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Zusammenfassung Hintergrund: Patienten mit unilateralen Hirnschäden zeigen häufig eine kontraversive Lateropulsion (»pusher syndrome«, PS). Prismenadaption (PA) führt zu einer Neuausrichtung des propriozeptiven und visuellen Koordinatensystems und könnte daher eine wirksame Behandlungsoption für das PS sein. Methode: Das Studiendesign bestand aus einer Serie von Einzelfallinterventionen (n=14) mit vor- und nachheriger Baseline Messung. Patienten in einem subakuten Zustand nach einem zerebrovaskulären Ereignis der rechten Hemisphäre und mit einem mindestens moderatem PS (gemessen anhand der Klinischen Skala für Contraversive PusherSymptomatik (SCP) und einer Abweichung der Sitzbalance zwischen der linken und rechten Körperhälfte von mindestens 10 Prozent) wurden eingeschlossen. Die quantitativ gemessene Veränderung der Gleichgewichtsverteilung beim Sitzen war der primäre Outcome Parameter. Nach drei Baseline-Messungen im Abstand von drei Tagen (Tage 1, 4, 7) folgten die Interventionssitzungen mit PA im Abstand von drei Tagen (Tage 10, 13, 16). Nach 14 Tagen (Tag 30) wurde eine Folgemessung durchgeführt. Wir erhoben auch den Kraftgrad, den Frührehabilitation Barthel-Index (FRBI) und die Functional Independence Measure (FIM) am Tag 1 und zum Zeitpunkt der Nachfolgeuntersuchung. Die Lokalisation der Läsion wurde manuell in den MRIcron-Standard-Hirnatlas übertragen. Ergebnisse: Jede PA-Intervention verbesserte das Sitzgleichgewicht signifikant, was auf eine sofortige Wirkung von PA auf PS hindeutet. Die PA-Effekte nahmen jedoch über den Zeitraum von drei Tagen teilweise wieder ab, und, wie die Baseline-Phase zeigte, es trat eine signifikante Erholung auch unabhängig von PA auf. Die Patienten verbesserten sich auch in der SCP, dem FRBI, der FIM und der Muskelkraft. Aufgrund der generell hohen Remission kann nicht geschlossen werden, dass letztere Verbesserungen mit den PA-Interventionen zusammenhingen. Die Patienten zeigten überlappende Läsionen im rechten Putamen, im präzentralen Gyrus und in der Corona radiata. Schlussfolgerung: PA führte zu einer kurzfristigen Verbesserung der kontraversiven Lateropulsion, die aber womöglich als Nacheffekt der PA zu interpretieren ist. Es bleibt damit unklar, ob sie sich auch nachhaltig auf die Erholung vom PS auswirkt Schlüsselwörter: Schlaganfallrehabilitation, Prismenadaption, Pusher-Syndrom, Lateropulsion, Sitzbalance

Objective Down Syndrome (DS) is the commonest form of chromosomal trisomy. Genetic factors in DS may increase the risk for diabetes. This study aimed to determine whether DS is associated with increased incidence of diabetes and the relationship with obesity across the lifespan compared to controls. Methods Matched population-based cohort study (UK Clinical Practice Research Datalink, 1990-2020). Results 9,917 DS and 38,266 control patients were analysed. Diabetes rates were higher in DS individuals (incidence rate ratio 3.67; 95% CI 2.43 – 5.55; p Conclusions At younger ages, the incidence of diabetes in DS patients is up to four times that of controls. Peak mean BMI is higher and established earlier in DS, contributing to T2DM risk. Further investigation into the relationship between obesity and diabetes in DS is required to inform treatment and prevention measures.

Following severe cerebrovascular accidents, patients are often unable to dress themselves. Little is known about the persistence and treatment of this impairment.

