[Obstructive sleep apnea syndrome in the setting of Gorlin-Goltz syndrome]. / Obstruktives Schlafapnoe-Syndrom bei Gorlin-Goltz-Syndrom.

Goltz-Gorlin syndrome is a rare autosomal dominant hereditary disease associated with a high rate of spontaneous mutation. Diagnosis is based on clinically defined major and minor criteria. The disease is caused by a gene mutation locating to chromosome 9q22-31. We report on a young Goltz-Gorlin syndrome patient with obstructive sleep apnea syndrome. Due to intolerance to continuous positive airway pressure (CPAP) therapy and in order to avoid a tracheotomy, we opted for an alternative therapy comprising interdisciplinary multi-level surgery.

Developments in therapeutic human papillomavirus vaccination.

Much progress has been made in the prevention and therapy of premalignant and malignant dysplasia caused by human papillomavirus by encouraging screening programs and recently by introducing preventive vaccines. To further reduce the worldwide burden of HPV-associated cancer supplementation of the established therapies with immunotherapeutic methods would have the potential for significant impact. Dysplastic epithelial lesions and cancer of the anogenital and the oropharyngeal region show strong association with HPV. Therefore cervical carcinoma and HPV-associated squamous cell carcinoma of the head and neck differ from most other malignancies in that they harbour HPV-derived antigens. Expression of the viral oncogenes is mandatory to maintain the cancerous phenotype. These antigens are unique to the tumour and attractive targets for "proof of concept" studies in the development of therapeutic vaccines showing the general applicability of tumour vaccination and prove the correlation of immune response and clinical response. To date numerous clinical trials have been performed with candidate vaccines predominantly testing the efficacy for cervical cancer and its precursors. Although a naturally induced anti-HPV T cell response in patients was shown, clinical success of therapeutic vaccines was sparse. This may be attributed to immunosuppression, immunoselection, and immunoediting by the tumour cells. Factors of the individual that led to the failure of autonomous clearance of the initial infection may also contribute. Overriding this failure, reversing immunosuppression and application of vaccines in early stages of the disease is the key task for the future. The aim of this article is to summarize recent developments of therapeutic vaccines and discuss obstacles that hinder their success.

An analysis of quality of life in 130 families having small children with cleft lip/palate using the impact on family scale.

Most parents are emotionally traumatized when confronted by the birth of a baby with an orofacial cleft (OFC). Affected families may have to compensate for increased financial, social and personal impacts before primary treatment is completed. This study was conducted to identify factors influencing the quality of life (QoL) of families having young children with OFC. A self-administered questionnaire containing the impact on family scale was applied in 130 consecutive families having children with OFC aged between 6 and 24 months. The results were related to the type of cleft and the time of initial diagnosis using non-parametric tests and multivariate correlation analysis (P<0.05). In families having children with isolated cleft lip, financial and social impacts were reduced, but problems in coping were increased when compared to families with children having cleft lip and palate or isolated cleft palate. Total impact was highest in families having children with isolated cleft palate, probably due to later surgery for reconstruction. Prenatal diagnosis of OFC did not reduce the general impact on affected families, but increased the social impact. The relation of certain impacts to distinct types of cleft might allow more tailored support of affected families and improve their QoL.

Reconstruction of orbital wall defects with calcium phosphate cement: clinical and histological findings in a sheep model.

The aim of this pilot study was to investigate the potential of calcium phosphate cement in the treatment of orbital wall defect fractures in an adult sheep model, and to compare this alloplastic material to autologous calvaria split-bone grafts. Clinical, volumetric and histological examinations were carried out of both reconstruction materials. The use of cement made intraoperative corrections easier to perform, and increased the precision of reconstruction of the orbital volume. This material also proved to be osseoconductive. The two materials were used successfully in combination. Regions of most intensive remodelling were the anterior orbital floor and the adjacent orbital rim. The preliminary results of this study demonstrate the potential of calcium phosphate cement as a useful biomaterial in the reconstruction of the anterior orbital region. Further animal and clinical trials are necessary to investigate its ability as a carrier for mediators where bone healing requires influence or support.

Preoperative paclitaxel/carboplatin radiochemotherapy for stage III/IV resectable oral and oropharyngeal cancer: seven-year follow-up of a phase II trial.

Numerous treatment concepts for advanced but resectable oral and oropharyngeal squamous cell carcinoma exist. In this study, we present the 7-year results of a promising treatment with preoperative simultaneous chemoradiation using paclitaxel and carboplatin within a prospective phase II trial comprising 56 patients. After determination of the local tumor extension, chemoradiation was applied for 4 weeks and up to 40 Gy. Following a recovery period of 3-4 weeks, tumor resection was performed within the initially tattooed resection margins, together with a functional modified neck dissection. The median follow-up time was 44.9 +/- 19.6 months (range 0.76-87.9). After 7 years, 35 (63.3%) patients were alive and 20 (36.4%) had died. In 2 patients (3.6%), the cause of death was related to treatment. After 7 years, the overall survival rate declined to 63.6%, whereas the local recurrence-free probability was still 84.2%. These results confirm the excellent local control and high survival rates of preoperative radiochemotherapy with the combination of paclitaxel/carboplatin.

The numbers of leukocyte subsets in lung sections differ between intercellular adhesion molecule-1-/-, lymphocyte function-associated antigen-1-/- mice and intercellular adhesion molecule-1-/- mice after aerosol exposure to Haemophilus influenzae type-b.

In order to investigate the role of the adhesion molecules intercellular adhesion molecule-1 (ICAM-1) and lymphocyte function-associated antigen-1 (LFA-1) in pulmonary immunological processes, leukocyte populations were stained immunohistochemically on cryostat lung sections of ICAM-1-/- and LFA-1-/- mice. A further group of ICAM-1-/- mice was exposed to Haemophilus influenzae type-b (Hib) 24 h before being sacrificed. Comparison of the numbers of leukocytes in these groups revealed different behaviors of the leukocyte subsets: granulocytes were significantly increased in all three groups. Lymphocytes were increased in ICAM-1-/- mice, while there was no significant difference in LFA-1-/- and even a decrease in ICAM-1-/- mice after Hib exposure. Neither in ICAM-1-/- nor in LFA-1-/- mice did macrophages and dendritic cells (DCs) show significant differences to control animals. After Hib exposure, a significant elevation of DCs was observed. The following conclusions can be drawn: (1) all investigated leukocyte subsets can use ICAM-1- and LFA-1-independent pathways in the lungs of mice; (2) the pathways used by the leukocytes are cell-type specific; (3) ICAM-1 plays an important role in the enhanced recruitment of lymphocytes during Hib challenge in the lung; and (4) the alternative migratory mechanisms are able to compensate for the absence of ICAM-1 or LFA-1 or even lead to increased cell numbers. This overcompensation can be seen as a result of a balance between active alternative migratory mechanisms, which takes place in the absence of ICAM-1 or LFA-1.