Current management of neonatal abstinence syndrome: a survey of practice in the UK and Ireland.

OBJECTIVE: To study the current management practices of neonatal abstinence syndrome (NAS) throughout the UK and Ireland and identify changes in practice from the most recent survey in 2008. DESIGN: Postal questionnaire to a consultant paediatrician or neonatologist in all 215 neonatal units in the UK and Ireland in January 2020. RESULTS: Response rate was 62%. An objective scoring tool was used in 97% of units and the Finnegan score was favoured by 70%. Morphine sulfate use as first line for the treatment of opiate withdrawal was almost universal and 70% used a dose of 40 µg/kg every 4 hours (240 µg/kg/day). Phenobarbitone administration as a second-line agent for opiate withdrawal increased to 61% of units with significant reductions in chloral hydrate and chlorpromazine use compared with the previous survey. Morphine sulfate and phenobarbitone remain the preferred first-line and second-line agents, respectively, for polysubstance withdrawal. There was a significant increase in chlorpromazine use as first line for polydrug withdrawal (1.5-14.2%). The practice of units discharging infants' home on medication increased to 46% from 29%. All units now permit breastfeeding in mothers taking methadone, compared with 81% previously. CONCLUSION AND RELEVANCE: Compared with the previous survey, improvements in evidence-based practices were noted, highlighting the benefits of this type of research. Nonetheless, significant variation still exists in some aspects of the management of NAS. Post-discharge follow-up varies widely, with particular deficits in ophthalmology follow-up.

Acute Diverticulitis in Young Patients: A Review of the Changing Epidemiology and Etiology.

Acute diverticulitis is one of the leading gastrointestinal causes for hospitalization. The incidence of acute diverticulitis has been increasing in recent years, especially in patients under 50 years old. Historically, acute diverticulitis in younger patients was felt to represent a separate entity, being more virulent and associated with a higher rate of recurrence. Accordingly, young patients were often managed differently to older counterparts. Our understanding of the natural history of this condition has evolved, and current clinical practice guidelines suggest age should not alter management. The purpose of this review is to evaluate the changing epidemiology of acute diverticulitis, consider potential explanations for the observed increased incidence in younger patients, as well as review the natural history of acute diverticulitis in the younger population.

The Epidemiology and Etiology of Right-Sided Colonic Diverticulosis: A Review.

Diverticulosis of the colon is a common condition in Western countries and most patients will remain asymptomatic, but some will present with symptoms of acute diverticulitis or bleeding. Our understanding of diverticulosis is evolving but is mostly derived from diverticulosis affecting the left-sided colon. In contrast, right-sided colonic diverticulosis (RCD) is more commonly seen in Asian countries but is much less common overall. Based on the marked differences in epidemiology, it is commonly thought that these are 2 distinct disease processes. A review of the literature describing the epidemiology and etiology of RCD was performed, with a comparison to the current understanding of left-sided diverticulosis. RCD is becoming increasingly common. The epidemiology of RCD shows it to be a mostly acquired condition, and not congenital as previously thought. Many factors in the etiology of RCD are similar to that seen in left-sided diverticulosis, with a few variations. It is therefore likely that most cases of RCD represent the same disease process that is seen in the left colon.

The prevalence of right-sided colonic diverticulosis in a New Zealand population.

BACKGROUND: Once considered to be a congenital condition, the epidemiology of right-sided colonic diverticulosis (RCD) is evolving. Acute diverticulitis (AD) is a complication of RCD which is frequently misdiagnosed as appendicitis, resulting in unnecessary surgery, as there is strong evidence supporting medical management for right-sided AD. In general, the incidence of AD correlates with the prevalence of RCD, which shows marked geographic variation. Few data reporting RCD prevalence come from Western countries, so the aim of this study is to define the prevalence of RCD in a New Zealand population. METHODS: Independent review of the imaging from 1000 consecutive patients undergoing a computed tomography Kidney/Ureter/Bladder scan for suspected urolithiasis at Christchurch Hospital between January and November 2017 was undertaken, to determine the presence or absence, and distribution of colonic diverticulosis. Patients were excluded if they had a history of colonic resection, known IBD, or were less than 18-years old. RESULTS: Thirty-one patients were excluded, leaving 969 eligible patients. Overall, 95 patients (9.8%) had RCD identified. The prevalence of RCD increased significantly with advancing age, being present in 2.3% of those aged 18-29, increasing to 20.3% in those greater than 70-years old (p < 0.001). CONCLUSION: The prevalence of RCD in a New Zealand population is relatively high and increases significantly with age. This adds support to the role of cross-sectional imaging in the evaluation of suspected appendicitis, to exclude right-sided AD. The association with advancing age supports RCD being an acquired condition rather than a congenital condition as was previously thought.

