What is Endocrinology - When should a pagetic bone lesion be suspected of having malignant sarcoma?6/30/2022 What is Endocrinology - When should a pagetic bone lesion be suspected of having malignant sarcoma?
The emergence of new or intensifying bone pain and/or soft tissue swelling typically signals the beginning of malignant transformation inside pagetic bone. Radiographs frequently reveal the progressive breakdown of pagetic bone. Less frequently, sclerosis that is worsening or masses of thick, amorphous deposits in the bone are indicators of malignant transformation. In an otherwise stable patient, the serum levels of alkaline phosphatase may increase quickly. In the vicinity of the tumour, radionuclide absorption is typically reduced as seen on bone scans. Additionally, gallium scans reveal higher uptake in the affected area (s).
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What is Endocrinology- What is the bone-related Paget's disease complication that is the most severe?
The development of malignant sarcoma in pagetic bone is the most severe side effect of Paget's disease. Although such tumours are mostly solitary, 20% may be multicentric. Fortunately, less than 1% of people with clinically obvious Paget's disease experience this uncommon consequence. Paget's sarcoma is a highly aggressive tumour, and the average patient survival time is under a year. The most typical locations for sarcomatous transformation are the pelvis and long bones (humerus, femur, and tibia). Although chondrosarcomas and fibrosarcomas have also been documented in bone afflicted by Paget's disease, the tumour is typically an osteogenic sarcoma. Typically, a biopsy of the affected bone serves as a diagnosis. Paget's disease is also linked to other bone neoplasms, such as benign giant cell tumours, however these tumours do not have such a dire prognosis. What is Endocrinology - What are the signs that Paget's illness has to be treated?
The presence of symptoms is the main cue for treatment. Not all symptoms, nevertheless, are treatable. Both bone pain and some neurologic compression symptoms typically react. Conversely, therapy is unlikely to help with hearing loss, bone abnormalities, or mechanically unsound joints. The prevention of local progression, planned surgery at a pagetic site, widespread pagetic involvement in patients who will likely be immobilised for an extended period of time (to reduce the risk of hypercalcemia), and potential prevention of future complications are additional indications for treating Paget's disease. It is debatable how to handle asymptomatic Paget's disease patients. Untreated Paget's illness, however, seems to worsen over time, and not all asymptomatic patients stay that way. As a result, many doctors treat patients with osteolytic Paget's disease or asymptomatic patients with an active disease affecting the skull, vertebral bodies, or bones that bear weight What is Endocrinology - What is osteonecrosis of the jaw, and does it occur in Paget's disease patients on bisphosphonates?
A region of exposed bone in the maxillofacial region that lasts longer than six weeks is known as osteonecrosis of the jaw (ONJ). Usually, this problem develops after dental surgery. The symptoms might range from sharp jaw discomfort to a painful exposed bone. Although there have been a few cases of this syndrome happening in people with Paget's disease, ONJ has also been recorded in patients taking extended intravenous bisphosphonates for cancer. So, using bisphosphonates to treat Paget's disease shouldn't be restricted due to the risk of ONJ. However, it is advised that bisphosphonate medication be postponed until after anticipated significant dental procedures or oral surgery is finished. Routine dental exams and oral care should be provided to all individuals using bisphosphonates. What is Endocrinology - Does Paget's disease of the bones experience treatment resistance?6/30/2022 What is Endocrinology - Does Paget's disease of the bones experience treatment resistance?
There is some resistance to calcitonin and bisphosphonates. Neutralizing antibodies are typically linked to resistance to salmon calcitonin treatment of Paget's disease. It has also been documented that some bisphosphonates can cause resistance to develop after therapy. A successful response to a second bisphosphonate may still be possible despite one's susceptibility to it, according to studies. What is Endocrinology - Which medications are preferred for Paget's disease of the bones?
