2011 - ISBTS 2011 Symposium
Plenary Session II: Physiology & Mucosal Biology + Oral Communications 8
10.201 - Hepatoadrenal syndrome in children with anatomic short gut and end-stage liver disease
Presenter: Hannah, Brotzman, Washington, United States
Authors: Gabriel Hauser1, Hannah Brotzman3, Stuart Kaufman2
Hepatoadrenal syndrome in children with anatomic short gut and end-stage liver disease
Gabriel Hauser1, Hannah Brotzman3, Stuart Kaufman2
1Division of Pediatric Critical Care Medicine, Department of Pediatrics, Georgetown University Hospital, Washington, DC, United States; 2Georgetown Transplant Institute and Department of Pediatrics, Georgetown University Hospital, Washington, DC, United States; 3School of Medicine, Georgetown University, Washington, DC, United States
Adrenal insufficiency (AI) in patients with liver failure, a condition referred to as hepatoadrenal syndrome (HAS), has been well characterized in adult patients but has not yet been described in children. We present a series of 22 children with end-stage liver disease and AI, diagnosed using corticotropin stimulation test (CST). Most of the 22 patients were infants with anatomic short gut suffering from severe total-parenteral nutrition-induced liver failure and awaiting liver and bowel transplantation. Many were critically ill as 68% required mechanical ventilation and 59% required vaspressor medications to support cardiovascular function. Average serum bilirubin was 25.7±14.0 mg/dL, serum albumin 2.6±1.0 mg/dL, international normalization ratio (INR) 2.4±1, platelet count 59,300±57,000/mcL and Pediatric End-Stage Liver Disease Model (PELD) scores 26 ± 11. Twelve (55%) patients died in the hospital, 11 prior to transplantation. There was no correlation between the severity of hepatic decompensation and degree of adrenal insufficiency. In addition, there were no differences in the clinical characteristics including serum electrolytes and vasopressor requirements between patients with HAS and group of 5 patients with liver failure and normal adrenal function. All patients had low baseline cortisol levels and 10 of them had an abnormal CST as well. Cortisol levels at baseline and increments of serum cortisol at 30 and 60 minutes post consyntropin were 9.3±5 μg/dL, 9.3±4 μg/dL, and 10.7±6 μg/dL, respectively, compared to values in the patients with normal adrenal function (21.3±3 μg/dL, 10.5±5 μg/dL, and 12.7±3 μg/dL, respectively). Baseline cortisol levels were significantly higher in patients who required vasopressors (11.1±5 μg/dL) compared to those who did not (6.6±4.3 μg/dL, p =0.04) and 60-minute increment in cortisol levels were significantly lower in non-survivors compared to survivors (8.6 ± 4.8 μg/dL vs 15.1 ± 5.1 μg/dL, p=0.002). Hydrocortisone therapy resulted in rapid weaning of vasopressor therapy but did not affect survival.
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