Home Diabetic ketoacidosis, hyperuricemia and encephalopathy intractable to regular-dose insulin
Article
Licensed
Unlicensed Requires Authentication

Diabetic ketoacidosis, hyperuricemia and encephalopathy intractable to regular-dose insulin

  • Jillian Gregory EMAIL logo and Sonali Basu
Published/Copyright: November 11, 2017

Abstract

Background:

Diabetic ketoacidosis (DKA) in children less than 1 year of age is a rare occurrence. Typical presentation includes a prodrome of weight loss and polyuria with subsequent presentation to medical care when acidosis becomes symptomatic.

Case presentation:

We describe an unusual case of a previously healthy infant with a 3 days’ history of constipation, presenting acutely with abdominal pain, lethargy, and dehydration. On initial evaluation, our patient had profound encephalopathy, with marked tachypnea and work of breathing. Arterial blood gas revealed a pH of 6.9, pCO2 of 20 and a bicarbonate level of <5. There was profound leukocytosis (WBC 77 K/μL), hyperuricemia (uric acid 15.9 mg/dL), and evidence of pre-renal azotemia [blood urea nitrogen (BUN) 54, Cr 0.82]. Blood glucose was >700 mg/dL. Despite fluid resuscitation and insulin infusion of 0.1 unit/kg/h, which are the mainstays of therapy for DKA, her severe metabolic acidosis and altered mental status did not improve. Differential diagnosis for her metabolic derangements included inborn errors of metabolism, insulin receptor defects, toxic ingestions, and septic shock secondary to an underlying oncologic or intra-abdominal process. The patient was treated with broad spectrum antibiotics and rasburicase. She continued to have significant shock for the first 30 h of her hospital stay, requiring moderate vasoactive support. Due to her refractory acidosis and persistent hyperglycemia, insulin infusion was increased to 0.15 units/kg/h. A hemoglobin A1C obtained on the second hospital day revealed a level of 7.4 and helped to solidify the diagnosis.

Conclusions:

Metabolic acidosis in an infant requires a broad differential. Rasburicase should be considered in hyperuricemia and DKA.


Corresponding author: Jillian Gregory, DO, Department of Critical Care, Children’s National Medical Center, Washington, DC 20010, USA

  1. Author contributions: All the authors have accepted responsibility for the entire content of this submitted manuscript and approved submission.

  2. Research funding: None declared.

  3. Employment or leadership: None declared.

  4. Honorarium: None declared.

  5. 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.

References

1. Orlowski JP, Cramer CL, Fiallos MR. Diabetic ketoacidosis in the pediatric ICU. Pediatr Clin North Am 2008;55:577–87.10.1016/j.pcl.2008.02.015Search in Google Scholar PubMed

2. Koh JS, Hahm JR, Jung JH, Jung TS, Rhyu SS, et al. Intussusception in a young female with Vibrio gastroenteritis and diabetic ketoacidosis. Intern Med 2007;46:171–3.10.2169/internalmedicine.46.1719Search in Google Scholar PubMed

3. Dejkhamron P, Wejapikul K, Unachak K, Sawangareetrakul P, Tanpaiboon P, et al. Isolated methylmalonic acidemia with unusual presentation mimicking diabetic ketoacidosis. J Pediatr Endocrinol Metab 2016;29:373–8.10.1515/jpem-2015-0228Search in Google Scholar PubMed

4. Kılıç M, Kaymaz N, Özgül RK. Isovaleric acidemia presenting as diabetic ketoacidosis: a case report. J Clin Res Pediatr Endocrinol 2014;6:59–61.10.4274/Jcrpe.1181Search in Google Scholar PubMed PubMed Central

5. Bai F, Jiang FF, Lu JJ, Ma SG, Peng YG, et al. The impact of hyperglycemic emergencies on the kidney and liver. J Diabetes Res 2013;2013:967097.10.1155/2013/967097Search in Google Scholar PubMed PubMed Central

6. Acosta AA, Hogg RJ. Rasburicase for hyperuricemia in hemolytic uremic syndrome. Pediatr Nephrol 2012;27:325–9.10.1007/s00467-011-2047-ySearch in Google Scholar PubMed

7. Rosner EA, Strezlecki KD, Clark JA, Lieh-Lai M. Low thiamine levels in children with type 1 diabetes and diabetic ketoacidosis: a pilot study. Pediatr Crit Care Med 2015;16: 114–8.10.1097/PCC.0000000000000302Search in Google Scholar PubMed

8. Clark JA, Burny I, Sarnaik AP, Audhya TK. Acute thiamine deficiency in diabetic ketoacidosis: diagnosis and management. Pediatr Crit Care Med 2006;7:595–9.10.1097/01.PCC.0000244463.59230.DASearch in Google Scholar PubMed

Received: 2017-6-5
Accepted: 2017-9-4
Published Online: 2017-11-11
Published in Print: 2017-11-27

©2017 Walter de Gruyter GmbH, Berlin/Boston

Articles in the same Issue

  1. Frontmatter
  2. Mini Review
  3. Marine-Lenhart syndrome in two adolescents, including one with thyroid cancer: a case series and review of the literature
  4. Original Articles
  5. The role of enterovirus infections in type 1 diabetes in Tunisia
  6. Serum progranulin levels in relation to insulin resistance in childhood obesity
  7. Relation between circulating oxidized-LDL and metabolic syndrome in children with obesity: the role of hypertriglyceridemic waist phenotype
  8. Etiologies of short stature in a pediatric endocrine clinic in Southern Thailand
  9. Thyroid evaluation of children and adolescents with Williams syndrome in Zhejiang Province
  10. The use of a radiolucent template to improve bone age X-ray quality (BASIC study)
  11. How often are clinicians performing genital exams in children with disorders of sex development?
  12. Efficacy and safety of percutaneous administration of dihydrotestosterone in children of different genetic backgrounds with micropenis
  13. Higher phthalate concentrations are associated with precocious puberty in normal weight Thai girls
  14. Identification of a novel mutation of NR0B1 in a patient with X-linked adrenal hypoplasia and symptomatic treatment
  15. Mutation analysis of the phenylalanine hydroxylase gene and prenatal diagnosis of phenylketonuria in Shaanxi, China
  16. Could a combination of heterozygous ABCC8 and KCNJ11 mutations cause congenital hyperinsulinism?
  17. Case Reports
  18. Diabetic ketoacidosis, hyperuricemia and encephalopathy intractable to regular-dose insulin
  19. A novel DAX-1 (NR0B1) mutation in a boy with X-linked adrenal hypoplasia congenita
  20. High aldosterone and cortisol levels in salt wasting congenital adrenal hyperplasia: a clinical conundrum
  21. A newborn with combined pituitary hormone deficiency developing shock and sludge
  22. Acknowledgment
Downloaded on 24.9.2025 from https://www.degruyterbrill.com/document/doi/10.1515/jpem-2017-0225/html
Scroll to top button