cherubism

..We have used the model of cherubism, a rare disorder characterized by giant-cell bone resorptive tumors of the mandible and maxilla, to explore ..

..Similarly, diseases such as cherubism and hyperparathyroid jaw tumor syndrome are restricted to the jaw bones and never been described in other sites...

..Similarly, diseases such as cherubism and hyperparathyroid jaw tumor syndrome are restricted to the jaw bones and never been described in other sites...

..In studies of a mouse model for cherubism, a genetic disorder in humans, the investigators seek to identify mechanisms in myeloid cells that are important ..

..In studies of a mouse model for cherubism, a genetic disorder in humans, the investigators seek to identify mechanisms in myeloid cells that are important ..

..Mutations in 3BP2 were found in cherubism, an lautosomal dominant inherited syndrome characterized by excessive bone degradation...

..Mutations in 3BP2 were found in cherubism, an lautosomal dominant inherited syndrome characterized by excessive bone degradation...

The authors reviewed 24 cases of familial or nonfamilial cherubism. The age at onset was between 6 and 10 years...

A mutation of the SH3BP2 gene is known to cause cherubism. As there are clinical and histopathological similarities between central giant cell granuloma and cherubism, we made a constitutional DNA analysis of the SH3BP2 gene in four ..

b>Cherubism is a rare hereditary condition characterised by progressive cystic proliferation of the mandible and maxilla in childhood, followed by post-pubertal involution of the process and jaw remodelling in adulthood...

..non-familial, generally unilateral osteoclast-rich lesions that are histopathologically indistinguishable from cherubism. Cherubism is an autosomal dominant disease that is characterised by bilateral radiolucencies of the jaw, and ..

..6). In 5 patients a clinical diagnosis of cherubism or concomitant neurofibromatosis type 1 was made (14 lesions)...

b>Cherubism is a rare developmental lesion of the jaw that is generally inherited as an autosomal dominant trait. Recent studies have revealed point mutations in the SH3BP2 gene in cherubism patients...

..to evaluate patients with dystrophies such as fibrous dysplasia, neurofibromatosis, Gorlin syndrome and cherubism. Some growth abnormalities lead to a pseudomalformative appearance...

The clinical picture of cherubism is similar to that of fibrous dysplasia. The initial clinical appearance involves the deformation of the maxillofacial area with orthodontic disorder...

b>Cherubism is a rare hereditary fibro-osseous childhood disease characterized by bone degradation and fibrous tissue replacement at the angles of the mandible and at the tuberosity areas of the maxilla that leads to prominence of the ..

..features of the MGCs in central giant cell granuloma (CGCG), peripheral giant cell granuloma (PGCG), cherubism, and aneurysmal bone cyst (ABC), and the mechanism underlying the interrelations between cellular components in ..

b>Cherubism (CBM) and central giant cell granuloma (CGCG) of the jaw and giant cell tumor (GCT) of the long bone are clinically different diseases. Histologically, they are all multinucleated giant cell (MGC)-containing lesions...

AIMS: The hereditary occurrence of cherubism indicates a probable genetic aetiology: a correlation with a mutation in the gene SH3BP2 has been demonstrated. A convincing concept of formal pathogenesis is not yet available...

OBJECTIVE: Cherubism is a rare hereditary multilocular cystic disease of the jaws, characterized by its typical appearance...

Two new cherubism cases have been documented and reported. Both were relatively mild clinically, being a Grade I within Motamedi's I to V clinical grading system...

b>Cherubism is a non-neoplastic bone disease characterized by clinically evident bilateral, painless enlargements of the jaws that are said to give the patient a cherubic appearance...

b>Cherubism is a paediatric disease affecting the jaws. It is a close radiological mimic of giant cell lesions of the mandible...

INTRODUCTION: Cherubism is an extensive kystic process of the mandibula. The diagnosis is often established in children presented swelling of the jaws...

BACKGROUND: Cherubism is a rare hereditary multi-cystic disease of the jaws, characterized by its typical appearance in early childhood, and stabilization and remission after puberty...

