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]

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.

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]
References in periodicals archive ?
Young, "ICVS (International Consortium for Vertebral Anomalies and Scoliosis) spondylocostal dysostosis, autosomal recessive," in GeneReviews[R], R.
Mode of Condition inheritance Autosomal (1) Jarcho-Levin syndrome (spondylocostal dysostosis): recessive shortened trunk, opisthotonus position of the head, short neck, barrel-shaped thorax, multiple wedge shaped and block vertebrae, spina bifida, and rib anomalies.
Orthodontic treatment in a patient with cleidocranial dysostosis. Angle Ortod.
Cleidocranial dysostosis. N Y State J Med 1963 May;63:1562-1566.Sonnenschein H
Treacher Collins syndrome also known as Mandibulofacial dysostosis or Franceschetti- Zwahlen-Klein Syndrome.
(1), (2) It was first described in 1962 by Maroteaux and Lamy as a form of dwarfism with craniofacial malformations similar to cleidocranial dysostosis. (2) Other authors have labeled it the Toulouse-Lautrec syndrome, as the French painter Henri de Toulouse-Lautrec is claimed to have suffered from the disorder.
Diagnosis: Based upon the history and clinical examination, a provisional diagnosis of mandibular hypo- and hyperdontia and chronic generalised gingivitis was made and the differential diagnosis of orodigitofacial dysostosis, Hallerman Streiff and Cleidocranial dysplasia syndromes were considered.
Mutations in a new gene in Ellis-van Creveld syndrome and Weyers acrodental dysostosis. Nat Genet 2000;24:283-6.
(2,6) Clinical manifestations of ML III include moderate to severe dysostosis multiplex, stiffness of fingers and shoulders, carpal and tarsal tunnel syndromes, claw-hand deformity, short stature, and scoliosis.
These syndromes such as Pierre Robin, Goldenhar, craniofacial dysostosis are common causes of difficult airway.
In Werth, the victim suffered cleidocranial dysostosis syndrome, a congenital bone development disorder characterized by a hump on his back, "absent or incompletely-formed collar bones, an abnormally shaped skull, characteristic facial appearance, short stature, and dental abnormalities." 472 F.