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BackgroundAccording to the world health organization, sexual health (SH) is “a state of physical, emotional, mental and social well-being in relation to sexuality”. Studies on the impact of juvenile idiopathic arthritis (JIA) on SH are scarce especially during the critical phase of adolescence. We can ask ourselves: are health professionals (HP) “good” interlocutors for JIA patients?ObjectivesWe aimed to determine the expectations of JIA adolescents (10-19 years) and the perceptions of their parents regarding exchanges with HP in the field of SH.MethodsA multicenter survey was performed in nine French rheumatology centers and three patient associations from September 2021 to April 2022, among JIA patients, aged 18-45 years and their parents. On the advice of two child psychologists and a psychiatrist, we interviewed an adult population to obtain convincing data about their adolescence with the necessary hindsight on the subject. Self-administered questionnaires were designed (for JIA patients and parents) after an extensive literature review and experts’ consensus and distributed to participants.Results76 patients and 43 parents completed the anonymous questionnaires.Most patients were women (75%), with a mean age of 26 (7.2) years and an education level higher than high school (89%). Parents were mainly mothers (88%), with a mean age of 54 (5.6) years and an education level higher than high school (56%).Half the patients considered that JIA impacted their love life. The main causes were body complexes (46%) and low self-esteem (40%). The impact on their sex life was not clear-cut. Love life was discussed with parents for 52% and sexual life for 20% of patients. 59% of patients reported they were comfortable to discuss SH with an HP (yet, only 26% had done). Their main sources of information were referees (at school (46%), family (43%)) or social networks (34%). If patients reported that SH has been discussed, it was mainly when the HP was proactive (56%), with the hospital rheumatologist (50%), from a biomedical perspective.Focusing the needs for optimal care, patients and parents agreed to address SH during an individual patient education session in hospital (51% vs 35%), a regular consultation (47% vs 53%) or a dedicated consultation by request of the adolescent without parents being informed (38% vs 21%). Most patients and parents agreed that the HP should be proactive (78% vs 70%).At hospital, for patients, the most competent or the most affordable HP were the gynecologist (68%; 47%, respectively), the rheumatologist (55%; 41%), and the psychologist (53%; 39%). Patients and parents both considered that a peer expert would make patients feel more comfortable (38% vs 37%); however, contrary to patients, fewer parents point out their skills (46% vs 25%, p>0.0276).The opportunity of a suitable moment (64% of patients vs 53% of parents), an HP comfortable with the subject (59% vs 53%), and availability of brochures (45% vs 49%) seemed to be helpful for both. The only statistically significant difference concerned HP gender, less cited by parents (7% vs 43%, p <0.0001).The use of digital resources was significantly more cited by patients than parents (video information (29% vs 9%, p=0.0127); smartphone application (25% vs 9%, p=0.0372)).79% of patients were looking for general information (impact of JIA and treatments on sexuality), discussion (68%), reassurance (65%), and listening (51%). General information (58%, p=0.0158) and discussion (39%, p=0.0022) were significantly less cited by parents.ConclusionTo our knowledge, this is the first study to address the SH needs of adolescents with JIA. HPs should take up this real need about SH, especially in hospital for adolescents and their parents. Indeed, there are expectations directly linked to the specifics of the disease. The main difference between patients and parents would be the use of digital tools. There could be an interesting vector of communication with adolescents if the sources are reliable and parents reassured about their content.AcknowledgementsThis work has obtained the financial support of the French Society of Rheumatology.Disclosure of InterestsNone Declared.

Background Systemic juvenile idiopathic arthritis (systemic JIA) is a severe disease with both systemic and joint inflammation. This study aims to identify predictors of disease evolution within the systemic JIA population enrolled in the Juvenile Inflammatory Rheumatism cohort (JIRcohort). Methods Observational patient cohort study with 201 recruited children from 4 countries (3 European, 1 North Africa) from 2005 until 2019, using retrospectively (2005–2015) and prospectively (2015–2019) routine care collected data. Results Sixty-five patients with complete follow-up data for 24 months after first diagnosis were classified as monophasic ( n  = 23), polyphasic ( n  = 6) or persistent group ( n  = 36) corresponding to their evolution (unique flare, recurrent flares, or persistent disease activity respectively). The patients of the persistent group were more likely to have an earlier disease onset, before the age of 6 (OR 2.57, 95%-CI 0.70–9.46), persistence of arthritis at 12-months post-diagnosis (OR 4.45, 95%-CI 0.58–34.20) and higher use of synthetic DMARD (sDMARD, OR 5.28, 95%-CI 1.39–20.01). Other variables like global assessment by physician and by patient and C Reactive Protein levels at 12-months post-diagnosis were assessed but without any predictive value after adjusting for confounding factors. Conclusions Our results suggest that the earlier disease onset, the persistence of arthritis throughout the first year of disease evolution and the need of sDMARD might predict a persistent disease course.

