Abstract

We present the case of a newborn who exhibited severe and persistent neonatal hypoglycemia without any apparent risk factors. The persistence of profound hypoglycemia, measured at 0.22 mmol/L, prompted an extensive biological workup that revealed a significant decrease of several pituitary hormones. The diagnosis of pituitary stalk interruption syndrome (PSIS) was confirmed by magnetic resonance imaging (MRI), which showed a complete interruption of the pituitary stalk. Prompt initiation of replacement therapy led to a notable clinical improvement and stabilization of blood glucose levels, thereby minimizing the risk of further hypoglycemic episodes.This case underscores the importance of broadening the etiological investigation in cases of persistent hypoglycemia without an obvious context, as hypoglycemia can be the sole clinical manifestation of PSIS. This congenital developmental anomaly of the pituitary gland represents a diagnostic emergency, and the prognosis is highly dependent on the prompt implementation of hormonal replacement therapy.

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