ESPE Abstracts

Background: The incidence of perinatal stress hyperinsulinism (PSHI) requiring diazoxide treatment is estimated to be 1:12,000 (Hoe et al., 2006). Diazoxide is used to treat PSHI, but it has been shown to have adverse effects including pulmonary hypertension. Gray et al, reported that of 1.25 million infants admitted to NICUs for hypoglycemia over a period of 18 years, only 1066 received diazoxide suggesting 1:1172 babies had HI however 92% of them were off diazoxide by the time of discharge and there was no diagnostic data given.

Objectives: 1) Determine the precise incidence rate of PSHI requiring diazoxide therapy in our service area.

Methods: We reviewed the charts of patients born in our service area who required diazoxide treatment of their PSHI (DrxPSHI). In our service area, all patients requiring diazoxide were referred to our institution during the study time frame of 2012 to 2019. We used public health records to determine the annual number of births and calculated the incidence. Outcome data was tracked in our IRB approved Congenital Hyperinsulinism Center REDCap registry.

Results: Forty-eight patients met our inclusion criteria (60% male, 67% Caucasian, 31% Hispanic). From 2012 to 2019, there were an average of 2.15 infants who had DrxPSHI per 10,000 live births (1/4652). The annual incidence of Infants with DrxPSHI ranged from a low of 1.41/10000 (1/7093) in 2015 to a high of 2.58/10000 (1/3880) in 2019. Of the 48 infants with DrxPSHI treated at our institution during this time, the average gestational age was 35.9 weeks and size was appropriate for gestational age for 48% (42% small for gestational age). Ninety (90) percent presented with hypoglycemia at birth (range 0 – 86 days) and were diagnosed with PSHI at 20 days on average (median: 10, range: 1-88, 384*).

Conclusions: We have shown the incidence of DrxPSHI is 1:4652. This contrasts to previous data showing 1/12000 (Hoe et al., 2006). Recent data suggesting the overall incidence of PSHI being around 1/1200-1700 suggests that 1:3-4 patients with PHSI need prolonged diazoxide therapy.