Complete agenesis of pancreas is a rare and lethal condition. Four cases have previously been reported in combination with other malformations, such as severe intrauterine growth retardation, hyperglycaemia and meconium ileus. We report a case of pancreatic agenesis as a single anomaly. The child died when 48 h old with severe metabolic acidosis. The literature is surveyed and the pathogenesis is discussed.
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Voldsgaard et al. (1994) studied this question.
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