Multicentre service evaluation of presentation of newly diagnosed cancers and type 1 diabetes in children in the UK during the COVID-19 pandemic.

Background: The COVID-19 pandemic led to changes in patterns of presentation to emergency departments. Child health professionals were concerned that this could contribute to the delayed diagnosis of life-threatening conditions, including childhood cancer (CC) and type 1 diabetes (T1DM). Our multicentre, UK-based service evaluation assessed diagnostic intervals and disease severity for these conditions. Methods: We collected presentation route, timing and disease severity for children with newly diagnosed CC in three principal treatment centres and T1DM in four centres between 1 January and 31 July 2020 and the corresponding period in 2019. Total diagnostic interval (TDI), patient interval (PI), system interval (SI) and disease severity across different time periods were compared. Results: For CCs and T1DM, the route to diagnosis and severity of illness at presentation were unchanged across all time periods. Diagnostic intervals for CCs during lockdown were comparable to that in 2019 (TDI 4.6, PI 1.1 and SI 2.1 weeks), except for an increased PI in January-March 2020 (median 2.7 weeks). Diagnostic intervals for T1DM during lockdown were similar to that in 2019 (TDI 16 vs 15 and PI 14 vs 14 days), except for an increased PI in January-March 2020 (median 21 days). Conclusions: There is no evidence of diagnostic delay or increased illness severity for CC or T1DM, during the first phase of the pandemic across the participating centres. This provides reassuring data for children and families with these life-changing conditions.

"The balloon was just the kick start, I had to do the rest myself": Adolescents living with severe obesity experiences of an intra-gastric balloon alongside a lifestyle support programme.

BACKGROUND: Few treatments exist for adolescents living with severe obesity. This qualitative study explored the experiences of severely obese adolescents and their families who participated in the BOB study. METHODS: Twelve adolescents (5 males;7 females; mean age 15 years; BMI > 3.5 s.d; puberty stage 4 +) who were engaged with the research study BOB (a non-randomised, pilot novel obesity treatment programme that involved the insertion of an intra-gastric balloon coupled with a family lifestyle behavioural support programme). Adolescents attended weekly lifestyle sessions before, during and post balloon insertion. All participants were interviewed at 3 months, (halfway through intra-gastric balloon insertion) and at 12 months follow-up (6 months post intra-gastric balloon removal, 3 months post lifestyle intervention). RESULTS: All BOB participants had exhausted all treatment options deeming this study their final option. Many alluded to feelings of desperation and referred to a sense of hope that this intervention would be effective. Family involvement and attendance within the structured sessions differed significantly. Adolescents and parents perceived support from the research study ceased when the intra-gastric balloon was removed at 6-months despite attendance post balloon removal being poor. All participants emphasised a need for further support longer term with the integration of the family a critical factor. CONCLUSIONS: Further research is needed to explore the specific role families play within treatment to optimise health and wellbeing outcomes. Adolescents perspectives should be integrated within treatment to inform and improve the effectiveness of future treatment programmes for severely obese adolescents and their families.

Fifteen-minute consultation: An approach to the child receiving glucocorticoids.

Glucocorticoids (GC) are used in paediatric practice for a broad range of conditions and all paediatricians will prescribe GC, in some form, during their career. A wide variety of GC formulations, doses and administration routes are used for periods of time ranging from days to years. Exposure to exogenous GC can result in hypothalamic-pituitary-adrenal axis suppression-otherwise known as adrenal suppression (AS). Patients with AS may be well most of the time but if GC therapy is reduced or stopped or if additional endogenous GC cannot be generated during illness, then an absolute or relative lack of GC can result in severe illness or death. Here, we highlight the relevance of AS to all paediatricians by providing an overview of the background and discussing the presentation and approaches to the management of this clinical entity.

Monogenic diabetes mellitus in cystic fibrosis.

We present a non-consanguineous family of three siblings who presented with diabetes mellitus (DM), two of whom had genetically confirmed cystic fibrosis (CF), with one pancreatic-sufficient mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene (ΔF508/R117H;IVS8-5T). A detailed history revealed family members from three successive generations diagnosed with 'type 1' or 'type 2' diabetes, leading to genetic investigations for monogenic DM. A heterozygous frameshift mutation in the hepatocyte nuclear factor 1 homeobox alpha (HNF1A) gene (c.404delA) was subsequently confirmed in all three siblings, which is known to cause monogenic diabetes and is exquisitely sensitive to sulfonylurea therapy. Following this diagnosis, both siblings with CF and HNF1A monogenic diabetes were started on gliclazide therapy, while their older brother who had been wrongly diagnosed with type 1 diabetes was switched from insulin to gliclazide, all with excellent therapeutic responses.

