A case of Klinefelter syndrome with retroperitoneal teratoma

Klinefelter syndrome (KS) is often associated with various neoplasms, especially germ cell tumors. Mediastinum is the most favored site of extragonadal germ cell tumors with KS, which is somewhat different from those without KS. The retroperitoneal germ cell tumor in KS is very rare. A five-month-old boy with an abdominal mass was found to have a retroperitoneal tumor. After surgical removal, he was diagnosed to have mature cystic teratoma. Cytogenetic study of his peripheral lymphocytes revealed that his karyotype was consistent with KS. This case suggests that patients with KS might be at risk of having germ cell tumors in sites other than mediastinum. It also suggests that all cases with these tumors should be screened for the presence of karyotypic abnormalities, and it might help to assess the exact correlation between germ cell tumors and KS, and to treat them accordingly.     

A novel lysophospholipid- and pH-sensitive receptor, GPR4, in brain endothelial cells regulates monocyte transmigration.

Abundant evidence documents the highly proinflammatory actions of lysophosphatidylcholine (LPC). Further, LPC, found in high amounts in oxidized low-density lipoprotein (LDL), is implicated as an atherogenic factor. In endothelial cells, LPC impairs endothelial barrier function through GPR4, a novel receptor hypothesized to be sensitive to LPC and protons. The authors investigated the stimulation by LPC or low pH of GPR4 in human brain microvascular endothelial cells (HBMECs) and whether the activated GPR4 regulates in vitro monocyte transmigration. The results indicated that HBMECs stimulated by LPC (5 microM), but not low pH, showed a twofold increase in monocyte transmigration. Using retroviruses containing siRNA to GPR4, a > 60% reduction of GPR4 expression resulted in blockade of the LPC-stimulated transmigration. The inhibited response was restored by co-expression with an small interference RNA (siRNA)-resistant, but functional, GPR4 mutant construct. To investigate potential signaling mechanisms, the siRNA-mediated knockdown of GPR4 also prevented LPC-induced RhoA activation. C3 transferase, a Rho inhibitor, prevented approximately approximately 65% of the LPC-stimulated transmigration. LPC also increased MLC phosphorylation by 5 min, which was inhibited by the Rho kinase inhibitor, Y-27632 (10 microM) or ML-7 (myosin light chain kinase (MLCK) inhibitor). The findings indicate that the proinflammatory and atherogenic LPC stimulated endothelial GPR4, which promoted monocyte transmigration through a RhoA-dependent pathway.     

Effect of aging on human aortic protein composition. II. Two-dimensional polyacrylamide gel electrophoretic analysis

Arterial intima proteins were extracted by 9 M urea from matching histologically atheroma-free areas of 27 human thoracic aortas of both sexes from younger (15-34) and older (35-82) age groups and studied after separation by high-resolution two-dimensional polyacrylamide gel electrophoresis. Seventeen specific protein groups on each gel were identified according to their relative charges and molecular weights and their distribution in the two age groups compared. Some plasma-derived proteins occurred rarely in young aortas while they were consistently found in those from older cases, i.e., protein group 4 (alpha 1-antichymotrypsin) 1/13 (8%) vs 12/14 (86%), group 7 (haptoglobin beta-chain) 1/13 (8%) vs 13/14 (93%) and groups 6 and 9 (IgG chains) 3/13 (23%) vs 9/14 (64%), respectively. Other plasma-derived proteins such as group 3 (albumin) and 5 (alpha 1-antitrypsin) were identified in all samples of both age groups but their expression in the aortic intima increased with age. Proteins which are typically found intracellularly such as those from groups 11 (actin), 12 (cytoskeleton proteins), and 13 (tropomyosin-like proteins) appeared in samples of intima of both age groups but were less apparent in older specimens. These studies suggest that the changes in aortic intima protein distribution in the absence of atherosclerosis closely correlate with histological changes such as intimal thickening often found with aging, providing new sensitive markers of vascular senescence.     