The preferred treatment for Paget's disease is bisphosphonates. The response to calcitonin is typically transient after treatment is stopped, in contrast to the treatment response to bisphosphonates, which frequently results in suppression of disease activity over extended periods of time, sometimes several years. Due to the availability of more strong drugs, etidronate and calcitonin are rarely utilised. In one research, patients with Paget's disease were given either a 15-minute zoledronic acid infusion or 60 days of oral risedronate. When compared to daily treatments with risedronate, a single infusion of zoledronic acid induced a quicker, more thorough, and longer-lasting response. Therefore, significant active disease or disease that does not respond to oral bisphosphonate medication may benefit more from intravenous bisphosphonate therapy. Other therapeutic methods, such as analgesics, nonsteroidal anti-inflammatory medications, canes, orthotics, hearing aids, and surgery, should also be used in the treatment of symptomatic patients. What is Endocrinology - What treatments are there for Paget's disease?
Although there is no known treatment for Paget's disease, there are a number of drugs that can be used to slow the disease's rapid osteoclastic bone resorption. Paget's bone disease is managed with a combination of calcitonin and bisphosphonates (Calcimar, Miacalcin injection). Currently, five bisphosphonates—etidronate (Didronel), alendronate (Fosamax), risedronate (Actonel), pamidronate (Aredia), and zoledronic acid—are authorised and accessible in the United States for the treatment of Paget's disease of the bone (Reclast, Aclasta). In clinical trials, the bisphosphonate ibandronate (Boniva) has been used to treat Paget's disease. However, the use of this medication to treat Paget's disease has not been authorised. Salmon calcitonin is a parenteral medication that must be administered intramuscularly or subcutaneously for six months, either daily or three times a week. Due to poor medication absorption, calcitonin nasal spray (Miacalcin, Fortical) is ineffective for the treatment of Paget's disease. What is Endocrinology - What gives rise to Paget's illness?
Both hereditary and nongenetic factors have been linked to the pathophysiology of Paget's disease, despite the fact that its aetiology is unknown. There are several polymorphisms that have been linked to Paget's disease. Up to 50% of patients with familial disease and 10% to 20% of instances that are sporadic have the SQSTM1 mutation. Patients with this mutation have more severe illness than those without it. A role for environmental factors in the aetiology of this disease is supported by the discovery of monostotic disease, the variable penetrance of Paget's disease in families with a genetic disposition, and the observation that the incidence of Paget's disease has been declining over the past 25 years. A viral origin is suggested by reports of paramyxovirus nucleocapsid-like structures in the osteoclasts of active pagetic bone. As etiologic agents, the measles virus, respiratory syncytial virus, and canine distemper virus have all been proposed; however, no virus has ever been cultivated from pagetic osteoclasts or osteoclast precursors up to this point. What is Endocrinology - Who among the patients is most susceptible to Paget's disease?
Age, gender, and place all affect the prevalence of Paget's disease. Although Paget's disease can develop in younger people, it most frequently affects people over the age of 50. Men are impacted more frequently than women (the male-to-female ratio is approximately 3:2). There is no clear familial pattern, however between 15% and 40% of affected patients have a first-degree relative who has the condition. Eastern and northern European people are more likely to contract the disease, as are populations in places where European immigrants have settled (including the United States, Australia, New Zealand, and South Africa). African Americans, people from Scandinavia, people from Asia, and people from Africa rarely acquire Paget's disease. What is Endocrinology - What histologic changes are caused by Paget's disease in bones?
An increased number of massive multinucleated osteoclasts, some of which have up to 100 nuclei, are present in greater numbers in the early lesions of Paget's disease. Large numbers of active osteoblasts are found producing bone at locations of earlier osteoclastic bone resorption during the mixed osteolytic-osteoblastic phase. In areas of high osteoblastic activity, bone deposition is chaotic (in a mosaic or woven pattern) as opposed to the regular bone's ordered lamellar pattern. Paget's disease-related woven bone is structurally weaker than healthy lamellar bone, which accounts for the propensity of pagetic bone to shatter or deform. |
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