dysostosis


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dysostosis

 [dis″os-to´sis]
defective ossification; a defect in the normal ossification of fetal cartilages.
cleidocranial dysostosis an autosomal dominant condition in which there is defective ossification of the cranial bones, complete or partial absence of the clavicles, so that the shoulders may be brought together, or nearly together, in front, and dental and vertebral anomalies. See illustration.
Cleidocranial dysostosis. From Dorland's, 2000.
craniofacial dysostosis an autosomal dominant condition marked by a pointed or conical skull, protruding wide-set eyes, strabismus, parrot-beaked nose, and hypoplastic maxilla with relative mandibular prognathism. Called also Crouzon's disease.
mandibulofacial dysostosis a hereditary disorder occurring in two different forms: the complete form is Franceschetti syndrome and the incomplete form is Treacher Collins syndrome. Persons with the condition have downslanting eyes (antimongoloid palpebral fissures); absence of all or part of the lower lid; underdeveloped cheekbones that appear depressed; a prominent nose, wide mouth, and small receding chin; underdeveloped, malformed, or prominent ears; and small tufts of hair in front of the ears. There is often, but not always, some degree of hearing loss, usually conductive.
metaphyseal dysostosis a skeletal abnormality in which the epiphyses are normal or nearly so, and the metaphyseal tissues are replaced by masses of cartilage, producing interference with endochondral bone formation and expansion and thinning of the metaphyseal cortices.
orodigitofacial dysostosis orofaciodigital syndrome.

dys·os·te·o·gen·e·sis

(dis'os-tē-ō-jen'ĕ-sis),
Defective bone formation.
Synonym(s): dysostosis
[dys- + G. osteon, bone, + genesis, production]

dysostosis

/dys·os·to·sis/ (dis″os-to´sis) defective ossification; defect in the normal ossification of fetal cartilages.
cleidocranial dysostosis  a hereditary condition marked by defective ossification of the cranial bones, absence of the clavicles, and dental and vertebral anomalies.
craniofacial dysostosis  a hereditary condition marked by acrocephaly, exophthalmos, hypertelorism, strabismus, parrot-beaked nose, and hypoplastic maxilla.
mandibulofacial dysostosis  a hereditary disorder occurring in a complete form (Franceschetti syndrome) and a less severe form (Treacher Collins syndrome), with antimongoloid slant of the palpebral fissures, coloboma of the lower lid, micrognathia and hypoplasia of the zygomatic arches, and microtia.
metaphyseal dysostosis  a skeletal abnormality in which the epiphyses are normal and the metaphyseal tissues are replaced by masses of cartilage, producing interference with enchondral bone formation.
dysostosis mul´tiplex  Hurler's syndrome.
orodigitofacial dysostosis  orofaciodigital syndrome.

dysostosis

[dis′ostō′sis]
Etymology: Gk, dys + osteon, bone, osis
an abnormal condition characterized by defective ossification, especially defects in the normal ossification of fetal cartilages. Kinds of dysostoses include cleidocranial dysostosis, craniofacial dysostosis, mandibulofacial dysostosis, metaphyseal dysostosis, and Nager's acrofacial dysostosis.

dys·os·te·o·gen·e·sis

(dis-os'tē-ō-jen'ĕ-sis)
Defective bone formation.
Synonym(s): dysostosis.
[dys- + G. osteon, bone, + genesis, production]

dysostosis

Defective bone formation.

dysostosis

defective bone formation

dysostosis (dis··stōˑ·sis),

n condition marked by defective ossification, particularly the abnormal ossification of fetal cartilages.

dys·os·te·o·gen·e·sis

(dis-os'tē-ō-jen'ĕ-sis)
Defective bone formation.
Synonym(s): dysostosis.
[dys- + G. osteon, bone, + genesis, production]

dysostosis (disostō´sis),

n defective ossification.
dysostosis, cleidocranial
(klī´dōkrā´nēəl),
n (Sainton's disease), See dysplasia, cleidocranial.
dysostosis, craniofacial,
dysostosis, mandibulofacial (TreacherCollins syndrome),
n a developmental disturbance of the cranial bones and hypoplasias of the upper part of the face. The mandibular body is underdeveloped, but the ramus is hyperplastic. The teeth are crowded and malposed.
dysostosis multiplex,

dysostosis

defective ossification; a defect in the normal ossification of fetal cartilages.
References in periodicals archive ?
A variety of anatomical abnormalities, including anomalies of the extraocular muscles, have been reported in patients with craniofacial dysostosis.
A histologic study of cemetum in a case of cleidocranial dysostosis.
The second form, called spondylothoracic dysostosis, also has an autosomal recessive pattern of inheritance and is characterized radiographically by fusion and/or absence of ribs and multiple segmentation defects involving all vertebral levels.
Condylar agenesis is associated with congenital syndromes, such as otomandibular dysostosis, hemifacial microsomia, and mandibulofacial dysostosis.
Crouzon disease, also called craniofacial dysostosis, is one of a large group of birth defects in which there is abnormal fusion (joining) between some of the bones of the skull and of the face.
Underdevelopment also occurs in first and second branchial arch anomalies such as Treacher Collins syndrome, mandibulofacial dysostosis, and thalassemia major when the demand for marrow prohibits sinus pneumatization.
Husler coined the term dysostosis multiplex to describe the skeletal findings.
generalized delay or failure of eruption--certain conditions like cleidocranial dysostosis, gingival fibromatosis and some clefts of lip and palate (16)
12] Human genes: SNRPN, small nuclear ribonucleoprotein polypeptide N; DMD, dystrophin (muscular dystrophy, Duchenne and Becker types); FGFR2, fibroblast growth factor receptor 2 (bacteria-expressed kinase, keratinocyte growth factor receptor, craniofacial dysostosis 1, Crouzon syndrome, Pfeiffer syndrome, Jackson-Weiss syndrome); KRIT1, KRIT1, ankyrin repeat containing.
Shwachman-Diamond syndrome (SDS) is a rare inherited disorder characterized by pancreatic insufficiency and neutropenia, as well as metaphyseal dysostosis in slightly less than half the cases.
MPS VI patients typically show dysostosis multiplex, a specific radiologic expression involving the skull, thorax, pelvis, hands and spine.
The term dysostosis means defective ossification or defect in the normal ossification of fetal cartilages (Gr.