The incidence of transient infantile pseudohypoaldosteronism in Ireland: A prospective study.

AIM: To review the clinical course, outcome and incidence of infantile salt wasting associated with urinary tract infection (UTI) and/or urinary tract malformation (UTM) over a two-year surveillance period on the island of Ireland. METHODS: A two-year (2013-14) prospective surveillance undertaken via the Irish and Ulster Paediatric Surveillance Units. Monthly prepaid postcards were circulated to consultant paediatricians (n = 260) at all paediatric units on the island of Ireland. Infants under one year of age presenting for the first time with hyponatraemia (Na < 130 mmol/L) and/or hyperkalaemia (K > 5.0 mmol/L) associated with urosepsis/UTM were reported. RESULTS: All 7 reported patients (6 male) had culture-proven UTI, and 5 (71%) also had an underlying UTM (one diagnosed antenatally). Four (57%) patients had a documented elevated serum aldosterone supporting secondary pseudohypoaldosteronism (PHA) as the underlying diagnosis. Data on aldosterone were not reported in the other 3 patients, but clinical features were suggestive of secondary PHA. The estimated incidence for the Irish population of transient PHA is 1 per 13,200 total live births per year. CONCLUSIONS: Salt wasting is a rare complication of UTI, especially if associated with underlying UTM. Boys appear to be at particular risk.

The clinical presentation, treatment and outcome of serologically confirmed paediatric Lyme disease in the Republic of Ireland over a 5-year period: a retrospective cohort study.

Lyme disease (LD) is the most common tick-borne illness in Europe. Population-based studies in European children are few. This study aimed to assess the incidence, clinical presentation, treatment and outcome of serologically confirmed paediatric LD in the Republic of Ireland over a 5-year period. A retrospective review of records from accredited laboratories performing Borrelia burgdorferi serological testing was undertaken. Proformas were distributed to clinicians of children and adolescents with positive Lyme serology. Data were requested regarding clinical presentation, treatment and outcome. Updated NICE guidelines were used to classify clinical cases. Serology testing for B. burgdorferi was performed on 2908 samples. Sixty-three (2.2%) children were two-tier positive, generating a crude annual incidence rate of 1.15/100,000. Proformas were returned for 55 (87%) and 47 met clinical and laboratory criteria for LD. Twenty-seven (57%) presented with non-focal symptoms (erythema migrans and/or influenza-like symptoms), and 20 (43%) with focal symptoms (cranial nerve involvement, 11; CNS involvement, 8; arthritis, 1). Median age at presentation was 8.2 (2.5-17.9) years. Seventeen (36%) acquired LD overseas. Twenty-five (83%) of the remaining 30 children acquired infection in the West/Northwest of Ireland. Full resolution of symptoms was reported in 97% of those with available data. Serologically confirmed LD in children is relatively rare in the Republic of Ireland. Ninety-eight percent of children tested were seronegative. Of the seropositive cases, 40% could have been diagnosed based on clinical findings alone. Neurological presentations (40%) were common. Full resolution of symptoms occurred in almost all (97%) where data were available.

Future career intentions of higher specialist trainees in general Paediatrics.

BACKGROUND: A survey of paediatric higher specialist trainees was carried out in 2002 assessing career intentions and perception of training. Fourteen years later, with increased numbers of trainees and a national model of care and a tertiary paediatric hospital on the horizon, we re-evaluated the career intentions of the current trainee workforce. AIMS: To assess the career intentions of the current paediatric higher specialist trainees. METHODS: A 28-item questionnaire was developed based on a previously validated instrument and distributed online using the Royal College of Physicians of Ireland trainee database. RESULTS: We distributed the questionnaire to 118 eligible trainees and received responses from 92 (78%). Seventy-nine (86%) respondents desire a consultant post in Ireland. Seventy-five (82%) indicated that their preferred consultant post location was in a tertiary paediatric centre. Sixty-two trainees (67%) intend to become subspecialists with 25 (27%) planning a career in general paediatrics. This contrasts with the 2002 survey when 76% wished to work in urban centres and 61% of trainees planned a career in general paediatrics. CONCLUSION: There appears to be a mismatch between the career goals of the future paediatric consultant workforce and the requirements for staffing paediatric units nationally. This has the potential to complicate the proposed expansion of general paediatricians in regional centres and result in a significant proportion of current trainees failing to secure a post in their desired location.

