Paget’s disease of the bone is also known as osteitis deformans (which in Latin would translate to deformity of the bones). The general mechanism of Paget’s disease involves the breakdown of bone (demineralization), which makes it brittle and weak, but then it is replacemed with abnormal and weak bone. Osteoclasts are the cells responsible for breaking down bone and releasing calcium into the blood serum, while osteoBlasts Build Bone and use the serum Ca+2 to do so. In Paget’s disease, initially there is an overactivity of osteoclasts breaking down bone, making it very weak. In attempts to remedy this situation, osteoclasts and osteoblasts have unregulated activity, which deposits an unorderly bone, not efficient for weight bearing, or activity in general, leading to weak bones.
An interesting aspect of this disease is that it is common in people of British descent. A diagnostic tool to help with figuring out whether or not Paget’s disease is present in a patient is the characteristic bone lesions seen in an X-Ray:
As you can see in this figure, the right hip bone is characteristically less dense (with more lytic lesions) than the left. This is due to the pathology of the disease, increasing bone remodeling by absorbing calcified bone.
What are the signs and symptoms of Paget’s disease? The patient will present with severe to moderate achy, deep, bone pain, or will not present with any bone pain at all. If bone pain is present, it will be worse at night. Most often affected areas of Paget’s disease are the femur, skull, tibia, vertebrae, and clavicles. If the long bones are affected, like the humerus or tibia, bowing of the bones can occur, due to the weak bone remodeling, weight bearing, and gravity associated with walking and daily living. Some patients may even have increased head circumference, due to the abnormal bone remodeling.
Lab tests will show elevated levels of serum alkaline phosphatase, which is a mineral released when hard bone is destroyed. Therefore, it will have no where to go other than into the blood, which can be detected.
You want to be careful with someone who has Paget’s disease, because it can present similarly like an infection of the bone (osteomyelitis) or osteosarcoma (cancer of the bone, which is common in children). They can have increased serum calcium, which can throw off condution to the heart and skeletal muscles, resulting in arrythmias or spasticity of muscles (abnormal involuntary contraction). Gout, kidney stones, or congestive heart failure can also ensue.
References:
http://www.mdconsult.com/das/pdxmd/body/266262956-15/0?type=med&eid=9-u1.0-_1_mt_1014936
Paget’s disease of the bone is also known as osteitis deformans (which in Latin would translate to deformity of the bones). The general mechanism of Paget’s disease involves the breakdown of bone (demineralization), which makes it brittle and weak, but then it is replacemed with abnormal and weak bone. Osteoclasts are the cells responsible for breaking down bone and releasing calcium into the blood serum, while osteoBlasts Build Bone and use the serum Ca+2 to do so. In Paget’s disease, initially there is an overactivity of osteoclasts breaking down bone, making it very weak. In attempts to remedy this situation, osteoclasts and osteoblasts have unregulated activity, which deposits an unorderly bone, not efficient for weight bearing, or activity in general, leading to weak bones.
An interesting aspect of this disease is that it is common in people of British descent. A diagnostic tool to help with figuring out whether or not Paget’s disease is present in a patient is the characteristic bone lesions seen in an X-Ray:
As you can see in this figure, the right hip bone is characteristically less dense (with more lytic lesions) than the left. This is due to the pathology of the disease, increasing bone remodeling by absorbing calcified bone.
What are the signs and symptoms of Paget’s disease? The patient will present with severe to moderate achy, deep, bone pain, or will not present with any bone pain at all. If bone pain is present, it will be worse at night. Most often affected areas of Paget’s disease are the femur, skull, tibia, vertebrae, and clavicles. If the long bones are affected, like the humerus or tibia, bowing of the bones can occur, due to the weak bone remodeling, weight bearing, and gravity associated with walking and daily living. Some patients may even have increased head circumference, due to the abnormal bone remodeling.
Lab tests will show elevated levels of serum alkaline phosphatase, which is a mineral released when hard bone is destroyed. Therefore, it will have no where to go other than into the blood, which can be detected.
You want to be careful with someone who has Paget’s disease, because it can present similarly like an infection of the bone (osteomyelitis) or osteosarcoma (cancer of the bone, which is common in children). They can have increased serum calcium, which can throw off condution to the heart and skeletal muscles, resulting in arrythmias or spasticity of muscles (abnormal involuntary contraction). Gout, kidney stones, or congestive heart failure can also ensue.
References:
http://www.mdconsult.com/das/pdxmd/body/266262956-15/0?type=med&eid=9-u1.0-_1_mt_1014936