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Barium enema findings in Type I hepatorenal glycogen storage disease

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Abstract

Four children with Type I hepatorenal glycogen storage have been studied by barium enema. All showed strikingly similar changes of a smooth-walled, slightly narrow but normal length colon without any haustration. The findings simulated colitis but the patients had either mild diarrhea or no gastrointestinal complaints. Small bowel series was normal. Endoscopy and biopsy failed to provide an adequate explanation, there being no evidence of glycogen storage in the biopsy material and the degree of colitis in two patients who were endoscoped was minimal. The explanation for these radiographic findings is not known at present, although they have similarities to the “cathartic colon syndrome” described in adults.

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Authors and Affiliations

  1. Department of Radiology and Pediatrics, Babies Hospital, Columbia-Presbyterian Medical Center, Columbia College of Physicians and Surgeons, 622 West 168 Street, 10032, New York, N. Y., USA

    Richard A. Fellows, Walter E. Berdon, David H. Baker & Ruth Harris

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  1. Richard A. Fellows
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  2. Walter E. Berdon
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  3. David H. Baker
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  4. Ruth Harris
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Additional information

Fellow in Pediatric Radiology, Maternal Child Health Bureau, Department of Health Education and Welfare Project No. 135-1972-1974.

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Fellows, R.A., Berdon, W.E., Baker, D.H. et al. Barium enema findings in Type I hepatorenal glycogen storage disease. Pediatr Radiol 3, 75–77 (1975). https://doi.org/10.1007/BF01000116

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