Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2018 Jul;29(7):1679-1681.
doi: 10.1007/s00198-018-4563-0. Epub 2018 May 17.

High levels of serum sclerostin and DKK1 in a case of Klippel-Trénaunay syndrome

P Muto  1 , A Lo Gullo  1 , G Mandraffino  1 , S Loddo  1 , M Atteritano  2
Affiliations
Case Reports

High levels of serum sclerostin and DKK1 in a case of Klippel-Trénaunay syndrome

P Muto et al. Osteoporos Int. 2018 Jul.

Abstract

Klippel-Trénaunay syndrome (KTS) is described as a complex syndrome characterized by various combinations of capillary, venous, and lymphatic malformations associated with bone and soft tissue hypertrophy. We report a case of a 67-year-old postmenopausal Caucasian women with KTS that shows elevated levels of sclerostin and Dickkopf-related protein 1 (DKK1). Dual-energy X-ray absorptiometry (DXA) BMD T-scores at lumbar spine and femur were normal. Serum calcium and phosphorus levels were consistently normal, 25-hydroxyvitamin D (25OHD) < 30 ng/mL, and normal parathyroid hormone (PTH). Turnover markers (serum osteocalcin [OCN], and carboxy-terminal cross-linking telopeptide of type 1 collagen [CTx]) were in the reference limits. It is interesting to note that the serum levels of sclerostin and DKK-1 were significantly higher in our patient with KTS than in a healthy volunteer (control), without impact on bone mineral density and bone formation markers. In fact, in our patient, the BMD at lumbar spine and femur was normal, and osteocalcin was not suppressed. Based on what is known, we would have expected to find low levels of the inhibitors of the Wnt system, perhaps we can explain the data as a response to the compensation for β-catenin hyper-transformation.

Keywords: Bone metabolism; Klippel-Trénaunay syndrome; Sclerostin.

PubMed Disclaimer

Similar articles

See all similar articles

Cited by

References

    1. Semin Pediatr Surg. 2014 Aug;23(4):208-15 - PubMed
    1. J Clin Invest. 2006 May;116(5):1202-9 - PubMed
    1. J Biol Chem. 2003 Jun 27;278(26):24113-7 - PubMed
    1. Exp Cell Res. 2013 May 15;319(9):1317-23 - PubMed
    1. Cell. 1995 Feb 10;80(3):371-8 - PubMed

Publication types

MeSH terms

LinkOut - more resources

  NODES
Note 1
twitter 2