Focal dermal hypoplasia: A novel finding in disguise.

Focal Dermal Hypoplasia (FDH) or Goltz-Gorlin syndrome is an unusual X-linked dominant syndrome characterised by anomalies of both ectodermal and mesodermal structures. We present a case report on the management of a 58 year old Caucasian male with Focal Dermal Hypoplasia. This report describes an additional clinical manifestation of an intraosseous mandibular lipoma, which has not been previously described in cases of FDH. Intraosseous lipomas in the head and neck region are reported in only seventeen cases in isolation of any associated syndrome. Diagnosis was hindered due to similitude with Nevoid Basal Cell Carcinoma Syndrome (Gorlin-Goltz Syndrome) which despite similar nomenclature, is an exclusively separate condition This novel finding encourages clinicians to consider unusual differential diagnoses in such cases and highlights the importance of avoiding eponyms to prevent confusion with similar conditions.

Craniometaphyseal Dysplasia: A review and novel oral manifestation.

Craniometaphyseal Dysplasia (CMD) is a sclerosing osseous dysplasia characterised by hyperostosis of craniofacial and long bones, resulting in distortion and cranial nerve palsies. We present a case report on the management of a 63 year old female with Craniometaphyseal Dysplasia. This report describes an additional clinical manifestation of hypercementosis, which although well recognised in other sclerosing osseous dysplasias, is not reported in the literature for Craniometaphyseal Dysplasia. We discuss established in vivo studies in mice which link the genetic mutations found in Craniometaphyseal Dysplasia to hypercementosis, and how this report describes the same manifestation in humans. This novel finding can aid the clinician in the management of patients with Craniometaphyseal Dysplasia, and complications that can arise in dentoalveolar surgery.

Novel oral anticoagulants and exodontia: the evidence.

Background Haemostasis is crucial for the success of oral surgical treatment as bleeding problems can cause complications both pre- and post-operatively. Patients on anticoagulant drugs present a challenge due to their increased risk of bleeding.Aims To review the evidence for the management of oral surgery patients on novel oral anticoagulant therapy.Methods A literature review was conducted in May 2016 of free-text and MESH searches (keywords: apixaban, dabigatran, rivaroxaban and dental extractions) in the Cochrane Library, PubMed and CINAHL. Trial registers, professional bodies for guidelines and OpenGrey for unpublished literature were also searched. Studies were selected for appraisal after limits were applied (adult, human and English only studies) and inclusion/exclusion criteria imposed.Results Five studies were identified for critical appraisal using the CASP tools. These were a combination of systematic reviews and case series. Two case series were excluded due to low quality evidence. Curtin et al., Davis et al. and Constantinides et al. together with guidelines from the Scottish Dental Clinical Effectiveness Programme, have highlighted a protocol in managing these patients in a dental surgical setting.Conclusion Patients on novel anticoagulant therapy requiring dental surgery can be managed appropriately either without discontinuation of therapy or a delay in dose. For those patients at higher risks of postoperative bleeding complications, it is advised to liaise with the specialist physician.

Exodontia in dual antiplatelet therapy: the evidence.

BACKGROUND: Haemostasis is crucial for the success of oral surgical treatment as bleeding problems can cause complications both pre- and post-operatively. Patients on antiplatelet drugs present a challenge due to their increased risk of bleeding. AIMS: To identify a protocol for the management of oral surgery patients on dual antiplatelet therapy (aspirin and clopidogrel). METHODS: A literature review was conducted in January 2016 of free-text and MESH searches (keywords: aspirin, clopidogrel and dental extractions) in the Cochrane Library, PubMed and CINAHL. Trial registers, professional bodies for guidelines and OpenGrey for unpublished literature were also searched. Studies were selected for appraisal after limits were applied (adult, human and English only studies) and inclusion/exclusion criteria imposed. RESULTS: Eight studies were identified for critical appraisal using the CASP tools. These were a combination of retrospective, prospective, cohort and case control studies. Napenas et al. and Park et al. found no statistically significant risk of postoperative bleeding complications in patients on dual antiplatelet therapy. Girotra et al., Lillis et al., Omar et al. and Olmos-Carrasco et al., however, found statistically significant risk of postoperative bleeding in this group of patients, all of which can be controlled with local measures. CONCLUSION: Patients on dual antiplatelet therapy - although at an increased risk of postoperative bleeding complications - can be managed safely with local haemostatic measures and without the need to discontinue antiplatelet therapy.

Successful attenuation of humoral immunity to viral capsid and transgenic protein following AAV-mediated gene transfer with a non-depleting CD4 antibody and cyclosporine.

The ability of transient immunosuppression with a combination of a non-depleting anti-CD4 (NDCD4) antibody and cyclosporine (CyA) to abrogate immune reactivity to both adeno-associated viral vector (AAV) and its transgene product was evaluated. This combination of immunosuppressants resulted in a 20-fold reduction in the resulting anti-AAV8 antibody titres, to levels in naïve mice, following intravenous administration of 2 × 10(12) AAV8 vector particles per kg to immunocompetent mice. This allowed efficient transduction upon secondary challenge with vector pseudotyped with the same capsid. Persistent tolerance did not result, however, as an anti-AAV8 antibody response was elicited upon rechallenge with AAV8 without immunosuppression. The route of vector administration, vector dose, AAV serotype or the concomitant administration of adenoviral vector appeared to have little impact on the ability of the NDCD4 antibody and CyA combination to moderate the primary humoral response to AAV capsid proteins. The combination of NDCD4 and CyA also abrogated the humoral response to the transgene product, that otherwise invariably would occur, following intramuscular injection of AAV5, leading to stable transgene expression. These observations could significantly improve the prospects of using rAAV vectors for chronic disorders by allowing for repeated vector administration and avoiding the development of antibodies to the transgene product.