Journal of Pediatric Urology
Volume 6, Issue 4 , Pages 389-395, August 2010

Detection of RXFP1 receptors in skin biopsies from children with congenital adrenal hyperplasia: A preliminary report

  • Timothy E. Cooney

      Affiliations

    • Department of Urology, Hamot Medical Center, Erie, PA, USA
    • Department of Orthopaedic Research, Hamot Medical Center, Erie, PA, USA
  • ,
  • Ronak A. Gor

      Affiliations

    • Lake Erie College of Osteopathic Medicine, Erie, PA, USA
  • ,
  • Donald W. Pfaff

      Affiliations

    • Department of Neurobiology and Behavior, The Rockefeller University, New York, NY, USA
  • ,
  • Justine M. Schober

      Affiliations

    • Department of Urology, Hamot Medical Center, Erie, PA, USA
    • Department of Neurobiology and Behavior, The Rockefeller University, New York, NY, USA
    • Corresponding Author InformationCorresponding author. Department of Urology, Hamot Medical Center, 333 State Street, Suite 201, Erie, PA 16507, USA. Tel.: +1 814 455 5900; fax: +1 814 456 0667.

Received 1 July 2009; accepted 5 October 2009. published online 09 November 2009.

Abstract 

Objective

Relaxin may potentiate the effect of topical estrogen treatment to eradicate post-incisional scarring in congenital adrenal hyperplasia (CAH) patients undergoing genitoplasty. The aim of this study was to determine whether CAH skin is capable of responding to relaxin.

Patients and methods

Skin biopsies were obtained from four female CAH patients (aged 2–9 years; Prader 4–5, salt-wasting, 21-hydroxylase deficiency, Caucasian) during routine genitoplasty surgery and screened for relaxin receptors. All received corticosteroid and mineralocorticoid replacement therapy. Specimens were sectioned, mounted and screened for the presence of the putative H2 relaxin receptor using conventional two-antibody immunohistochemistry. Tissue controls were processed concurrently.

Results

Tissue controls evidenced appropriate staining. Biopsies from CAH patients stained positively for RXFP1 expression while some variation between specimens was evident. Staining occurred adjacent to the basement membrane of the epithelium, localized to germinative basal keratinocytes.

Conclusion

Based on a limited patient sample, germinative keratinocytes in CAH patients appear competent to respond to relaxin perhaps topically applied. Given that relaxin downregulates collagen accretion and upregulates collagenases, its use may potentiate the effects of estradiol and abrogate post-incisional wound scarring. More research is needed to confirm or refute this thesis.

Keywords: Relaxin, Receptors, Keratinocytes, Congenital adrenal hyperplasia

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PII: S1477-5131(09)00456-2

doi:10.1016/j.jpurol.2009.10.003

Journal of Pediatric Urology
Volume 6, Issue 4 , Pages 389-395, August 2010