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)
Open accessAbstract
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.
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It is estimated that about 1 in 10,000,000 people are diagnosed with idiopathic/sporadic adult-onset nesidioblastosis annually.
“It is estimated that about 1 in 10,000,000 people are diagnosed with this disease annually [45-48].”
From An Uncommon Cause of Recurrent Presyncope, Dizziness, and Tachycardia: A Case Report of Diffuse, Adult-Onset Nesidioblastosis/Non-Insulinoma Pancreatogenous Hypoglycemia Syndrome (NIPHS) (2023) · cites this paper as [45]
The Results (Non-insulinoma pancreatogenous hypoglycemia syndrome; page unavailable) report 10 patients with adult-onset nesidioblastosis, and the Discussion describes NIPHS as rare. Neither passage estimates one diagnosis per 10,000,000 people annually; the reported prevalence of 0.09 per 100,000 is for the broader NIPHS category, not annual diagnoses of nesidioblastosis. (LLM, conf=0.84)
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- Authors
- Yamada, Y., Kitayama, K., Oyachi, M., Higuchi, S., Kawakita, R., Kanamori, Y., & Yorifuji, T.
- Affiliations
- Osaka City General Hospital (JP)
- National Center For Child Health and Development (JP)
- Corresponding author
- Yorifuji, Tohru
- Venue
- Journal of Diabetes Investigation
- Publisher
- Wiley
- Published
- 2019-12-24
- Volume
- 11
- Issue
- 3
- Pages
- 554-563
- ISSN
- 2040-1116 (ISSN-L)
- 2040-1124
- PMC
- PMC7232294
- OpenAlex
- W2990929279
- Cited by
- 63
- References
- 33
- Citations by year
- 2026: 4 · 2025: 4 · 2024: 16 · 2023: 12 · 2022: 10 · 2021: 12 · 2020: 4 · 2019: 1
- FWCI
- 6.45
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- Locations
- Journal of Diabetes Investigation (publishedVersion, cc-by-nc)
- PubMed Central (publishedVersion, cc-by-nc)
- PubMed (publishedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Funders
- Ministry of Health, Labour and Welfare (19FC1008)
- Topics
- Health Sciences › Medicine › Surgery › Pancreatic function and diabetes
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Diabetes Management and Research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Diabetes and associated disorders
- Keywords
- endogenous hyperinsulinemic hypoglycemia, congenital hyperinsulinism, insulinoma, insulin autoimmune syndrome, nesidioblastosis, postprandial hypoglycemia, quality of life, diabetes mellitus, incidence, Japan
- MeSH terms
- Adolescent, Adult, Aged, Aged, 80 and over, Child, Child, Preschool, Female, Humans, Hyperinsulinism, Hypoglycemia, Infant, Infant, Newborn, Insulinoma, Japan, Male, Middle Aged, Surveys and Questionnaires, Congenital Hyperinsulinism, Nesidioblastosis, Young Adult
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