Treatment of rapid recurrence of severe steatosis with combined GLP-1 agonist and growth hormone therapy in a pediatric patient with hypopituitarism transplanted for NASH cirrhosis
Stephanie Saaybi1,2, Henry Shiau1,2, Goo Lee3, Babak Orandi4,5, Luz Helena Gutierrez Sanchez 1,2.
1Department of Pediatric Gastroenterology, Hepatology and Nutrition , University of Alabama at Birmingham , Birmingham, AL, United States; 2Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Hospital of Alabama , Birmingham, AL, United States; 3Department of Pathology , University of Alabama at Birmingham , Birmingham, AL, United States; 4Department of Surgery , New York University , New York City , NY, United States; 5Department of Medicine , New York University , New York City , NY, United States
The association between hypopituitarism and NAFLD/NASH is increasingly recognized in pediatrics; however, existing literature has been limited to pre-transplant care without much data on successful treatment modalities for rapid recurrence post-transplant in children.
A female patient with obesity and panhypopituitarism secondary to craniopharyngioma resection with multiple polyendocrinopathies was referred for elevated transaminases and diagnosed with NAFLD at 6-years-old. Over two years, she progressed to end-stage liver disease, and underwent whole orthotopic liver transplant at 8 years old. In the 2 months post-transplant, recurrence of elevated transaminases prompted a liver biopsy, which demonstrated rapid re-accumulation of severe macrosteatosis, with >70% hepatocytes involved. She was started on vitamin E, Omega 3, with maximum increase in GLP-1 agonist (Dulaglutide) dose. Patient lost considerable weight while on Dulaglutide. Growth hormone (GH) injections were started following undetectable levels on a provocative levodopa test. Her transaminases normalized after 2.5 months, and one-year post-transplant surveillance biopsy demonstrated steatosis of 10-15%.
This is one of the youngest reported patients with hypopituitarism to have undergone transplantation secondary to rapidly progressing NASH with rapid reaccumulation of severe steatosis post-transplantation. Patients with panhypopituitarism are predisposed to developing NAFLD/NASH due to hyperphagia, insulin resistance, central obesity and GH deficiency. GH replacement in conjunction with GLP-1 agonist therapy improved her hepatosteatosis after initial concerns for rapid reaccumulation post-transplantation. If started early, this therapy combination could help slow NAFLD progression and prevent early recurrence of steatosis post-transplantation in this patient population. More studies are needed to determine long-term effects and establish pediatric protocols.
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