Management of type 1 diabetes in primary schools in Ireland: a cross-sectional survey.

BACKGROUND: Parents reported experiences of support for diabetes management in schools are variable. Recent data from European countries are sparse and experiences in the Irish primary school setting have not been described previously. AIM: To describe parents' experiences of support for diabetes management in primary schools in Ireland. METHODS: Questionnaires were distributed through nine regional and tertiary paediatric diabetes services to parents of children aged 4-13 years with type 1 diabetes attending primary school. Data sought included patient demographics, treatment regimens, diabetes education of school staff, assistances received, and interactions between the school and family. RESULTS: Responses were received from 418 parents of primary school children with type 1 diabetes. Twenty-six percent of children were not on intensive insulin therapy. Children on a multiple daily injection regime who were unable to self-administer insulin had administration facilitated by attendance of a parent in 95% of cases. Seventy-eight percent of parents were phoned by the school regarding diabetes management, particularly those of younger children (p < 0.001). More than half of parents attended the school at least once per month to assist with diabetes management, particularly those of younger children (p < 0.001). Younger children were also more likely to have a special needs assistant (p < 0.001) and have a written management plan (p = 0.001). CONCLUSIONS: Our research has demonstrated deficits in care with respect to access to intensive insulin therapy, individualised care plans and a high burden on families which should be addressed through the National Clinical Programme for Paediatrics and Neonatology and relevant government departments.

A multicentre, benchmarking study of rectal cancer management in provincial New Zealand hospitals.

AIM: The quality of rectal cancer management within New Zealand provincial hospitals is largely unknown. This study was conducted to appraise and benchmark the management of rectal cancer in provincial New Zealand centres as compared to specialist tertiary centres. METHOD: Retrospective data was collected for all patients who underwent elective rectal cancer resection in six provincial New Zealand hospitals from January 2012 to December 2013. This was then compared with data from two tertiary hospitals over the same time period. The complete management pathway was evaluated. RESULTS: A total of 124 provincial and 145 tertiary rectal cancer resections were analysed. Completeness of preoperative staging was comparable between provincial and tertiary centres, as was type of surgical procedure performed and rates of clear surgical margins. A statistically significant difference was observed in mean number of lymph nodes analysed (10.3 v 17.2), reporting of mesorectal grade (61% v 77%), and completion colonoscopy rates (91% v 99%), all of which were lower in provincial hospitals. Multidisciplinary team discussion, rates of neoadjuvant therapy and post-operative parameters such as 30-day mortality (0.8% v 1.4%), length of stay (11.9 v 12.4 days), anastomotic leak (7% v 5%) and return to theatre (8% v 8%), were similar. CONCLUSION: Management of rectal cancer in provincial hospitals is comparable to specialist centres, however lymph node harvest, reporting of mesorectal grade and complete colonoscopy were factors identified which were lower in the provincial group. Provincial rectal cancer management remains an important resource for patients living outside major centres.

Expanding the clinical spectrum of chromosome 15q26 terminal deletions associated with IGF-1 resistance.

Haploinsufficiency of the insulin-like growth factor-1 receptor (IGF1R) gene on chromosome 15q26.3 is associated with impaired prenatal and postnatal growth, developmental delay, dysmorphic features and skeletal abnormalities. Terminal deletions of chromosome 15q26 arising more proximally may also be associated with congenital heart disease, epilepsy, diaphragmatic hernia and renal anomalies. We report three additional cases of 15q26 terminal deletions with novel features which may further expand the spectrum of this rarely reported contiguous gene syndrome. Phenotypic features including neonatal lymphedema, aplasia cutis congenita and aortic root dilatation have not been reported previously. Similarly, laboratory features of insulin-like growth factor 1 (IGF-1) resistance are described, including markedly elevated IGF-1 of up to +4.7 SDS. In one patient, the elevated IGF-1 declined over time and this coincided with a period of spontaneous growth acceleration. CONCLUSION: Deletions of 15q26 are a potential risk factor for aortic root dilatation, neonatal lymphedema and aplasia cutis in addition to causing growth restriction. What is Known: • Terminal deletions of chromosome 15q26 are associated with impaired prenatal and postnatal growth, developmental delay, dysmorphic features and skeletal abnormalities. What is New: • Neonatal lymphedema, aplasia cutis congenita and aortic root dilatation have not been previously described in 15q26 terminal deletions and may represent novel features. • IGF-1 levels may be increased up to 4.7 SDS.

