BackgroundNeck lymphangioma is the most common lymphatic malformation of the newborn, representing about 5% of benign tumours of infancy and childhood. Due to its action on angiogenesis and cellular growth, the use of sirolimus (m-Tor inhibitor) has been proposed for treatment, as it has produced satisfactory results in some studies in children and in a few cases in neonatology.PurposeTo describe the use of sirolimus as treatment for a left laterocervical lymphangioma in a newborn.Material and methodsWe report the case of a term male newborn (3.3 kg, 51 cm, 0.22 m2) with a left laterocervical mass (13 cm) diagnosed on a prenatal ultrasound. According to the neonatal cases in the literature, an oral solution of sirolimus 1 mg/mL was started at 0.8 mg/m2/day (divided into 12 hourly doses) for 10 days of life. Sirolimus plasma levels were monitored (LC/MS/MS), with a desired target of 4–8 µg/L, as well as adverse effects and the evolution of the mass.Results Day Dose Levels (ng/mL) Triglycerides (mg/dL) Cholesterol-LDL (mg/dL) 10.08 mg bid672860.08 mg bid2389941018.531999128.7382140.1 mg qd190.1 mg qd8103123400.1 mg qd4.1167470.15 mg qd550.15 mg qd7.418096The first 2 months of treatment are described. On day 6, treatment was interrupted owing to high plasma levels. On day 14, sirolimus was reintroduced at a lower dose and once daily schedule (estimated elimination half-life of 33 hours, concordant with the neonatal literature). Posterior levels were correct and the dose was titrated according to them and the weight gain of the child. Regarding side effects, the patient experienced hypertriglyceridaemia and hypercholesterolaemia, with unaltered hepatic and renal functions associated with high sirolimus levels. He also had occasional nausea and vomiting that were appropriately managed. Posterior controls showed normalisation of triglycerides and cholesterol levels. From the beginning of treatment, the cervical mass showed a progressive reduction in size and a marked reduction in consistency.ConclusionSirolimus can be a useful option for the treatment of lymphatic malformations with few short term side effects. More data are needed to characterise the pharmacokinetics of sirolimus in the neonatal population, in order to define optimal dosing.No conflict of interest

BackgroundRecent progress in pulmonary arterial hypertension (PAH) research has made possible the expansion of treatment options improving the prognosis of patients. Due to multimorbidity and increasing age, patients are much more likely to be exposed to polypharmacy. As a result, there is an increased risk of drug–drug interactions affecting efficacy as well as the toxicity of the treatment. In 2012, the pulmonary hypertension unit of our hospital was designated as a reference unit by regional health authorities.PurposeTo identify potential drug–drug interactions (PDDIs) between oral drugs (OD) for PAH and medications used to treat concomitant illnesses.Material and methodsA cross sectional study was conducted in April 2016 in a tertiary hospital. All outpatients on OD for PAH with their chronic medication recorded in the regional electronic database were included. We collected demographic data, concomitant pharmacotherapy and PAH treatment. PDDIs were identified using the local Guide of Interactions in Pulmonary Hypertension.Results174 patients (224 OD) were included: 63.8% women, mean age 59 years (range 18–91). Most of the OD prescribed were bosentan and sildenafil (both 29%) followed by tadalafil (15.2%), ambrisentan (13.8%), riociguat (8.5%) and macitentan (4.5%). Approximately 30% of the patients received combined oral therapy. Median number of drugs per patient was 9 (range 2–19) and 83.9% were polymedicated (≥ 5 drugs). We identified a total of 237 PDDIs (67% of patients had at least one and over 30% more than two): 83% were moderate and 16% contraindicated according to their possible clinical relevance; 62% were pharmacokinetic and 38% pharmacodynamic; 70% affected the co-medication, 27% the OD and 3% both; 68% resulted in an increase in toxicity, 29% in decreased of efficacy and 3% both. The main contraindicated PDDIs were: beta-blockers with bosentan (n=10) and sildenafil (n=8), and bosentan with immunosuppressants (n=7). Polymedicated patients were more likely to have PDDIs (p<0.05).ConclusionPDDIs represent a significant issue among patients with PAH. Evaluation of these PDDIs could result in the establishment of a better treatment plan for these patients. However, more prospective studies are required to investigate the clinical relevance of PDDIs and their influence on therapeutic outcomes.References and/or acknowledgementsCiraci R, et al. Pulm Pharmacol Ther2014;28:1–8.No conflict of interest