International survey on high- and low-dose synacthen test and assessment of accuracy in preparing low-dose synacthen.

OBJECTIVE: The short synacthen test (SST) is widely used to assess patients for adrenal insufficiency, but the frequency and protocols used across different centres for the low-dose test (LDT) are unknown. This study aimed to survey centres and test the accuracy of ten different synacthen preparation strategies used for the LDT. METHODS: Members of 6 international endocrine societies were surveyed regarding diagnostic tests used for adrenal insufficiency, and in particular the SST. Synacthen was diluted for the LDT and concentrations measured using a synacthen ELISA. RESULTS: Survey responses were received from 766 individuals across 60 countries (52% adult, 45% paediatric endocrinologists). The SST is used by 98% of centres: 92% using high-dose (250 µg), 43% low-dose and 37% both. Ten low-dose dilution methods were assessed and variation in synacthen concentration was demonstrated with intramethod coefficients of variation (CV) ranging from 2.1% to 109%. The method using 5% dextrose as a diluent was the least variable (CV of 2.1%). The variation in dilution methods means that the dose of synacthen administered in a LDT may vary between 0.16 and 0.81 µg. CONCLUSIONS: The high-dose SST is the most popular diagnostic test of adrenal insufficiency, but up to 72% of paediatric endocrinologists use a LDT. There is considerable variation observed both within and between low-dose synacthen dilution methods creating considerable risk of inaccurate dosing and thereby invalid results.

Bariatric surgery in severely obese adolescents: a single-centre experience.

BACKGROUND: Increasing numbers of severely obese young people undergo bariatric surgery in the USA with reports of substantial weight loss after 1 year. National Institute for Clinical Excellence 2006 suggests considering surgery for young people in 'exceptional circumstances'. We present six patients operated upon 2004-2012 at our centre in the UK. CASE SERIES: Six patients (4 male) aged 14-16 years (mean age 15.10) underwent surgery. Mean preoperative body mass index (BMI) was 62.7 kg/m(2) and BMI SDS +4.4. Comorbidities included hypertension, insulin resistance, obstructive sleep apnoea, limited mobility, benign intracranial hypertension and psychosocial issues. All six patients had prior involvement with local lifestyle weight management services and had pharmacological intervention. Four laparoscopic gastric bypass procedures, one laparoscopic gastric banding (patient had a gastric balloon prior to band) and one laparoscopic sleeve gastrectomy were performed. RESULTS: There were no major postoperative procedural complications (one patient had a port rotation). Mean percentage of weight loss, as a percentage of total body weight at 6 and 12 months, was 22 and 27%, respectively. Average absolute weight loss at current follow-up is 54 kg. Mean BMI at 12 months postprocedure was 46.5 kg/m(2)-a mean fall of 16.2 kg/m(2). Mean BMI SDS fell from +4.4 to +3.8 at 12 months and +3.1 at 2 years. Resolution of hypertension, improved school attendance and no progression to T2DM were the benefits noted. CONCLUSIONS: Recent systematic reviews and meta-analyses suggest that bariatric surgery results in sustained and clinically significant weight loss in paediatric populations. The surgical option should continue to be exercised with extreme caution only in severely obese adolescents and done so in appropriate case results in positive outcomes.

The short Synacthen test: a questionnaire survey of current usage.

BACKGROUND: Supported by meta-analyses, the low-dose Synacthen test (LDST) has gained in popularity, with many believing it to be more sensitive than the supraphysiological standard (250 µg) short ST (SSST), particularly when assessing children prescribed high-dose inhaled corticosteroids (HDICS). However, consensus is lacking about its specific clinical application, what is considered 'low dose' and how that dose is made up. METHODS: To ascertain current use of the short Synacthen test (SST), a questionnaire was emailed to members of the British Society of Paediatric Endocrinology and Diabetes in the UK and Ireland (N=257), requesting a response from each department (N=92). A reminder was sent a month later to members of departments which had not responded. RESULTS: The authors received 39 replies, giving a response rate of 42%. All departments use the SST: 82% use an LDST, 87% use the SSST and 69% use both. The 1 µg dose was used by 44% of hospitals, with the other 56% using seven different doses based on age, weight and body surface area. There were 14 different methods of preparing the low dose test. Additionally, variations in the timings of cortisol sampling and the diagnostic cut-offs for adrenal insufficiency were found. Increased requests for SSTs in children with asthma prescribed HDICS were noted by 44% of respondents, with 67% reporting the detection of adrenal suppression in this group. CONCLUSION: Standardisation of the SST is required to address the considerable variation in the methodology and application of this test in the UK and Ireland.