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Unusual jaw lesions in the paediatric and adolescent patient: a management challenge.

While major maxillofacial pathology in the young patient is relatively uncommon, non-malignant conditions may pose a significant treatment dilemma due to their aggressive or unpredictable behaviour. Several such diseases managed by the Oral and Maxillofacial Surgery Unit at the Royal Children's Hospital of Melbourne have been selected for review. Illustrative case reports to highlight the principles of management are presented.

Adolescent↗

[Pycnodysostosis--common ancestor of some Danish patients. Examination and diagnosis based on molecular genetics].

Eight patients with pycnodysostosis from six Danish families were examined for mutations in the cathepsin K gene. Three different mutations are the cause of pycnodysostosis in the six families--five of whom come from Ringkøbing County and one from Vejle County. One mutation has a high frequency in the families from Ringkoebing County. The five families are related through a common ancestor, who introduced the mutation around the year 1100. The disease is described with respect to aetiology, symptoms, prognosis, diagnosis, and symptomatic treatment. Research in pycnodysostosis may bring important knowledge to the understanding of related diseases, such as osteoporosis.

Cathepsins↗

[Involvement of the facial skull in Erdheim-Chester disease].

We report on a patient suffering from Erdheim-Chester-disease (ECD). ECD represents a very rare entity with lipogranulomatosis of mesenchymal origin. The most common radiological manifestation is the involvement of the long bones of the extremities. Here we find sclerosis of the spongiosa combined with a thinning of cortical structures. This often results in a small crack of hyperlucency between corticals and spongiosa. Our case demonstrates an involvement of the craniofacial part of the skull showing sclerosis of the upper jaw bone. This manifestation has not yet been reported in the literature.

Adult↗

Traumatic bone cyst: report of two cases and review of the literature.

Traumatic bone cysts usually are found in young individuals and most frequently have the radiographic appearance of well-circumscribed radiolucent lesions with a tendency to scallop between the roots of the teeth. Two well-documented cases of traumatic bone cysts are presented in which some differences related to their radiographic features were observed. The possible explanations for these differences are discussed and a brief review of the literature regarding the main characteristics of traumatic bone cyst lesions also is provided.

Adolescent↗

[Proceedings: The clinical effects of human growth hormone. Report on an international symposium of the German Kabi Corp. on February 15, 1975 in Munich].

Report of an International Symposion (Febr. 15, 1975, Munich). Human growth hormone (HGH) acts on proliferation of cartilage, the basal process of growth, by the way of somatomedine and changes in protein metabolism. Growth can be enhanced in hypopituitary dwardism and with higher dosage in other forms of failure to thrive. HGH also seems to have a beneficial effect in patients with muscular dystrophy as shown in a preliminary report but not in thrombocytopenia. First observations on patients with osteogenesis imperfecta and on patients with non healing fractures of the long bones (pseudarthroses) have shown significant improvement. Of importance are the reports on stopping severe gastric hemorrhage in patients with stress ulcera.

Bone Development↗

[Clinical aspects of osteomyelofibrosis and osteomyelosclerosis].

The clinical and radiological parameters, the laboratory findings as well as the results of the nuclear-medical examination established in 78 patients with an osteomyelofibrosis or osteomyelosclerosis are compared with the data existing in literature and the problems of diagnostics and therapy of these diseases are derived from this.

Adult↗

Garre's osteomyelitis: a case report.

Garre's Osteomyelitis is a distinctive type of chronic osteomyelitis associated with gross thickening of the periosteum of the bones and peripheral reactive bone formation resulting from mild irritation or infections. The condition is seen exclusively in children or young adults. Mandible is more often affected than the maxilla. Clinically it manifests as bony hard, non-tender swelling which is slowly progressive and associated with painful carious tooth. In this article, a case report of a eight year old female is presented along with complete investigations and treatment.

Child↗

Bone lesions in malignant diseases--I. Current concepts of major pathogenetic mechanisms and forms.

Bones are among the major parts of the body that are targeted in neoplastic diseases. Bone lesions increasing in number and size and diffuse osteoporosis are found in 30-80% of cancer patients. All literature data about the pathogenetic factors of osteolytic and osteosclerotic bone lesions are classified into four groups: 1. Directly connected with the neoplastic tumour mass. 2. Humoral interaction between the tumour cells and bone cells. 3. Systemic effects and complications of the neoplastic process. 4. Extracancerous factors. We discuss here the basic pathogenetic forms of bone lesions in neoplasias: 1. Local osteolysis in the area of neoplastic infiltration. 2. Humorally activated demineralisation with hypercalcemia. 3. Light chain osteomalacia. 4. Hypophosphatemic osteomalacia. We consider the major current biochemical markers of bone remodelling and their use in diagnosing and monitoring bone disease in neoplastic conditions.

Biomarkers↗

A 5-year follow-up of occlusal status and radiographic findings in mandibular condyles of the elderly.

PURPOSE: The aim of the present 5-year follow-up was to clarify the nature of occlusal support status and radiographic changes in condyles of the elderly, and the association between these two variables. MATERIALS AND METHODS: The present study is part of a comprehensive medical survey of a random sample born in 1904, 1909, and 1914. A total of 364 subjects living in Helsinki participated in the dental part of the examination during 1990 to 1991, and after 5 years a total of 103 were reexamined. Comprehensive data on occlusal support status were available for 94 subjects, and radiographic data were available for 88 subjects. Occlusal support status was assessed on the basis of the Eichner index, radiographic changes were assessed from panoramic radiographs, and symptoms of temporomandibular disorders were assessed using Helkimo's anamnestic index. RESULTS: The most frequent radiographic finding in the mandibular joint was flattening of the articular surface of the condyle associated with osteoarthrosis, found at baseline in 17% and during follow-up in 13% of the subjects. During the 5-year follow-up, Eichner index for natural dentition remained unaltered in 94% of the subjects and in 85% of the subjects when removable dentures were included. There were no radiographic changes in 92% of the cases. No differences based on age or gender were found. A logistic regression model revealed associations between the selected baseline factors. The odds ratio for baseline Helkimo's anamnestic index was 4.1, 5.7 for Eichner index with the support of removable dentures, and 356 for radiographic findings. CONCLUSION: Radiographic changes in condyles of elderly people were small during the 5-year follow-up, but baseline radiographic findings, Helkimo's anamnestic index, and Eichner index with removable dentures were risk factors for radiographic findings at the end of the follow-up.

Age Factors↗

Recent progress in the molecular genetics of sclerosing bone dysplasias.

Bone mineral density is a quantitative trait influenced by both genetic and environmental factors. Epidemiological and twin studies have shown heritability for the variance of bone density of up to 80%. The role of genetic factors in bone homeostasis also is clearly illustrated by the existence of an extended group of genetic conditions associated with an abnormal bone density. These conditions are mainly monogenic in that they are caused by mutations in one gene. Recent developments in molecular genetics and genomics have resulted in a dramatically increased power to identify such disease-causing genes. The group of conditions with increased bone density has been poorly studied and understood at the molecular genetic level but recently for some of them, major breakthroughs have been made. These findings will make the molecular analysis of such patients an additional tool in diagnostics and in genetic counseling.

Camurati-Engelmann Syndrome↗