Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Perinatal/Neonatal Case Presentation
  • Published:

Perinatal/Neonatal Case Presentation

Pyloric stenosis in a newborn baby with polycystic kidneys

Abstract

Patients with infantile hypertrophic pyloric stenosis (IHPS) have an increased incidence of renal anomalies. Association of IHPS and polycystic kidney disease (PKD) has been reported in three case histories. It is uncertain whether the association of PKD and IHPS is due to genetics, a pathophysiological link or a chance phenomenon. One hypothesis linking the two conditions is nitric oxide synthetase (NOS) deficiency. NOS deficiency leads to lack of locally available nitric oxide causing a failure of smooth muscle relaxation. It is therefore plausible that NOS deficiency plays a part in the pathogenesis of pyloric stenosis, hypertension and cyst development in autosomal-dominant PKD.

This is a preview of subscription content, access via your institution

Access options

Buy this article

Prices may be subject to local taxes which are calculated during checkout

Similar content being viewed by others

References

  1. Atwell JD, Lewick P . Congenital hypertrophic pyloric stenosis and associated anomalies in the genitourinary tract. J Paediatr Surg 1981; 16: 1029.

    Article  CAS  Google Scholar 

  2. Loh JP, Haller JO, Kassner EG, Aloni A, Glassberg K . Dominantly-inherited polycystic kidneys in infants; association with pyloric stenosis. Pediatr Radiol 1977; 6: 27.

    Article  CAS  Google Scholar 

  3. Gupta AK, Berry M . Recessive polycystic kidney disease and congenital hypertrophic pyloric stenosis. Paediatr Radiol 1991; 21: 160.

    Article  CAS  Google Scholar 

  4. Lieberman E, Salinas-Madrigal L, Gwinn JL . Infantile polycystic disease of the kidney and liver: clinical pathological correlations and comparison with congenital hepatic fibrosis. Medicine 1971; 50: 277.

    Article  CAS  Google Scholar 

  5. Gagnadoux MF, Habib R, Levy M, Brunelle F, Broyer M . Cystic renal diseases in children. Adv Nephrol 1989; 18: 33.

    CAS  Google Scholar 

  6. Kaplan BS, Kaplan P, Rosenberg HK, Lamothe E, Rosenblatt DS . Polycystic kidney diseases in childhood. J Pediatr 1989; 115: 867.

    Article  CAS  Google Scholar 

  7. Fick-Brosnahan GM, Tran ZU, Johnson AM, Stran JD, Gabow PA . Progression of autosomal-dominant polycystic kidney disease in children. Kidney Int 2001; 59: 1654.

    Article  CAS  Google Scholar 

  8. Kaplan BS, Kaplan P, Dechadarievan JP, Jequier S, O'Regan S, Russo P . Variable expression of autosomal recessive polycystic kidney disease and congenital hepatic fibrosis within a family. Am J Med Genet 1988; 29: 639.

    Article  CAS  Google Scholar 

  9. Zerres K, Hansmann M, Mallman R, Gembruch U . Autosomal recessive polycystic kidney disease. Problems of prenatal diagnosis. Prenat Diagn 1988; 8: 215.

    Article  CAS  Google Scholar 

  10. Fernbach SK, Morello FP . Renal abnormalities in children with hypertrophic pyloric stenosis: fact or fallacy? Paediatr Radiol 1993; 23: 286–288.

    Article  CAS  Google Scholar 

  11. MacMahon B . The continuing enigma of pyloric stenosis of Infancy. Epidemiology 2006; 17 (2): 195–201.

    Article  Google Scholar 

  12. Barbosa IM, Frernte SMR, Mandarim-de-Lacerda CA . The role of nitric oxide synthase in infantile pyloric stenosis. J de Pediatr 2001; 77 (4): 307–312.

    CAS  Google Scholar 

  13. Subramaniam R, Doig CM, Moore L . Nitric oxide synthase is absent in only a subset of cases of pyloric stenosis. J Pediatr Surg 2001; 36: 616–619.

    Article  CAS  Google Scholar 

  14. Wang D, Braendstrup OB, Larsen S, Horn T, Strandgaard S . The expression and activity of renal nitric oxide synthase and circulating nitric oxide in polycystic kidney disease rats. APMIS 2004; 112: 358–368.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to J Tennakoon.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Tennakoon, J., Koh, T. & Alcock, G. Pyloric stenosis in a newborn baby with polycystic kidneys. J Perinatol 27, 125–126 (2007). https://doi.org/10.1038/sj.jp.7211634

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/sj.jp.7211634

This article is cited by

Search

Quick links