Abstract
To determine the spectrum of urologic disease and the value of ultrasound as a screening mechanism, renal imaging was performed on 23 patients with Bardet-Biedl syndrome. On intravenous urography (IVU), abnormal calices were present in 22 patients, with communicating cortical cysts/diverticula in 17. Ultrasound detected caliceal or cystic changes in 70%. On IVU, 21 patients had fetal-type lobular outlines that were detected on sonography in 95%.
Renal structural abnormalities are characteristic of the Bardet-Biedl syndrome, and are still best imaged by urography.
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References
- 1.
McKunik VA:Mendelian Inheritance in Man. 3rd ed, Baltimore: Johns Hopkins Press, 1971
- 2.
Churchill DN, McManamon P, Hurley RM: Renal disease—a sixth/cardinal feature of the Laurence-Moon-Biedl syndrome.Nephrology 16:151–154, 1981
- 3.
Alton DJ, McDonald P: Urographic findings in the Bardet Biedl syndrome.Radiology 109:659–663, 1973
- 4.
Srinivas V, Winsor GM, Dow D: Urologic manifestations of Laurence-Moon-Biedl syndrome.Urology 21:581–583, 1983
- 5.
Hurley RM, Dery P, Nogrady MB, Drummond KN: The renal lesion of the Laurence-Moon-Biedl syndrome.J Pediatr 87:206–209, 1975
- 6.
Linne T, Wikstad I, Zetterstrom R: Renal involvement in the Laurence-Mood-Biedl syndrome.Acta Paediatr Scand 75:240–244, 1986
- 7.
Bluett NH, Chantler C, Singer JD, Saxton HM: Congenital renal abnormalities in the Laurence-Moon-Biedl syndrome.Arch Dis Child 52:968–979, 1977
- 8.
Lee WJ, Turetz F: Laurence-Moon-Biedl syndrome.NY State J Med 78:959–960, 1978
- 9.
Price D, Gartner JG, Kaplan BS: Ultrastructural changes in the glomerular basement membrane of patients with Laurence-Moon-Biedl-Bardet syndrome.Clin Nephrol 16:283–288, 1981
- 10.
McLaughlen TG, Shanklin DR: Pathology of Laurence-Moon-Bardet-Biedl syndrome.J Pathol Bacteriol 93:65–79, 1967
- 11.
Falkner B, Langman C, Katz S: Renal histopathological changes in a child with Laurence-Moon-Biedl syndrome.J Clin Pathol 30:1077–1081, 1977
- 12.
Harnett JD, Green JS, Cramer BC, Johnson G, Chafe L, McManamon PJ, Farid NR, Pryse-Phillips W, Parfrey PS: The spectrum of renal disease in Laurence-Moon-Biedl syndrome.N Engl J Med 319:615–618, 1988
- 13.
Patriquin HB: Fetal lobation of the kidney: an ultrasonoanatomic correlation. Poster exhibit, International Pediatric Radiology meeting, Toronto, 1987
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Cramer, B., Green, J., Harnett, J. et al. Sonographic and urographic correlation in Bardet-Biedl syndrome (Formerly Laurence-Moon-Biedl Syndrome). Urol Radiol 10, 176–180 (1988). https://doi.org/10.1007/BF02926564
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Key words
- Bardet-Biedl syndrome
- Laurence-Moon-Biedl syndrome
- Renal abnormalities
- Renal imaging