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The clinical picture of cherubism is similar to that of fibrous dysplasia. The initial clinical appearance involves the deformation of the maxillo-facial area with orthodontic disorder. Usually it is found in the mandible, giving the child a chubby-faced appearance, and it often occurs together with symmetric submandibular lymph node enlargement. This appearance reminds one of the cherubs seen in art. Only histological evidence for cherubism is inconclusive. The presence of multi-nucleated giant cells resembles fibrous dysplasia. A combination of clinical, radiographical, and histological findings eventually leads to the correct diagnosis.An example is given of a patient displaying the typical disease process. Over a period of 12 years, we observed the progression of the disease from its initial appearance in a young child, through the full and characteristic display of a cherubic youth, and finally its regression.In conclusion, we advise restraint in planning surgical intervention. The disease's etiology is not entirely clear. The latest research points to genetic defects that lead to failure in the expression of matrix proteins.  相似文献   

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Background

The aim of this retrospective study was to evaluate prognostic factors for the development of secondary local lymph node metastases in patients with oral squamous cell carcinoma who had undergone selective neck dissection for primary node-negative disease.

Patients and Methods

The study included 331 patients with primary squamous cell carcinoma of the oral cavity who underwent 431 selective neck dissections between January 1986 and December 2002 in Germany at the Hannover Medical School’s Department of Oral and Maxillofacial Surgery. Several potential prognostic factors were evaluated for their influence on the development of secondary metastases following primary neck dissection.

Results

No statistically significant relationship to the appearance of secondary local metastasis following selective neck dissection was detected concerning: patient age or sex, histopathologic tumor stage, primary tumor grade, or adjuvant therapies such as pre- or postoperative radiotherapy and radiochemotherapy. The only study factor with a statistically significant influence was the extent of lymphadenectomy, in which particularly the region of the carotid bifurcation played a decisive role.

Conclusion

Significantly fewer secondary metastases occurred following neck dissections that included the carotid trigone. In light of these results, we recommend that neck dissection for primary oral squamous cell cancer always include the region of the carotid bifurcation, regardless of the above mentioned associated patient and tumor factors.  相似文献   

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Galvan O  Sprinzl GM  Widner B  Hackl JM  Gunkel AR  Thumfart WF 《HNO》2000,48(12):928-936
Background and objective. Patients with advanced head and neck cancer often suffer from malnutrition even before the start of therapy. Hence, the demand for nutritional support increases particularly before and during radiochemotherapy. Though nutritional therapy has been shown to substantially improve individual outcome, neither the criteria for patient candidacy nor the indications for therapeutic intervention have been established. We performed a retrospective analysis to determine the indications for nutritional support and to evaluate the benefits of measures actually taken against malnutrition before and during radiochemotherapy as well as perioperatively. Patients/methods. Data taken from a prospective study for the evaluation of oral mucositis during radiochemotherapy was analysed retrospectively. To calculate the indication for nutritional support, a nutritional scoring system (Hackl) was employed for the first time, which contained biochemical and anthropometric parameters as well as the period of starvation. The results were then compared to a nutritional support program implemented by the subjective examinations of the attending physician. Results. Changes in body weight and body mass index (BMI) remained the most impressive parameters. Catabolic metabolism developed preoperatively and a significant loss of whole-body protein followed surgical therapy. Clinically, the results of the nutritional score correlated with the observation of malnutrition. Furthermore, our findings suggest that nutritional therapy was commonly delayed until late in the clinical course. Conclusion. The results indicate the necessity of objective and reproducible diagnosis and control of malnutrition. The scoring system used may provide a useful and yet simple tool for assessing individual indications for timely nutritional support.  相似文献   

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Background

In the presence of a (central) auditory processing disorder [(C)APD] central hearing processes are disturbed, leading to impaired speech perception in noise. Poor acoustics in educational institutions exacerbates the problem. In children affected by (C)APD this can result in impaired development of language, reading and writing acquisition as well as other skills.

Materials and methods

A total of 66 children aged 6–11 years completed the Oldenburg sentence test in noise (OlSa) with and without an FM system. Children with normal OlSa test results formed the control group (group 1), while group 2a comprised children with an unremarkable re-test result and group 2b comprised children who produced abnormal results even in the re-test.

Results

All children achieved improved speech intelligibility using the FM system. The greatest difference between results with and without FM system was seen in group 2b (9.53 dB S/N). Group 1 achieved an improvement of 8.86 dB S/N and group 2a 7.89 dB S/N.

