Background. There are few data on anakinra use after failure of conventional medications for crystal-induced peripheral arthritis and/or crowned dens syndrome among complex hospitalized patients. Methods. We retrospectively analyzed the outcome of six patients affected with subacute crystal-induced arthritis who had received anakinra in second or third line therapy, including three patients with crowned dens syndrome and three others with gouty arthritis. Patients' comorbidities, reasons for anakinra use and associated drugs, and outcomes were recorded. Results. All patients presented with elevated inflammatory syndrome, systemic symptoms with poly/oligoarthritis. Except for absolute contraindications, all patients were previously treated with full or decreased dose of NSAID, colchicine, and/or glucocorticoids, with unsatisfactory response. All three gouty patients exhibited complete responses in all acute involvements under anakinra within 3 to 5 days, including one of them who needed the reintroduction of colchicine treatment that was previously unsuccessful. Crowned dens syndrome patients, including two with pseudogout and one with subacute hydroxyapatite deposition disease, needed 9 to 11 days to achieve complete response. Tolerance to anakinra was good. Conclusion. In case series of complex hospitalized patients, anakinra showed good activity in crowned dens syndrome and associated crystal-induced peripheral arthritis, with longer treatment duration than in gouty arthritis.

In Schnitzler syndrome, which is mostly diagnosed with a low and asymptomatic monoclonal peak, anakinra has always exhibited a complete but only transient control of the auto-inflammatory signs, which are induced by interleukin (IL)-1 auto-activation. We focused on the treatment of a case of Schnitzler syndrome with moderate macroglobulinemia peak. Anakinra failed to improve the severe inflammatory anaemia and the dysglobulinemia, but rituximab-dexamethasone-cyclophosphamide chemotherapy alone allowed a complete response. The correlation between the clinical, pro-inflammatory cytokines and dysglobulinemia complete controls with chemotherapy proves the following: (1) the dual action of this treatment in both the auto-inflammatory and dysglobulinemia components of the syndrome and (2) a different but entangled cytokine network in the pathogenesis of the auto-inflammatory and dysglobulinemia components of the syndrome.

Purpose Birmingham Children's Hospital in the UK provides family centred care for post-transplant medication (PTM) management. Nevertheless, PTM is often considered a burden for patients, and can be difficult to manage. This study was designed to identify the main medication issues encountered by children and their parents in order to find suitable means to improve their care management. Method Anonymised multifactorial, cross-sectional, single-site survey. Parents of children transplanted from January 2011 were recruited following two strategies: (1) self-completion of questionnaire at the outpatient department over a period of 4 weeks during May 2013, (2) telephone completion by random selection of 30 parents. Results were analysed using MS Excel 2010. Results 37 parents were recruited. Medication supply problems concerned 12 (32.4%) parents. 30 (81.1%) parents declared having almost run out of medicines, but no children missed an immunosuppressant dose. 9 (24.3%) parents experienced medication-administration problems, and 14 (37.8%) blood test issues. 14 (37.8%) parents felt they had an inadequate knowledge of side effects. However, 30 (81.1%) parents thought that their training was excellent or very good, and 36 (97.5%) that the written information provided was comprehensible and accurate. Among proposed improvements, parents’ preferences were for an online learning tool (OLT), an explanatory comic book (CB) for children and pharmaceutical consultations (PC). Conclusions To our knowledge, this study is the most comprehensive published audit concerning the care process of PTM. Although parents seemed particularly satisfied by the current training and written information, many experienced issues linked to PTM management. Based on parents’ preferences, a working party is currently considering service development options (OLT, CB, PC).

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Background Immunosuppressant treatment is a critically important aspect of post-transplant patients’ care. In our English paediatric hospital, a multidisciplinary team including doctors, specialist nurses and pharmacy staff is involved in family-centred care with post-transplant medicines (PTM) management. Although transplantation is perceived as a new chance of life, PTM are often felt as a constraint that can sometimes be difficult to manage. Purpose To identify the main medicines issues encountered by children and their parents in order to find suitable means to improve their care management. Materials and methods Anonymised multi-factorial cross-sectional survey, single-site. A questionnaire was designed to obtain parents’ experiences in the 12 months prior to data collection regarding obtaining, administering and monitoring PTM, side effects (SEs) and training. A cognisance test was conducted with 4 parents and the questionnaire modified accordingly. Parents of children transplanted from January 2011 were recruited following two strategies 1- self completion during clinic attendance at the outpatient department over a period of 4 weeks during May 2013, 2- telephone completion by randomisation of 30 parents. Results 37 parents were recruited (80.3%). Medicines supply problems (MSP) concerned 32.4% (n = 12) of parents. The main issue was that the General Practitioner declined to prescribe the medicine (s) required (35.3%). 81.1% (n = 30) of parents declared that they had almost run out of medicines but no children missed a dose because of MSP. 24.3% (n = 9) of parents experienced medicines administration problems (usually vomiting n = 7). 37.8% (n = 14) of parents were concerned by medicines-related blood test problems: obtaining the results was the main issue (n = 6). Regarding SE management, 56.6% (n = 21) of parents declared that their child had had SEs in the last year and 37.7% (n = 14) of parents felt that they had an inadequate knowledge on how to manage SEs. Concerning parents’ opinions of their training, 81.1% (n = 30) of them thought that the service was excellent or very good and 97.5% (n = 36) thought that the patient support information was comprehensible and accurate. Finally, among proposed service improvements, parents’ preferences were for an online learning tool (OLT) on medicines, a comic book (CB) to explain transplantation and medicine to the child, and the opportunity to have pharmaceutical consultations (PC). Conclusions Numerous medicines-related problems were identified. However, the current multidisciplinary team seem effective in preventing children from missing doses. Due to the problems reported concerning SE management, the development of thorough information sessions for parents in this domain should be considered. Although parents seemed particularly satisfied by the current training and documentation about medicines, many experienced issues linked to PTM management. Thus, based on parents’ preferences, a working party is currently considering service development option...