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Table 3 Drugs for medical therapy of hyperinsulinaemic hypoglycaemia [1, 110, 113]

From: Diagnosis and treatment of hyperinsulinaemic hypoglycaemia and its implications for paediatric endocrinology

 

Route

Dose

Mode of action

Side effects

Conventional medicines

 Diazoxide

Oral

5–20 mg/kg/day, in 3 divided doses

Bind to SUR1 subunit of KATPchannels, opens the channels and inhibits insulin secretion

Requires an intact KATP channel activity to work properly

Common: Water and salt retention, hypertrichosis, loss of appetite

Rare: Cardiac failure, hyperuricaemia, blood dyscrasias (bone marrow suppression, anaemia, eosinophilia etc.), paradoxical hypoglycaemia

 Chlorothiazide

Oral

7–10 mg/kg/day, in 2 divided doses

Prevents fluid retention, synergistic effects with diazoxide on KATP channels to inhibit insulin secretion

Hyponatraemia, hypokalaemia

 Nifedipine

Oral

0.25–2.5 mg/kg/day, in 2–3 divided doses

Inhibits Ca-channels of the β-cell membrane

Hypotension

 Octreotide

s.c

5–35 μg/kg/day, divided to 3–4 doses or continuous subcutaneous infusion

Activation of SSTR-2 and SSTR-5 inhibits calcium mobilization and acetylcholine activity, and decreases insulin gene promoter activity, reduces insulin biosynthesis and insulin secretion.

Acute: Anorexia, nausea, abdominal discomfort, diarrhoea, drug induced hepatitis, elevated liver enzyme, long QT syndrome, tachyphylaxis, necrotizing enterocolitis

Long-term: Decreases intestinal motility, bile sludge and gallstone, suppression of pituitary hormones (Growth hormone, TSH)

 Glucagon

s.c/i.m bolus or s.c/i.v infusion

0.02 mg/kg/dose or 5–10 μg/kg/h infusion

G-protein coupled activation of adenylate cyclase, increases cAMP, Induces glycogenolysis and gluconeogenesis

Nausea, vomiting, skin rash and rebound hypoglycaemia in high doses (>20 μg/kg/h) due to paradoxical activation of insulin secretion

New medicines

 Rapamycin (sirolimus, everolimus)

Oral

An initial dose of 1 mg/m2 per day may require dose adjustment according to blood sirolimus concentration usually to keep between 5 and 15 ng/ml

mTOR inhibitor. Inhibits insulin release and β-cell proliferation through different mechanism which have not been clarified yet

Immune suppression, mucositis, hyperlipidemia, elevation of liver enzyme, thrombocytosis, impaired immune response to BCG vaccine

 Octreotide LAR/ Lanreotide

Deep s.c

Total 4 weekly dose of octreotide given every 4 weekly or 15–60 mg/every 4 weekly

These long acting somatostatin analogues have similar effects as daily multidose octreotide.

Similar to daily multiple injection octreotide. However, long-term follow up is not known yet