ASDC Journal Abstracts, Sept 1998 Sahikh, Rafat and Shusterman, Stephen Delayed dental maturation in cleidocranialdysplasia. cleidocranial dysplasia; Dental Maturity Ratio. http://cudental.creighton.edu/asdc/htm/absep98.htm
Extractions: Abstracts, September 1998 Petersen, Kerstin and Wetzel, Willi-Eckhard: Recent findings in classification of osteogenesis imperfecta by means of existing dental symptons. J Dent Child, 65:305-309, September-October 1998. The findings are based on a clinical investigation conducted on forty-nine patients suffering from osteogenesis imperfecta (OI), as well as on a questionnaire study in which 117 osteogenesis imperfecta-affected persons or their parents were involved. The survey established pathological tooth discolorations as well as tooth abrasions. Dentinogenesis imperfecta (DI) was more frequently found in primary teeth than in permanent teeth. There were no sex-specific differences. Radiological abnormalities were found in both, abraded and/or discolored teeth, as well as in clinically normal appearing teeth. In most cases there were club-shaped extensions of the pulp chambers and obliterations of the root canals. The probability that dentinogenesis imperfecta occurs as an accompanying symptom of osteogenesis imperfecta, was not dependent on the degree of skeletal severity. The self-assignment according to A and B forms of osteogenesis imperfecta types I and IV in accordance with the presence/absence of dental symptoms was contradictory, since the literature was based on varying classifications. Dentinogenesis imperfecta type I; Osteogenesis imperfecta-classification; Fracture rate
Katalog - Wirtualna Polska Serwis Katalog w Wirtualna Polska S.A. pierwszy portal w Polsce. http://katalog.wp.pl/DMOZ/Health/Conditions_and_Diseases/Rare_Disorders/Cleidocr
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Listings Of The World Health Conditions And Diseases Rare Listings World Health Conditions and Diseases Rare Disorders CleidocranialDysplasia. Listings World, Over 2 million http://listingsworld.com/Health/Conditions_and_Diseases/Rare_Disorders/Cleidocra
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Extractions: A three-year-old child came to our hospital with the complaints of "a large depression in the front of the skull". The mental status and developmental milestones were normal. The child was otherwise clinically asymptomatic. Her older sib had similar complaints. Both children could touch their shoulders under their chin (Fig 1). A routine skull radiograph frontal and lateral views shows an open anterior fontanelle and presence of Wormian bones. The chest radiograph shows absence of both clavicles. These findings on radiography led to the diagnosis of Cleidocranial dysplasia. The patient also had this abnormality. The three-year-old also had severe dental abnormalities. All these clinical findings with radiological evidence to support it helped to reach the diagnosis of Cleidocranial dysplasia. Discussion: Synonyms
Extractions: Overview of Rare Disease Research Activities Recent Scientific Advances in Rare Disease Research McCune-Albright Syndrome (MAS) McCune-Albright syndrome is characterized by precocious puberty, skin pigmentation and weak, deformed bones with marrow replaced by fibrous tissues and sharp needle-like bony deposits (fibrous dysplasia of bone). While mutations leading to the activation of a gene regulating signaling pathways in cells have been identified in patients, the mechanism leading to fibrous dysplasia in bone is not clear. NIDR scientists have shown that the expression of the gene in question is upregulated during maturation of precursor cells to normal bone-forming cells (osteoblasts), and that this pattern of expression is retained in fibrous dysplasia. Characterization of fibrous dysplastic tissues revealed that the fibrotic areas consist of an excess of cells that resemble pre-osteogenic cells, whereas the abnormal bone formed de novo within fibrotic areas represents the output of mature, but abnormal osteoblasts. The abnormal osteoblasts produce a bone matrix enriched in certain anti-adhesion molecules, and poor in certain pro-adhesive molecules found at high levels in normal bone formation. These data indicate that fibrous dysplasia of bone is a disease of cells in the osteogenic lineage, related to the upregulation of gene expression and its effects on bone cell function. They further suggest that a critical, physiological, maturation-related regulation of the gene involved in signaling pathways makes cells in the osteogenic lineage a natural target for the effects of mutations in the gene and may provide a clue as to why bone itself is affected in this painful, crippling disease.
SearchUK Home Top Health Conditions_and_Diseases Rare_Disorders CleidocranialDysplasia. ADULT (18+), SHOPPING, FINANCE, GAMBLING, JOBS, TRAVEL, http://www.searchuk.co.uk/Top/Health/Conditions_and_Diseases/Rare_Disorders/Clei
