Osteoporosis International

, Volume 29, Issue 7, pp 1679–1681 | Cite as

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

  • P. Muto
  • A. Lo Gullo
  • G. Mandraffino
  • S. Loddo
  • M. Atteritano
Case Report


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.


Bone metabolism Klippel-Trénaunay syndrome Sclerostin 


Compliance with ethical standards

Conflict of interest



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Copyright information

© International Osteoporosis Foundation and National Osteoporosis Foundation 2018

Authors and Affiliations

  • P. Muto
    • 1
  • A. Lo Gullo
    • 1
  • G. Mandraffino
    • 1
  • S. Loddo
    • 1
  • M. Atteritano
    • 1
  1. 1.Department of Clinical and Experimental MedicineUniversity of MessinaMessinaItaly

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