ESPE Abstracts (2021) 94 P1-151

ESPE2021 ePoster Category 1 Fetal Endocrinology and Multisystem Disorders B (10 abstracts)

Duration of Fasting Studies Required to Demonstrate a Cure for Patients with Focal Congenital Hyperinsulinism.

Lisa Truong , Courtney Reynolds & Paul S Thornton

Cook Children’s Medical Center, Fort Worth, USA

Introduction: Focal disease of the pancreas causing hyperinsulinism (FHI) has 2 essential characteristics 1) the inheritance of a paternally derived mutation in ABCC8 or KCNJ11 in conjunction with loss of a region of the maternal chromosome 11p13.1 and 2) the ability to be cured by resection of the focal lesion while sparing the majority of the pancreas. At the time of surgery the surgeon resects the focal lesion until clear margins are available. We perform a fasting study postoperatively to determine if the patient is cured or not. In our multidisciplinary team approach we terminate our fasting studies when either a plasma glucose is < 2.7 mmol/l, beta-hydroxybutyrate (BOHB) is >1.7 mmol/l or a max fasting duration appropriate for the patient’s age has been reached. There is current debate as to how long one should fast an infant to demonstrate cure of FHI. The aim of this study is to demonstrate the length of time to either hypoglycemia <2.7 mmol/l or BOHB to >1.7 mmol/l in order to prove cure in patients with FHI in whom surgery has been deemed successful at the time of frozen section.

Methods: We conducted a retrospective analysis on 36 patients with focal disease. Patients were divided into 3 groups. Group 1: those not cured, Group 2 those cured and fasted until glucose < 2.7 mmol/l or BOHB >1.7 mmol/l and group 3 those cured and fasted based on time.

Results: Group 1 n = 7, Group 2 n = 22 and Group 3 n = 7. The mean duration of fasting in: group 1 was 11.4 h with 5 fasting more than 9 hours and 4 fasting >12 h, Group 2 was 16.6 h (range 13 to 22h) with 8/22 patients fasting >18 hrs and Group 3 was 20.6 h (range 15-30 h) and 7/7 were ≥18 hours. Of the patients determined cured (group 2 and 3) the 9 hour glucose was above 3.88 mmol/l in 26/29 patients whereas it was only >3.88 mmol/l in 1 who was not cured. Extending fasting studies to 18-24 hours will add 0.37 to 0.62 extra days of hospitalization. In our case an average length of stay for cured focal patients is 22.1 days.

Conclusion: We recommend that fasting studies to demonstrate cure in patients with FHI be performed until either the glucose is <2.7 mmol/l, the BOHB >1.7 mmol/l or 18 hours for those <6m or 24 hours for those >6m, whichever comes first.

Volume 94

59th Annual ESPE (ESPE 2021 Online)

22 Sep 2021 - 26 Sep 2021

European Society for Paediatric Endocrinology 

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