The validity of dementia diagnostic criteria depends on their ability to distinguish dementia symptoms from pre-existing cognitive impairments. The study aimed to assess inter-rater reliability and concurrent validity of DSM-5 criteria for neurocognitive disorder in Down syndrome. The utility of mild neurocognitive disorder as a distinct diagnostic category, and the association between clinical symptoms and neurodegenerative changes represented by the plasma biomarker neurofilament light were also examined. 165 adults with Down syndrome were included. Two clinicians independently applied clinical judgement, DSM-IV, ICD-10 and DSM-5 criteria for dementia (or neurocognitive disorder) to each case. Inter-rater reliability and concurrent validity were analysed using the kappa statistic. Plasma neurofilament light concentrations were measured for 55 participants as a marker of neurodegeneration and between group comparisons calculated. All diagnostic criteria showed good inter-rater reliability apart from mild neurocognitive disorder which was moderate (k = 0.494). DSM- 5 criteria had substantial concurrence with clinical judgement (k = 0.855). When compared to the no neurocognitive disorder group, average neurofilament light concentrations were higher in both the mild and major neurocognitive disorder groups. DSM-5 neurocognitive disorder criteria can be used reliably in a Down syndrome population and has higher concurrence with clinical judgement than the older DSM-IV and ICD-10 criteria. Whilst the inter-rater reliability of the mild neurocognitive disorder criteria was modest, it does appear to identify people in an early stage of dementia with underlying neurodegenerative changes, represented by higher average NfL levels.

Mental health problems are often a feature of Down syndrome (DS). Depression in particular has features in common with physical health conditions and dementia, from which it needs to be distinguished. Regression syndrome has recently been recognized as a rare but important condition in younger individuals. The assessment and treatment approaches to these and other conditions are summarized.

During the COVID-19 pandemic, people with Down syndrome (DS) have experienced a more severe disease course and higher mortality rates than the general population. It is not yet known whether people with DS are more susceptible to being diagnosed with COVID-19.To explore whether DS is associated with increased susceptibility to COVID-19.Matched-cohort study design using anonymised primary care electronic health records from the May 2021 release of Clinical Practice Research Datalink (CPRD) Aurum.Electronic health records from approximately 1400 general practices (GPs) in England.8854 people with DS and 34,724 controls matched for age, gender and GP who were registered on or after the 29th January 2020.The primary outcome was COVID-19 diagnosis between January 2020 and May 2021. Conditional logistic regression models were fitted to estimate associations between DS and COVID-19 diagnosis, adjusting for comorbidities.Compared to controls, people with DS were more likely to be diagnosed with COVID-19 (7.4% vs 5.6%, p ≤ 0.001, odds ratio (OR) = 1.35; 95% CI = 1.23-1.48). There was a significant interaction between people with DS and a chronic respiratory disease diagnosis excluding asthma and increased odds of a COVID-19 diagnosis (OR = 1.71; 95% CI = 1.20-2.43), whilst adjusting for a number of comorbidities.Individuals with DS are at increased risk for contracting COVID-19. Those with underlying lung conditions are particularly vulnerable during viral pandemics and should be prioritised for vaccinations.

After treatment, many rectal cancer survivors are confronted with ongoing bowel problems, called low anterior resection syndrome. The cancer diagnosis and treatment poses a burden on informal caregivers and results in higher levels of psychological distress and loneliness which is persistent after treatment as well. Our study aimed to investigate the experiences and needs of partners as informal caregivers of patients with major low anterior resection syndrome.A ground theory study was conducted. Semi-structured interviews with partners as informal caregivers of patients with major low anterior syndrome were performed in three hospitals between 2017 and 2019. In the first phase, maximum variation sampling was used and later theoretical sampling. Data analysis was done using the constant comparative method and investigators triangulation.Twenty partners as informal caregivers were interviewed until data saturation. Low anterior resection syndrome of their partner was overwhelming and they failed to live a normal life. They had the feeling that they stood at the side-line and partly because of that felt lonely. Partners experienced three levels of loneliness: because of their changed own life, the changed life of their partner and the changed life in the environment.Low anterior resection syndrome has a large impact on the lives of partners as informal caregivers and induces loneliness at three levels. It is key that the HCPs of the interdisciplinary team understand this impact. A clinical nurse specialist/oncology nurse navigator could help in alleviate that burden by addressing both patients' and partners' needs.

