Biomaterial Database

Progressive diaphyseal dysplasia: Camurati-Engelmann's disease. 1983

C Lazzarone, and M Cartesegna, and M Crova, and D Calorio

The authors have studied two cases of progressive diaphyseal dysplasia (Camurati Engelmann's disease). In one case their investigations included bone marrow pressure tests, phlebography and blood gas analysis of the medullary blood. The BMP was raised, and the phlebogram showed venous stasis. The blood gas analysis revealed reduced oxygenation of the medullary blood. On the hypothesis that the vascular change could be of pathogenetic significance, the authors proceeded in both cases to open the medullary canal. This produced immediate remission of the pain, which still persists in one case after six years, while it lasted for three years in the second case.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D011241	Prednisone	A synthetic anti-inflammatory glucocorticoid derived from CORTISONE. It is biologically inert and converted to PREDNISOLONE in the liver.	Dehydrocortisone,delta-Cortisone,Apo-Prednisone,Cortan,Cortancyl,Cutason,Dacortin,Decortin,Decortisyl,Deltasone,Encorton,Encortone,Enkortolon,Kortancyl,Liquid Pred,Meticorten,Orasone,Panafcort,Panasol,Predni Tablinen,Prednidib,Predniment,Prednison Acsis,Prednison Galen,Prednison Hexal,Pronisone,Rectodelt,Sone,Sterapred,Ultracorten,Winpred,Acsis, Prednison
D001848	Bone Diseases, Developmental	Diseases resulting in abnormal GROWTH or abnormal MORPHOGENESIS of BONES.	Bone Dysplasias,Developmental Bone Disease,Bone Disease, Developmental,Bone Dysplasia,Developmental Bone Diseases,Dysplasia, Bone,Dysplasias, Bone
D003966	Camurati-Engelmann Syndrome	An autosomal dominant form of dysplasia that is characterized by progressive thickening of diaphyseal cortex of long bones. Mutations in the gene that encodes TRANSFORMING GROWTH FACTOR BETA1 are one cause of this disorder.	Diaphyseal Dysplasia, Progressive,Engelmann's Disease,Camurati-Engelmann Disease,Diaphyseal Dysplasia 1, Progressive,Diaphyseal Hyperostosis,Engelmann Disease,Progressive Diaphyseal Dysplasia,Camurati Engelmann Disease,Camurati Engelmann Syndrome,Diaphyseal Dysplasias, Progressive,Diaphyseal Hyperostoses,Dysplasia, Progressive Diaphyseal,Dysplasias, Progressive Diaphyseal,Hyperostoses, Diaphyseal,Hyperostosis, Diaphyseal
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013977	Tibia	The second longest bone of the skeleton. It is located on the medial side of the lower leg, articulating with the FIBULA laterally, the TALUS distally, and the FEMUR proximally.	Tibias