神经导航中脑组织变形补偿的点云处理

有限元方法是解决神经导航中脑组织变形的重要方法,需要手术过程中的脑皮层信息作为其边界条件.本文通过非结构点云进行了脑皮层信息的表示,并通过对其进行处理来获取有限元方法的边界条件.点云处理包括纹理映射、分割、简化和去噪,其中非结构点云的简化与去噪采用了改进的基于表面特性k邻域的聚类方法.实验结果证明所采用的点云处理方法是可靠的.     

Increased myeloperoxidase and lipid peroxide-modified protein in gynecological malignancies

Oxidative stress has been implicated in several diseases, including cancer. Oxidants induce oncogenes and their products associated with cell growth. Even though epidemiological studies implicate oxidants in promoting cancer, there is still a lack of in vivo evidence for the same. In this study, we measured the levels of myeloperoxidase (MPO), an enzyme associated with oxidation and autoantibodies to lipid peroxide-modified protein (LOOH-RSA), in the plasma of subjects with gynecological cancers. The gynecological cancer subjects (n = 201) had higher plasma MPO and LOOH-RSA levels compared with control subjects (n = 60). Immunohistochemical analysis of tissues revealed that immunostaining for MPO and LOOH-RSA was higher in cancer tissues compared with controls. The staining was specific to cell types and not ubiquitously present. Neutrophils, monocytes/macrophages, and natural killer cells have been proposed to play a role in cancer promotion and progression. This study proposes a role for oxidative stress and especially MPO in cancer.     

Alterations of hepatocellular intermediate filaments during extrahepatic cholestasis in rat liver

Intermediate filaments (IF) maintain the structural and functional integrity of cells. To investigate whether IF change as a consequence of increased mechanical pressure and what the significance of such alterations is for the integrity of hepatocytes, we investigated alterations of IF in rat liver following common bile duct ligation (CBDL). Immunofluorescence of cytokeratin 18 was performed on extracted cryostat sections which were also used for electron microscopy. Ultrathin sections of mildly extracted liver tissue were applied to reveal the relationship between IF and intercellular junctions and cytoplasmic organelles. Our results showed that hepatocellular IF underwent striking changes during CBDL. The so-called pericanalicular sheath disappeared and IF were rigidly rearranged at the cell periphery, appearing as honeycomb-like structures. Increased amounts of IF were found in close association with increased numbers of desmosomes at the lateral membranes of hepatocytes, and electron-dense desmosome-like bodies were even observed in the ectoplasm at bile canaliculi. Rearrangement of IF in the cytoplasm resulted in segregation of subcellular compartments. The increased density of the IF network and desmosomes are compensatory mechanisms of hepatocytes to resist increased mechanical load and disperse the tension. However, the intracellular rearrangement of IF leading to segregation of subcellular compartments may also have distinct effects on hepatocellular metabolic functions.     

Haptoglobin genotypic distribution (including Hp0 allele) and associated serum haptoglobin concentrations in Koreans

BACKGROUND: Haptoglobin polymorphism is associated with the prevalence of infections, autoimmune diseases, cardiovascular diseases, and other disorders. Congenital haptoglobin deficiency is associated with anaphylactic transfusion reactions in anhaptoglobinaemic patients with antihaptoglobin antibody. AIMS: To investigate haptoglobin genotypic distribution (including the Hp(0) allele) and associated serum haptoglobin concentrations in Koreans. METHODS: Five hundred and nine healthy Korean adults were randomly selected. Two methods were used: haptoglobin genotyping based on a polymerase chain reaction (PCR) system that exploited the structural difference of the Hp(1) and Hp(2 )alleles, and another PCR method that detected haptoglobin gene deletion by amplification of the junctional region of the Hp(0) allele. Serum haptoglobin concentrations were measured by nephelometry. RESULTS: The haptoglobin genotypes of 509 subjects were as follows: Hp(1)Hp(1), 7.1%; Hp(2)Hp(1), 37.7%; Hp(2)Hp(2), 49.3%; Hp(0)Hp(1), 2.2%; Hp(0)Hp(2), 3.5%; Hp(0)Hp(0), 0.2%. The gene frequency of Hp(0) in Koreans was calculated to be 0.031. Significant differences were seen among the concentrations of each haptoglobin genotype (Kruskal-Wallis test). Hp(0)Hp(2), but not Hp(0)Hp(1), was associated with hypohaptoglobinaemia. CONCLUSIONS: PCR methods for differentiating between haptoglobin genotypes, including the Hp(0) allele, may be useful in a broad spectrum of basic studies and clinical examinations.     

