ESPE2023 Poster Category 1 Diabetes and Insulin (55 abstracts)
Management of severe diabetic ketoacidosis with extremely high doses of insulin in a girl with severe insulin resistance syndrome due to compound heterozygous mutations in the insulin receptor gene
Clemens Kamrath , Nora Genthner , Hande Rakicioglu & Stefan Wudy
Justus-Liebig-University, Giessen, Germany
We report a girl born small for gestational age with a birth weight of 1970g. At the age of 2 years she developed marked dystrophy, height was -4.39 SDS and BMI was -3.1 SDS. Later she developed severe insulin restistance and hyperglycaemia due to compound heterozygous mutations in the insulin receptor gene: exon 16: c.2986A>G (paternal) and intron 9: c.2029+1G>T (maternal). Clinical findings included severe acanthosis nigricans, mild hypertrophic cardiomyopathy, abnormal enamel formation, polycystic ovarian hyperplasia and hyperandrogenemia. She was treated with rhIGF-1, resulting in catch-up growth. At the age of 11, the patient developed severe diabetic ketoacidosis following an influenza A infection. Only with an extremely high insulin dose of up to 2,000 U/kg BW/h = 50,000 U/d and an extremely high sodium bicarbonate dose of >1 mmol/kg/h did a slow improvement occur. In the course of time, the insulin intake could be reduced to about 3000 U/day at discharge. Discharge was by insulin pump therapy with U500 insulin.
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