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Paget's disease is uncommon in Asians. We present 3 patients with varying manifestations. A 50 years old lady was detected to have raised alkaline phosphatase at a routine health check-up. Isoenzyme studies confirmed its bony origin. Bone scan revealed polyostotic disease. A 65 years old male patient with known Paget's disease of 6 years duration p...
Citations
... Vertebral monostotic lesion of lumbar spine in Indian subcontinent are rare with only few cases reported. [11][12][13][14][15][16] Paget disease consist of an initial lytic phase, intermediate mixed phase and a late blastic phase. In bones with less trabecular/cortex ratio like skull, femur and humerus, Paget disease produce a leading edge in lytic phase. ...
Paget disease is a metabolic disorder involving abnormal bone turnover that consists of 3 phases: the initial lytic phase, the intermediate mixed phase, and the chronic sclerotic phase. Paget disease mostly presents as polyostotic or monostotic lesions of the spine, and is rare on the Indian subcontinent. We present a case of isolated Paget disease of the lumbar third vertebra, which was confirmed only at biopsy. The patient presented with chronic low backache with a developing neurological deficit. We managed the patient with posterior spinal stabilisation, using pedicular screws and vertebroplasty of the collapsed vertebrae to regain the height. Upon the diagnosis of Paget disease, the patient was treated with calcitonin and bisphosphonates for 3 months, along with regular monitoring of alkaline phosphatase levels. Through this case, we hope to emphasize that Paget disease should be considered in the differential diagnosis of lytic lesions. Additionally, the alkaline phosphatase level in this patient was high-normal, which was an aspect of the role of Paget disease as a mimicker. Confirmatory biopsy is mandatory in all lesions.
... Antiresorptive treatment with bisphosphonates are the benchmark of treatment in PD. 12 Historically, parenteral calcitonin has been used but the short half-life, frequent relapses after stoppage of therapy and resistance to treatment have limited its use. 13 The goal of the therapy is to induce full remission, depicted by symptom improvement and normal levels of markers of bone turnover (alkaline phosphatase). Bisphosphonates reduce pain and improve the osteogenic remodelling. ...
... 14 Post-treatment osteolytic lesions on skull appear sclerotic whereas bone scan analyses show marked improvement in up to eighty percent of the patients; in up to 20% of cases there is still residual uptake despite adequate control of the disease. 13 Our case was a monostotic Paget's disease of skull without any neurological or otological complications. Diagnosis was made by typical radiological picture and high serum alkaline phosphatase levels; so aggressive interventional diagnostic modalities like bone biopsy were omitted. ...
... Antiresorptive treatment with bisphosphonates are the benchmark of treatment in PD. 12 Historically, parenteral calcitonin has been used but the short half-life, frequent relapses after stoppage of therapy and resistance to treatment have limited its use. 13 The goal of the therapy is to induce full remission, depicted by symptom improvement and normal levels of markers of bone turnover (alkaline phosphatase). Bisphosphonates reduce pain and improve the osteogenic remodelling. ...
... 14 Post-treatment osteolytic lesions on skull appear sclerotic whereas bone scan analyses show marked improvement in up to eighty percent of the patients; in up to 20% of cases there is still residual uptake despite adequate control of the disease. 13 Our case was a monostotic Paget's disease of skull without any neurological or otological complications. Diagnosis was made by typical radiological picture and high serum alkaline phosphatase levels; so aggressive interventional diagnostic modalities like bone biopsy were omitted. ...
Paget’s disease (PD) of the bone is a chronic metabolic disorder involving increased bone turnover with abnormal repair, leading to bony deformities and associated pain. It is characterised by a disturbance in bone modelling and remodelling because of increased osteoclastic activity followed by improper osteoblastic repair. Although PD of bone is an uncommon entity, axial skeleton involving pelvic bones, spine and skull are the commonest sites. Here we report a case of 65-yearold postmenopausal female who presented with generalised bone pain and subsequently was diagnosed as a case of Paget’s disease and managed accordingly.J Enam Med Col 2016; 6(2): 106-109
... Paget's disease is a chronic non-hormonal bone remodelling disease characterised by aggressive bone resorption and imperfect deposition [1,2] resulting in a patchwork of structurally abnormal bone with extensive vascularity and fibrous tissue in the marrow [3]. It is commoner in males [4] and rare before the age of 55 years, but increases in prevalence thereafter with advancing age [5,6]. Though it is usually asymptomatic and diagnosed incidentally, most clinical symptoms are skeletal [1,7] It is a common disease in Caucasian Population, and rare in non-Caucasians, particularly Asians [6,8]. ...
... Bone that is remodelled due to Paget's Disease becomes enlarged, disorganized in structure, and weakened. Patient usually present with complains of pain(80%), skeletal deformity(15%), and pathological fracture (9%) due to structurally inferior bone and neurological compression syndromes due to bony expansion [1,4]. The bowing deformity is usually due to stress fractures that heal over time. ...
Paget's Disease is a chronic bone remodelling disease characterised by aggressive bone resorption and imperfect deposition resulting in structurally abnormal bone. It is a common in Caucasian population, and is rare in non-Caucasians, particularly Asians, with only stray case reports from India. We report a case of Paget's Disease of bone from Northeast region of India. A 55 year old male presented with bony deformity, pain and tenderness in upper third of his right leg for 1 year. There was no history of trauma or local infection. Blood investigations revealed raised ESR, CRP and Alkaline phosphatase. X-Rays revealed bowing and two healed fracture lines at upper third of tibia. Patient was operated where internal fixation with Interlocking nail was done after deformity correction and removal of pathological tissue. Histopathology report confirmed Paget's Disease. Calcium and vitamin D supplementation and medical treatment with Risedronate was started and it resulted in improvement in pain and reduction of serum Alkaline phosphatase levels. In conclusion, genetic factors as well as environmental triggers have been implicated in etiology of Paget's Disease. Though rare, it can occur in Indian population as well, so a high index of clinical suspicion is necessary for diagnosis.
