1/12. Otolaryngological complications of osteopetrosis.osteopetrosis is a rare inherited bone disease that affects both humans and various mammals. The authors report on two cases of osteopetrosis with otolaryngological complications. One patient had the childhood form and presented with chronic otitis media and brain abscess. The second patient had the adult form and presented with sinusitis from tooth extraction which developed into chronic osteomyelitis of the maxillary bone.- - - - - - - - - - ranking = 1keywords = bone disease (Clic here for more details about this article) |
2/12. Intrathoracic extramedullary hematopoiesis arising in marble bone disease. Clarifying the genetic disease mechanism.We report a man with intrathoracic extramedullary hematopoiesis accompanied by marble bone disease who underwent thoracoscopic resection safely and definitively, followed by a radionuclide scan. Although intrathoracic extramedullary hematopoiesis is almost always accompanied by hemolytic anemia, this man's hematological data normalized. A consensus on the tumor genesis mechanism has yet to be reached, but the existence of marble bone disease in our case throws new light on the genetic mechanism in intrathoracic extramedullary hematopoiesis.- - - - - - - - - - ranking = 6keywords = bone disease (Clic here for more details about this article) |
3/12. role of osteoclastic dysfunction in the development of renal bone disease.A 47-year-old-man was referred for treatment for end-stage renal failure. He had been diagnosed with type II adult onset osteopetrosis before the deterioration of his renal function. He presented with anaemia, severe hypocalcaemia, secondary hyperparathyroidism and azotaemia. An iliac bone biopsy revealed increased bone volume, disturbed osteoid calcification, active osteoclastic bone resorption and fibrous transformation in the bone marrow space. Incomplete osteoclastic dysfunction strongly suggested hypocalcaemia and secondary hyperparathyroidism, and the osteoclastic bone resorption also indicated secondary hyperparathyroidism, even though bone resorption was potentially suppressed. The present case shows that evidence of the involvement of osteoclastic dysfunction in the development of renal bone disease can be found in bone histology.- - - - - - - - - - ranking = 5keywords = bone disease (Clic here for more details about this article) |
4/12. rickets in osteopetrosis--a paradoxical association.osteopetrosis is a hereditary bone disease with intense positive balance of body calcium. Infantile variety is often associated with rickets--a paradoxical association. Two siblings with osteopetro rickets are reported in the article. The pathophysiologic mechanism of the paradoxical association has been explained and various management options have been discussed. Both cases were treated with high dose calcitriol and calcium supplements.- - - - - - - - - - ranking = 1keywords = bone disease (Clic here for more details about this article) |
5/12. Elevated serum levels of creatine kinase BB in autosomal dominant osteopetrosis type II--a family study.A markedly elevated BB isoenzyme fraction of serum creatine kinase was noted in four male siblings and correlated with typical radiographic findings of autosomal dominant osteopetrosis Type II (ADO Type II). patients with other sclerosing bone diseases had no elevation of CK-BB. The precision of the electrophoretic mobility patterns and correlation by I-125 tagged radioimmunoassay method confirms that this is CK-BB. We postulate that the dysfunctional and/or immature osteoclasts in ADO are more dependent on CK-BB than on the usual tricarboxylic acid cycle for the production of energy. The correlation of marked elevation of serum CK-BB with radiographic evidence of ADO Type II may prove to be of value as a biologic marker in the early diagnosis of the illness and lead to better understanding of the metabolism of bone.- - - - - - - - - - ranking = 1keywords = bone disease (Clic here for more details about this article) |
6/12. Paget's disease of bone or osteopetrosis?We report a previously undescribed case of diffuse, scan-negative, and low active form of bone disease carrying clinical, x-ray, and biochemistry signs of Paget's disease of bone, which is analyzed in comparison with different forms of osteopetrosis.- - - - - - - - - - ranking = 1keywords = bone disease (Clic here for more details about this article) |
7/12. osteomyelitis of the mandible in marble bone disease.Marble bone disease is a rare skeletal disorder. Osteomyelitic changes in the jaw bones are frequently seen in this disease. Considering the rarity of the disease, a case of osteomyelitis of the mandible in marble bone disease is presented, together with a review of the available literature.- - - - - - - - - - ranking = 6keywords = bone disease (Clic here for more details about this article) |
8/12. osteopetrosis (marble bone disease)--agenesis of paranasal sinuses.A case of osteopetrosis (Marble Bone Disease) in a child is presented. Agenesis (non-pneumatization) of all paranasal sinuses has been one of the salient features in this case.- - - - - - - - - - ranking = 4keywords = bone disease (Clic here for more details about this article) |
9/12. Neurotologic manifestations of the osteopetroses.facial nerve paralysis or symptoms of audiovestibular nerve dysfunction may be the first indication of one of the group of bone diseases known as the osteopetroses. We describe three such patients. The pathophysiology of these diseases and the treatment of the resulting symptoms of facial and audiovestibular nerve dysfunction are discussed.- - - - - - - - - - ranking = 1keywords = bone disease (Clic here for more details about this article) |
10/12. osteopetrosis in trauma.A 19-year-old male with a bone disease that predisposes to pathological fractures was involved in an automobile accident. Although the physician must always look for multiple injuries in any trauma patient, this case illustrates that in the patient with bone disease, the probability of multiple fractures is extremely high. The differential diagnosis of dense-bone disease is discussed as are considerations relevant to caring for the trauma patient with bone disease.- - - - - - - - - - ranking = 4keywords = bone disease (Clic here for more details about this article) |
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