PURPOSE: To report a case of cherubism with extensive, bilateral orbital involvement occurring in a 27-year-old woman who had the diagnosis established at the age of 4 years. DESIGN: Single interventional case report...

b>Cherubism is a benign lesion that causes painless symmetrical enlargement of the jaws, usually with a familial tendency...

b>Cherubism is a benign disease of bones affecting the jaws and giving a characteristic cherubic appearance to the patient. On radiography, the lesions exhibit bilateral multilocular radiolucent areas...

..D419N (g.1371G>A, c.1255G>A) within exon 9 of SH3BP2 in a patient with cherubism, an autosomal dominant syndrome characterized by excessive osteoclastic bone resorption of the jaw...

..radiographic characteristics, giant cells lesions with Noonan-like phenotype can be considered a form of cherubism. Therefore, surgical intervention is not necessary, but radiographic follow-up and observation is very important ..

OBJECTIVE: We sought to describe the radiographic and imaging features of cherubism. CONCLUSION: Cherubism is a rare osseous disorder of children and adolescents...

b>Cherubism is a hereditary childhood disease of autosomal dominant inheritance that is more common in the male sex. Some cases have also revealed a mutation of the gene FGF-RIII (fibroblast growth factor receptor III)...

..It may be sporadic or inherited as an autosomal dominant trait and occurs between one in 1000-2500. Cherubism is a giant cell lesion of the jaws thought to be transmitted as an autosomal dominant trait...

..was revised in the light of laboratory investigations that were within the reference ranges, and normal appearance of the parathyroids on exploration to that of an extreme case of cherubism that behaved in a locally aggressive manner.

..identify its function or explain why SH3BP2 missense mutations cause bone loss and inflammation in patients with cherubism. We demonstrate that Sh3bp2 "cherubism" mice exhibit trabecular bone loss, TNF-alpha-dependent ..

..Genetic study identified the point mutations of 3BP2 gene in human-inherited disease cherubism. The multiple cysts in cherubism lesion of jaw bones are filled with the activated osteoclasts and stromal cells,..

Mutations in the SH3-domain binding protein 2 (SH3BP2) are known to cause a rare childhood disorder called cherubism that is characterized by inflammation and bone loss in the jaw, but the mechanism has remained unclear...

b>Cherubism is a rare autosomal dominant inherited condition caused by mutations in the c-Abl-binding protein SH3BP2...

b>Cherubism (MIM 118400) is an autosomal dominant inherited syndrome characterized by excessive bone degradation of the upper and lower jaws followed by development of fibrous tissue masses, which causes a characteristic facial swelling...

..b>Cherubism is a benign lesion of the maxilla and mandible histologically similar to giant cell tumor of bone and giant cell ..

..Point mutations of the 3BP2 gene cause the rare human inherited disorder cherubism, characterized by excessive bone resorption in the jaw bones...

BACKGROUND: Cherubism is a rare fibro-osseous disorder that almost exclusively affects the maxilla and mandible. CASE REPORT: We report on three affected males in three generations in family A, and ten affected patients in family B...

..of the bone is a clinical feature of both chronic recurrent multifocal osteomyelitis and (to a lesser degree) cherubism. The genes responsible for Majeed syndrome (LPIN2), murine chronic multifocal osteomyelitis (pstpip2), and ..

b>Cherubism is a rare autosomal dominant fibro-osseous disorder of childhood, mostly limited to the maxilla and mandible. Extra-cranial skeletal involvement is rare...

Many cases have been published on cherubism since Jones described it first time in three children of the same family [Am. J. Cancer 17 (1933) 946]...

b>Cherubism is a rare, inherited condition characterized by fibro-osseous lesions of the maxilla and mandible. It has recently been localized to chromosome 4p16.3...

b>Cherubism is a disease that usually affects the jaws in the paediatric population, mostly below five years of age. Radiologically, it closely resembles fibrous dysplasia and other giant cell lesions of the mandible...

b>Cherubism is an autosomal dominant disorder that may be related to tooth development and eruption...

b>Cherubism is an autosomal-dominant syndrome characterized by excessive bilateral maxillomandibular bony degeneration followed by fibrous tissue hyperplasia...

b>Cherubism is a rare benign, non-neoplastic condition characterized by facial alteration due to symmetrically expansion of bilateral mandibles and maxillas...

b>Cherubism is an early childhood disease that primarily involves the mandible and consists of painless mandibular enlargement with or without maxillary involvement and progresses rapidly over the course of several years...