Effectiveness of a Predictive Algorithm in the Prevention of Exercise-Induced Hypoglycemia in Type 1 Diabetes.

BACKGROUND: Sensor-augmented pump therapy (SAPT) with a predictive algorithm to suspend insulin delivery has the potential to reduce hypoglycemia, a known obstacle in improving physical activity in patients with type 1 diabetes. The predictive low glucose management (PLGM) system employs a predictive algorithm that suspends basal insulin when hypoglycemia is predicted. The aim of this study was to determine the efficacy of this algorithm in the prevention of exercise-induced hypoglycemia under in-clinic conditions. METHODS: This was a randomized, controlled cross-over study in which 25 participants performed 2 consecutive sessions of 30 min of moderate-intensity exercise while on basal continuous subcutaneous insulin infusion on 2 study days: a control day with SAPT alone and an intervention day with SAPT and PLGM. The predictive algorithm suspended basal insulin when sensor glucose was predicted to be below the preset hypoglycemic threshold in 30 min. We tested preset hypoglycemic thresholds of 70 and 80 mg/dL. The primary outcome was the requirement for hypoglycemia treatment (symptomatic hypoglycemia with plasma glucose <63 mg/dL or plasma glucose <50 mg/dL) and was compared in both control and intervention arms. RESULTS: Results were analyzed in 19 participants. In the intervention arm with both thresholds, only 6 participants (32%) required treatment for hypoglycemia compared with 17 participants (89%) in the control arm (P = 0.003). In participants with a 2-h pump suspension on intervention days, the plasma glucose was 84 ± 12 and 99 ± 24 mg/dL at thresholds of 70 and 80 mg/dL, respectively. CONCLUSIONS: SAPT with PLGM reduced the need for hypoglycemia treatment after moderate-intensity exercise in an in-clinic setting.

Prevention of Insulin-Induced Hypoglycemia in Type 1 Diabetes with Predictive Low Glucose Management System.

BACKGROUND: Sensor-augmented pump therapy (SAPT) with algorithms to predict impending low blood glucose and suspend insulin delivery has the potential to reduce hypoglycemia exposure. The aim of this study was to determine whether predictive low glucose management (PLGM) system is effective in preventing insulin-induced hypoglycemia in controlled experiments. METHODS: Two protocols were used to induce hypoglycemia in an in-clinic environment. (A) Insulin bolus: Insulin was administered as a manual bolus through the pump. (B) Increased basal insulin: Hypoglycemia was induced by increasing basal rates overnight to 180%. For both protocols, participants were randomized and studied on 2 separate days; a control day with SAPT alone and an intervention day with SAPT and PLGM activated. The predictive algorithm was programmed to suspend basal insulin infusion when sensor glucose was predicted to be <80 mg/dL in 30 min. The primary outcome was the requirement for hypoglycemia treatment (symptomatic hypoglycemia or plasma glucose <50 mg/dL) and was compared in both control and intervention arms. RESULTS: With insulin bolus, 24/28 participants required hypoglycemia treatment with SAPT alone compared to 5/28 participants when PLGM was activated (P ≤ 0.001). With increased basal rates, all the eight SAPT-alone participants required treatment for hypoglycemia compared to only one with SAPT and PLGM. There was no post pump-suspend hyperglycemia with insulin bolus (P = 0.4) or increased basal rates (P = 0.69) in participants with 2-h pump suspension on intervention days. CONCLUSIONS: SAPT with PLGM reduced the requirement for hypoglycemia treatment following insulin-induced hypoglycemia in an in-clinic setting.

Aromatase inhibitors for short stature in male children and adolescents.

