Priority 9 from the Rare Musculoskeletal Diseases in Adulthood PSP
| UNCERTAINTY: How are other parts of the body affected by rare metabolic bone disorders to cause other symptoms? (JLA PSP Priority 9) | |
|---|---|
| Overall ranking | 9 |
| JLA question ID | 0076/9 |
| Explanatory note | Not available for this PSP |
| Evidence |
A study compared muscle size, density and fuction in 34 children and adults with XLH and 34 age and sex matched control subjects. The authors reported no difference in muscle size, but lower muscle density and muscle force and power in XLH ( Veilleux LN et al, J Clin Endocrinol Metab 2012; 97: 1492-1498). Patients with McCune-Albright syndrome have a broad spectrum of abnormalities in the gastrointestinal tract and pancreas.Wood LD, Noë M, Hackeng W, Brosens LA, Bhaijee F, Debeljak M, Yu J, Suenaga M, Singhi AD, Zaheer A, Boyce A, Robinson C, Eshleman JR, Goggins MG, Hruban RH, Collins MT, Lennon AM, Montgomery EA. Virchows Arch. 2017 Apr;470(4):391-400. |
| Health Research Classification System category | Musculoskeletal |
| Extra information provided by this PSP | |
|---|---|
| Original uncertainty examples | Are hearing loss and migraines associated a xlh? ~ I think OI is affecting some of my organs is there any evidence? Effect on other parts of body e.g. kidneys. Liver etc ~ Is high blood pressure linked to XLH? ~ Why is hearing of XLH patients affected? Is this linked to phosphates or calcification? |
| Submitted by | Individual survey submissions categorised by Health or Social Care Professionals, Organisations representing people with rare musculoskeletal diseases, people with rare musculoskeletal diseases, relatives/carers/friends, Other. For full details of the type of submitter for each individual question, please see the spreadsheet of data held on the JLA website. |
| PSP information | |
|---|---|
| PSP unique ID | 0076 |
| PSP name | Rare Musculoskeletal Diseases in Adulthood |
| Total number of uncertainties identified by this PSP. | 39 (To see a full list of all uncertainties identified, please see the detailed spreadsheet held on the JLA website) |
| Date of priority setting workshop | 18 June 2018 |