Pitfalls in the diagnosis of insulin autoimmune syndrome (Hirata’s disease) in a hypoglycemic child: a case report and review of the literature

Abstract

Background Insulin autoimmune syndrome (IAS) is a rare cause of hyperinsulinemic hypoglycemia (HH) not addressed as a potential differential diagnosis in current pediatric guidelines. We present a case of IAS in a child with no previous history of autoimmune disease, no previous intake of triggering medications and absence of genetic predisposition. Case presentation A 6-year-old boy presented with recurrent HH (blood glucose of 26 mg/dL [1.4 mmol/L] and insulin of 686 μU/mL). Abdominal imaging was normal. After multiple therapeutic failures, we hypothesized misuse of exogenous insulin and factitious hypoglycemia. Council of Guardianship had the child separated from his mother, but insulin levels remained high. A chromatography test was then performed which showed high titers of endogenous insulin autoantibody (IAA) with early dissociation from the insulin molecule. The human leukocyte antigen (HLA) test showed a DRB1 *13:01/*08:02 genotype. The patient was advised to control food intake and physical activity routines. During a 5-year follow-up, hypoglycemic episodes were sparse, despite high insulin levels. Conclusions Misdiagnosis of IAS with factitious hypoglycemia may happen if IAS is not considered as a differential diagnosis, leading to potential traumatic consequences. Further efforts should be made to increase awareness of IAS as a differential diagnosis of hypoglycemia and to include it in pediatric guidelines.

Nationwide survey of endogenous hyperinsulinemic hypoglycemia in Japan (2017–2018): Congenital hyperinsulinism, insulinoma, non‐insulinoma pancreatogenous hypoglycemia syndrome and insulin autoimmune syndrome (Hirata’s disease)

“…Hirata’s disease has also been reported as the cause of transient hypoglycemia in children, who could have lower levels of blood insulin than in older patients, making this disorder as an important pitfall in the differential diagnosis of childhood HH.…”

A Case of Insulin Autoimmune Syndrome Accompanied With Systemic Joint Pain: A Case Report

“…This is generally due to the body's stress response and increased muscle activity, rather than directly affecting the musculoskeletal organs. Over time, severe and frequent hypoglycemia can have various detrimental effects on the body; however, specific musculoskeletal damage is not commonly reported [10]. This case report showed that controlling serum blood glucose levels can alleviate systemic joint pain; therefore, higher and lower glucose levels can affect joint pain.…”

Complete laboratory diagnosis of Insulin Autoimmune Syndrome

“…Our patient was only recommended to eat small and frequent meals low in carbohydrates. This is effective because postprandial hyperglycemia is reduced and consequently, the insulin release [ 18 ].…”