A case of minute intraductal papillary mucinous tumor of the pancreas presenting with recurrent acute pancreatitis

Intraductal papillary mucinous tumor (IPMT) of the pancreas, a lesion consisting of mucin-producing cells with neoplastic potential, is characterized by duct ectasia, mucin hypersecretion, often extensive papillary intraductal growth, varying degrees of cytologic atypia, and relatively indolent growth. The clinical presentation of IPMT of the pancreas is characterized by chronic or recurrent attacks of abdominal discomfort often in association with low level pancreatic enzyme elevations. Less commonly these lesions may be detected as asymptomatic radiographic abnormalities. Interestingly, a case of a minute IPMT (2 mm in height and 7 mm in length, adenoma) in the main pancreatic duct presenting with acute pancreatitis in a 55 year-old man has been reported in the Japanese literature. Recently, we also experienced a case of a minute IPMT in a branch pancreatic duct causing repeated bouts of acute pancreatitis in a 75 year-old man. A filling defect at the neck of the main pancreatic duct seen on an endoscopic retrograde pancreatogram performed after recovery of the second attack of acute pancreatitis led the patient to undergo an exploratory laparotomy. After a near-total pancreatectomy was carried out, a minute (3 x 7 mm) IPMT of borderline malignancy was discovered in a branch duct at the head portion near the pancreatic neck without any lesions in the main pancreatic duct. Surprisingly, despite the resective surgery the patient died of carcinomatosis 8.5 months after the operation. We herein report a case of a minute but aggressive IPMT of the pancreas with a review of the literature.     

Evaluation of recombinant antigen-based assays for diagnosis of bullous autoimmune diseases

The diagnosis of autoimmune bullous diseases is based on clinical observation and on the presence of autoantibodies directed to molecules involved in the adhesion systems of the skin. Immunofluorescence assays are the currently accepted method for detection of autoantibodies; such assays depend greatly on the skill of operators and are difficult to standardize. Recombinant desmoglein-1 (Dsg1), Dsg3, and BP180 peptides, the main autoantigens in pemphigus or bullous pemphigoid, have been used to develop new quantitative enzyme immunoassays (EIA) for the detection of specific antibodies. The present study was undertaken to evaluate the sensitivity and specificity of these immunoassays and to determine the correlation between the results and the clinical aspects of diseases. Serum samples from patients with pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, or mucous membrane pemphigoid, from healthy individuals, and from patients with unrelated autoimmune conditions were tested. Anti-desmoglein reactivity was detected in all the patients with pemphigus and in none of the controls. Patients with the more benign form of cutaneous disease had anti-Dsg1 antibodies, while patients with deeper cutaneous lesions or with mucosal involvement had anti-Dsg3 reactivity also, or exclusively. The BP180-based assay was positive for 66.6% of patients with bullous pemphigoid and for none of the patients with mucous membrane pemphigoid, and no reactivity was detected in the control sera. In conclusion, the anti-Dsg1 and anti-Dsg3 assays are useful in the diagnosis of pemphigus and provide information on the clinical phenotype of the disease. However, the sensitivity of EIA for detection of autoantibodies in bullous pemphigoid should be improved by the use of additional antigens or epitopes.