ESPE Abstracts (2021) 94 P2-107

ESPE2021 ePoster Category 2 Diabetes and insulin (72 abstracts)

A paradoxical conundrum: diabetic ketoacidosis resulting in treatment-resistant hypokalemic alkalosis

Hande Rakicioglu , Clemens Kamrath , Platonas Karatsiolis , Nora Genthner & Stefan Wudy


Justus Liebig University, Centre of Child and Adolescent Medicine, Divison of Pediatric Endocrinology and Diabetology, Giessen, Germany


From time to time pediatric endocrinologists may be confronted with paradoxical constellations. Here, we report on a 13-year-old boy with type-1 diabetes. He had polyuria and polydipsia for the previous 2 weeks. Three days before presentation, he got increasingly exhausted and developed recurrent vomiting. At presentation his weight was 41,4 kg (P 25-50) and his height was 154 cm (P 25-50). Initial labs were HbA1c 10.2%, glucose 530 mg/dl, pH 7.15, base excess - 21.1 mmol/l, bicarbonate 9.8 mmol/l, Na 131 mmol/l, K 2.4 mmol/l. Type-1 diabetes was diagnosed and the boy treated with i.v. insulin, fluid and electrolyte substitution. However, despite a high potassium substitution of up to 6 mmol/kg body weight/24h, therapy-resistant hypokalemia with hypokalemic metabolic alkalosis persisted after regression of ketoacidosis: pH 7.48, base excess 10.8 mmol/l, bicarbonate 33.7 mmol/l, Na 136 mmol/l, K 2.7 mmol/l. Further lab diagnostics showed a plasma renin of >300ng/l and a serum aldosterone of 141.1 ng/dl. The constellation of therapy-resistant hypokalemic alkalosis and secondary hyperaldosteronism with normal blood pressures made us think of the additional presence of Bartter’s syndrome. Bartter’s syndrome is a clinically and genetically heterogeneous renal salt loss disorder with hypokalemic metabolic alkalosis and secondary hyperaldosteronism. Bartter`s syndrome type 3 is caused by pathogenic changes in the CLCNKB gene and is inherited as an autosomal recessive trait. Analysis by next generation sequencing showed a complete deletion of the CLCNKB gene in the homozygous state, which was confirmed by multiplex ligation-dependent probe amplification analysis. To the best of our knowledge, we describe the first case of persisting hypokalaemia and alkalosis after regression of diabetic ketoacidosis due to a previously undiagnosed Bartter`s syndrome. During the course of long-term therapy with spironolactone and potassium substitution, the potassium levels normalized.

Volume 94

59th Annual ESPE (ESPE 2021 Online)

Online,
22 Sep 2021 - 26 Sep 2021

European Society for Paediatric Endocrinology 

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