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BackgroundAn automatic storage and picking system linked to the electronic prescription was introduced into the pharmacy of our children’s university hospital. The pharmacy prepares and distributes unit dose patient specific medication to 213 paediatric inpatients on a daily basis. Safety is one the most important objectives in our hospital, so automation of the pharmacy was introduced in order to increase it.PurposeThe aim of this study was to determine whether dispensing errors were reduced in preparing daily unit dose drugs in a paediatric hospital after the introduction of an automated storage system in comparison with traditional manual picking.Material and methodsData were collected over 2 months by checking the whole amount of medication units contained in every patient’s daily unit dose (dispensed in an individual container) before sending them to wards. It was done a month prior to the introduction of the automated storage system and the same month once the system was fully implemented. We used a chart to register every incident detected and classify it as: wrong medicine, wrong dosage, wrong pharmaceutical form, wrong patient container, excess of units or missing units.ResultsA total of 30 114 units were analysed, 17 062 of which were checked before the automated storage system was implemented in the pharmacy, and the rest (13 052 units) were examined after its implementation. Recorded errors were 186 (1.09% regarding the total units dispensed) in the first stage, before automation, and 41 (0.31%) in the second stage, after automation, resulting in a risk ratio of 3.52.Analysing the type of errors, it is important to remark that wrong medicine and wrong dosage were dramatically reduced, whereas the excess of units remained steady.ConclusionBy implementing this automatic storage and picking system, patient safety has increased on account of the decrease in the number of dispensing errors made. Indeed, we have been able to reduce those errors related to dispensing the wrong medicine or dosage, which are the most hazardous and likely to happen in a paediatric hospital owing to the large number of available pharmaceutical forms and dosages for the same drug.References and/or AcknowledgementsAcknowledgements to Juan Antonio and to all of the pharmacy staff.No conflict of interest.

BackgroundHuman normal immunoglobulin (HNIg) indications are replacement. This therapy can be administered intravenously monthly (IVIG) or subcutaneously weekly (SCIG). Due to the possibility of self-administration and a better safety profile of SCIG, this route is being increasingly preferred by patients and physicians.PurposeTo describe the changes in HNIg requirements as replacement therapy in paediatric patients with primary immunodeficiency (PID), focusing on the IVIG to SCIG switching.Material and methodsBased on medical history records, we collected the dosage of HNIg treatments of paediatric PID patients both on IVIG and SCIG, in our hospital over 12 months.Then we analysed the subgroup of patients treated with SCIG: we conducted a retrospective data collection of the previous IVIG requirements, the SCIG doses and the IgG plasma levels reached.ResultsA total of 34 patients on HNIg treatment were identified, 28 were treated with IVIG and six were treated with SCIG with a median monthly dose of 441 mg/kg and 410.8 mg/kg respectively.Focusing on the SCIG-treated patients (six active patients at the time of the study and two previously treated), with a median of 15.8 months of treatment (11–23), all of them were treated previously with IVIG, with a monthly dose of 541 mg/kg/month (442.5–702.5), reaching IgG plasma levels of 8882 mg/L (8454.5–9725). All the SCIG switches were performed using dose equivalence 1/1 of the monthly IVIG as a weekly regimen, achieving plasma levels of IgG of 10212.5 mg/L (9790.5–10847.5) on the first control (1 to 3 months after the switch). During follow-up, the monthly SCIG dose was reduced in six/eight patients (mainly by widening the administration interval from weekly to every 10 to 14 days) still keeping plasma IgG levels of 10000.5 mg/L (8515.5–10635). This dose optimisation means a 24.1% reduction between IVIG dose required previously (541.3 mg/kg/month (355–739)) to the SCIG dosing at the end of the study (410.8 mg/kg/month (332–504)).ConclusionA priori, SCIG treatments have similar dose requirements as IVIG, but we have shown that in our patients, the switch allowed a HNIg dose reduction of 24%, still keeping correct IgG plasma levels. The SCIG pharmacoeconomic profile seems to be more interesting, although other studies are lacking in validating these results.References and/or AcknowledgementsColleagues.No conflict of interest