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Review
. 2023 Oct 30:14:1231043.
doi: 10.3389/fendo.2023.1231043. eCollection 2023.

Standardised practices in the networked management of congenital hyperinsulinism: a UK national collaborative consensus

M Guftar Shaikh  1 , Angela K Lucas-Herald  1 , Antonia Dastamani  2 , Maria Salomon Estebanez  3 , Senthil Senniappan  4 , Noina Abid  5 , Sumera Ahmad  3 , Sophie Alexander  2 , Bindu Avatapalle  6 , Neelam Awan  3 , Hester Blair  7 , Roisin Boyle  1 , Alexander Chesover  2 , Barbara Cochrane  1 , Ross Craigie  8 , Annaruby Cunjamalay  2 , Sarah Dearman  9 , Paolo De Coppi  10   11 , Karen Erlandson-Parry  4 , Sarah E Flanagan  12 , Clare Gilbert  2 , Niamh Gilligan  3 , Caroline Hall  3 , Jayne Houghton  13 , Ritika Kapoor  14 , Helen McDevitt  1 , Zainab Mohamed  15 , Kate Morgan  2 , Jacqueline Nicholson  16 , Ana Nikiforovski  3 , Elaine O'Shea  3 , Pratik Shah  17 , Kirsty Wilson  1 , Chris Worth  3 , Sarah Worthington  3 , Indraneel Banerjee  3
Affiliations
Review

Standardised practices in the networked management of congenital hyperinsulinism: a UK national collaborative consensus

M Guftar Shaikh et al. Front Endocrinol (Lausanne). .

Abstract

Congenital hyperinsulinism (CHI) is a condition characterised by severe and recurrent hypoglycaemia in infants and young children caused by inappropriate insulin over-secretion. CHI is of heterogeneous aetiology with a significant genetic component and is often unresponsive to standard medical therapy options. The treatment of CHI can be multifaceted and complex, requiring multidisciplinary input. It is important to manage hypoglycaemia in CHI promptly as the risk of long-term neurodisability arising from neuroglycopaenia is high. The UK CHI consensus on the practice and management of CHI was developed to optimise and harmonise clinical management of patients in centres specialising in CHI as well as in non-specialist centres engaged in collaborative, networked models of care. Using current best practice and a consensus approach, it provides guidance and practical advice in the domains of diagnosis, clinical assessment and treatment to mitigate hypoglycaemia risk and improve long term outcomes for health and well-being.

Keywords: congenital hyperinsulinism; consensus; glucose; hypoglycaemia; patient organisation; standardised practice; treatment.

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Conflict of interest statement

IB has received honoraria for advisory opinion from Merck and Diurnal Pharmaceuticals. He is the UK CI for clinical trials funded by Zealand Pharmaceuticals and has received grant funding from Merck, Diurnal and Crinetics Pharmaceuticals. IB is the Chair of the BSPED-NIHR Clinical Studies Group and the Chair of the ESPE Communications Committee. He is a co-opted member of the NICE Head Injury Update Committee. IB has advisory roles with several patient organisations including the Pituitary Foundation, Living with CAH, CHC and CHI. MGS has received funding/speaker honorium from Novo Nordisk, Sandoz, Pfizer and received honorarium for consultancies/advisory boards from Novo Nordisk, Pfizer and Merck. RK has received grant funding and honoraria for advisory opinion from Merck. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Referral pathways and criteria for congenital hyperinsulinism diagnosis and treatment in a networked model of care. CHI, congenital hyperinsulinism; DGH, district general hospital.
Figure 2
Figure 2
Clinical pathway of diagnosis and management for patients with congenital hyperinsulinism (CHI). Clinical decisions hinge on criteria such as diazoxide responsiveness, genetic investigations, 18 Fluoro Dopa PET-CT/MR imaging and response to medical therapy.
Figure 3
Figure 3
Flowchart for blood glucose monitoring for families. BG, blood glucose.
Figure 4
Figure 4
Quotes from parents describing their feelings in living with children with CHI.
See this image and copyright information in PMC

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