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  Vol. 128 No. 5, November 1974 TABLE OF CONTENTS
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Sporadic Peutz-Jeghers Syndrome in Early Childhood

A Diagnostic Dilemma

MAJ Philip Yosowitz, MC; LTC Robert Hobson, MC; LTC Frederick Ruymann, MC

Am J Dis Child. 1974;128(5):709-712.


Abstract

The sporadic case of Peutz-Jeghers syndrome presenting during childhood poses a continuing challenge. Mucocutaneous pigmentation represents the most reliable diagnostic sign and should be sought in any child who complains of episodic abdominal pain. Recurrent iron deficiency anemia points to gastrointestinal blood loss, even when periodic stool examinations are negative for occult blood. A conservative surgical approach is advocated with local excision of symptomatic and accessible polyps. The risk of a gastrointestinal malignant neoplasm in adults with Peutz-Jeghers syndrome may be slightly greater than in the general population, and in women there is an increased incidence of ovarian tumors. These factors, combined with the unpredictable appearance of new polyps, make continued follow-up of individuals and families with Peutz-Jeghers syndrome necessary.



Author Affiliations

USA; USA; USA

From the departments of general surgery (Drs. Yosowitz and Hobson), and pediatrics (Dr. Ruymann), Walter Reed Army Medical Center, Washington, DC.


Footnotes

Received for publication Oct 18, 1973; accepted April 3, 1974.

The opinions expressed herein are those of the authors and do not necessarily represent the views of the Department of the Army.

Reprint requests to Department of Pediatrics, Walter Reed Army Medical Center, Washington, DC 20012 (Dr. Ruymann).



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