Fibrous dysplasia of the jaws: Integrating molecular pathogenesis with clinical,radiological, and histopathological features

Fibrous dysplasia is a non‐neoplastic developmental process that affects the craniofacial bones, characterized by painless enlargement as a result of bone substitution by abnormal fibrous tissue. Postzygotic somatic activating mutations in the GNAS1 gene cause fibrous dysplasia and have been extensively investigated, as well as being helpful in the differential diagnosis of the disease. Fibrous dysplasia may involve one (monostotic) or multiple bones (polyostotic), sporadically or in association with McCune‐Albright syndrome, Jeffe‐Lichenstein syndrome, or Mazabreud syndrome. This review summarizes the current knowledge on fibrous dysplasia, emphasizing the value of integrating the understanding of its molecular pathogenesis with the clinical, radiological, and histopathological features. In addition, we address important aspects related to the differential diagnosis and patient management.     

Lipid peroxidation, occupational stress and aging in workers of a prehospital emergency service.

BACKGROUND: Stressful conditions lead to formation of excessive free radicals, and lipid peroxidation is one of the major outcomes of free radical-mediated injury that directly damages membranes and generates a number of secondary products. OBJECTIVES: To determine the levels of malondialdehyde, an end product of lipid peroxidation, according to demographic and occupational variables in workers of a prehospital emergency service and to analyse the relationship between malondialdehyde levels and burnout. MATERIAL AND METHODS: One hundred and eleven healthy workers of a prehospital emergency service and eighty aged-matched healthy individuals of both sexes as a control group were surveyed. Malondialdehyde levels were measured by the Bull and Marnett method. To measure burnout, the Maslach Burnout Inventory was used. RESULTS: Professional category is associated with lipid peroxidation and burnout levels (Malondialdehyde levels were: physicians 338.10+/-14.47, nurses 329.17+/-12.62 and technicians 296.74+/-14.28; burnout levels were: physicians 41.29+/-3.59, nurses 37.38+/-6.05 and technicians 35.33+/-5.87). Working at night and in the evening increased malondialdehyde and burnout levels. Malondialdehyde levels increase with age. No significant variations with respect to sex were detected. Significant variations in malondialdehyde levels were detected between singles (303.13+/-12.74) and married people (344.43+/-13.43) but not with respect to divorcees (326.44+/-11.74). Significant differences were detected in erythrocyte malondialdehyde levels between smokers (341.37+/-17.09) and nonsmokers (302.21+/-12.38), but not for alcohol consumption. CONCLUSIONS: These findings suggest a positive correlation between malondialdehyde, a biomarker of lipid peroxidation and occupational stress, as estimated by elements of the Maslach Burnout Inventory, and oxidative stress.     

Quantitative diagnostic performance of myocardial perfusion SPECT with attenuation correction in women.

Attenuation correction (AC) for myocardial perfusion SPECT (MPS) had not been evaluated separately in women despite specific considerations in this group because of breast photon attenuation. We aimed to evaluate the performance of AC in women by using automated quantitative analysis of MPS to avoid any bias. METHODS: Consecutive female patients--134 with a low likelihood (LLk) of coronary artery disease (CAD) and 114 with coronary angiography performed within less than 3 mo of MPS--who were referred for rest-stress electrocardiography-gated 99mTc-sestamibi MPS with AC were considered. Imaging data were evaluated for contour quality control. An additional 50 LLk studies in women were used to create equivalent normal limits for studies with AC and with no correction (NC). An experienced technologist unaware of the angiography and other results performed the contour quality control. All other processing was performed in a fully automated manner. Quantitative analysis was performed with the Cedars-Sinai myocardial perfusion analysis package. All automated segmental analyses were performed with the 17-segment, 5-point American Heart Association model. Summed stress scores (SSS) of > or =3 were considered abnormal. RESULTS: CAD (> or =70% stenosis) was present in 69 of 114 patients (60%). The normalcy rates were 93% for both NC and AC studies. The SSS for patients with CAD and without CAD for NC versus AC were 10.0 +/- 9.0 (mean +/- SD) versus 10.2 +/- 8.5 and 1.6 +/- 2.3 versus 1.8 +/- 2.5, respectively; P was not significant (NS) for all comparisons of NC versus AC. The SSS for LLk patients for NC versus AC were 0.51 +/- 1.0 versus 0.6 +/- 1.1, respectively; P was NS. The specificity for both NC and AC was 73%. The sensitivities for NC and AC were 80% and 81%, respectively, and the accuracies for NC and AC were 77% and 78%, respectively; P was NS for both comparisons. CONCLUSION: There are no significant diagnostic differences between automated quantitative MPS analyses performed in studies processed with and without AC in women.     