Controlled-rate freezing and storage in vapour phase nitrogen are used by most transplantation teams for the cryopreservation and storage of peripheral blood haematopoietic stem cells (PBSC). In this study, we analysed 666 autologous PBSC transplants after uncontrolled freezing and storage of PBSC at −80 °C. Statistical analysis showed that neutrophil recovery was associated with both the infused CD34 + cell dose ( P =0.01) and the post transplantation use of growth factors ( P <0.001) and that platelet recovery was associated with the infused CD34 + cell dose ( P <0.001) and with the diagnosis ( P =0.02). We analysed three groups according to the duration of the cryopreservation period (less than 6 months, between 6 and 12 months or more than 1 year). Haematopoietic recovery was not found to be adversely affected by longer storage at −80 °C. The haematopoietic recoveries of 50 pairs of sequential transplantations from the same PBSC mobilization were analysed. Despite prolonged cryopreservation, there were no statistically significant differences in neutrophil ( P =0.09) or platelet ( P =0.22) recovery in the second compared with the first transplant. In conclusion, the long-term storage of PBSC at −80 °C after uncontrolled-rate freezing is an easy and comparatively inexpensive cryopreservation method that leads to successful haematopoietic recovery even after prolonged storage.

We report French prospective experience with reduced-intensity conditioning–allo-SCT in 46 patients (median age: 15.5 years, 4.8–20.2) presenting high-risk AL ( n =11), Hodgkin’s lymphoma ( n =15) or solid tumors ( n =20). Graft sources were BM ( n =21), PBSC ( n =20) and cord blood (CB; n =5) from related ( n =20) or unrelated ( n =26) donors. For CB grafts, only one patient out of five achieved sustained engraftment. For PBSC/BM grafts, engraftment rate was 95%, hematopoietic recovery times were not significantly different between BM, PBSC, sibling or unrelated grafts, day+100. Full donor chimerism was achieved in 94% of patients, and incidences of primary acute GVHD and chronic GVHD were 49% and 14%, respectively. Underlying disease was fatal in 39% of patients. TRM was 6.9%. Three-year OS was 49.15%. OS and EFS were not significantly different between patients transplanted with different grafts and with or without primary GVHD. Patients with solid tumor or measurable disease at transplant had poorer outcomes. Three-year EFS: 33.3% for ALL, 75.0% for AML, 51.8% for Hodgkin’s lymphoma, 28.6% for neuroblastoma and 22.2% for sarcoma patients. This multicentre study concluded that Bu/fludarabine/anti-thymocyte globulin conditioning with PB or BM, related or unrelated grafts in patients with various malignancies at high-risk for transplantation toxicity results in high engraftment rates, low TRM and acceptable survival.

Background: Leukemia is the most common cancer in pediatrics, with many late effects such as higher risk of dyslipidemia, insulin resistance, obesity, and metabolic syndrome. The objective of this work was to investigate substrate oxidation during submaximal exercise in survivors of childhood acute leukemia. Methods: A total of 20 leukemia survivors and 20 healthy children were matched by sex, age, and Tanner stage. They all took a submaximal incremental exercise test to determine fat and carbohydrate oxidation rates. Results: Cardiorespiratory fitness was significantly lower in leukemia survivors, with lower relative VO2 peaks (p < 0.001), lower heart rate values (p = 0.02), and lower exercise power (p = 0.012), whereas rest metabolism and body mass index did not differ between the two groups. During exercise, upward of heart rate relative to VO2 peak was significantly higher (p < 0.001) in childhood leukemia survivors. We found lower carbohydrate and fat oxidation rates (p = 0.07) in leukemia survivors compared with healthy children, and also a significantly lower relative maximal fat oxidation rate (p = 0.014). Conclusion: Despite impaired physical fitness and metabolic response to exercise, childhood leukemia survivors remained sensitive to physical activity interventions, and could readily adapt to submaximal exercise intensity.

