Guidelines
Guidelines Summary
Clinical practice guidelines regarding congenital hyperinsulinism (congenital hyperinsulinemic hypoglycemia), created through a collaboration between The Japanese Society for Pediatric Endocrinology and the Japanese Society of Pediatric Surgeons, were published in 2017. The recommendations, targeted at patients under age 18 years, include the following [20] :
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When the diagnosis of hyperinsulinemic hypoglycemia is made, acquired hyperinsulinemia should be excluded by the following - History of gastric bypass, fundoplication for gastroesophageal reflux, and the use of insulin or oral hypoglycemia agents for diabetes mellitus, as well as through blood insulin/C-peptide ratio, anti-insulin antibody, imaging studies of the pancreas (contrast-enhanced computed tomography [CT] scanning, contrast-enhanced magnetic resonance imaging [MRI], endoscopic ultrasonography)
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Treatment goals should be set at blood glucose over 70 mg/dL for congenital hyperinsulinism
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The threshold of blood glucose to circumvent neurologic sequelae cannot be set because it is patient specific
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Regarding first-line treatment, maintain blood glucose above the target range by continuous glucose infusion
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When blood glucose is not maintained by continuous glucose infusion or when it is difficult to withdraw glucose infusion for an extended period, a 5-day trial of oral diazoxide, in 2-3 divided doses, at 5-15 mg/kg/d should be attempted unless contraindicated by cardiac failure or pulmonary hypertension
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While on diazoxide, the patient should be on a glucose self-monitoring regimen to detect episodes of hypoglycemia; furthermore, a complete blood count (CBC), blood chemistry, and physical examination should be performed to detect frequent adverse events, such as hypertrichosis, tachycardia, or edema
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When euglycemia is not achieved by the first-line treatment and continuous glucose infusion cannot be withdrawn, second-line treatment should be initiated
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In patients unresponsive to diazoxide, subcutaneous octreotide, intravenous (IV) glucagon, and oral calcium antagonist should be sequentially attempted as second-line therapies
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Glucocorticoid is not recommended as a second-line therapy
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In patients unresponsive to diazoxide, etiologic diagnosis should be performed
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In patients unresponsive to diazoxide, mutational analysis of the K ATP channel genes ( ABCC8, KCNJ11) should be performed
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In patients unresponsive to diazoxide, a 18F-L-3,4-dihydroxyphenylalanine ( 18F-DOPA) positron emission tomography (PET) scan should be performed
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Partial pancreatectomy should be performed when a focal lesion in the body-tail of the pancreas is identified by 18F-DOPA-PET scan and blood glucose cannot be stabilized by nutritional support and diazoxide
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Partial pancreatectomy should be considered when a focal lesion in the head of the pancreas is identified by 18F-DOPA-PET scan and blood glucose cannot be stabilized by nutritional support and diazoxide
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Consider pancreatectomy for a diffuse lesion diagnosed by mutational analysis or by 18F-DOPA-PET scan that is resistant to medical treatment, including second-line treatments
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It is desirable to avoid pancreatectomy of greater than 95%
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When a focal lesion cannot be identified by visual inspection or palpation, intraoperative biopsies should be taken from multiple parts of the pancreas for rapid diagnosis by frozen section
Media Gallery
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Normal hypoglycemic counterregulation.
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Interpretation of the critical sample.
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