ABSTRACT
We have described three children with serum levels of TSH that are too high for the concomitant serum T4 level. Two of them meet the criteria for combined central and peripheral resistance to thyroid hormone. The third has long-standing central resistance to TSH suppression previously reported in congenital hypothyroidism. The recognition of this syndrome by physicians is important to avoid unnecessary and potentially dangerous treatment.
Subject(s)
Thyroid Function Tests , Thyroid Hormones/physiology , Child , Child, Preschool , Congenital Hypothyroidism , Female , Humans , Hyperkinesis/physiopathology , Hyperthyroidism/diagnosis , Hypothyroidism/diagnosis , Thyroid Gland/pathology , Thyroid Hormones/bloodABSTRACT
Hyperinsulinemic hypoglycemia secondary to nesidioblastosis persisted in a 4-year-old girl despite a 95% pancreatectomy and high-dose diazoxide treatment. Current literature suggests that dietary treatment, specifically protein restriction, of patients who are receiving diazoxide is ineffective and that further surgical pancreatectomy is required. However, in this patient, marked leucine sensitivity was demonstrated and a subsequent reduction in leucine intake eliminated hypoglycemia and the need for total or nearly total pancreatectomy. Therefore, dietary alteration should be considered in some patients with hyperinsulinism as an adjunct to other medical treatment.
