is to identify patients who require total neoadjuvant treatment with the aim of curative surgery to obtain a cure without local or distant relapse, and those for whom therapeutic de-escalation can be envisaged.

[Administration of anti-HER2 and satisfaction of patients treated for breast cancer]. / Administration des anti-HER2 et satisfaction des patients avec un cancer du sein.

INTRODUCTION: Quality of life (QoL) and patient satisfaction are major concerns in oncology. METHODS: The aim of this prospective observational study was to evaluate these parameters according to the mode of administration of anti-HER2 (subcutaneous [SC] versus intravenous [IV]), the place of administration (Home Hospitalization or HOD versus hospital) for patients supervised by an advanced practice nurse (APN). RESULTS: Between January 2022 and June 2023, 32 patients were included. They were statistically more satisfied with subcutaneous management (P=0.0004), a result explained by the speed of administration (43.5%), comfort during administration (26%) even though some expressed pain on injection and felt less anxiety (26%). Management by the APN seems more appropriate when anti-HER2 drugs were administered in HOD. In HOD, patients perceived an overall improvement in their quality of life, appetite and cognitive abilities, with a reduction in fatigue, pain and depression (P<0.05). However, the rate of outsourcing to HOD remained too low (30.4%), as 56.3% of patients would have liked to be cared for in HOD if they had had the opportunity. CONCLUSION: SC administration of anti-HER2 under the supervision of an APN has advantages for the patient, resulting in greatest satisfaction and improved patient QOL, preferably in HOD.

[Children with atopic eczema and their environment]. / L'enfant atteint d'eczéma atopique et son environnement.

Taking an interest in the environment of a child suffering from eczema means understanding the word "environment" in the broadest possible sense: the child's lifestyle, family, social and cultural environment. By taking all these aspects into account, we can optimize the effectiveness of treatments, and avoid the multiple problems and comorbidities associated with moderate and severe eczema. It's up to caregivers to be vigilant about this, and to reposition the right gestures by spotting errors right from the start, even in the case of mild eczema. The best way to respond to this challenge, i.e. to help parents understand, is to draw on the principles of therapeutic patient education.

[What is the cross-disciplinary pathway for a patient receiving gene therapy?] / Quel parcours transversal pour un patient recevant une thérapie génique ?

The Department of Hematology-Immunology, headed by Professor Jean-Hugues Dalle, is the leading department in France for the care of patients receiving pediatric hematopoietic stem cell transplants. Since 2018, it has been a "major player" in the development of innovative treatments, such as gene therapy. To date, it is the only care service in France, which offers this treatment to stop the neurodegenerative progression of two genetic diseases, in collaboration with the neuropediatric service of the Kremlin-Bicêtre hospital headed by Prof Kumaran Deiva, in conjunction with the national reference center for rare disaeses didicated to leucodystrophies. It is a complex care that requires all the actors a cooperation (parents-children-caregivers).

[Nurse coordinators and IPAs in Parkinson's expert centers: what are their roles?] / Infirmières coordinatrices et IPA en centre expert Parkinson : quels sont leurs rôles ?

The creation of Parkinson's expert centers (PEC) led to the emergence of new nurses, known as coordination nurses. And in 2018, the emergence of advanced practice nursing led to the creation of a new category of professionals, advanced practice nurses (APN). Nurses coordinators and APN play a major role in meeting the public health challenges posed by Parkinson's disease. They are the guarantors of the implementation of a personalized care pathway, the aim of which is to offer patients an optimized quality of life. They forge an indispensable link between the city's healthcare services and the PEC, collaborating with the many healthcare professionals who work with people suffering from Parkinson's disease.