Biomaterial Database

[Clinical and radiological aspects of maxillo-nasal dysostosis: "naso-maxillo-vertebral syndrome". A study of 34 new cases (author's transl)]. 1979

J Delaire, and P Tessier, and J F Tulasne, and F Resche

Maxillo-nasal dysostosis is not uncommon as we have noted 34 new cases in eight years. The most characteristic anomalies, well described by Binder, are nasal hypoplasia both of bone and cartilage with agenesis of the nasal spine, as well as a characteristic rearrangement of the naso-labial muscles. On first seeing the patient the disease is generally recognized by the very typical aspect of the nose. There very often exists however other dental, mandibular and cervical spine anomolies. It is indispensable to look for these anomalies in the work up of patients with this malformation syndrome "the naso-maxillo-vertebral syndrome", in order to better understand the disease and better treat the patients.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D008313	Malocclusion, Angle Class III	Malocclusion in which the mandible is anterior to the maxilla as reflected by the first relationship of the first permanent molar (mesioclusion).	Angle Class III,Habsburg Jaw,Hapsburg Jaw,Prognathism, Mandibular,Underbite
D008437	Maxilla	One of a pair of irregularly shaped bones that form the upper jaw. A maxillary bone provides tooth sockets for the superior teeth, forms part of the ORBIT, and contains the MAXILLARY SINUS.	Maxillae,Maxillary Bone,Bone, Maxillary,Bones, Maxillary,Maxillary Bones,Maxillas
D009666	Nose	A part of the upper respiratory tract. It contains the organ of SMELL. The term includes the external nose, the nasal cavity, and the PARANASAL SINUSES.	External Nose,External Noses,Nose, External,Noses,Noses, External
D009958	Orofaciodigital Syndromes	Two syndromes of oral, facial, and digital malformations. Type I (Papillon-Leage and Psaume syndrome, Gorlin-Psaume syndrome) is inherited as an X-linked dominant trait and is found only in females and XXY males. Type II (Mohr syndrome) is inherited as an autosomal recessive trait.	Gorlin-Psaume Syndrome,Mohr Syndrome,Papillon-Leage and Psaume Syndrome,Dysplasia Linguofacialis,Oral-Facial-Digital Syndrome,Oral-Facial-Digital Syndrome, Type I,Oral-Facial-Digital Syndrome, Type II,Oro-Facio-Digital Syndrome,Orodigitofacial Dysostosis,Orodigitofacial Syndrome,Orofaciodigital Syndrome,Orofaciodigital Syndrome I,Orofaciodigital Syndrome II,Gorlin Psaume Syndrome,Oral Facial Digital Syndrome, Type I,Oral Facial Digital Syndrome, Type II,Orofaciodigital Syndrome IIs,Orofaciodigital Syndrome Is,Papillon Leage and Psaume Syndrome,Syndrome, Gorlin-Psaume,Syndrome, Mohr,Syndrome, Orofaciodigital,Syndromes, Orofaciodigital
D011859	Radiography	Examination of any part of the body for diagnostic purposes by means of X-RAYS or GAMMA RAYS, recording the image on a sensitized surface (such as photographic film).	Radiology, Diagnostic X-Ray,Roentgenography,X-Ray, Diagnostic,Diagnostic X-Ray,Diagnostic X-Ray Radiology,X-Ray Radiology, Diagnostic,Diagnostic X Ray,Diagnostic X Ray Radiology,Diagnostic X-Rays,Radiology, Diagnostic X Ray,X Ray Radiology, Diagnostic,X Ray, Diagnostic,X-Rays, Diagnostic
D002508	Cephalometry	The measurement of the dimensions of the HEAD.	Craniometry
D002574	Cervical Vertebrae	The first seven VERTEBRAE of the SPINAL COLUMN, which correspond to the VERTEBRAE of the NECK.	Cervical Spine,Cervical Spines,Spine, Cervical,Vertebrae, Cervical
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D003394	Craniofacial Dysostosis	Autosomal dominant CRANIOSYNOSTOSIS with shallow ORBITS; EXOPHTHALMOS; and maxillary hypoplasia.	Crouzon's Disease,Dysostosis, Craniofacial,Craniofacial Dysarthrosis,Craniofacial Dysostosis Syndrome,Craniofacial Dysostosis Type 1,Craniofacial Dysostosis, Type I,Crouzon Craniofacial Dysostosis,Crouzon Disease,Crouzon Syndrome,Craniofacial Dysarthroses,Craniofacial Dysostoses,Craniofacial Dysostosis Syndromes,Craniofacial Dysostosis, Crouzon,Crouzons Disease,Dysarthroses, Craniofacial,Dysarthrosis, Craniofacial,Dysostoses, Craniofacial