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. 2020 May;11(3):554-563.
doi: 10.1111/jdi.13180. Epub 2019 Dec 24.

Nationwide survey of endogenous hyperinsulinemic hypoglycemia in Japan (2017-2018): Congenital hyperinsulinism, insulinoma, non-insulinoma pancreatogenous hypoglycemia syndrome and insulin autoimmune syndrome (Hirata's disease)

Yuki Yamada  1 , Kana Kitayama  1 , Maki Oyachi  1 , Shinji Higuchi  1 , Rie Kawakita  1 , Yutaka Kanamori  2 , Tohru Yorifuji  1
Affiliations

Nationwide survey of endogenous hyperinsulinemic hypoglycemia in Japan (2017-2018): Congenital hyperinsulinism, insulinoma, non-insulinoma pancreatogenous hypoglycemia syndrome and insulin autoimmune syndrome (Hirata's disease)

Yuki Yamada et al. J Diabetes Investig. 2020 May.

Abstract

Aims/introduction: We aimed to investigate the nationwide incidence, treatment details and outcomes of patients with endogenous hyperinsulinemic hypoglycemia (EHH), including those with transient/persistent congenital hyperinsulinism (CHI), insulinoma, non-insulinoma pancreatogenous hypoglycemia syndrome and insulin autoimmune syndrome (Hirata's disease) in Japan.

Materials and methods: A nationwide, questionnaire-based survey was carried out to determine the number of patients with EHH who were treated for hypoglycemia or hypoglycemia-related complications in 2017-2018. The questionnaires were sent to all hospitals in Japan with >300 beds, and with pediatric and/or adult clinics likely managing EHH patients. The secondary questionnaires were sent to obtain the patients' date of birth, sex, age at onset, treatment details and post-treatment outcomes.

Results: A total of 447 patients with CHI (197 transient CHI, 225 persistent CHI and 25, unknown histology), 205 with insulinoma (118 benign, 18 malignant and 69 unknown subtype), 111 with non-insulinoma pancreatogenous hypoglycemia syndrome (33 post-gastric surgery HH, 57 postprandial HH, 10 nesidioblastosis and 11 unknown subtype) and 22 with insulin autoimmune syndrome were identified. Novel findings included: (i) marked improvement in the prognosis of persistent CHI over the past 10 years; (ii) male dominance in the incidence of transient CHI; (iii) non-insulinoma pancreatogenous hypoglycemia syndrome emerging as the second most common form of EHH in adults; (iv) frequent association of diabetes mellitus with insulin autoimmune syndrome; and (v) frequent post-treatment residual hypoglycemia and impaired quality of life.

Conclusions: The first nationwide, all age group survey of EHH showed the current status of each type of EHH disorder and the unmet needs of the patients.

Keywords: Hyperinsulinism; Hypoglycemia; Surveys.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Age at onset of patients with transient and persistent congenital hyperinsulinism. CHI, congenital hyperinsulinism.
Figure 2
Figure 2
Age at onset of patients with insulinoma.
Figure 3
Figure 3
Age at onset of non‐insulinoma pancreatogenous hypoglycemia syndrome. HH, hyperinsulinemic hypoglycemia.
Figure 4
Figure 4
Age at onset of patients with insulin autoimmune syndrome (Hirata’s disease).
See this image and copyright information in PMC

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