ObjectivesThis study explores the hospital journey of patients with intellectual disabilities (IDs) compared with the general population after admission for COVID-19 during the first wave of the pandemic (when demand on inpatient resources was high) to identify disparities in treatment and outcomes.DesignMatched cohort study; an ID cohort of 506 patients were matched based on age, sex and ethnicity with a control group using a 1:3 ratio to compare outcomes from the International Severe Acute Respiratory and emerging Infections Consortium WHO Clinical Characterisation Protocol UK.SettingAdmissions for COVID-19 from UK hospitals; data on symptoms, severity, access to interventions, complications, mortality and length of stay were extracted.InterventionsNon-invasive respiratory support, intubation, tracheostomy, ventilation and admission to intensive care units (ICU).ResultsSubjective presenting symptoms such as loss of taste/smell were less frequently reported in ID patients, whereas indicators of more severe disease such as altered consciousness and seizures were more common. Controls had higher rates of cardiovascular risk factors, asthma, rheumatological disorder and smoking. ID patients were admitted with higher respiratory rates (median=22, range=10–48) and were more likely to require oxygen therapy (35.1% vs 28.9%). Despite this, ID patients were 37% (95% CI 13% to 57%) less likely to receive non-invasive respiratory support, 40% (95% CI 7% to 63%) less likely to receive intubation and 50% (95% CI 30% to 66%) less likely to be admitted to the ICU while in hospital. They had a 56% (95% CI 17% to 102%) increased risk of dying from COVID-19 after they were hospitalised and were dying 1.44 times faster (95% CI 1.13 to 1.84) compared with controls.ConclusionsThere have been significant disparities in healthcare between people with ID and the general population during the COVID-19 pandemic, which may have contributed to excess mortality in this group.

Purpose After the reversal of the temporary stoma, rectal cancer survivors are often confronted with bowel complaints largely impacting on their quality of life. This systematic review aims to identify and synthesise the experiences and needs of patients with rectal cancer confronted with bowel problems after stoma reversal. Methods A systematic search was performed through Pubmed, CINAHL and Web of Science . Only studies with a qualitative design were included in this review. Quality assessment was done by the critical appraisal skill programme (CASP) Qualitative Studies Checklist. A thematic-synthesis was performed. Results Of 2713 identified papers, 10 were included in this systematic review. Two general themes were identified: ‘experiences and needs about bowel function before surgery’ and ‘experiences and needs afterwards’. Before restoration of continuity patients had to cope with the temporary stoma, and they felt uncertain about what to expect. Patients indicated that the timing of providing information was crucial but varied. Bowel problems after surgery had a physical and emotional impact on patients' family life. They were also confronted with shame and stigma. Patients were happy to be alive and cancer free but were hopeful that the bowel problems would resolve. They used several strategies to manage and cope with these symptoms. Peers and healthcare professionals proved valuable resources of support. Conclusion Rectal cancer survivors experience ongoing bowel problems after treatment. Patients describe experiences and needs before rectal cancer surgery and afterwards when confronting with bowel problems. Follow-up care should be organised proactively and focus on management strategies and emotional support.

