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PAGOD Syndrome: A New Abdominal Finding and Risk of Sudden Death

Journal of Perinatology volume 25, pages 349–351 (2005)Cite this article

PAGOD syndrome is a rare familial syndrome of multiple congenital anomalies associated with very high mortality during infancy. Until now it has been described only in the genetics literature. We wish to report a patient with this syndrome in order to demonstrate the imaging findings, present a new finding, the spherical spleen, and emphasize the importance of sudden death and arrhythmias in these patients.

The pulmonary hypoplasia seen in PAGOD syndrome is most frequently hypogenetic right lung with small right pulmonary artery in most cases. In addition to hypogenetic right lung muscular defects in the right hemidiaphragm have also occurred.3 Cardiac disease and fatal arrhythmias have also been described.4 Cardiac lesions have varied from mitral and aortic atresia to simple PDA, VSD and ASD and even one patient without heart disease.1 Wolff–Parkinson–White syndrome has been observed in approximately 25% of reported patients.4

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References

  1. Kennerknecht I, Sorgo W, Oberhoffer R, et al. Familial occurrence of agonadism and multiple internal malformations in phenotypically normal girls with 46,XY and 46,XX karyotypes, respectively: a new autosomal recessive syndrome. Am J Med Genet 1993;47:1166–1170.

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Authors and Affiliations

  1. Department of Radiology, St Louis Children's Hospital, Mallinckrodt Institute of Radiology, Washington University School of Medicine, St. Louis, MO, USA

    Thomas E Herman MD, William H McAlister MD & Madelyn M Stazzone MD

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  1. Thomas E Herman MD
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  2. William H McAlister MD
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  3. Madelyn M Stazzone MD
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Herman, T., McAlister, W. & Stazzone, M. PAGOD Syndrome: A New Abdominal Finding and Risk of Sudden Death. J Perinatol 25, 349–351 (2005). https://doi.org/10.1038/sj.jp.7211292

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