... [2] Paget's disease is uncommon in Asians. [3] The disease is more common in men of older adults and mean age of occurrence is 59 ± 14 years. But it is rarely encountered in patients younger than 40 years of age. ...
Paget's disease of bone (PDB) is characterized by a focal alteration of bone remodeling, which leads to bone with anomalous structure and altered mechanical properties associated with pain and complications. It is more common in Western Europe where as rare in Africa and Asia. The disease is more common in men of older adults and mean age of occurrence is 59 ± 14 years. But it is rarely encountered in patients younger than 40 years of age.
... 4 In Asia, especially in India, the disease is rare. 5,6 In Pakistan this disease appears to be very rare but it might be because the cases here are underreported. The reported patient might not be the first person suffering from this disease, however, to the best of authors' knowledge it is the first reported case of Paget's disease of bone from Pakistan. ...
... Researchers have postulated that prevalence in India is underestimated as most cases were incidental findings. 5,6 Underreporting of cases was also one of the possible cause of low prevalence in India. 7 The disease might be sporadic or familial, while looking at the comparison between Chinese and Caucasian data; in all of the Chinese patients and in Indians the disease was sporadic. ...
Paget's disease of bone is a common metabolic bone disease in most of the European countries. The disease has distinct geographical distribution being rare in Asia. We report Paget's disease in a 70 years old Pakistani male who presented with history of pain in right leg with difficulty in walking for the last 5 months. Plain X-ray raised suspicion of Paget's disease of bone. Technetium-99m MDP bone scan showed involvement of multiple bones. Bone biopsy confirmed the diagnosis. This case illustrates that Paget's disease of bone does exist in Pakistan.
... The disease has been considered rare in Asian and African population. In India, according to recent studies the total number of reported cases are only about 100 [15][16][17][18][19][20]. These reports along with studies from china and Japan have shown that PD is not totally absent among Asians [6,12]. ...
Pagets disease of bone is a rare condition in India and other Asian countries, although it is a common metabolic bone disorder in west. The disease affects elderly and exact etiology is not known. It clinically presents as longstanding bone pain and deformity. We report a case of pagets disease in India affecting humerus and presented with pathological fracture twice before being diagnosed as pagets disease. Fracture was treated conservatively and patient was started on alendronate. Patient responded favourably at the end of six months of treatment.
... It is common in males and tends to increase in prevalence with age. Although viral aetiology has been proposed, the exact cause is unknown (2). Majority (>90%) are asymptomatic and are detected incidentally due to raised alkaline phosphatase or when radiographs are done for other reasons. ...
We herein describe a case with calvarial Paget's disease associated with basilar impression and platybasia leading to bulbomedullary junction compression. The patient was presented with the symptoms of increasing deafness, loss of balance, dysphagia, and neck pain spreading to the arms and skull. The diagnosis of basilar impression and detailed measurements of the craniocervical junction could be possible by the use of magnetic resonance imaging. MRI should be the preferred technique in such cases.
Introduction:
Paget disease of bone (PDB) is a disorder of altered bone remodeling mainly characterized by increased osteoclastic activity. While the exact Indian prevalence remains unknown, a clustering of published cases suggests South Indian predominance.
Objective:
To study the clinico-biochemical profile and therapeutic response of patients with PDB and briefly review the epidemiology of PDB from an Indian perspective.
Materials and methods:
Retrospective data was collected from the charts of patients who have been seen in endocrine out-patient clinics in Tamil Nadu over a 12-year period. Published literature on PDB from India was reviewed.
Results:
A total of 66 patients (71% males) predominantly from Tamil Nadu were studied. The mean age at presentation was 67 ± 8 years. Polyostotic involvement was seen in 89% and familial occurrence of PDB in 5 patients. Symptoms at presentation mainly included bone pain (51%) and skeletal deformities (18%). Scalp vein sign (21%) and sensorineural hearing loss (64%) were also noted. Incidental PDB detection by raised serum alkaline phosphatase (SAP) levels was observed in 17% and by abnormal fluorodeoxyglucose-positron emission tomography (FDG-PET) scan in 6% of cases. Mean SAP at presentation was 606 ± 438 IU/L (Normal, 76-140). Major skeletal site involvement includes pelvis (62.1%) and spine (34.8%). Mean (range) follow-up of the cohort was 3.4 yrs (1-12 yrs). In all, 64 subjects received zoledronate and two received alendronate, and mean (SD) SAP at 1-year was 73 ± 42 IU/L. All but two showed remission at the end of 1 year. Two had pathological fractures and two had sarcomas. A review of epidemiology of PDB in Indian literature clearly showed a South Indian predilection for unclear reasons.
Conclusion:
In our cohort of PDB, male gender, polyostotic involvement, and hearing impairment were noted in more than two-thirds of patients and single-dose intravenous zoledronate was effective in normalizing SAP in almost all patients. PDB is intriguingly more common in South India and this needs more exploration.
Lesions of the tibial tuberosity are very rare but often present a diagnostic dilemma due to their unusual location and appearances. Although some pathologies affecting this region are commonly seen elsewhere in the skeleton, they may have atypical imaging appearances and morphological characteristics when present at the tibial tuberosity. The present review describes the developmental stages of the tibial tuberosity, explaining why this area is prone to degenerative tractional changes and injury, and also highlights various pathologies including benign and malignant osseous lesions and trauma/degenerative related abnormalities.