Summary Background Adults with Down syndrome are at an ultra-high risk of Alzheimer's disease, but diagnosis of Alzheimer's disease in this population is challenging. We aimed to validate the clinical utility of plasma neurofilament light chain (NfL) for the diagnosis of symptomatic Alzheimer's disease in Down syndrome, assess its prognostic value, and establish longitudinal changes in adults with Down syndrome. Methods We did a multicentre cohort study, including adults with Down syndrome (≥18 years), recruited from six hospitals and university medical centres in France, Germany, Spain, the UK, and the USA, who had been assessed, followed up, and provided at least two plasma samples. Participants were classified by local clinicians, who were masked to biomarker data, as asymptomatic (ie, no clinical suspicion of Alzheimer's disease), prodromal Alzheimer's disease, or Alzheimer's disease dementia. We classified individuals who progressed along the Alzheimer's disease continuum during follow-up as progressors. Plasma samples were analysed retrospectively; NfL concentrations were measured centrally using commercial kits for biomarker detection. We used ANOVA to evaluate differences in baseline NfL concentrations, Cox regression to study their prognostic value, and linear mixed models to estimate longitudinal changes. To account for potential confounders, we included age, sex, and intellectual disability as covariates in the analyses. Findings Between Aug 2, 2010, and July 16, 2019, we analysed 608 samples from 236 people with Down syndrome: 165 (70%) were asymptomatic, 32 (14%) had prodromal Alzheimer's disease, and 29 (12%) had Alzheimer's disease dementia; ten [4%] participants were excluded because their classification was uncertain. Mean follow-up was 3·6 years (SD 1·6, range 0·6–9·2). Baseline plasma NfL concentrations showed an area under the receiver operating characteristic curve of 0·83 (95% CI 0·76–0·91) in the prodromal group and 0·94 (0·90–0·97) in the dementia group for differentiating from participants who were asymptomatic. An increase of 1 pg/mL in baseline NfL concentrations was associated with a 1·04-fold risk of clinical progression (95% CI 1·01–1·07; p=0·0034). Plasma NfL concentrations showed an annual increase of 3·0% (95% CI 0·4–5·8) per year in the asymptomatic non-progressors group, 11·5% (4·9–18·5) per year in the asymptomatic progressors group, and 16·0% (8·4–24·0) per year in the prodromal Alzheimer's disease progressors group. In participants with Alzheimer's disease dementia, NfL concentrations increased by a mean of 24·3% (15·3–34·1). Interpretation Plasma NfL concentrations have excellent diagnostic and prognostic performance for symptomatic Alzheimer's disease in Down syndrome. The longitudinal trajectory of plasma NfL supports its use as a theragnostic marker in clinical trials. Funding AC Immune, La Caixa Foundation, Instituto de Salud Carlos III, National Institute on Aging, Wellcome Trust, Jerome Lejeune Foundation, Medical Research Council, National Institute for Health Research, EU Joint Programme–Neurodegenerative Disease Research, Alzheimer's society, Deutsche Forschungsgemeinschaft, Stiftung fur die Erforschung von Verhaltens und Umwelteinflussen auf die menschliche Gesundheit, and NHS National Institute of Health Research Applied Research Collaborations East of England, UK.

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Rectal cancer survivors are often confronted with long-term bowel function impairment, called low anterior resection syndrome. This has a large impact on patients' lives. The aim was to explore the experiences of patients with major low anterior resection syndrome with a specific focus on hope and loneliness.A grounded theory approach was used. Individual semi-structured interviews (n = 28) were performed between 2017 and 2019. Patients were recruited in three hospitals and a call was distributed in two patients' organizations. During data analysis, the constant comparative method and investigators' triangulation was used.Hope was important at different stages in the trajectory. After treatment patients hoped to pick up their previous life. Confrontation with low anterior resection syndrome was unexpected and patients hoped to manage it through trial and error. They lost hope if further improvement was absent. At that turning point some tried to accept the new lifestyle, others pushed their boundaries and some opted for a permanent stoma. Loneliness presented itself in several layers. Patients experience loneliness due being toilet-bound, changes in their lives and activities outside their homes and the impact on their family life.Assessment of the impact on patients' lives and tailored counselling is possible if healthcare professionals gain more insight into the different layers of loneliness and where the patient is located in the trajectory of hope.

