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Licensed Unlicensed Requires Authentication Published by De Gruyter October 9, 2020

Characterization and outcome of 11 children with non-diabetic ketoacidosis

Elsayed Abdelkreem ORCID logo, Rofaida M. Magdy and Abdelrahim A. Sadek



To study the clinical and laboratory features, management, and outcome of pediatric non-diabetic ketoacidosis (NDKA).


Between May 2018 and April 2020, we prospectively collected children under 18 years who presented with ketoacidosis, defined as ketosis (urinary ketones ≥++ and/or serum β-hydroxybutyrate level ≥3 mmol/L) and metabolic acidosis (pH <7.3 and HCO3 <15 mmol/L). Children with HbA1c level ≥6.5% at initial presentation and those meeting the diagnostic criteria for DM during follow-up were excluded. Data were collected on demographics, clinical and laboratory features, management, and outcome.


Eleven children with 19 episodes of NDKA were identified. The median age was 12 months (range from 5 months to 5 years). They manifested dehydration and disturbed conscious level (all cases), convulsions (n=6), hypoglycemia (n=6), hyperglycemia (n=2) and significant hyperammonemia (n=4). Most cases required intensive care management. Death or neurodevelopmental impairment occurred in six cases. Seven cases had inborn errors of metabolism (IEMs). Other cases were attributed to starvation, sepsis, and salicylate intoxication.


This is the largest case series of pediatric NDKA. Ketoacidosis, even with hyperglycemia, is not always secondary to diabetes mellitus. IEMs may constitute a significant portion of pediatric NDKA. Increased awareness of this unfamiliar condition is important for prompt diagnosis, timely management, and better outcome.

Corresponding author: Elsayed Abdelkreem, MD, PhD, Department of Pediatrics, Faculty of Medicine, Sohag University, Sohag 82524, Egypt, E-mail:


The authors would like to appreciate the invaluable inputs from Prof. Toshiyuki Fukao (Gifu University, Japan) during study design and implementation. Prof. Fukao died at the beginning of 2020. He had a long-term and considerable contribution to scientific research, particularly in the field of ketone body metabolism and its defects.

  1. Research funding: None declared.

  2. Author contributions: Elsayed Abdelkreem conceived and designed the study, shared in data collection, and wrote the first draft of the manuscript. Rofaida M. Magdy was involved in clinical management and follow up of patients, shared in data collection, and revised manuscript. Abdelrahim A. Sadek supervised the whole study and critically revised the manuscript. All authors have accepted responsibility for the entire content of this submitted manuscript and approved submission.

  3. Competing interests: The funding organization(s) played no role in the study design; in the collection, analysis, and interpretation of data; in the writing of the report; or in the decision to submit the report for publication.

  4. Informed consent: Informed consent was obtained from all individuals included in this study.

  5. Ethical approval: The local Institutional Review Board approved this study.


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Received: 2020-06-01
Accepted: 2020-09-03
Published Online: 2020-10-09
Published in Print: 2021-01-27

© 2020 Walter de Gruyter GmbH, Berlin/Boston