ReviewAnnals of pediatric endocrinology & metabolism2014
Congenital hyperinsulinism: current status and future perspectives.
Review in Annals of pediatric endocrinology & metabolism, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT03053284 (Pasireotide for Prevention of Hypoglycemia in Patients With Hyperinsulinemic Hypoglycemia), which is not on this map. Cited by 44 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Pasireotide for Prevention of Hypoglycemia in Patients With Hyperinsulinemic Hypoglycemia
Who cites it
44 citing papers in PubMed, 152 citations in OpenAlex.
- Congenital Hyperinsulinism With Paternally InheritedJournal of the ASEAN Federation of Endocrine Societies · 2026Article
- Somatostatin and Its Analogues as Second-Line Treatments in Non-Neoplastic Conditions.International journal of molecular sciences · 2026Review
- Dulaglutide as a Bridging Therapy Before Insulin for Diabetes Following Pancreatectomy on Congenital Hyperinsulinism.JCEM case reports · 2026Article
- Congenital hyperinsulinism due to NEUROD1 gene mutation.Annals of pediatric endocrinology & metabolism · 2025Article
- Glutamate dehydrogenase hyperinsulinism: mechanisms, diagnosis, and treatment.Orphanet journal of rare diseases · 2023Review
- Investigating Genetic Mutations in a Large Cohort of Iranian Patients with Congenital HyperinsulinismJournal of clinical research in pediatric endocrinology · 2022Article
- Molecular Characterization and Management of Congenital Hyperinsulinism: A Tertiary Centre Experience.Indian pediatrics · 2022Article
- Long-Term Outcome and Treatment in Persistent and Transient Congenital Hyperinsulinism: A Finnish Population-Based Study.The Journal of clinical endocrinology and metabolism · 2021Article
- Intraoperative ultrasound imaging in the surgical treatment of congenital hyperinsulinism: prospective, blinded study.BJS open · 2021Observational
- Congenital hyperinsulinism: 2 case reports with different rare variants in ABCC8.Annals of pediatric endocrinology & metabolism · 2021Article
- Health-Related Quality of Life of Children and Adolescents With Congenital Hyperinsulinism - A Scoping Review.Frontiers in endocrinology · 2021Article
- Diagnosis of congenital Hyperinsulinism can occur not only in infancy but also in later age: a new flow chart from a single center experience.Italian journal of pediatrics · 2020Article
- Suspected congenital hyperinsulinism in a Shiba Inu dog.Journal of veterinary internal medicine · 2020Article
- 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).Journal of diabetes investigation · 2020Article
- Current and Emerging Agents for the Treatment of Hypoglycemia in Patients with Congenital Hyperinsulinism.Paediatric drugs · 2019Review
- Congenital hyperinsulinism in two siblings with ABCC8 mutation: same genotype, different phenotypes.Archives of endocrinology and metabolism · 2018Article
- The burden of congenital hyperinsulinism in the United Kingdom: a cost of illness study.Orphanet journal of rare diseases · 2018Article
- Management of hypoglycemia in newborn: Turkish Neonatal and Pediatric Endocrinology and Diabetes Societies consensus report.Turk pediatri arsivi · 2018Article
- Article
- Clinical Diversity in Focal Congenital Hyperinsulinism in Infancy Correlates With Histological Heterogeneity of Islet Cell Lesions.Frontiers in endocrinology · 2018Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
1 author at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The diagnosis and treatment of congenital hyperinsulinism (CHI) have made a remarkable progress over the past 20 years and, currently, it is relatively rare to see patients who are left with severe psychomotor delay. The improvement was made possible by the recent developments in the understanding of the molecular and pathological basis of CHI. Known etiologies include inactivating mutations of the KATP channel genes (ABCC8 and KCNJ11) and HNF4A, HNF1A, HADH, and UCP2 or activating mutations of GLUD1, GCK, and SLC16A1. The understanding of the focal form of KATP channel CHI and its detection by (18)F-fluoro-L-DOPA positron emission tomography have revolutionized the management of CHI, and many patients can be cured without postoperative diabetes mellitus. The incidence of the focal form appears to be higher in Asian countries; therefore, the establishment of treatment systems is even more important in this population. In addition to diazoxide or long-term subcutaneous infusion of octreotide or glucagon, long-acting octreotide or lanreotide have also been used successfully until spontaneous remission. Because of these medications, near-total pancreatectomy is less often performed even for the diazoxide-unresponsive diffuse form of CHI. Other promising medications include pasireotide, small-molecule correctors such as sulfonylurea or carbamazepine, GLP1 receptor antagonists, or mammalian target of rapamycin inhibitors. Unsolved questions in this field include the identification of the remaining genes responsible for CHI, the mechanisms leading to transient CHI, and the mechanisms responsible for the spontaneous remission of CHI. This article reviews recent developments and hypothesis regarding these questions.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.