Paget's disease with craniofacial and skeletal bone involvement

Prakash, Narendra; Anekar, Jayaprasad; Mustafa, Shabil Mohamed; Divakar, Darshan Devang

doi:10.1136/bcr-2016-216173

Cited by 5 publications

(3 citation statements)

References 12 publications

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“… A cartoon of the number of published case reports/series on Paget Disease of Bone from India from Pubmed and Google Scholar from 1963 to 2019. [ 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60 61 62 63 64 ] The size of the dots is representative of the number of case reports from that state. Size expressed as a fraction (0.1cm X Number of cases) and for purpose of rep-resentation, the largest representation is shown as Square root of (0.1cm X Number of cases) …”

Section: Discussionmentioning

confidence: 99%

Is paget disease of bone more common in South India? Clinical characteristics, therapeutic outcome and follow-up of 66 patients from Tamil Nadu

Asirvatham

Kannan

Mahadevan

et al. 2020

Indian J Endocr Metab

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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.

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Section: Discussionmentioning

confidence: 99%

Is paget disease of bone more common in South India? Clinical characteristics, therapeutic outcome and follow-up of 66 patients from Tamil Nadu

Asirvatham

Kannan

Mahadevan

et al. 2020

Indian J Endocr Metab

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“…It is commonly diagnosed in northern Europe, North America, Australia, and New Zealand, but is rare in the Asia, including Malaysia. 4,5 About 3.7% of individuals of Anglo-Saxon origin older than the age of 55 are afflicted with PDB. [6][7][8][9] It rarely manifest itself clinically before age 40, and the frequency of the condition increases with advancing age.…”

Section: Discussionmentioning

confidence: 99%

Metastatic Bone Disease Secondary to Bronchial Adenocarcinoma in a Patient with Paget’s Disease of the Bone

Lim

Kok

Kamaruddin

2018

JAFES

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A 69-year-old female complained of intermittent left hip pain for the past 3 years. Biochemical tests revealed normal serum calcium and phosphorus with markedly raised alkaline phosphatase. MRI of the hip revealed extensive marrow signal abnormalities at the left pelvic bone, while CT of the thorax revealed a spiculated lung nodule at the left lower lung lobe. In order to diagnose either primary, metastatic bone tumour or Paget's disease of the bone (PDB), an open biopsy of the left iliac bone was performed. The histopathology of bone biopsy of the left iliac bone was consistent with PDB. A CT guided biopsy of the lung mass done later revealed adenocarcinoma of the lung. She had 18 F-FDG PET-CECT Scan for staging evaluation and result was suggestive of new bony metastases. Patient was started on IV Zoledronic acid for treatment of the PDB. In view of the stage 4 lung adenocarcinoma with bony metastases, patient was scheduled for palliative chemotherapy.

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“…Histologically, the severity of the disease correlates with frequency of intranuclear inclusions. The skull bones are affected in addition to the pelvis, cervical, thoracic, and lumbar bones of the vertebral column ( Rai et al, 2016 ). Studies showed involvement of maxilla and mandible to be about 17 % with slight predilection for the maxilla relative to the mandible ( Fig.…”

Section: Endocrine/metabolic Related Craniofacial Deformitiesmentioning

confidence: 99%

Craniofacial disorders and dysplasias: Molecular, clinical, and management perspectives

Akintoye,

Adisa,

Okwuosa

et al. 2024

Bone Reports

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Paget's disease with craniofacial and skeletal bone involvement

Cited by 5 publications

References 12 publications

Is paget disease of bone more common in South India? Clinical characteristics, therapeutic outcome and follow-up of 66 patients from Tamil Nadu

Is paget disease of bone more common in South India? Clinical characteristics, therapeutic outcome and follow-up of 66 patients from Tamil Nadu

Metastatic Bone Disease Secondary to Bronchial Adenocarcinoma in a Patient with Paget’s Disease of the Bone

Craniofacial disorders and dysplasias: Molecular, clinical, and management perspectives

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