Roentgenographic Findings in Familial Osteodysplasia

Buchignani, John S.; Cook, Albert J.; Anderson, Linda

doi:10.2214/ajr.116.3.602

Cited by 10 publications

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“…The ribs were gracile and the superior pubic rami of the pelvis were slender. 2 Upon investigation of the patient's family, similar facial characteristics were found in three of the woman's four living siblings (2 brothers and 1 sister affected, 1 sister normal). This sibship was the product of a consanguinous marriage (first cousins once removed) in which neither the parents nor other members of this kindred demonstrated the same stigmata.…”

mentioning

confidence: 79%

“…Physical and laboratory examinations were done on all of them, and the positive results are summarized in Table 1 along with the findings from the original family reported by Anderson et al' and Buchignani et a1. 2 A cephalometric analysis using the architectural and structural analysis was done for each member. The major similarity found in all members was a greatly reduced anterior face height secondary to a n absence of alveolar bone and a high palatal plane in relation to the atlantooccipital articulation.…”

Section: Clinical Observations Of the Familymentioning

confidence: 99%

“…The middle phalanges of the second, third, and fourth digits were relatively shorter than normal. 2 Anderson et al stated that the most distinctive abnormality of this syndrome involved the facial bones.' They also stated that the hyperuricemia and hypertension could be "familial" and unrelated to the bony dysplasia since they existed in the father, who they stated had no obvious facial malformation.…”

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confidence: 99%

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Familial osteodysplasia

Schendel

Delaire

1982

Head Neck

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A family with characteristics that have striking similarities to the syndrome of familial osteodysplasia is reported. The inheritance pattern appears to be autosomal dominant. The typical facies has a lack of vertical facial development and a functional prognathism caused by overclosure of the mandible and opening of the gonial angle. Midfacial hypoplasia and a pointed chin are characteristic. Alveolar and sutural bone growth in the face is diminished and associated with partial dental agenesis. Variable cranial digital markings and possible synostosis are also seen. These typical facial changes are associated with other bony and systemic abnormalities. The long bones have a flask shape and occasionally have increased cortical thickness. The superior pubic ramus is often thin and scoliosis is common. The dermatoglyphic pattern demostrates a distal triradius and small whorl abnormalities. The most significant laboratory values are an increased erythrocyte sedimentation rate and C3 complement found on the erythrocytes. The plasma fibrinogen level was low in all family members.

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confidence: 79%

Section: Clinical Observations Of the Familymentioning

confidence: 99%

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confidence: 99%

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Familial osteodysplasia

Schendel

Delaire

1982

Head Neck

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“…The craniofacial defects consist of midfacial hypoplasia, flat nasal bridge, pointed chin, depressed zygomatic bones, hypoplasia of the petrous bone, calvarial thinning, prominent eyebrows, large ear bones, mandibular prognathism, mandible with wide angle, micromaxilla, recurrent mandibular fractures and malocclusion. Diastolic hypertension and hyperuricaemia also appear as common clinical findings (Anderson et al , 1972; Buchignani et al , 1972).…”

Section: Introductionmentioning

confidence: 99%

“…The syndrome has an apparent autosomal recessive pattern of inheritance (Anderson et al , 1972; Buchignani et al , 1972). It was defined in detail with genetic, radiologic and physical properties, but no recent cases have been reported since 1972 when the syndrome was first described.…”

Section: Introductionmentioning

confidence: 99%

Dental findings and rehabilitation in familial osteodysplasia (Anderson type): a case report

Değer¹,

Tabar²,

Şermet³

et al. 2006

Oral Diseases

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Familial osteodysplasia is a disorder of osteogenesis with an autosomal recessive pattern of inheritance which predominantly affects facial bones. No recent case had been reported, particularly from a dental point of view since the syndrome was first described by Anderson et al (JAMA 1972;220:1687-93). A 23-year-old male with familial osteodysplasia was presented in maxillofacial and dental aspects with clinical and radiological manifestations including malocclusion, abnormal teeth alignment, impacted teeth, shape disturbances including uncompleted coronal formation, root shortening with bulbous form, high angled mandible and elongation of the corpus of mandible. Recognition of the syndromal features prior to any dental intervention is of paramount importance because of increased inclination to spontaneous mandibular fractures. Hence, no surgical intervention was performed for impacted teeth. Following the extractions of severely mobile teeth, a definitive restoration was fabricated as distal-extension removable partial dentures with conus crown telescopic system. The aesthetic and functional outcome was satisfactory for the patient. In conclusion, dentists appear to play an important role in the recognition of familial osteodysplasia, based on maxillofacial and dentoalveolar findings. Awareness of the syndromal features, especially of spontaneous fractures, would detect the limitations for dental interventions and treatment planning.

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Osteodysplasty (Melnick-Needles Syndrome)

Beighton

Cremin

1980

Sclerosing Bone Dysplasias

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Roentgenographic Findings in Familial Osteodysplasia

Cited by 10 publications

References 0 publications

Familial osteodysplasia

Familial osteodysplasia

Dental findings and rehabilitation in familial osteodysplasia (Anderson type): a case report

Osteodysplasty (Melnick-Needles Syndrome)

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