Obesity and Bone Fractures in PWS Children Tied to High Blood Levels of Protein, Study Says – Prader-Willi News


Posted: November 17, 2019 at 1:49 pm

Children with Prader-Willi syndrome (PWS) have high levels of a protein called LIGHT/TNFSF14, which may contribute to obesity and low bone mineral density (BMD), a study suggests.

The research, High levels of LIGHT/TNFSF14 in Prader-Willi syndrome, was presented at theEuropean Society of Pediatric Endocrinology 2019 meeting in Vienna (Session 2, pg 84) and published in the journal Hormone Research in Paediatrics.

Low bone mineral density (BMD) affects almost half of all teenagers and adults with PWS, putting them at a higher risk of bone fractures. However, the reason why low BMD exists in such a large proportion of these patients remains to be understood.

LIGHT/TNFSF14 is a protein implicated in the activation of lymphoid cells and proliferation of immune T-cells.

As studies also indicate that LIGHT/TNFSF14 is involved in bone remodeling and obesity, researchers in Italy assessed blood serum levels of this protein in 19 children with PWS (average age, about 11). And the team tested whether these levels correlate with parameters of obesity and bone quality.

Bone status, lean mass, and fat mass were assessed by Dual-energy X-ray absorptiometry (DXA) scan, a common technique to determine bone density and body composition.

Results revealed that PWS patients have significantly higher blood levels of LIGHT/TNFSF14 levels compared to controls (426.73 pg/ml vs. 162.26 pg/ml).

Higher protein levels significantly correlated with greater amounts of parathyroid hormone (PTH), a higher proportion of trunk fat, as well as with more total fat and left/right arm fat.

PTH increases the release of calcium from bones. When its levels are too high, the amount of calcium in bones drops low, increasing the risk for fracture.

In turn, higher LIGHT/TNFSF14 levels were linked with a lower concentration of IGF-1 (a critical mediator of bone growth and bone health), proportion of lean mass, femoral z-score, and lumbar z-score.

A Z-score compares the bone density of children with PWS to that of healthy children of the same age and sex. As such, these results link high LIGHT/TNFSF14 levels with lower BMD in the femur (thigh) and lower spine (lumbar region).

These results suggest that LIGHT/TNFSF14 levels are associated with those of parameters that contribute to both obesity and low BMD.

We demonstrated high serum levels of LIGHT/ TNFSF14 in PWS children that correlated with both fat and bone mass, the researchers wrote.

This protein, the team added, could contribute to obesity and bone disease affecting PWS patients, therefore representing a good pharmacological target.

Iqra holds a MSc in Cellular and Molecular Medicine from the University of Ottawa in Ottawa, Canada. She also holds a BSc in Life Sciences from Queens University in Kingston, Canada. Currently, she is completing a PhD in Laboratory Medicine and Pathobiology from the University of Toronto in Toronto, Canada. Her research has ranged from across various disease areas including Alzheimers disease, myelodysplastic syndrome, bleeding disorders and rare pediatric brain tumors.

Total Posts: 12

Jos is a science news writer with a PhD in Neuroscience from Universidade of Porto, in Portugal. He has also studied Biochemistry at Universidade do Porto and was a postdoctoral associate at Weill Cornell Medicine, in New York, and at The University of Western Ontario in London, Ontario, Canada. His work has ranged from the association of central cardiovascular and pain control to the neurobiological basis of hypertension, and the molecular pathways driving Alzheimers disease.

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Obesity and Bone Fractures in PWS Children Tied to High Blood Levels of Protein, Study Says - Prader-Willi News

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