ASPP2 maintains the integrity of mechanically stressed pseudostratified epithelia during morphogenesis.

During development, pseudostratified epithelia undergo large scale morphogenetic events associated with increased mechanical stress. Using a variety of genetic and imaging approaches, we uncover that in the mouse E6.5 epiblast, where apical tension is highest, ASPP2 safeguards tissue integrity. It achieves this by preventing the most apical daughter cells from delaminating apically following division events. In this context, ASPP2 maintains the integrity and organisation of the filamentous actin cytoskeleton at apical junctions. ASPP2 is also essential during gastrulation in the primitive streak, in somites and in the head fold region, suggesting that it is required across a wide range of pseudostratified epithelia during morphogenetic events that are accompanied by intense tissue remodelling. Finally, our study also suggests that the interaction between ASPP2 and PP1 is essential to the tumour suppressor function of ASPP2, which may be particularly relevant in the context of tissues that are subject to increased mechanical stress.

Treatment of Kimura disease with photodynamic therapy: a case study.

We report on the application of photodynamic therapy (PDT) in the management of Kimura disease. A 58-year-old Asian male was offered this modality to assess the possibility to control disease progression. The patient was managed with surgery and the disease recurred and caused facial disfigurement. PDT was offered after careful discussion at UCLH multidiscipline meeting. The photosensitiser "mTHPC" was introduced intravenously 96h prior to delivering the light under ultrasound guidance. Magnetic resonance images showed moderate-significant reduction of the disease volume. Fourteen months post-PDT, the disease started re-growing and the patient subsequently underwent one further round of PDT which was as successful as the first round in reducing the facial disfigurement. Photodynamic therapy was very effective in controlling disease progression in this patient who suffers from Kimura disease.

TMJ arthroscopy in patients with Ehlers Danlos syndrome: case series.

OBJECTIVE: The purpose of this report was to assess the outcome of temporomandibular joint (TMJ) arthroscopy in patients with temporomandibular disorders (TMDs) associated with Ehlers Danlos syndrome (EDS). STUDY DESIGN: This retrospective case series describes 18 patients with EDS who underwent arthroscopy for temporomandibular disorders. The patients' demographics were recorded, along with preoperative TMJ symptoms, Wilkes classification, mouth opening, and the presence of systemic involvement. The incidence of early and late postoperative complications and the final outcome were noted. RESULTS: All of the patients were females, with EDS Type III, and had a mean age of 34 years. A high proportion of the patients had joints other than the TMJ affected. Five patients were classified as stage II according to the Wilkes classification, 9 patients were stage III, 3 patients were stage IV, and only 1 patient was diagnosed with stage V Wilkes before intervention. Arthroscopy, followed by arthrocentesis and balloon dilatation of the affected TMJs was performed and intra-articular morphine injections were given to all patients. The main pre- and early postoperative complaint was pain, but this resolved in most cases. Improvement of mouth opening was noted from 23.4 +/- 4.2 to 27.8 +/- 5.1 mm after arthroscopy. Patients were followed for an average of 62 months and all were asymptomatic at their last review appointment. CONCLUSION: For patients where conservative measures of treating TMD are not effective, arthroscopy is a minimally invasive surgical procedure that has been shown to result in a satisfactory outcome, with no need to resort to open joint surgery. This case series is limited by its size and further research on surgical intervention on EDS patients with temporomandibular disorders is recommended.

Management of deep-seated malformations with photodynamic therapy: a new guiding imaging modality.

Photodynamic therapy is an emerging technology and results from the interactions between a photosensitiser, oxygen and light. The delivery of light may either be by surface illumination or by interstitial application. We describe the first clinical application of ultrasound-guided interstitial photodynamic therapy (US-iPDT). A total of 23 treatments with meta-tetra-hydroxyphenyl chlorine (mTHPC) and ultrasound-guided interstitial photodynamic therapy were performed on 21 patients with various conditions at the Head & Neck Centre, University College London Hospital. The needles could be clearly identified during insertion in all 23 treatments, and it was possible to guide parallel needle insertions using ultrasound. Although the resolution of ultrasound is not as high as that of other imaging modalities [i.e. computed tomography (CT) and magnetic resonance imaging (MRI)] it was satisfactory in identifying the centre and the peripheries of the pathological lesions. Ultrasound is very easy to perform, non-invasive, relatively inexpensive, quick, convenient, non-ionising, suited to the imaging of soft tissues and does not cause any discomfort. Ultrasound can be used to guide 'real-time' photodynamic therapy in deep-seated tumours and other malformations and can augment the information from other imaging modalities without affecting the patient's treatment outcome.