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Congenital galactosaemia reveals usually in the second and third weeks of life with a severe liver dysfunction.We report on a case of congenital galactosaemia with, on the one hand, an early onset liver failure, without any free interval, and on the other hand, an hemophagocytic syndrome as a severe secondary outbreak with pulmonary haemorrhage.Appropriate diet led to normalisation of liver function.Hemophagocytosis, probably linked to an associated Klebsiella Pneumoniae sepsis, had a favourable outcome after antibiotic and corticosteroid therapy.
Congenital galactosaemia reveals usually in the second and third weeks of life with a severe liver dysfunction. We report on a case of congenital galactosaemia with, on the one hand, an early onset liver failure, without any free interval, and on the other hand, an hemophagocytic syndrome as a severe secondary outbreak with pulmonary haemorrhage. Appropriate diet led to normalization of liver function. Hemophagocytosis, probably linked to an associated Klebsiella pneumoniae sepsis, had a favorable outcome after antibiotic and corticosteroid therapy.