Esophageal cancer (EC) remains an aggressive disease with a poor survival. Management of metastatic EC is limited to palliative chemotherapy (CT). Scientific contributions regarding the role of surgery are scarce and controversial. We analysed outcome of surgically treated metastatic EC patients.We retrospectively identified surgically treated metastatic EC patients from our esophagectomy database. The aim of this study was to evaluate surgical complications, pathological response, oncological outcome and mean survival of these aggressively treated stage IV cancer patients.Twelve stage IV patients with disease presentation limited to outfield lymph node (LN) and/or liver metastasis were treated with an aggressive multimodality treatment including surgery. Mean age was 58 years (75% male, 75% Adenocarcinomas). Median postoperative hospital stay was 15 d. Radiological anastomotic leakage occurred in one patient. In hospital, mortality was nil. Complete resection was achieved in all but one. Metastatic recurrence occurred in 64% of R0 resected patients. At date of censoring, after a median follow-up of 22 months, 50% of the surgical resected patients are still alive and 33% are free of disease recurrence. Kaplan-Meier curves show a possibility to long-term survival after aggressive multimodality therapy including surgery.In selected metastatic EC patients, multimodality treatment including surgery has an acceptable surgical outcome with a potentially long-term survival.

Background: People with Down syndrome are at ultra-high risk of developing Alzheimer’s dementia. At present, there are no preventative or curative treatments. Evidence from sporadic Alzheimer’s disease literature suggests that lifestyle factors including physical activity may help maintain cognitive and functional skills and reduce dementia risk. Our study aimed to explore the association between regular exercise undertaken by participants with Down syndrome and changes in dementia-related domains of cognition and function. This was to consider whether physical activity may be a protective measure to delay cognitive decline and dementia in Down syndrome. Methods: Demographic, lifestyle, and health information was collected at baseline and at a two year follow up from 214 adults with Down syndrome without dementia, who also underwent assessment using the Cambridge Examination for Mental Disorders of Older People with Down Syndrome and Others with Intellectual Disabilities (CAMDEX-DS) and genetic analysis. Logistic regression models were used to examine the potential associations between decline in CAMDEX-DS domains and exercise whilst controlling for key variables. Results: At baseline, engaging in moderate intensity exercise was associated with a 47% reduced risk of everyday skills decline and engaging in high intensity exercise was associated with a 62% reduced risk of decline in personality and behaviour. At follow-up, high levels of exercise were associated with an 87% reduced risk of decline in personality and behaviour. Moderate intensity exercise at baseline was associated with a 62% reduction in risk of decline during the follow-up period in memory and orientation. Discussion: Based on our data it appears that regular moderate and high intensity exercise could reduce the risk of clinically detectable decline in a Down syndrome population with possible long-term benefits. People with Down syndrome may engage in less physical activity than their peers, and barriers remain which can prevent people with Down syndrome engaging in exercise. Our work highlights how important it is that people with Down syndrome are supported to be physically active, and to promote exercise as part of a healthy ageing plan. Clinical trials in this area would be justified to determine if engaging in exercise can lead to realistic improvements in maintaining functioning and delaying dementia onset in Down syndrome and to help develop guidance in this area.

