Early Postnatal Use of Glibenclamide in Permanent Neonatal Diabetes Secondary to Antenatally Diagnosed KJCN11 Mutation.

INTRODUCTION: Heterozygous activating mutations in KCNJ11 cause both permanent and transient neonatal diabetes. A minority of patients also have neurological features. Early genetic diagnosis has important therapeutic implications as treatment with sulfonylurea provides good metabolic control and exerts a protective effect on neuromuscular function. CASE PRESENTATION: A term female infant with normal birth weight (2.73 kg, z-score: -1.69) was admitted to the Neonatal Unit at Addenbrookes Hospital. She had been antenatally diagnosed with KCNJ11 mutation-R201C inherited from her glibenclamide-treated mother who continued sulfonylurea treatment throughout pregnancy. A continuous glucose-monitoring system inserted at 20 h of age showed progressive rise of blood glucose concentrations, prompting treatment with glibenclamide on day 2 of life. Initial attempts to treat with an extemporaneous solution of glibenclamide (starting dose 0.2 mg/kg/day) resulted in inconsistent response and significant hypoglycaemia and hyperglycaemia. A licenced liquid formulation of glibenclamide (AMGLIDIA) at a starting dose of 0.05 mg/kg/day was used with stabilization of blood glucose profile within 24 h. Other than a mild transient elevation in transaminase, treatment was well tolerated. At most recent review (age 12 months), the patient remains well with age-appropriate neurodevelopment. Overall glucose control is reasonable with estimated HbA1c of 7.6% (59.9 mmol/mol). CONCLUSION: Early postnatal glibenclamide treatment of insulin-naive patients with KATP-dependent neonatal diabetes is safe, provides good metabolic control, and has a potential protective effect on neurological function. The formulation of the medicine needs to be carefully considered in the context of the very small doses required in this age group.

ASSESSING THE IMPACT OF A NEWLY INTRODUCED ELECTRONIC PRESCRIBING SYSTEM ACROSS A PAEDIATRIC DEPARTMENT - LESSONS LEARNED.

AIM: Prescribing audits have shown that the Women's and Children's Directorate reported higher number of prescription errors on the paediatric and neonatal wards compared to other areas in the Trust. Over the last three years a multidisciplinary prescribing team (PT), which included senior clinicians, pharmacists and trainees introduced a number of initiatives to improve the quality of prescribing. Strategies included structured departmental inductions, setting up of designated prescribing areas and reviewing errors with the prescriber. Year on year there were fewer prescribing errors.1 With the introduction of a new electronic prescribing system in October 2014 prescribing error rates were expected to decrease further, eradicating omissions around allergy recording, ward location and drug names. The aim of this abstract is to highlight the impact of the new system and describe lessons learned. METHOD: In the summer of 2014, all inpatient drug charts across the department were reviewed on three non-consecutive days over a period of three weeks. Prescribing errors were identified by the ward pharmacist. Errors were grouped according to type and further analyzed by the PT. Errors deemed to have no clinical significance were excluded. Error rates were compared to the previous audits performed with identical methodology. Following the introduction of the electronic prescribing system, the ward pharmacists continued to review prescription charts on daily basis and generate regular error reports to notify the staff of new challenges. RESULTS: There were 174 (14%) errors out of 1225 prescriptions on 181 drug charts. The most commonly made mistakes included drug name errors, strength of preparation, allergies and ward documentation, prescriber's signature omissions, and antibiotic review and end dates. The introduction of an electronic system has eliminated drug name, strength of preparation, allergy recording and ward errors. However, serious challenges have been identified: entering of an incorrect weight resulted in all drug dosages being inaccurate; the timing of drug levels for Vancomycin and Gentamicin and the administration of subsequent doses have been problematic. Communication difficulties between all staff groups has led to dosage omission, duplicate administration and confusion around start and stop dates. The ability to prescribe away from the bedside and indeed the ward has compounded some of these problems. CONCLUSION: The implementation of a new electronic system has reduced prescribing errors but has also resulted in new challenges, some with significant patient safety implications. The lessons learned and good practice introduced following previous audits of "traditional paper based" prescribing are equally important with electronic prescribing. Communication between staff groups is crucial. It is likely that the full benefits of the system will be realized a year after its introduction. On-going audit is required to assess the impact and safety of the electronic prescribing and lessons learned.

Accurately administering oral medication to children isn't child's play.

OBJECTIVE: Parents administer oral medications with various measuring devices including metal teaspoons, calibrated spoons and oral syringes. We aimed to determine which was the most accurate. DESIGN: Self-controlled, non-randomised, experimental study. SETTING: Caregivers attending paediatric outpatient clinics. METHODOLOGY: Caregivers measured 5 ml of 120 mg/5 ml paracetamol suspension using a 5.0 ml metal teaspoon, 5.0 ml calibrated spoon and 5.0 ml oral syringe. Samples were weighed and converted to mls. MAIN OUTCOME MEASURES: The mean volume and variance of volumes were measured for each device. RESULTS: We recruited 277 caregivers (98% parents). Volumes measured ranged from 0.83-6.52 ml. Accuracy did not vary with caregivers' age, gender, instrument preference, number and age of children. The mean volumes measured with the oral syringe (95% CI 5.09 to 5.17 ml) and metal spoon (95% CI 3.90 to 4.08 ml) were significantly different to the desired 5 ml volume (p<0.0001), dissimilar to the mean volume measured using the calibrated spoon (95% CI 4.91 to 5.09 ml, p=0.99). The variance of volumes measured with the oral syringe (SD 0.348 ml) was significantly smaller (p<0.0001) than that measured using a calibrated spoon (SD 0.762 ml) or metal spoon (SD 0.749 ml). CONCLUSIONS: The calibrated spoon was the most accurate producing a mean volume of 5 ml, while the oral syringe had the smallest variance. The increased variability of calibrated or metal spoons may result in under or overdosing especially when administering drugs with a narrow therapeutic window. Health care professionals must make a case-by-case decision regarding which device is preferable depending on the medication in question. Parental education could improve measuring accuracy.