Oral Surgery in Patients With Glanzmann Thrombasthenia: A Case Series.

Glanzmann thrombasthenia is a severe defect of platelet function caused by an inherited deficiency or dysfunction of the glycoprotein IIb/IIIa complex, the platelet fibrinogen receptor. Patients with Glanzmann thrombasthenia experience lifelong spontaneous and post-traumatic mucocutaneous bleeding diathesis. Surgery is usually very challenging, requiring close cooperation among surgeons, hematologists, and anesthesiologists. For anatomic reasons, oral surgery is particularly difficult owing to the inherent risk of hemorrhage and the difficulty in achieving local hemostasis. In the present report, we describe 3 successful cases of oral surgery in patients with Glanzmann thrombasthenia and report the surgical and hematologic management of each case.

Cockayne syndrome b maintains neural precursor function.

Neurodevelopmental defects are observed in the hereditary disorder Cockayne syndrome (CS). The gene most frequently mutated in CS, Cockayne Syndrome B (CSB), is required for the repair of bulky DNA adducts in transcribed genes during transcription-coupled nucleotide excision repair. CSB also plays a role in chromatin remodeling and mitochondrial function. The role of CSB in neural development is poorly understood. Here we report that the abundance of neural progenitors is normal in Csb(-/-) mice and the frequency of apoptotic cells in the neurogenic niche of the adult subependymal zone is similar in Csb(-/-) and wild type mice. Both embryonic and adult Csb(-/-) neural precursors exhibited defective self-renewal in the neurosphere assay. In Csb(-/-) neural precursors, self-renewal progressively decreased in serially passaged neurospheres. The data also indicate that Csb and the nucleotide excision repair protein Xpa preserve embryonic neural stem cell self-renewal after UV DNA damage. Although Csb(-/-) neural precursors do not exhibit altered neuronal lineage commitment after low-dose UV (1J/m(2)) in vitro, neurons differentiated in vitro from Csb(-/-) neural precursors that had been irradiated with 1J/m(2) UV exhibited defective neurite outgrowth. These findings identify a function for Csb in neural precursors.

Evolution of ultrasound guided axillary brachial plexus blockade: retrospective analysis of 662 blocks.

PURPOSE: Ultrasound (US) is being used increasingly to guide needle placement during axillary brachial plexus blockade (AXB). This retrospective study investigated whether US guidance can increase the success rate, decrease block onset time, and reduce local anesthetic (LA) volume for AXB compared to a traditional (TRAD) approach, namely, peripheral nerve stimulation (PNS) and transarterial (TA) techniques. METHODS: The anesthetic records, operative reports, discharge summaries, and surgical consultation notes of all patients who had undergone AXB for surgical anesthesia at the Toronto Western Hospital, between October 2003 and November 2006 were, retrospectively reviewed for evidence of block success and associated complications. Block success was defined as the achievement of surgical anesthesia without additional LA supplementation. RESULTS: Among the 662 patients, 535 patients underwent AXB using US guidance (US group), and 127 using TRAD techniques (TRAD group), namely, 56 using PNS (PNS subgroup) and 71 using the TA technique (TA subgroup). The block success rate was higher in the US group compared to the TRAD group (91.6% vs 81.9%, P = 0.003). The LA volume used for AXB was less in the US group compared to the TRAD group (39.8 +/- 6.4 mL vs 46.7 +/- 17.1 mL, P < 0.0001). Ultrasound group patients spent less time in the block procedure room than those in the TRAD group (30.6 +/- 14.2 min vs 40.1 +/- 27.3 min, P < 0.0001). When analyzed by subgroup, the US group demonstrated significantly greater success and shorter duration in the block room compared to the PNS subgroup, but not the TA subgroup. Complications (inadvertent intravenous LA injection, and transient neuropathy) were lower in the US group compared to the TRAD group (0.37% vs 3.15%, P = 0.014). CONCLUSIONS: Our results suggest that US-guided AXB may improve block success, reduce the local anesthetic volume used, and shorten the time spent in the block room compared to traditional nerve localization techniques.