Congenital absence of islets of Langerhans

A small-for-dates male infant who developed acute metabolic acidosis shortly after birth had diabetes and died aged 40 hours. At necropsy there was an absence of any recognizable islets of Langerhans though lymphorecticular aggregates were found. This was the fourth child of healthy unrelated parents whose first child, also a male, died at 48 hours under similar circumstances. It is suggested that both boys had the same underlying pathology and this might be a previously undescribed recessive or x-linked inherited condition.