Challenges in management of transient hyperinsulinism – a retrospective analysis of 36 severely affected children
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Henrike Hoermann
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Marcia Roeper
Abstract
Objectives
Transient hyperinsulinism (THI) is a hypoglycemia disorder which resolves spontaneously within the first weeks or months of life. The pathomechanism of THI is not elucidated yet; however, it is known that perinatal stress predisposes for THI. We aimed to characterize the clinical phenotype and treatment of children with THI, and to identify options for improved management.
Methods
A retrospective analysis of 36 children with THI treated at the University Children’s Hospital Düsseldorf between 2007 and 2019 was performed.
Results
All children had risk factors for neonatal hypoglycemia or indicators of perinatal stress. Eighty three percent were diagnosed with hypoglycemia on day of life (DOL)1. None of the six diagnosed later had routine blood glucose screening and showed significantly lower blood glucose levels at the time of first blood glucose measurement compared to the children diagnosed on DOL1. Ninety seven percent of all children received intravenous glucose, 42% received continuous glucagon and 81% were started on diazoxide. Diazoxide withdrawal and subsequent fasting tests lacked standardization and were based on clinical experience. Three patients had a subsequent episode of hypoglycemia, after fasting studies only demonstrated “clinical” remission without proving the ability to ketogenesis.
Conclusions
Any kind of perinatal stress might pose a risk to develop THI, and postnatal monitoring for hypoglycemia still needs to be improved. Diazoxide is effective in children with THI; however, further studies are needed to guide the development of criteria and procedures for the initiation and discontinuation of treatment. Furthermore, establishing consensus diagnostic criteria/definitions for THI would improve comparability between studies.
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Research funding: None declared.
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Author contributions: All the authors have accepted responsibility for the entire content of this submitted manuscript and approved submission.
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Competing interests: None declared.
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Informed consent: Not applicable.
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Ethical approval: The research related to human use has complied with all the relevant national regulations, institutional policies, and in accordance with the tenets of the Helsiniki Declaration, and has been approved by the authors’ Institutional Review Board (Ethikkommission an der Medizinischen Fakultät der HHU Düsseldorf, 2019-384-ProspDEuA).
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Supplementary Material
The online version of this article offers supplementary material (https://doi.org/10.1515/jpem-2020-0639).
© 2021 Walter de Gruyter GmbH, Berlin/Boston
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Articles in the same Issue
- Frontmatter
- Review Article
- Delivery factors and neonatal thyroid hormone levels: a systematic review
- Original Articles
- Birth prevalence of tetrahydrobiopterin deficiency in China: data from the national newborn screening program, 2013–2019
- Analysis of the CAG tract length in the Androgen Receptor gene in Mexican patients with nonsyndromic cryptorchidism
- Pituitary hypoplasia is the best MRI predictor of the severity and type of growth hormone deficiency in children with congenital growth hormone deficiency
- Urinary gonadotropin measurements by enhanced luminometric assays (LIA) for the evaluation of pubertal development
- Challenges in management of transient hyperinsulinism – a retrospective analysis of 36 severely affected children
- Who should return for an oral glucose tolerance test? A proposed clinical pathway based on retrospective analysis of 332 children
- Investigation of the prevalence of cardiovascular risk factors in obese patients diagnosed with metabolic syndrome in childhood and examination of left ventricular function by echocardiography
- Effect of l-carnitine supplementation on children and adolescents with nonalcoholic fatty liver disease (NAFLD): a randomized, triple-blind, placebo-controlled clinical trial
- Management of patients with X-linked hypophosphatemic rickets during Covid-19 pandemic lockdown
- Evaluation of the pathophysiological role of Fetuin A levels in adolescents with polycystic ovary syndrome
- Gonadotropins for testicular descent in cryptorchid congenital hypogonadotropic hypogonadism males beyond infancy
- Clinical characteristics and outcome of hospitalized children and adolescent patients with type 1 diabetes during the COVID-19 pandemic: data from a single center surveillance study in Egypt
- Letter to the Editor
- Revisiting the effect of GnRH analogue treatment on bone mineral density in young adolescents with gender dysphoria
- Case Reports
- Pulmonary and cutaneous mucormycosis in two children with diabetes mellitus type 1
- Pyruvate carboxylase deficiency type C as a differential diagnosis of diabetic ketoacidosis
- Barth syndrome with severe dilated cardiomyopathy and growth hormone resistance: a case report
- The utility of reverse phenotyping: a case of lysinuric protein intolerance presented with childhood osteoporosis
