Hirata Syndrome in a Woman With Graves’ Disease Without Exposure to Thionamides and Summary of Cases from South America

TL;DR

A 65-year-old woman with Graves’ disease developed Hirata syndrome, a rare autoimmune condition causing hypoglycemia, without exposure to thionamides, and was successfully treated with corticosteroids and dietary changes.

Contribution

This is the third reported case of Hirata syndrome in Peru and highlights its occurrence without thionamide exposure.

Findings

Hirata syndrome was diagnosed in a patient with Graves’ disease and no prior thionamide use.

Treatment with prednisone and dietary adjustments resolved the hypoglycemia within six months.

Corticosteroid therapy led to a complication of unilateral avascular necrosis of the hip.

Abstract

We report here the third case of Hirata syndrome from Peru, a 65-year-old woman who presented to the emergency room with obtunded sensorium caused by severe hypoglycemia secondary to endogenous autoimmune hyperinsulinism after 1 year of many episodes of postprandial hypoglycemia. Six months before admission, both arterial hypertension and Graves’ disease were disclosed, receiving nebivolol 5 mg day only. Eight days before admission, flavonoids were prescribed for mild venous insufficiency. Treatment of hypoglycemia included drug withdrawal, a fractionated diet with low-glycemic index carbohydrates, prednisone, and radioactive iodine for definitive treatment of Graves’ disease, with resolution of the condition 6 months later. She presented unilateral avascular necrosis of the hip as a complication of corticosteroid therapy. We did not identify any trigger factor for hypoglycemia besides Graves’ disease as she did not receive any drug at hypoglycemia onset. We recommend checking for anti-insulin antibodies in cases of endogenous hyperinsulinism associated with an autoimmune disease, despite the absence of any drug treatment.