Pediatric Nephrology

, Volume 7, Issue 4, pp 375–378 | Cite as

Glomerulocystic disease: unilateral involvement of a horseshoe kidney and in trisomy 18

  • Randall D. Craver
  • Joseph Ortenberg
  • Radhakrishna Baliga
Original Article


Two occurrences of glomerulocystic kidney disease (GCD) in children younger than 1 year are described. One child was 3 months old with trisomy 18; the other child was 6 months old with GCD localized to one side of a horseshoe kidney. Lectin and immunohistochemical studies in tissue from the second child suggested that the entire nephron may be affected in GCD. There may also be overlap of morphological features between GCD and early stages of autosomal dominant polycystic kidneys.

Key words

Glomerulocystic disease Autosomal dominant polycystic kidney disease Horseshoe kidney Trisomy 18 Renal dysplasia 


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Verani RR, Silva FG (1988) Histogenesis of the renal cysts in adult (autosomal dominant) polycystic kidney disease: a histochemical study. Mod Pathol 1:457–463Google Scholar
  2. 2.
    Verani RR, Walker P, Silva F (1989) Renal cystic disease of infancy: results of histochemical studies. Pediatr Nephrol 3:37–42Google Scholar
  3. 3.
    Faraggiana T, Bernstein J, Strauss L, Churg J (1985) Use of lectins in the study of histogenesis of renal cysts. Lab Invest 53:575–579Google Scholar
  4. 4.
    Bernstein J, Landing BH (1989) Glomerulocystic kidney disease. Prog Clin Biol Res 305:27–43Google Scholar
  5. 5.
    Eulderink F, Hogewind BL (1978) Renal cysts in premature children: occurrences in a family with polycystic kidney disease. Arch Pathol Lab Med 102:592–595Google Scholar
  6. 6.
    Bengtsson U, Hedman L, Svalander C (1975) Adult type of polycystic kidney disease in a new-born child. Acta Med Scand 197: 447–450Google Scholar
  7. 7.
    Ross DG, Travers H (1975) Infantile presentation of an adult type polycystic kidney disease in a large kindred. J Pediatr 87:760–763Google Scholar
  8. 8.
    Chevalier RL, Garland TA, Buschi AJ (1981) The neonate with adult type autosomal dominant polycystic kidney disease. Int J Pediatr Nephrol 2:73–77Google Scholar
  9. 9.
    Kossow AS, Meek JM (1982) Unilateral adult polycystic kidney disease. J Urol 127:297–300Google Scholar
  10. 10.
    Lee JKT, McClennan BL, Kissane JM (1978) Unilateral polycystic kidney disease. Am J Roentgenol 130:1165–1167Google Scholar
  11. 11.
    Gagnadoux MF, Hibib R, Levy M, Brunelle F, Broyer M (1989) Cystic renal diseases in children. Adv Nephrol 18:33–58Google Scholar
  12. 12.
    Fellows RA, Leonidas JC, Beatty EC (1976) Radiologic features of “adult type” polycystic kidney disease in the neonate. Pediatr Radiol 4:87–92Google Scholar
  13. 13.
    Proesmans W, Van Damme B, Casaer P, Marchal G (1982) Autosomal dominant polycystic kidney disease in the neonatal period: association with a cerebral arteriovenous malformation. Pediatrics. 70:971–975Google Scholar
  14. 14.
    Ritter R, Siafarikas K (1976) Hemihypertrophy in a boy with renal polycystic disease: varied patterns of presentation of renal polycystic disease in his family. Pediatr Radiol 5:98–102Google Scholar
  15. 15.
    Cole B, Conley SB, Stapleton FB (1987) Polycystic kidney disease in the first year of life. J Pediatr 111:693–698Google Scholar
  16. 16.
    Anton PA, Abramowsky CR (1982) Adult polycystic renal disease presenting in infancy: a report emphasizing the bilateral involvement. J. Urol 128:1290–1291Google Scholar
  17. 17.
    Kaplan BS, Rabin I, Nogrady MB, Drummond KN (1977) Autosomal dominant polycystic renal disease in children. J Pediatr 90: 782–783Google Scholar
  18. 18.
    Blythe H, Ockenden BG (1971) Polycystic disease of kidneys and liver presenting in childhood. J Med Genet 8:257–284Google Scholar
  19. 19.
    Porch P, Noe HN, Stapleton FB (1986) Unilateral presentation of adult type polycystic kidney disease in children. J Urol 135: 744–746Google Scholar
  20. 20.
    Dobranowski J, Somers S, Pirani M (1986) Adult polycystic horseshoe kidney. J Can Assoc Radiol 37:208–209Google Scholar
  21. 21.
    George CRP (1981) Familial association of polycystic kidneys, horseshoe kidney and loin-pain-hematuria syndrome. J R Soc Med 74:77–78Google Scholar
  22. 22.
    Van Every MJ (1992) In utero detection of horseshoe kidney with unilateral multicystic dysplasia. Urology 40:435–437Google Scholar
  23. 23.
    Parfrey PES, Bear JC, Morgan J, Kramer BC, McManamon PJ, Gault MH, Churchill DN, Singh M, Hewitt R, Somlo S, Readers ST (1990) The diagnosis and prognosis of autosomal dominant polycystic kidney disease. N Engl J Med 323:1085–1090Google Scholar
  24. 24.
    Bernstein J (1968) Developmental abnormalities of renal parenchyma-renal hypoplasia and dysplasia. In: Sommers SC (ed) Pathology annual. Appleton Sentry Cross, New York, pp 213–247Google Scholar
  25. 25.
    Bartman J, Barraclough G (1965) Cystic dysplasia of the kidneys studied by micro-dissection in a case of 13–15 trisomy. J Pathol Bacteriol 89:233–238Google Scholar
  26. 26.
    Joshi VJ, Kasznica J (1984) Clinicopathologic spectrum of glomerulocystic kidneys: report of two cases and a brief review of literature. Pediatr Pathol 2:171–186Google Scholar

Copyright information

© IPNA 1993

Authors and Affiliations

  • Randall D. Craver
    • 1
  • Joseph Ortenberg
    • 1
  • Radhakrishna Baliga
    • 1
  1. 1.New Orleans Children's HospitalLouisiana State University Medical CenterNew OrleansUSA

Personalised recommendations