The literature contains a substantial amount of information about factors that adversely influence the linear growth in up to 85% of patients undergoing haematopoietic SCT (HSCT) with TBI and/or cranial irradiation (CI) for acute leukaemia (AL). By contrast, only a few studies have evaluated the impact of growth hormone (GH) therapy on growth rate and final height (FH) in these children. We evaluated growth rates during the pre- and post-transplant periods to FH in a group of 25 children treated with HSCT ( n =22), TBI ( n =21) or/and CI ( n =8) for AL and receiving GH therapy. At the start of GH treatment, the median height Z -score was −2.19 (−3.95 to 0.02), significantly lower than at AL diagnosis ( P <0.001). Overall height gain from start of GH treatment to FH was 0.59 Z (−2.72 to 2.93) with a median height Z -score at FH of −1.35 (−5.35 to 0.27). This overall height gain effect was greater in girls than in boys ( P =0.04). The number of children with heights in the reference population range was greater after than before GH therapy ( P =0.07). At FH the GVHD and GH treatments lasting <2 years were associated with shorter FH ( P =0.02 and 0.05). We found a measurable beneficial effect of GH treatment on growth up to FH.

New concepts of allogeneic hematopoietic SCT (allo-HSCT) for neuroblastoma and other solid tumors do not rely on escalation of chemotherapy intensity and tumor load reduction but rather on a graft-vs-tumor effect. At this point, this is still an investigational and unusual application of allogeneic transplant, with 78 neuroblastoma patients reported to the European Group for Blood and Marrow Transplantation activity survey from 2002 to 2007 and less than 100 published cases. Two trends can be observed in the reviewed data: some teams have used allo-HSCT in children with refractory or progressive disease and significant tumor burden and other teams in children with CR, PR or minimal residual disease earlier in their disease process. Early studies of allo-HSCT in children with high-risk neuroblastoma suggest that this is a feasible approach that may improve outcome in this deadly disease. However, the proper timing for allo-HSCT during the disease course remains to be determined.

Background KD is the main vasculitis affecting children before 5 years and the leading cause of acquired heart disease in childhood. The epidemiologic characteristics of KD are few reported in Europe and especially in France within a population from different ethnic backgrounds. Even IVIG is still the standard treatment of KD; the management of patients at risk for cardiac complications may change toward reinforced (and new) therapeutic approaches. Objectives Kawanet is a clinical and biological data repository aimed to define the epidemiological characteristics of KD, in France. Kawanet will compare clinical characteristics between distinct ethnic backgrounds and will define risk factors for resistance to standard treatment (IVIG) and for cardiac complications Methods Targeted institutional physicians received information on a national registry for KD. All patients suspected with KD and seen since January 2011 were eligible to enter the study. An eCRF was implemented in a web secured database (CLEANWeb). IRB approval and authorization for data storage were obtained. Results 419 cases were entered into the registry and complete information was available for 351 of them, in January 2014. Patients (57% males/43% females) were enrolled by 77 physicians from 52 centres: they originated from 5 main ethnical backgrounds: 67% European Caucasian, 15% afro-Caribbean, 13% Eastern Caucasian or North African, 4% Asian and 1% of mixed ancestry. The median age at diagnosis was 2.4y (3m-26y). A family history of KD was present in 5 patients. The clinical symptoms were (%): conjunctivitis 85, cheilitis 83, diffuse exanthema 74, fever ≥5 days 63, oral erythema 61, modification of the extremities 57, cervical adenopathy 49, oedema 47, raspberry tongue 45, desquamation 40, seat erythema 26 and perineal desquamation 17. Cardiac complications were reported in 186/328 patients (42%): coronary dilatation 28%, pericarditis 17%, coronary aneurysm 4%, and myocarditis 3%. At diagnosis of 351 patients: 135 had complete KD according to the AAC definition, 56 were incomplete, 118 doubtful and 42 not classified. 327/331 (99%) patients received IVIG, 20% (n=64) required 2 courses, and 5 patients 3 courses. 11% required steroids, 93% received Aspirin and 1 Anti-TNF. The mean delay between fever onset and treatment was 6 days. Conclusions The awareness on KD appears very high in France, even the completed forms are relatively rare (38%). The rate of coronary abnormalities was elevated, in comparison to other series even though 1/3 of patients receiving IVIG had doubtful diagnosis of KD. The IVIG resistance rate was 20%. Factors associated with IVIG resistance in our population are currently analysed Acknowledgements To the French KD study group Supported by a grant from the French ministry of health: PHRCN2009 Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.3534