Conclusions

Children with detected auditory selection problems benefit most from the use of an FM system for improved speech understanding. This should be borne in mind in the diagnosis and therapy of these children.  相似文献   

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Zusammenfassung Es wird über den Fall eines 8jährigen scharlachkranken Mädchens berichtet, bei dem es metastatisch — vermutlich ausgehend von den Tonsillen — zur Entstehung einer primären Osteomyelitis der Pars petrosa des Schläfebeins mit konsekutiver Meningitis und Otitis kam. Eingehende Erörterung der Pathogenese des Falles an der Hand des klinischen, pathologisch-anatomischen und histologischen Befundes. Da die Otitis eine Frühotitis war und histologische Befunde solcher selten sind, wurde diesem besondere Beachtung gewidmet.Mit 4 Textabbildungen.Über den Fall wurde kurz in der Sitzung der österr. otol. Ges., Dezember 1937, berichtet.  相似文献   

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Zusammenfassung Ein subperiostaler Luftbruch am Hinterhaupt ist auf dem Boden einer umfangreichen Pneumatisation entstanden, welche sich vom Warzenfortsatz auf Scheitelbein und Hinterhauptsbein einer Seite ausgedehnt hat. Die Ursache dieser einseitigen Luftraumbildung ist durch die Vorstellungen der herrschenden Pneumatisationslehren kaum zu erklären. Einzig die allerdings rein hypothetische Annahme eines durch anatomische Besonderheit der Luftkanalbildung bedingten Ventilverschlusses würde für unseren Fall die vonKrainz allgemein für die Pneumatisation vertretene Annahme stützen, daß die Binnendruckwirkung der Luft die treibende Kraft für die Zellbildung darstellen kann.Mit 3 Textabbildungen.  相似文献   

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Härter M  Maurischat C  Weske G  Laszig R  Berger M 《HNO》2004,52(2):125-131
OBJECTIVES: The majority of patients with chronic decompensated forms of tinnitus have comorbid psychological disorders, possibly accompanied by restricted quality of life and participation in everyday activities. There is no standardized tool to measure these clinically relevant dimensions. SAMPLE AND METHOD: Both the Hospital Anxiety and Depression Scale (HADS) and the Short Form 36 (SF-36) were administered to a consecutive sample of patients ( n=110) who were recruited from the tinnitus outpatient service in the department of psychiatry and psychotherapy. Using the Tinnitus Questionnaire (TF), associations of the HADS and SF-36 scales with tinnitus grading were determined and the classification of patients (mild-medium-severe-very severe) was evaluated. RESULTS: A severe tinnitus grading is correlated to a high extent of anxiety and depression and a low quality of life. With the exception of the SF-36 subscale on physical functioning, all scales discriminated between the extreme groups (mild vs severe/very severe), but less between the two medium groups. CONCLUSIONS: Patients with severe decompensated tinnitus (grades III and IV) should be seen for diagnosis of psychiatric comorbidity and supportive psychotherapy/psychopharmacotherapy used when necessary.  相似文献   

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The reliability of skin prick tests (SPT) may be insufficient for the screening of occupational inhalant allergies. The influence of different flour extracts on the SPT in flour allergic subjects has not been compared previously. In this study, SPT reactions against two commercially available rye and wheat flour extracts and individually prepared extracts from flour samples were compared in 35 patients with known bakers' rhinitis. Flour sensitization was confirmed by a positive nasal provocation test (NPT) and/or serum-specific IgE. The sensitivity of NPT with a combination of rye and wheat flour extracts of individual flour samples was 94%. Wheat and/or rye flour specific IgE (RAST>/=2) was true positive in 86%. The sensitivity of the SPT was 94% for individual rye flour extracts compared to 38% and 59% for two commercially available rye flour extracts and 88% for individual wheat flour extract compared to 53% and 48% for commercially available wheat flour extracts. SPT and sIgE did not reveal a significant difference in prevalence between rye and wheat flour sensitization. Thirty healthy volunteers served as the control group. Three control subjects with histamine equivalent SPT reactions to grass pollen had a positive SPT reaction against individual flour extracts, whereas NPT with undiluted individual flour extracts was negative in all controls. SPT with individually prepared flour extracts appears to be sensitive for the demonstration of inhalant flour allergy. Our findings show that extracts of individual flour samples rather than commercially available extracts should be used for both SPT and NPT if flour allergy is suspected.  相似文献   

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BACKGROUND: ML-1 standardized mistletoe extracts have been recommended for increasing the health-related quality of life in cancer patients. PATIENTS AND METHODS: The EORTC questionnaire QLQ-C30((V2)) was given to a randomly chosen subgroup of 399 patients of a prospective, randomized, open, multi-center trial. A total of 200 patients from this trial were randomized for ML-1 treatment (1 ng/kg body weight ML-1 was injected subcutaneously twice weekly over a 60-week period. Treatment cycles of 12 weeks were followed by a break of 4 weeks (between weeks 12-16, 28-32, and 44-48)). The remaining 199 patients formed the control group. RESULTS: Patients completed questionnaires before the start of their treatments at week 0 and continued until week 156. The compliance rate was high: 3611 questionnaires were available, which equals a median of nine longitudinal measurements per patient between weeks 0 and 156. Analysis did not indicate any improvement in the quality of life for either group. A significant decrease in quality of life, however, was seen in patients undergoing radiotherapy. In these patients, the global state of health was reduced and four symptom scales were significantly worse. CONCLUSION: Our results demonstrated no improvement in the quality of life in head and neck cancer patients when treated with ML-1 extract.  相似文献   

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