Purpose The purpose of this study was to assess the relationship between the low anterior resection syndrome (LARS) and quality of life (QOL). Furthermore, in patients with major LARS, therapeutic management options were explored. Methods A cohort of surviving patients, who underwent a low anterior resection for rectal cancer after long course of radiochemotherapy, were identified. These patients were treated in Ghent University Hospital between 2006 and 2016. QOL was assessed using the European Organization for Research and Treatment of Cancer Quality Of Life questionnaire-C30 and the bowel function using the LARS-score. The relationship between LARS and QOL was analysed. Patients with major LARS (≥30 points) were contacted to explore their therapeutic management of LARS. Results 69% of the participants had major LARS. QOL was closely associated with LARS. Significant differences were found between those with and without LARS in the global health status (p ≤ 0.001) and in the following functional scales: physical (p ≤ 0.001), role (p ≤ 0.001), cognitive (p = 0.04) and social (p ≤ 0.001). Patients with major LARS experienced more diarrhea (p ≤ 0.001), fatigue (p = 0.002), insomnia (p ≤ 0.001) and pain (p = 0.02), compared to patient with no/minor LARS. Most patients tried dietary regimens (71%), medication (71%) and incontinence material (63.8%) in an attempt to manage their LARS and found some of them useful. The level of the anastomosis (low) was a significant risk factor for major LARS (p=0.03). Conclusion More than half of the patients in this cohort still suffered from major LARS. Patients confronted with major LARS had a lower QOL than patients with no/minor LARS. Currently, there is no gold standard for the management of LARS. Patients manage it through trial and error.

Introduction/Objective Schistosomiasis is extremely rare in the United States. Most patients diagnosed in the US have an international travel history or recently immigrated from endemic areas. We report a case of a 9-year-old boy who presented with a three-month history of daily abdominal pain, decreased appetite, weight loss, and microcytic anemia. The patient had alternating symptoms of constipation and diarrhea mimicking Irritable Bowel Syndrome (IBS). Methods CT scans showed distal sigmoid colon with wall thickening and hyperenhancement. Stool Helicobacter pylori antigen test was positive and hemoglobin levels were low (11 g/dL). The blood eosinophil count was initially within the normal range but was significantly elevated three months later (2,115 cells/µL). Esophagogastroduodenoscopy and colonoscopy were performed, which showed patchy areas of erythema and punctate bleeding within the descending and sigmoid colon. Results Gastrointestinal tract biopsies revealed focal, prominent eosinophilic infiltrates in the lamina propria. There were multifocal granulomas and fibrosis surrounding Schistosoma ova in the lamina propria and submucosa of duodenum, cecum, colon, and rectum. Acid-fast stain highlights the shell and spine of the ova. A retrospective chart review revealed that the patient had traveled to Yemen one month before the onset of symptoms. Schistosomiasis is among the top differentials for marked, sustained eosinophilia, especially with relevant travel history. Distortions caused by processing and sectioning can make speciation difficult on tissue sections. Acid-fast staining of the shell would favor S. mansoni over S. intercalatum; however, the S. intercalatum-S. hematobium hybrid also has an acid fast–positive shell. The duodenum is an uncommon location to find Schistosoma ova, which are usually found in the rectum and sigmoid colon. Conclusion The symptoms of gastrointestinal schistosomiasis may mimic IBS, and eosinophilia may initially be not evident as in this case. However, a high degree of suspicion is required in diagnosing schistosomiasis in patients from endemic areas.

Nous rapportons un cas d’intoxication aiguë volontaire au baclofène avec une dose ingérée probable de 3 900 mg. Le tableau clinique était celui d’un coma profond avec mydriase aréactive bilatérale et disparition des réflexes du tronc à l’exception du réflexe oculocardiaque et de la ventilation spontanée. Ce tableau persistait pendant cinq jours et évoluait vers une réversion complète des troubles et la restauration ad integrum de l’état de conscience du patient après traitement purement symptomatique. Une interaction potentielle entre baclofène, valpromide et tiapride, médicaments pris de façon chronique par le patient, est évoquée.