BACKGROUND: As a result of the essential role of oestrogens in epiphyseal closure, aromatase inhibitors have been trialled as an intervention to improve height outcomes in male children and adolescents by inhibiting the conversion of testosterone to oestradiol. OBJECTIVES: To assess the effects of aromatase inhibitors in male children and adolescents with short stature. SEARCH METHODS: To identify relevant trials, we searched the Cochrane Library (2014, Issue 7), MEDLINE, EMBASE, and the World Health Organization (WHO) ICTRP trial register from their inception until August 2014. In addition, we conducted citation searches and screened reference lists of included trials. SELECTION CRITERIA: We included randomised controlled trials (RCTs) if they compared use of an aromatase inhibitor with placebo in male children and adolescents with short stature. DATA COLLECTION AND ANALYSIS: Two authors independently screened titles and abstracts for relevance. Both authors carried out screening for inclusion, data extraction, and risk of bias assessment, with any disagreements resolved following discussion. We assessed trials for quality of evidence using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) instrument. We contacted study authors regarding missing information. Primary outcomes were final or near-final height, adverse events, and health-related quality of life. Secondary outcomes included all-cause mortality, cognitive outcomes, socioeconomic effects, laboratory measures, short-term growth parameters, and assessment of effects on bone health. Meta-analysis was not appropriate due to the substantial clinical heterogeneity between trials; we presented the findings of the review in narrative format. MAIN RESULTS: We included four RCTs involving 207 participants (84 on interventions) in the review. Trials included males with constitutional delay of growth and puberty (CDGP), idiopathic short stature (ISS), and growth hormone (GH) deficiency. Three of the trials had an overall low or unclear risk of bias for primary outcomes. Short-term growth outcomes, such as predicted adult height, improved in all trials. Just one trial reported the primary outcome of final and near-final height as an extension under non-randomised conditions. None of the trials assessed health-related quality of life. One publication provided detailed information regarding the incidence of adverse events. A significant proportion (45%) of prepubertal boys with ISS treated with letrozole developed mild morphological abnormalities of their vertebrae, compared with none in the placebo group. AUTHORS' CONCLUSIONS: Available evidence suggested that aromatase inhibitors improved short-term growth outcomes. There was no evidence to support an increase in final adult height, based on limited data, with only one of four trials publishing final height data under non-randomised conditions.

Sideroblastic anaemia and primary adrenal insufficiency due to a mitochondrial respiratory chain disorder in the absence of mtDNA deletion.

A fatigued 8-year-old boy was found to have sideroblastic anaemia (haemoglobin 7.8 g/dL) which over time became transfusion dependent. Subtle neurological dysfunction, initially manifesting as mild spastic diplegia, was slowly progressive and ultimately led to wheelchair dependence. Elevated plasma lactate and urinary 3-methylglutaconate led to a muscle biopsy which confirmed partial complex IV deficiency. PCR in leucocytes and muscle was negative for mitochondrial DNA (mtDNA) deletions. Faltering growth prompted an insulin tolerance test which confirmed growth hormone sufficiency and adrenal insufficiency. Plasma renin was elevated and adrenal androgens were low, suggesting primary adrenal insufficiency. Glucocorticoid and mineralocorticoid replacement therapy was initiated. A renal tubular Fanconi syndrome and diabetes mellitus developed subsequently. Sideroblastic anaemia and primary adrenal insufficiency, both individually and collectively, are associated with mtDNA deletion; however, absence of the same does not exclude the possibility that sideroblastic anaemia and primary adrenal insufficiency are of mitochondrial origin.

How might immunization rates change if cost sharing is eliminated?

OBJECTIVES: There is a debate regarding the effect of cost sharing on immunization, particularly as the Affordable Care Act will eliminate cost sharing for recommended vaccines. This study estimates changes in immunization rates and spending associated with extending first-dollar coverage to privately insured children for four childhood vaccines. METHODS: We used the 2008 National Immunization Survey and peer-reviewed literature to generate estimates of immunization status for each vaccine by age group and insurance type. We used the Truven Health Analytics 2006 MarketScan Commercial Claims and Encounters Database of line-item medical claims to estimate changes in immunization rates that would result from eliminating cost sharing, and we used the Kaiser Family Foundation/Health Research and Educational Trust Employer Health Benefits Survey to determine the prevalence of coverage for patients with first-dollar coverage, patients who face office visit cost sharing, and patients who face cost sharing for all vaccine cost components. We assumed that once cost sharing is removed, coverage rates in plans that impose cost sharing will rise to the level of plans that do not. RESULTS: We estimate that immunization rates would increase modestly and result in additional direct spending of $26.0 million to insurers/employers. Further, these payers would have an additional $11.0 million in spending associated with eliminating cost sharing for children already receiving immunizations. CONCLUSIONS: The effects of eliminating cost sharing for vaccines vary by vaccine. Overall, immunization rates will rise modestly given high insurance coverage for vaccinations, and these increases would be more substantial for those currently facing cost sharing. However, in addition to the removal of cost sharing for immunizations, these findings suggest other strategies to consider to further increase immunization rates.