Partial Portal Vein Ligation Plus Thioacetamide: A Method to Obtain a New Model of Cirrhosis and Chronic Portal Hypertension in the Rat

To obtain a new model of chronic portal hypertension in the rat, two classical methods to produce portal hypertension, partial portal vein ligation and the oral administration of thioacetamide (TAA), have been combined. Male Wistar rats were divided into four groups: 1 (control; n?=?10), 2 [triple partial portal vein ligation (TPVL); n?=?9], 3 (TAA; n?=?11), and 4 (TPVL plus TAA; n?=?9). After 3 months, portal pressure, types of portosystemic collateral circulation, laboratory hepatic function tests (aspartate aminotransferase, alanine aminotransferase, bilirubin, alkaline phosphatase, and gamma-glutamyl transpeptidase) and liver histology were studied. The animals belonging to group 2 (TPVL) developed extrahepatic portosystemic collateral circulation, associated with mesenteric venous vasculopathy without hepatic destructurization or portal hypertension. Animals from group 3 (TAA) developed cirrhosis and portal hypertension but not extrahepatic portosystemic collateral circulation, or mesenteric venous vasculopathy. Finally, the animals from group 4 (TPVL?+?TAA) developed cirrhosis, portal hypertension, portosystemic collateral circulation, and mesenteric venous vasculopathy. The association of TPVL and TAA can be used to obtain a model of chronic portal hypertension in the rat that includes all the alterations that patients with hepatic cirrhosis usually have. This could, therefore, prove to be a useful tool to study the pathophysiological mechanisms involved in these alterations.     

Predisposition to atypical hemolytic uremic syndrome involves the concurrence of different susceptibility alleles in the regulators of complement activation gene cluster in 1q32

Some MCP SNP and aHUS-associated MCP mutation     

Idiopathic transient osteoporosis of the knee: five cases and revision of literature

Five patients with idiopathic transient osteoporosis were examined. Bone scanning and MRI was helpful in the diagnosis to differentiate of necrosis and all patients recovered completely with conservative and symptomatic treatment. There was no history of trauma in all patients. MRI was realized previously in all cases to confirm the diagnosis and after the resolution of symptomathology.     

Robotic-assisted heller myotomy versus laparoscopic heller myotomy for the treatment of esophageal achalasia: multicenter study

Laparoscopic Heller myotomy (LHM) has become the standard treatment option for achalasia. The incidence of esophageal perforation reported is about 5%–10%. Robotically assisted Heller myotomy (RAHM) is emerging as a safe alternative to LHM. Data comparing the two approaches are scant. The aim of this study was to compare RAHM with LHM in terms of efficacy and safety for treatment of achalasia. A total of 121 patients underwent surgical treatment of achalasia at three institutions. A retrospective review of prospectively collected perioperative data was performed. Patients were divided into two groups: group A (RAHM), 59 patients, and group B (LHM), 62 patients. All the operations were completed using minimally invasive techniques. There were 63 women and 58 men, with a mean age of 45 ±19 years (14–82 years). Fifty-one percent of patients in group A and 95% of patients in group B reported weight loss. Duration of symptoms was equal for both groups. Dysphagia was the main complaint in both groups (P = NS). There was no difference in preoperative endoscopic treatment in both groups (44% versus 27%, P = NS). Operative time was significantly shorter for LHM in the first half of the experience (141 ± 49 versus 122 ± 44 minutes, P < .05). However, in the last 30 cases there was no difference in operative time between the groups (P = NS). Intraoperative complications (esophageal perforation) were more frequent in group B (16% versus 0%). The incidence of postoperative heartburn did not differ by group. There were no deaths. At 18 and 22 months, 92% and 90% of patients had relief of their dysphagia. This study suggests that RAHM is safer than LHM, because it decreases the incidence of esophageal perforation to 0%, even in patients who had previous treatment. At short-term follow-up, relief of dysphagia was equally achieved in both groups. Presented at the Forty-Sixth Annual Meeting of The Society for Surgery of the Alimentary Tract, Chicago, Illinois, May 14–18, 2005 (oral presentation). This study was supported in part by a grant provided by Intuitive Surgical, Inc. and Ethicon Endo-Surgery, Inc.     

Assay of cytomegalovirus susceptibility to ganciclovir in renal and heart transplant recipients

Ganciclovir (GCV) prophylaxis or pre-emptive therapy significantly reduce the rate of cytomegalovirus (CMV) disease and viremia, but increase the potential for emergence of ganciclovir-resistant CMV strains. The inhibitor concentration at 50% (IC(50)) of GCV from 156 CMV isolates from 59 renal or heart transplant recipients was calculated by means of a rapid phenotypic susceptibility assay. Twenty-seven strains were from 14 patients undergoing GCV therapy. The IC(50) was higher in patients under the prophylaxis regimen. One CMV strain, from a heart transplant recipient, became GCV-resistant after 1 month of therapy (IC(50)=13.7 micromol/l). These data, together with clinical and virological markers, suggested that a switch to foscarnet was necessary, and good evolution was observed. Thus, assay of CMV susceptibility to GCV could be helpful in clinical management.