# Secondary Hirata Syndrome in a Neonate: Practical Approach and Management

**Authors:** Alessandro Perri, Simona Fattore, Annamaria Sbordone, Luca Viti, Dario Pitocco, Giovanni Vento

PMC · DOI: 10.1210/jcemcr/luae109 · JCEM Case Reports · 2024-07-01

## TL;DR

This paper presents the first reported case of a neonate with secondary Hirata syndrome, a rare condition causing hypoglycemia due to insulin autoantibodies passed from the mother.

## Contribution

The novelty lies in documenting the first neonatal case of secondary insulin autoimmune syndrome and discussing its management.

## Key findings

- A neonate presented with hypoglycemia caused by secondary insulin autoimmune syndrome.
- Insulin autoantibodies were transferred from the mother to the fetus through the placenta.
- Management strategies for neonatal hypoglycemia due to this syndrome were discussed.

## Abstract

Hirata disease, also known as insulin autoimmune syndrome (IAS), is a rare cause of hypoglycemia, due to the presence of insulin autoantibodies (IAA) in the circulating blood. These antibodies are immunoglobulin G (IgG), making placental transfer to the fetus possible. To our knowledge, no reports of IAS have been previously described in the neonatal population. We present a case report of hypoglycemia due to a secondary IAS in a neonate and discuss the management and treatment of the disease.

## Linked entities

- **Diseases:** hypoglycemia (MONDO:0004946), Hirata disease (MONDO:0018465), insulin autoimmune syndrome (MONDO:0018465)

## Full-text entities

- **Genes:** INS (insulin) [NCBI Gene 3630] {aka IDDM, IDDM1, IDDM2, ILPR, IRDN, MODY10}
- **Diseases:** Hirata disease (MESH:D004194), Hirata Syndrome (MESH:D013577), IAS (MESH:D007333), hypoglycemia (MESH:D007003)

## Full text

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## Figures

2 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11216324/full.md

## References

17 references — full list in the complete paper: https://tomesphere.com/paper/PMC11216324/full.md

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Source: https://tomesphere.com/paper/PMC11216324