Canterbury Hospital

About: Canterbury Hospital is a healthcare organization based out in Sydney, New South Wales, Australia. It is known for research contribution in the topics: Population & Randomized controlled trial. The organization has 830 authors who have published 882 publications receiving 22544 citations.

Autosomal recessive lissencephaly with cerebellar hypoplasia is associated with human RELN mutations

Abstract: Normal development of the cerebral cortex requires long-range migration of cortical neurons from proliferative regions deep in the brain. Lissencephaly ("smooth brain," from "lissos," meaning smooth, and "encephalos," meaning brain) is a severe developmental disorder in which neuronal migration is impaired, leading to a thickened cerebral cortex whose normally folded contour is simplified and smooth. Two identified lissencephaly genes do not account for all known cases, and additional lissencephaly syndromes have been described. An autosomal recessive form of lissencephaly (LCH) associated with severe abnormalities of the cerebellum, hippocampus and brainstem maps to chromosome 7q22, and is associated with two independent mutations in the human gene encoding reelin (RELN). The mutations disrupt splicing of RELN cDNA, resulting in low or undetectable amounts of reelin protein. LCH parallels the reeler mouse mutant (Reln(rl)), in which Reln mutations cause cerebellar hypoplasia, abnormal cerebral cortical neuronal migration and abnormal axonal connectivity. RELN encodes a large (388 kD) secreted protein that acts on migrating cortical neurons by binding to the very low density lipoprotein receptor (VLDLR), the apolipoprotein E receptor 2 (ApoER2; refs 9-11 ), alpha3beta1 integrin and protocadherins. Although reelin was previously thought to function exclusively in brain, some humans with RELN mutations show abnormal neuromuscular connectivity and congenital lymphoedema, suggesting previously unsuspected functions for reelin in and outside of the brain.

99Tcm sestamibi--a new agent for parathyroid imaging.

Abstract: Parathyroid imaging using 99Tcm sestamibi has been carried out prior to surgery in five patients with hyperparathyroidism and the results compared with a standard preoperative localization technique using 201Tl (thallous chloride). The 99Tcm sestamibi correctly localized all abnormal glands and showed higher parathyroid to thyroid uptake in three of four parathyroid adenomas. Both agents showed localization in a thyroid adenoma. The higher uptake of sestambi and better imaging properties of its 99Tcm radiolabel means that the agent may replace thallium for routine preoperative parathyroid localization.

A neurophysiological grading scale for carpal tunnel syndrome.

Abstract: Different ways of expressing the severity of carpal tunnel syndrome (CTS) are found in the existing literature and in clinical records. This paper documents the distribution of patients on a scale based upon the nerve conduction study findings, which are largely independent of the exact normal values used in any given laboratory and demonstrate a highly significant linear relationship between the neurophysiological grading and a numerical score derived from the clinical history. Patients with more characteristic stories of CTS generally have higher neurophysiological grades. The scale is as follows: normal (grade 0); very mild (grade 1), CTS demonstrable only with most sensitive tests; mild (grade 2), sensory nerve conduction velocity slow on finger/wrist measurement, normal terminal motor latency; moderate (grade 3), sensory potential preserved with motor slowing, distal motor latency to abductor pollicis brevis (APB) 6.5 ms; extremely severe (grade 6), sensory and motor potentials effectively unrecordable (surface motor potential from APB < 0.2 mV amplitude).

Parathyroid imaging with technetium-99m-sestamibi: preoperative localization and tissue uptake studies.

Abstract: The accepted radionuclide method for imaging abnormal parathyroid tissue has been the combined use of [99mTc]pertechnetate 201Tl-chloride. Various problems with this approach, however, have suggested the need for an improved parathyroid imaging agent. This study examined the use of 99mTc-sestamibi as a parathyroid imaging agent compared with 201Tl-chloride. Fifty-seven patients were scanned with both 99mTc-sestamibi and 201Tl preoperatively. There were 40 adenomas, of which 37 were localized with 201Tl and 39 with sestamibi. Fifteen patients had hyperplastic glands, of which 29 glands were localized with 201Tl and 32 with sestamibi. Possible differences in uptake of the two agents by thyroid and parathyroid tissue were examined by administering 10 MBq of each agent to patients undergoing surgical exploration and biopsy. Preoperatively 20 patients were studied (13 adenomas and 7 with hyperplasia). Thallium-201 uptake was higher in both the parathyroid and thyroid tissue than sestamibi. However, the uptake per gram of parathyroid tissue of sestamibi was higher than the uptake per gram of thyroid tissue. This was not true for 201Tl. Technetium-99m-sestamibi was at least as effective as 201Tl in parathyroid localization. This may be partly due to a higher target-to-background ratio, but also to the superior physical characteristics of 99mTc.