7819 Hypoglycemia with Hereditary Hemorrhagic Telangiextsia in a Boy After Liver Transplantation

Abstract Disclosure: H. Miyagi: None. M. Igarashi: None. E. Tanimoto: None. R. Horikawa: None. Introduction: Hereditary hemorrhagic telangiectasia (HHT) causes arteriovenous malformation (AVM) in liver, lung, and brain. It is not clear whether it also causes hypoglycemia. We show a case of HHT combined with hypoglycemia after liver transplantation, improved by nutrition management and growth hormone treatment. Case: 11 months old boy born from a mother with HHT was diagnosed as having with AVM prenatally. He was once operated for the liver AVM but developed a new collateral circulation which caused severe heart failure and lead to liver transplantation. He showed severe growth retardation and malnutrition. His body weight was 4.6 kg and height were 60 cm (-5.8 SD) when he was 1 year old, and blood test showed low albumin for 3 g/dL (3.4 - 4.7 g/dL), and total protein for 4 g/dL (5.7 - 7.5 g/dL). Hypoglycemia was found while switching his nutrition from parenteral to enteral nutrition after operation. His insulin was under 0.3 µU/mL when his blood glucose was 49 mg/dL IGF1 was 17 ng/mL (ref.11 - 149 ng/mL), suggested growth hormone (GH) deficiency. GH stimulation test showed blunted response. GH treatment was started. Other pituitary hormones were within normal range. Nutritional support was added using high-calory milk. After GH treatment, IGF1 level increased to 87 ng/mL. GH and nutritional management improved not only his weight and height gain but also his glycemic control. Discussion: Hypoglycemia may be caused by both GHD and malnutrition. GHD and low IGF1 might be caused by malnutrition. Retesting GH secretion after improvement of nutrition is planned to clarify his pituitary function. Presentation: 6/2/2024