Continuous Glucose Monitoring in the Management of Neonatal Hypoglycaemia

A quality improvement project was carried out in a Level 3 Neonatal Intensive Care Unit (NICU) which aimed to successfully implement the use of continuous glucose monitoring systems (CGMS) in hypoglycaemic infants. Piloting of the device revealed several potential practical barriers to its reliably successful implementation. Five Plan-Do-Study-Act (PDSA) cycles followed, tackling these problems and other issues inductively identified throughout the project. Parents and multi-professional stakeholders were involved and consulted throughout. Change was measured on a runchart using qualitative and quantitative feedback. Problem rate per patient was reduced to zero by the end of a one-month study period. This study used basic quality improvement methodologies to implement a change intervention in a structured manner and elucidated aspects of its use that need to be adapted for its successful incorporation into real-life clinical practice.

The Prolonged Neonatal Admission: Implications for our National Children's Hospital.

A significant number of neonates are admitted to tertiary paediatric units for prolonged stays annually, despite limited availability of neonatal beds. As the three Dublin paediatric hospitals merge, this pressure will be transferred to our new National Children's Hospital. We analysed epidemiological trends in prolonged neonatal admissions to the 14-bed neonatal unit in The Children's University Hospital, Temple Street, Dublin. This was with a view to extrapolating this data toward the development of a neonatal unit in the National Children's Hospital that could accommodate for this complex, important, and resource-heavy patient population. Four hundred and thirty-six babies between 0 and 28 days of life were admitted to our neonatal unit for prolonged stays (three cohorts: >1 month and <3months, >3months and <6months, and >6months), between 2000-2014. Mean number of prolonged admissions >1 month was 29.1 per year (range 18-43). Median length of stay (LOS) was 42 days (range 29-727). 363 babies were admitted for >1month but <3months with a median LOS 38 days (range 28-90); 54 babies were admitted for >3months but <6months with a median LOS 111 days (range 91-179); 19 babies were admitted for >6months with a median LOS 331 (range 196-727). There has been a statistically significant upward trend in the number of prolonged admissions over last fifteen years (Spearman's rho p=0.01, correlation coefficient 0.848). There has been no significant increase in the median length of stay over time. It can be extrapolated, that in the new children's hospital must be capable of dealing with at least 80 neonatal long-stay patients annually.

Failure of oral penicillin as secondary prophylaxis for rheumatic heart disease: a lesson from a low-prevalence rheumatic fever region.

Our patient is an 18-year-old Caucasian woman from the UK who developed severe mitral stenosis on a history of childhood acute rheumatic fever (ARF) and rheumatic heart disease (RHD). She had been reporting of her oral penicillin secondary prophylaxis regimen since diagnosis. At the age of 15 years, a new murmur was discovered during routine cardiac follow-up. An echocardiogram confirmed moderate-severe mitral stenosis. One year later, her exercise tolerance significantly deteriorated and she subsequently underwent balloon valvuloplasty of her mitral valve to good effect. Our case emphasises the evidence base supporting the use of monthly intramuscular penicillin injection to prevent ARF recurrence and RHD progression; it also emphasises the reduced efficacy of oral penicillin prophylaxis in this context. It particularly resonates with regions of low rheumatic fever endemicity. The long-term cardiac sequelae of ARF can be devastating; prescribing the most effective secondary prophylaxis regimen is essential.

Is the NHS best practice tariff for type 1 diabetes applicable in the Irish context?

The National Health Service in the UK has identified thirteen key standards of paediatric diabetes care. Funding depends on services meeting these standards. The aim of this study was to determine if these standards are applicable in an Irish setting. All patients attending the diabetes service during 2012 were included. Patient charts, electronic appointments, nursing notes and computerised results were used to ascertain relevant information for comparison with the NHS standards. Patients attended a mean 2.97 (SD 0.7) medical and 2.2 (SD 2.9) nursing appointments per year, with a median additional contacts of 8 nurse phone calls (range 0 - 125). Most standards were met by this service. In comparing our service to the NHS standard, we have identified a number of areas for improving our service provision. Limited resources and staff shortages make a number of these standards unachievable, namely annual dietetic review and three monthly outpatient appointments.

Difficulties associated with diabetes management during the Junior Certificate examination.

The aim of this study was to describe the adherence to recommended diabetes care during the Junior Certificate, and the utilisation of available allowances for children with type 1 diabetes. Questionnaires were sent within 3 months of the examination to all adolescents and their families attending our service completing the Junior Certificate in June of 2012. Fifteen of the 25 (60%) patients/parents completed the questionnaires. Five (33%) had higher than usual glucose readings during the examination period and three (20%) experienced hypoglycaemia during at least one exam. Nine (60%) never checked capillary glucose levels during the exams. No patients left the examination area to perform diabetes related tasks. Thirteen (86%) brought fast acting glucose into the examination centre while only six (40%) brought a glucometer. Just four (27%) patients availed of the rest breaks allowed and six (40%) felt that their diabetes affected their examination performance.

The evolving course of HNF4A hyperinsulinaemic hypoglycaemia--a case series.

BACKGROUND: Hepatocyte nuclear factor 4 alpha (HNF4A) gene mutations have a well-recognized role in maturity-onset diabetes of the young and have recently been described in congenital hyperinsulinism. A biphasic phenotype has been postulated, with macrosomia and congenital hyperinsulinism in infancy, and diabetes in young adulthood. In this case series, we report three children with HNF4A mutations (two de novo) and diazoxide-responsive congenital hyperinsulinism, highlighting the potential for ongoing diazoxide requirement and the importance of screening for these mutations even in the absence of family history. CASE REPORTS: All patients presented with macrosomia (mean birthweight 4.26 kg) and hyperinsulinaemic hypoglycaemia soon after birth (median age 1 day). All three (age range 7 months to 11 years 10 months) remain on diazoxide therapy, with dose requirements increasing in one patient. There was no prior family history of diabetes, neonatal hypoglycaemia or macrosomia. Parents were screened for HNF4A mutations post-diagnosis and one father was subsequently found to have maturity-onset diabetes of the young. CONCLUSIONS: This case series follows the evolving course of three patients with confirmed HNF4A-mediated congenital hyperinsulinism, highlighting (1) the variable natural history of these mutations, (2) the potential for prolonged diazoxide requirement, even into adolescence, and (3) the need for screening, regardless of family history.

Short stature in child with early-onset diabetes.

We present a girl who initially presented at 12 weeks of age with antibody negative diabetes. Genetic screening for common mutations of monogenic diabetes was negative. She was noted to have short stature at 8 years of age (height <0.4 centile), as well as overlapping toes and distal abnormalities of her fingers. On reevaluation, further investigation revealed an EIF2AK3 mutation, and a diagnosis of Wolcott Rallison syndrome was made. This case highlights the importance of close follow up of patients with neonatal diabetes for the development of syndromic features that may lead to a unifying diagnosis.