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We report 40 cases in one family of an autosomal dominant bone dysplasia, which, though similar in some aspects to Paget's disease, seems unique in some features and in its natural history. The disease shows both general and focal skeletal changes, the latter being mainly in the limbs with an onset from the second decade. Progressive osteoclastic resorption is accompanied by medullary expansion which leads to pain, severe deformity and a tendency to pathological fracture. The serum alkaline phosphatase and urinary hydroxyproline are variably elevated, while other biochemical indices are normal. Most patients had an associated deafness of early onset and loss of dentition. No previous description of this disease has been found in the literature.
P H Osterberg, and
R G Wallace, and
D A Adams, and
R S Crone, and
G R Dickson, and
J A Kanis, and
R A Mollan, and
N C Nevin, and
J Sloan, and
P G Toner
February 1990,
Journal of oral pathology & medicine : official publication of the International Association of Oral Pathologists and the American Academy of Oral Pathology,
P H Osterberg, and
R G Wallace, and
D A Adams, and
R S Crone, and
G R Dickson, and
J A Kanis, and
R A Mollan, and
N C Nevin, and
J Sloan, and
P G Toner
January 1990,
Skeletal radiology,
P H Osterberg, and
R G Wallace, and
D A Adams, and
R S Crone, and
G R Dickson, and
J A Kanis, and
R A Mollan, and
N C Nevin, and
J Sloan, and
P G Toner
January 1991,
Bone,
P H Osterberg, and
R G Wallace, and
D A Adams, and
R S Crone, and
G R Dickson, and
J A Kanis, and
R A Mollan, and
N C Nevin, and
J Sloan, and
P G Toner
November 2006,
Skeletal radiology,
P H Osterberg, and
R G Wallace, and
D A Adams, and
R S Crone, and
G R Dickson, and
J A Kanis, and
R A Mollan, and
N C Nevin, and
J Sloan, and
P G Toner
January 2020,
Pediatric dentistry,
P H Osterberg, and
R G Wallace, and
D A Adams, and
R S Crone, and
G R Dickson, and
J A Kanis, and
R A Mollan, and
N C Nevin, and
J Sloan, and
P G Toner
September 1990,
Oral surgery, oral medicine, and oral pathology,
P H Osterberg, and
R G Wallace, and
D A Adams, and
R S Crone, and
G R Dickson, and
J A Kanis, and
R A Mollan, and
N C Nevin, and
J Sloan, and
P G Toner
March 2002,
Medicine,
P H Osterberg, and
R G Wallace, and
D A Adams, and
R S Crone, and
G R Dickson, and
J A Kanis, and
R A Mollan, and
N C Nevin, and
J Sloan, and
P G Toner
June 2016,
Journal of medical imaging and radiation oncology,
P H Osterberg, and
R G Wallace, and
D A Adams, and
R S Crone, and
G R Dickson, and
J A Kanis, and
R A Mollan, and
N C Nevin, and
J Sloan, and
P G Toner
March 2008,
Best practice & research. Clinical rheumatology,
P H Osterberg, and
R G Wallace, and
D A Adams, and
R S Crone, and
G R Dickson, and
J A Kanis, and
R A Mollan, and
N C Nevin, and
J Sloan, and
P G Toner
March 2010,
Journal of pediatric hematology/oncology,
