Plasma Exchange for Hemolytic Crisis and Acute Liver Failure in Wilson Disease

Wilson disease (WD) is a rare autosomal recessive disorder of copper metabolism which primarily involves the liver and the central nervous system. Rarely, WD can present as acute liver failure (ALF) and this disease is universally fatal in the absence of liver transplantation. The authors report a young girl with WD ALF, who showed signs of recovery after prompt initiation of plasma exchange (PE) and chelation therapy. Though liver transplantation could not be done in this child and the child died 8 d after stopping PE, this case highlights that PE can be a successful medical treatment in WD ALF and should be considered as a therapeutic measure to stabilize a patient by decreasing serum copper, reducing hemolysis, and helping to prevent renal tubular injury from copper and copper complexes until liver transplantation is possible.     

Costs of laparoscopic and open liver and pancreatic resection: A systematic review

AIM: To study costs of laparoscopic and open liver and pancreatic resections, all the compiled data from available observational studies were systematically reviewed.METHODS: A systematic review of the literature was performed using the Medline, Embase, PubMed, and Cochrane databases to identify all studies published up to 2013 that compared laparoscopic and open liver [laparoscopic hepatic resection (LLR) vs open liver resection (OLR)] and pancreatic [laparoscopic pancreatic resection (LPR) vs open pancreatic resection] resection. The last search was conducted on October 30, 2013.RESULTS: Four studies reported that LLR was associated with lower ward stay cost than OLR (2972 USD vs 5291 USD). The costs related to equipment (3345 USD vs 2207 USD) and theatre (14538 vs 11406) were reported higher for LLR. The total cost was lower in patients managed by LLR (19269 USD) compared to OLR (23419 USD). Four studies reported that LPR was associated with lower ward stay cost than OLR (6755 vs 9826 USD). The costs related to equipment (2496 USD vs 1630 USD) and theatre (5563 vs 4444) were reported higher for LPR. The total cost was lower in the LPR (8825 USD) compared to OLR (13380 USD).CONCLUSION: This systematic review support the economic advantage of laparoscopic over open approach to liver and pancreatic resection.     

Analysis of thirteen cases with hyperthyroidism-associated myocardial infarction

Background The close relationship between the thyrotoxic state and abnormalities in cardiac function,arrhythmias and congestive heart failure is well known to clinicians. Patients with hyperthyroidism-induced acute myocardial infarction(AMI) are unusual and almost all reported cases have been associated with coronary vasospasm. There is quite consistent evidence that hyperthyroidism is directly associated with the presence of a prothrombotic state. Methods The present article reports a 69-year-old female with hyperthyroidism who developed myocardial infarction(MI) and reviews AMI in hyperthyroidism cases reported in the literature. We conducted a comprehensive review of the English literature since 1990 to analyze data on MI in hyperthyroidism patients who had developed AMI. Results From 11 English articles, we identified 12 hyperthyroidism patients, plus our cases, with AMI. They consisted of 11 females and 2 males.Their mean age was 49 ± 14.4 years(range 28-71). Coronary angiography revealed normal coronary arteries in 6 patients, coronary spasm in 6 patients. Only our patient received percutaneous coronary intervention through coronary thrombus suction and other patients were treated mainly with medicine. Twelve patients had good prognosis and 1 patient died of cardiogenic shock. Conclusions Hyperthyroidism-induced AMI is rare.Coronary thrombus or spasm without coronary atherosclerotic stenosis should be considered. Different therapies should be chosen according to different types of coronary involvement.     

Regulatory element copy number differences shape primate expression profiles

Gene expression differences are shaped by selective pressures and contribute to phenotypic differences between species. We identified 964 copy number differences (CNDs) of conserved sequences across three primate species and examined their potential effects on gene expression profiles. Samples with copy number different genes had significantly different expression than samples with neutral copy number. Genes encoding regulatory molecules differed in copy number and were associated with significant expression differences. Additionally, we identified 127 CNDs that were processed pseudogenes and some of which were expressed. Furthermore, there were copy number-different regulatory regions such as ultraconserved elements and long intergenic noncoding RNAs with the potential to affect expression. We postulate that CNDs of these conserved sequences fine-tune developmental pathways by altering the levels of RNA.     

Evaluation of Dimensional Accuracy of Panoramic Cross‐Sectional Tomography,Its Ability to Identify the Inferior Alveolar Canal,and Its Impact on Estimation of Appropriate Implant Dimensions in the Mandibular Posterior Region

Objectives: To evaluate the dimensional accuracy of panoramic cross‐sectional tomography, its impact on implant size estimation and its ability in identifying the inferior alveolar canal in the mandibular posterior region. Material and Methods: Eight partially edentulous mandibles with 18 edentulous sites were obtained. Orthopantomograms and tomograms were made and the mandible's outline and the position of mandibular canals on tomograms were traced on a clear acetate paper. Horizontal and vertical magnification factors were calculated. The mandibular height, distance between mandibular canal and alveolar crest, maximum bucco‐lingual width, distance between buccal cortex and mandibular canal, and cortical thickness at the inferior border of the mandible were measured. Potential implant sites were identified and implant sizes were estimated. Location and visibility of mandibular canals were also evaluated. The mandibles were sectioned at each site and all the above mentioned parameters were assessed which served as gold standard. Results: Mean horizontal and vertical magnification factors were 1.47 ± 0.048 and 1.53 ± 0.038. Total height and maximum bucco‐lingual width were underestimated by 1.88% and 1.59%. Crest to canal distance, cortical thickness at the inferior border of the mandible and buccal cortex to mandibular canal were overestimated by 0.59%, 5.16%, and 3.64%. Implant sizes were estimated for 11 sites and changes were recorded at 2 sites between record 1 and record 2. However, there was no disagreement between record 2 and record 3. Of the canals, 61.11% were located lingually and the visibility of mandibular canals was poor in 44.44% of cases. Conclusions: The tomograms were found to be accurate for the measurements in both horizontal and vertical planes and reliable for implant size estimation, taking into consideration proper magnification factors. They were also found to be useful in assessing the location of mandibular canal but were not very effective in discerning it.