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January 1967

Dietary Treatment of Maple Syrup Urine Disease

Author Affiliations

From the Medical Unit, University College Hospital Medical School, London.

Am J Dis Child. 1967;113(1):58-59. doi:10.1001/archpedi.1967.02090160108011

ABOUT three years ago, the dietary treatment of a newborn infant with maple syrup urine disease was reported (Westall, 19631). At that time, the child was 15 months old and the belief was expressed that the child's mental and neurological status was normal and that the controlled diet had prevented the serious cerebro-degenerative changes which occur when this disease is untreated. The purpose of this paper is to report on the further progress of this child, now nearly 5 years old.

When 5½ months old, the patient was allowed to go home. Her mother was instructed in the preparation of the diet by a dietitian and she was provided with the necessary equipment. Apart from occasional short stays in hospital, not exceeding two weeks at any one time, the child has been cared for at home since that time. She now attends a nursery school for physically handicapped children