Calcitonin treatment in osteoectasia with hyperphosphatasia (juvenile Paget's disease): radiographic changes after treatment

Abstract

Osteoectasia with hyperphosphatasia is a rare skeletal disorder, characterised by demineralisation and expansion of tubular bones and elevated serum alkaline phosphatase. We present a girl diagnosed as having osteoectasia with hyperphosphatasia who had swelling of phalanges of both hands and motor retardation. She was treated with synthetic human calcitonin. Clinical and radiological findings showed remarkable improvement after 2 years' treatment.

Juvenile Paget's Disease with Paranasal Sinus Aplasia

“…JPD is a bone modeling disorder, and the basic pathology is believed to be due to a blockage in the transformation of coarsely woven bone into mature lamellar bone (4). The pathogenesis of JPD is unknown.…”

“…The pathogenesis of JPD is unknown. The increased serum alkaline phosphatase activity is a reflection of increased bone turnover, and excessive bone turnover is responsible for the decreased levels of mature lamellar bone (4). …”

“…The administration of calcitonin for JPD can slow the disease progression by promoting normal bone formation, resulting in an enhanced quality of life (2, 4, 5, 8). Furthermore, calcitonin is reported to not only arrest the disease progress, but it can also reverse the long bone abnormalities in terms of external dimensions and shape (8).…”

Intermediate type of Juvenile Paget's disease: A rare case in Indian population

“…Only few case reports have described dental findings such as missing incisors, resorbed or loose teeth, loss of lamina dura, premature exfoliation of deciduous teeth, or delayed eruption of the dentition. [259] The present case showed impacted 33 and missed 45 with over retained 85.…”

“…Imaging of the skull shows increased thickness of skull vault and expansion of jaw bones with osteolytic and sclerotic areas. [79101112] Involvement of the paranasal sinus in JPD is extremely rare.…”