Despite the multiple clinical clues which these infants give us and despite the passage of 300 years since its first discovery, we remain still quite ignorant of the pathogenesis. The essential given clinical characteristics regularly documented include the 5/1 male sex ratio/preponderance of first born infants presentation when the tumour is acquired at around 4 weeks of age spontaneous self-cure if the infant survives for several weeks with medical treatment problems with gastric hyperacidity in later life dangerous hypokalaemic alkalosis from loss of acid before the obstruction is relieved.
Author(s): I. M. Rogers*
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