Intraoperative PTH Assay during Minimally Invasive Parathyroidectomy May Be Helpful in the Detection of Double Adenomas and May Minimise the Risk of Recurrent Surgery

Background. Minimally invasive parathyroidectomy (MIP) is increasingly replacing the traditional bilateral neck exploration in the treatment of primary hyperparathyroidism (PHP). Intraoperative PTH (IOPTH) measurement has recently been introduced as a useful adjunct in confirming successful excision of abnormal parathyroid gland. Aims. We evaluate the safety, efficacy, and clinical usefulness of IOPTH measurement during MIP in a district general hospital. Methods. Retrospective review of eleven consecutive patients with PHP who underwent MIP with IOPTH, following preoperative assessment with ultrasound and sestamibi scans. Results. All patients had successful removal of the abnormal parathyroid gland. The concordance rate between ultrasound and sestamibi scan in localising the parathyroid adenoma was 82%. IOPTH measurement confirmed the removal of adenoma in all cases and, in one case, led to identification of a second adenoma, not localised preoperatively. The median hospital stay was 2 days (range 1–7 days). All patients remained normocalcaemic after a median of 6 months (range 1–10 months). Conclusions. Minimally invasive parathyroidectomy is a feasible, safe, and effective method for treatment of PHP. The use of IOPTH monitoring potentially offers increased sensitivity in detecting multiglandular disease, can minimise the need and risk associated with recurrent operations, and may facilitate cost-effective minimally invasive surgery

Cholesterol Metabolism Is Required for Intracellular Hedgehog Signal Transduction In Vivo

We describe the rudolph mouse, a mutant with striking defects in both central nervous system and skeletal development. Rudolph is an allele of the cholesterol biosynthetic enzyme, hydroxysteroid (17-beta) dehydrogenase 7, which is an intriguing finding given the recent implication of oxysterols in mediating intracellular Hedgehog (Hh) signaling. We see an abnormal sterol profile and decreased Hh target gene induction in the rudolph mutant, both in vivo and in vitro. Reduced Hh signaling has been proposed to contribute to the phenotypes of congenital diseases of cholesterol metabolism. Recent in vitro and pharmacological data also indicate a requirement for intracellular cholesterol synthesis for proper regulation of Hh activity via Smoothened. The data presented here are the first in vivo genetic evidence supporting both of these hypotheses, revealing a role for embryonic cholesterol metabolism in both CNS development and normal Hh signaling

The use of preoperative radiotherapy in the management of patients with clinically resectable rectal cancer: a practice guideline

BACKGROUND: This systematic review with meta-analysis was designed to evaluate the literature and to develop recommendations regarding the use of preoperative radiotherapy in the management of patients with resectable rectal cancer. METHODS: The MEDLINE, CANCERLIT and Cochrane Library databases, and abstracts published in the annual proceedings of the American Society of Clinical Oncology and the American Society for Therapeutic Radiology and Oncology were systematically searched for evidence. Relevant reports were reviewed by four members of the Gastrointestinal Cancer Disease Site Group and the references from these reports were searched for additional trials. External review by Ontario practitioners was obtained through a mailed survey. Final approval of the practice guideline report was obtained from the Practice Guidelines Coordinating Committee. RESULTS: Two meta-analyses of preoperative radiotherapy versus surgery alone, nineteen trials that compared preoperative radiotherapy plus surgery to surgery alone, and five trials that compared preoperative radiotherapy to alternative treatments were obtained. Randomized trials demonstrate that preoperative radiotherapy followed by surgery is significantly more effective than surgery alone in preventing local recurrence in patients with resectable rectal cancer and it may also improve survival. A single trial, using surgery with total mesorectal excision, has shown similar benefits in local recurrence. CONCLUSION: For adult patients with clinically resectable rectal cancer we conclude that: • Preoperative radiotherapy is an acceptable alternative to the previous practice of postoperative radiotherapy for patients with stage II and III resectable rectal cancer; • Both preoperative and postoperative radiotherapy decrease local recurrence but neither improves survival as much as postoperative radiotherapy combined with chemotherapy. Therefore, if preoperative radiotherapy is used, chemotherapy should be added postoperatively to at least patients with stage III disease

Acute Intermittent Porphyria: Diagnostic Conundrums

Peer Reviewe