GA3-78
Rebound Hyperkalemia Post Subtotal Pancreatectomy in a Patient with Diffuse Congenital Hyperinsulinism from ABCC8 Mutation
1Liu C, 2Groener R, 2Shahrawat S, 2Sharma A
1Washington University/St. Louis Children’s Hospital, St Louis, MO, USA; 2St Louis Children's Hospital, St Louis, Missouri, United states
Introduction:
Congenital hyperinsulinism (CHI) is a disease characterized by persistent insulin secretion unresponsive to hypoglycemia1. Mutation in the pancreatic ATP-sensitive potassium channel protein sulfonylurea receptor 1, encoded by ABCC8, is one of the most common causes1. Due to the pervasive nature of the channelopathy, the disease often requires pancreatic resection.
Case description:
The patient, born at 37 weeks gestation without complications, weighing 4.55kg, had low serum glucose at birth requiring intravenous glucose infusion. He was the 3rd child of consanguineous Amish parents and 5th child of this family to be affected with CHI. Genetic testing confirmed ABCC8 homozygosity.
At 15 days old, he underwent subtotal pancreatectomy. General anesthesia was performed with a caudal catheter with the goal of reducing the glucose requirement during surgery by suppressing the stress response. Despite closely monitoring hemodynamic stability and serum glucose, within an hour after 97% of the pancreas was removed, we observed rebound hyperkalemia to 7.3 from baseline 4.6 without EKG changes. The potassium level normalized postoperatively without intervention while his glucose infusion requirement increased. The patient’s insulin/glucose balance was finally achieved on post-operative day (POD) 18 when he was completely off glucose infusion and subsequently discharged home on continuous tube feeding and octreotide.
Discussion:
In ABCC8 homozygosity causing CHI, pancreatic beta cells are depolarized causing calcium channel opening that triggers insulin secretion in the absence of glucose. Pancreatic resection is a necessity as medical treatment with diazoxide and octreotide is limited1. Managing the insulin/glucose balance in CHI is challenging during the perioperative period; yet we present another obstacle – potassium hemostasis as shown in our case. We postulate the patient’s hyperkalemia was due to abrupt withdrawal of excessive endogenous insulin causing potassium efflux into the extracellular compartment following pancreatectomy. Post-operative potassium normalization without intervention is likely due to inflammation of the unresected pancreas causing excess insulin production discernible in the increased glucose demand postoperatively. This is the first case report demonstrating transient rebound hyperkalemia following subtotal pancreatectomy. Although the patient did not experience hyperkalemia-induced arrhythmia, it remains a potential complication according to Austin, et al2. Thus, we recommend potassium level and EKG monitoring while maintaining normoglycemia when caring for CHI patients perioperatively.
Conclusion:
Healthcare providers should be vigilant regarding potassium homeostasis and potential arrhythmia during pancreatectomy, and maintain normoglycemia in spite of changing glucose level.
Reference:
1. Arnoux JB, et al. Congenital hyperinsulinism: current trends in diagnosis and therapy. Orphanet J Rare Dis 2011;6(63).
2. Austin JD, et al. Life-threatening hyperkalemia following partial pancreatectomy for neonatal hyperinsulinism. Pediatr Crit Care Med 2008; 9(3):e17-9.
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