Desmopressin is a synthetic vasopressin analog that increases the plasma levels of coagulation factor VIII, von Willebrand factor, and tissue plasminogen activator. This hemostatic agent, which can be administered either parenterally or intranasally, has been approved for use in the prevention and treatment of hemorrhagic events during surgery in patients with hemophilia A, in cases of prolonged idiopathic bleeding, and for complications associated with platelet antiaggregant therapy. This case report describes cardiac toxicity associated with desmopressin administered according to the recommended indications: a 55-year-old woman diagnosed with Wegener's granulomatosis (WG) was treated with desmopressin to improve hemostasis and shorten bleeding time before a planned renal biopsy. She developed cardiac arrest within 60 minutes of the desmopressin injection. Cardiopulmonary resuscitation began immediately and was successful, although the patient subsequently died of WG-associated complications. Desmopressin administration thus appears, in some cases, to be associated with a high risk of thrombotic events, possibly by stimulating the rapid release of endothelial factors such as an abnormal multimeric form of von Willebrand factor, which might cause platelet aggregation. Clinicians should be aware of the possible occurrence of this little-known but potentially serious cardiac event associated with desmopressin administration and be prepared to initiate cardiopulmonary resuscitation immediately if needed.

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Introduction Les recommandations pour la prise en charge therapeutique de l’urticaire chronique spontanee (UCS) ont place l’omalizumab (OMZ) (biotherapie anti-IgE) en seconde ligne therapeutique apres les antihistaminiques a posologie augmentee. Notre etude rapporte l’experience en vraie vie de l’utilisation de cette molecule dans le service de dermatologie du CHRU de Lille. Materiel et methodes Il s’agit d’une etude descriptive, retrospective et monocentrique realisee dans le service de Dermatologie du CHRU de Lille. L’evolution dans le temps des parametres Urticaria Activity Score (UAS7) et Dermatology Life Quality Index (DLQI) a ete analysee par le test du Wilcoxon apparie ou le test du Student apparie. Une reponse complete (RC) etait definie par un score UAS7 = 0, une reponse partielle (RP) entre 1 et 6 et une absence de reponse (NR) par un UAS7 > 6. Resultats Du 1er janvier 2013 au 1er novembre 2017, 58 patients atteints d’urticaire chronique ont ete traites par OMZ dont 26 urticaires inductibles et 32 urticaires chroniques spontanees (UCS). Vingt-huit patients (5 hommes et 23 femmes) atteints d’UCS ont ete inclus pour l’analyse. Quatorze patients (50 %) presentaient un angiœdeme associe. A trois mois de l’introduction de l’OMZ, 10 RC (n = 10/22, 45,5 %), quatre RP (n = 4/22, 18,2 %) et huit NR (n = 8/22, 36,4 %) etaient observees. En moyenne, les patients recevaient 13,1 ± 8,4 injections pour une duree moyenne de traitement de 13,5 ± 8,8 mois. Pour sept patients sur 28 (26,9 %) une intensification du traitement etait necessaire pour 3 RC et 4 NR. La dose de 300 mg/15 jours etait la plus frequemment efficace (permettant d’obtenir 3 RC). Pour huit patients sur 28 (30,7 %) le traitement a pu etre espace permettant de maintenir 7 RC. La dose minimale la plus frequemment efficace etait le schema a 300 mg/5 semaines pour quatre patients. Douze patients ont arrete leur traitement dont cinq en raison d’une RC et deux en raison d’un effet indesirable. Discussion A ce jour, aucun consensus n’existe sur la maniere d’arreter le traitement en cas de reponse complete (decroissance progressive ou arret brutal). De meme, en cas d’inefficacite pour la dose usuellement reconnue et utilisee dans cette indication, aucun consensus n’existe sur la maniere d’incrementer le traitement. Faut-il privilegier une augmentation de dose tout en maintenant l’intervalle de 4 semaines ou faut-il privilegier le raccourcissement du delai inter-dose ? Ou voire modifier les deux, en proposant une dose de charge dans les UCS les plus severes notamment avec angiœdeme ? Nos donnees suggerent que plusieurs schemas sont possibles et doivent etre proposes au cas par cas dans l’attente de donnees prospectives robustes permettant la hierarchisation des schemas en comparant le schema classique de l’AMM (300 mg/mois) a un schema optimise a 300 mg/15 jours potentiellement etendu a 450 mg/15 jours en cas de reponse insuffisante.