Paget's Syndrome - Bone Disorders Message Board

Frustrated1 · 12-07-2003, 03:58 PM

Does anyone have any information on this syndrome. I believe that this is uneven marrow production, which causes thin spots in bone. I would like to know if there are tests that can be performed to determine if you have it or is it genetic.

Thanks,

butterflytrans · 12-08-2003, 05:11 PM

Quote:

Originally Posted by Frustrated1

Does anyone have any information on this syndrome. I believe that this is uneven marrow production, which causes thin spots in bone. I would like to know if there are tests that can be performed to determine if you have it or is it genetic.

Thanks,

hi there....Paget's disease isn't a disease of marrow production, but a disease at the level of the bone itself. Bone is constantly being built and broken down by osteoblasts and osteoclasts respectively. Normally, this is in an equilibrium so no bone is lost and no bone is gained. In paget's disease, it's not that the equilibrium is being shifted, but there is excessive bone remodelling. This eventually leads to bones that are poorly constructed at the microscopic level and they begin to do funny things....long bones like the femur start to bow like a thin branch. The skull begins to flatten (one of the most common complaints is that their hats don't fit them anymore)....bones are also at risk for fracture, and eventually leading to things like osteopenia. A treatment for the disease is through the use of drugs called bisphosphonates which inhibit osteoclast (bone breakdown cells) and help keep the bones from breaking down.

Frustrated1 · 12-09-2003, 07:49 PM

This information is very nice and explains it very well. Is this condition heriditary?
When would symptoms occur? I would assume they would be slow to detect.
Any more information would be extremely helpful

butterflytrans · 12-09-2003, 09:40 PM

Quote:

Originally Posted by Frustrated1

This information is very nice and explains it very well. Is this condition heriditary?
When would symptoms occur? I would assume they would be slow to detect.
Any more information would be extremely helpful

I'm not sure if it's hereditary to be quite honest. In terms of symptoms, patients often first complain that their hats are all of a sudden to small for them. They may complain of joint pain (due to formation of osteoarthritis at the joints), problems walking (due to bowing of long bones), loss in height, chest pain etc. etc. Patients may also be totally asymptomatic. One thing I didn't mention above is, when the bone is abnormally reconstructed in Paget's disease, you get areas where there is excessive bone deposition, you get areas where there is not a lot of bone deposition, and you also get areas where there is increased deposition of blood vessels. Because this can often be extensive, there is a lot of extra blood flow to these areas of bone which can cause a strain on your heart due to the extra work. Because there is an intricate series of nerves running through the holes in your skull, deformities of it can cause things like deafness (compression of the auditory nerve). You can also get spinal cord compression with/without back pain if the vertebra are involved.

Because of the often lack of symptoms, it is hard to detect Paget's, though there are tests like alkaline phosphatase levels in the blood along with a clinical picture which can be used as a guide.