Submitted to: Annals of Clinical and Laboratory Science
Publication Type: Peer Reviewed Journal
Publication Acceptance Date: 3/27/2007
Publication Date: 6/1/2007
Citation: Balazs, A.E., Athanassaki, I., Gunn, S.K., Tatevian, N., Huang, S.A., Haymond, M.W., Karaviti, L.P. 2007. Rapid resolution of consumptive hypothyroidism in a child with hepatic hemangioendothelioma following liver transplantation. Annals of Clinical and Laboratory Science. 37(3):280-284. Interpretive Summary: This paper describes a new approach in the prevention of hypo- and hyperglycemia in children with Type 1 diabetes. The purpose of this study was to see if giving pramalintide (manmade amylin) and insulin before a meal lower hyperglycemia and if a glucagon shot given in the late "after meal" time would prevent hypoglycemia. This study for the first time investigated the role of glucagon in the causation of hyperglycemia and its role in the prevention of hypoglycemia. We found that the combination of insulin and pramlintide was better than insulin alone in lowering blood sugar after a meal in children with Type 1 diabetes. Low doses of glucagon prevent hypoglycemia. The significance of this study is that we target 3 naturally occurring hormones that are abnormal in children with type 1 diabetes (insulin, glucagon and amylin) and that showed improvement of the blood sugars in the "after meal" period.
Technical Abstract: We report a unique case of a 3-mo-old female with consumptive hypothyroidism and liver hemangioendothelioma who required pharmacological doses of thyroid hormones and was cured following liver transplantation. Liver hemangioendotheliomas are capable of producing an excess of the thyroid hormone inactivating enzyme, type-3 iodothyronine deiodinase. The increased tumoral enzyme activity leads to rapid degradation of thyroid hormones, resulting in consumptive hypothyroidism. Review of similar cases indicated variable outcomes. We focus on our patient's clinical course and describe in detail the thyroid hormone replacement therapy and a unique outcome of this rare type of hypothyroidism. This first example of a prompt and complete resolution of consumptive hypothyroidism in an infant after liver transplantation confirms the concept and the reversibility of consumptive hypothyroidism and provides novel insights into the rapidity of response of the infant's hypothalamic-pituitary-thyroid axis to thyroid hormone replacement.