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A 25-day-old girl born from consanguineous parents presented with seizures. She was apyretic, and, except for axial hypotonia, her neurological examination was normal. A cerebral scan and cardiac and abdominal ultrasonography did not detect any abnormality. First-line biological investigations showed hypoketotic hyperinsulinemic hypoglycemia [glucose, 1 mmol/L (18 mg/dL); reference, 4–6 mmol/L (70–110 mg/dL); insulin, 19.32 mUI/L; reference, 2.00–12.00 mUI/L] and a constant hyperlactatemia (5.7 mmol/L; reference, 0.5–2.2 mmol/L). Cortisol (258 nmol/L; reference, 250–800 nmol/L), ammonia (34 μmol/L; reference

Background Hyper-IgA is not a rare finding in children although its causes are less reported than hypergamma-globulinemia in other classes of immunoglobulin. However, an isolated hyper-IgA might play a role as a diagnostic marker, in particular in children with an incomplete clinical picture at disease onset. Results We reported the case of a 3-year-old girl hospitalized for acute abdominal symptoms and suspicion of ruptured appendicitis. She presented severe inflammatory syndrome and her medical history related recurrent fever episodes. Serum immunoglobulin analysis was not in favor of an infection; indeed, IgA concentration alone increased and reached a surprising extremely high value in a young child (17-fold of the upper reference value). Conclusions This case highlights the potential clinical significance of an isolated hyper-IgA that is known to be mostly found in serious diseases in children; it might contribute to reduce the delay in diagnosis and treatment of hyperimmunoglobulinemia D syndrome, an autoinflammatory disease.