Paget’s disease, also known as osteitis deformans is a medical condition in which there are the cellular remodeling and abnormal formation of one bone or more. Bones affected do have remodeling, even at the microscopic level. In Paget’s disease, there is a significant breakdown of the bones, followed by an abnormal formation of the bones. The remodeling and abnormal formation of the new bones causes a structural weakness in the bone, and might also lead to conditions such as pain, fracture, and deformity. The main cause of this disease is not known, however, some factors that contribute to the development of this disease, have been identified. Some of these include genetics and environmental factors. This disease can affect any bone of the body, although, it has been noted that affects the femur, pelvic bones, skull and the lumbar vertebrae more. Paget’s disease has no cure yet, however, there are some medications that have proved useful in the treatment of the disease. Some of the medications include bisphosphonates and calcitonin. These are helpful in reducing the symptoms of the disease such as pain. Stem cell therapy and exosomes also have a huge potential of becoming one of the ways of treating this disease. Drug therapy does treat the disease successfully, especially when medications are started before complications set in. It’s more predominant in old adults, and in people above the age of fifty-five. It’s also more common in the male gender than in women.
What Are The Signs And Symptoms Of Paget’s Disease?
A large number of people affected by the Paget’s disease of the bone do not present with any symptoms. However, when they do, the most common complaint is bone pain. This is because the disease induces a remodeling of the bones, making them weak and fragile. This usually causes pain, deformities, and fractures. Below are some of the signs and symptoms of Paget’s disease;
- Skull: Paget’s disease of bone leads to an abnormal growth of bones in the skull. This usually leads to conditions such as loss of hearing, headaches, and even compressions of some vital parts of the skull. For example, some major vessels such as nerves and arteries might become compromised due to the abnormal formation of the bones. This might eventually lead to the loss of vision.
- Pelvis: This disease can also cause patients to feel some pain in the hips, and other bones in the pelvic region.
- Lower limb: Persons affected by the Paget’s disease of the bone tends to become bowlegged. This occurs because, as new bone tissues are formed rapidly, bones of low quality are formed, which might eventually lead to leg deformities such as the bending of the knee. Patients might also experience pains and stress on the joints. Osteoarthritis might occur when the disease is not well managed. This osteoarthritis might affect the knee, and the bones of the hip joint.
- Vertebrae: A lot of complications can occur when the spine is affected by this disease. For example, the patient’s nerve roots can be compressed by the bones. They might experience some loss of sensitivities in some parts of their body. In addition, they can have pains and some tingling sensations in the upper or lower extremities.
What Are the Risk Factors Of Paget’s Disease?
There are some factors that increase the chances of developing Paget’s disease. Some of these factors include the following;
- Age: Age plays an important role in the development of Paget’s disease. As an illustration, individuals above the age of 40 are at more risk of developing the disease.
- Gender: Statistics have shown that Paget’s disease is more prevalent in women.
- Race: It has been observed that Paget’s disease is more prevalent in Europe, especially in the central part of the country, and in England, Scotland and the Scandinavian countries.
- Family: People who have had a relative or sibling that got affected by this disease have a high chance of having Paget’s disease.
What Are The Causes Of Paget’s Disease?
The exact cause of Paget’s disease is not known yet, however, some factors that contribute to the development of the disease have been identified. These factors include genetics, environmental and some other acquired factors have been noted to contribute to the development of this disease. Below are some of the causes of this disease;
- Infections: One of the main causes of Paget’s disease is an infection. A common cause of this disease is viral infections. Some viral disease has been linked to the development of this disease. Some of them include the respiratory syncytial virus and paramyxoviridae Measles has also been identified as a cause of this disease. An individual might have these infections for a long time before the symptoms would manifest.
- Genetics: According to studies, some genes have been identified to contribute to the development of Paget’s disease. According to statistics, about fifty percent of all cases of Paget’s disease is caused by genetics. The people affected by genetics usually inherited the gene responsible for this disease from their parents. The gene is named the “SQSTM1” gene. The gene encodes the protein that regulates the action of osteoclastic cells in the body. Osteoclast cells are cells that break down the bone tissues. Meanwhile, another set of patients have a mutation of “RANK”. RANK is a receptor on the osteoclasts, that is responsible for breaking down the bone tissues.
What Are The Complications Of Paget’s Disease?
Some of the complications of Paget’s disease include the following;
- Fractures and bone deformities
- Loss of vision, mainly due to the compression of nerves associated with vision, in the skull.
- Heart failure
- Cancer
Stem Cell Therapy Of Paget’s Disease
Stem cells are cells that can proliferate, and also regenerate. They are a special type of cells that can develop into many kinds of cells. Stem cells also have the ability to repair and replace dead tissues. These features make them useful and important in the treatment of Paget’s disease. These stem cells are extracted from the body, then made to undergo some processes. They’re then released back into the body, to correct the deformities, and also regulate the rate of bone remodeling.
References
Colman, A. (2008). Induced Pluripotent Stem Cells and Human Disease. Cell Stem Cell, 3(3), pp.236-237.
SHIMIZU, M. (2011). Origin of Paget’s cells in Paget’s disease. Skin Cancer, 26(1), pp.21-27.
Wagner, G. and Sachse, M. (2011). Extramammary Paget disease – clinical appearance, pathogenesis, management. JDDG: Journal der Deutschen Dermatologischen Gesellschaft, 9(6), pp.448-454.
Wagner, G. and Sachse, M. (2011). Extramammary Paget disease – clinical appearance, pathogenesis, management. JDDG: Journal der Deutschen Dermatologischen Gesellschaft, 9(6), pp.448-454.
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