Endocrine Abstracts

We present this case of a 7-year-old female who had been well, without fever, polyuria or polydipsia, nor temperature intolerance. There was no known family history of auto-immune disorders. Physical exam was unremarkable but further labs revealed hyperglycemia (329 mg/dl), venous pH 7.37, glycosuria (no ketonuria). Her hyperglycemia resolved with intravenous and then subcutaneous insulin. Further labs revealed glutamic acid decarboxylase antibodies to be high (>30.0), and HbA1c 8.9%. Interestingly, she was also noted to have a decreased TSH (<0.008 μIU/ml) and elevated thyroid peroxidase antibodies (>600 U/ml). She was diagnosed as a new onset type 1 diabetes and Grave’s disease and started on an insulin regimen with methimazole daily.

Grave’s disease is the most common cause of hyperthyroidism in children and adults. While type 1 diabetes also presents in childhood, both conditions presenting coincidentally is extremely rare and uniquely categorized as a polyglandular autoimmune syndrome (PAS). There are three types of PAS; type 3 is when autoimmune thyroiditis occurs with another organ-specific autoimmune disease, 3A being with diabetes mellitus. This condition, associated with HLA type 2, has been noted to have an autosomal dominant pattern of inheritance with incomplete penetrance, usually occurring after the age of 30. Patients with PAS III must undergo lifelong monitoring of hormones and/or vitamin replacement therapy to avoid the development of new glandular failures.

Management of this condition in the pediatric population is challenging primarily because of the impact on families who have to learn to manage multiple conditions. Moreover, the pediatric age group may be asymptomatic, or have symptoms but remain unnoticed for a long time, putting them at risk for chronic lymphocyte infiltration. This case proves an important teaching point to routinely screen pediatric patients for autoimmune abnormalities in multiple organ systems because autoimmune endocrinopathy can be insidious in onset and necessitates adequate surveillance.