PEDIATRICS Vol. 40 No. 2 August 1967, pp. 289-304
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COMMITTEE ON NUTRITION

NUTRITIONAL MANAGEMENT IN HEREDITARY METABOLIC DISEASE

CHARLES U. LOWE , DAVID BAIRD COURSIN , FELIX P. HEALD , MALCOLM A. HOLLIDAY , DONOUGH O'BRIEN , GEORGE M. OWEN , HOWARD A. PEARSON , CHARLES R. SCRIVER , L. J. FILER JR. , and O. L. KLINE

THIRTEEN YEARS AGO a dietary approach to the therapy of phenylketonuria was proposed, and data on the usefulness as well as the very real limitations of this program have accumulated in the intervening years. At the present time studies on the application of special diets for use in this disease, as well as for many other hereditary metabolic diseases, are in progress. As wider use is made of procedures for detection of hereditary metabolic disease in the newborn, an increasingly larger number of patients who may benefit from appropriate nutritional therapy will be identified very early in life. For example, calculations based on the current birth rate and apparent incidence of phenylketonuria indicate that as many as 4,000 infants with this disorder in the United States alone could require dietary therapy in the next decade. There is, therefore, a need to evaluate the principles governing nutritional management of hereditary metabolic disease in order to develop optimal treatment facilities for use in conjunction with new detection methods. It seems anomalous that comparatively little has been done either to establish good treatment practices in hereditary metabolic disease or to mobilize scientific resources to ensure an optimistic out-come for therapeutic endeavors, while so much emphasis has been placed on detection.

Dietary treatment of hereditary metabolic disease is simple in theory; however, practical application may be unexpectedly difficult, or even hazardous, if not carefully supervised. It should be determined whether: (1) the untreated disease is in fact harmful, (2) the treatment is useful in preventing or reversing the unfavorable progression of the disease, (3) the therapy may be harmful by interfering with growth or development, and (4) the program may be harmful to others to whom it is inadvertently or inappropriately given.




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J. P. Brosco, L. M. Sanders, M. I. Seider, and A. C. Dunn
Adverse Medical Outcomes of Early Newborn Screening Programs for Phenylketonuria
Pediatrics, July 1, 2008; 122(1): 192 - 197.
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