A Newborn with Congenital Hyperinsulinism

Hyperinsulinaemic hypoglycaemia (HH) is a biochemical finding of low blood glucose levels due to the dysregulation of insulin secretion from pancreatic β-cells. Under normal physiological conditions, glucose metabolism is coupled to β-cell insulin secretion so that blood glucose levels are maintained within the physiological range of 3.5–5.5 mmol/L. However, in HH this coupling of glucose metabolism to insulin secretion is perturbed so that insulin secretion becomes unregulated. HH typically occurs in the neonatal, infancy and childhood periods and can be due to many different causes. Adults can also present with HH but the causes in adults tend to be different. Somatostatin (SST) is a peptide hormone that is released by the delta cells (δ-cells) in the pancreas. It binds to G protein-coupled SST receptors to regulate a variety of location-specific and selective functions such as hormone inhibition, neurotransmission and cell proliferation. SST plays a potent role in the regulation of both insulin and glucagon secretion in response to changes in glucose levels by negative feedback mechanism. The half-life of SST is only 1–3 min due to quick degradation by peptidases in plasma and tissues. Thus, a direct continuous intravenous or subcutaneous infusion is required to achieve the therapeutic effect. These limitations prompted the discovery of SST analogues such as octreotide and lanreotide, which have longer half-lives and therefore can be administered as injections. SST analogues are used to treat different forms of HH in children and adults and therapeutic effect is achieved by suppressing insulin secretion from pancreatic β-cells by complex mechanisms. These treatments are associated with several side effects, especially in the newborn period, with necrotizing enterocolitis being the most serious side effect and hence SS analogues should be used with extreme caution in this age group.

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“… 73 Many studies document the use of SST analogues for treating childhood HH, dating back to 1977. 14 , 27 , 67 …”

Section: The Role Of Sst Analogues In Treatment Of Hhmentioning

confidence: 99%

Somatostatin analogues for the treatment of hyperinsulinaemic hypoglycaemia

Haris

Saraswathi

Hussain

2020

Therapeutic Advances in Endocrinology

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“…Мутации указанных генов могут наследоваться как по аутосомно-рецессивному, так и аутосомно-доминантному типу. Однако в 30-40% случаев не удается установить молекулярно-генетическую основу врожденного гиперинсулинизма [17,18]. Кроме того, выделены синдромальные состояния, в структуру которых входит врожденный гиперинсулинизм.…”

Section: врожденный гиперинсулинизмunclassified

“…Существует несколько препаратов, оказывающих инсулиностатическое действие, среди которых наиболее эффективны диазоксид (селективный агонист АТФ-зависимых калиевых каналов β-клеток) и октреотид (аналог соматостатина пролонгированного действия) [16][17][18][19]. В настоящее время в клинической практике начинать лечение предпочтительнее с диазоксида, эффективность и безопасность использования которого доказана во многих исследованиях.…”

Section: врожденный гиперинсулинизмunclassified

Neonatal carbohydrate metabolism disorders: a personalized approach to diagnostics and treatment

Алимова¹

2020

Ross. vestn. perinatol. pediatr.

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The article discusses the current state of the problem of providing personalized medical care to newborns with impaired carbohydrate metabolism: neonatal diabetes and congenital hyperinsulinism. Approaches to the diagnosis, prognosis of the disease, the choice of treatment and the evaluation of its effectiveness, based on the results of molecular and genetic research, are considered. It is shown that the introduction into clinical practice of the principles of the personalized medicine contributed to improving the quality of medical care for newborns with endocrine diseases and substantiated the directions for the further development of neonatal endocrinology.

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A Newborn with Congenital Hyperinsulinism

Cited by 2 publications

References 11 publications

Somatostatin analogues for the treatment of hyperinsulinaemic hypoglycaemia

Somatostatin analogues for the treatment of hyperinsulinaemic hypoglycaemia

Neonatal carbohydrate metabolism disorders: a personalized approach to diagnostics and treatment

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