Valosin‐containing protein immunoreactivity in tauopathies,synucleinopathies, polyglutamine diseases and intranuclear inclusion body disease

Valosin‐containing protein (VCP) is associated with multiple cellular functions, including ubiquitin‐dependent protein degradation. Mutations in VCP are known to cause inclusion body myopathy with Paget's disease and frontotemporal dementia and familial amyotrophic lateral sclerosis (fALS; ALS14), both of which are characterized by trans‐activation response DNA protein 43 (TDP‐43)‐positive neuronal cytoplasmic and nuclear inclusions. Recently, immunoreactivity for fALS‐associated proteins (TDP‐43, fused in sarcoma (FUS), optineurin and ubiquilin‐2) were reported to be present in cytoplasmic and nuclear inclusions in various neurodegenerative diseases. However, the extent and frequency of VCP‐immunoreactive structures in these neurodegenerative diseases are uncertain. We immunohistochemically examined the brains of 72 cases with neurodegenerative diseases and five control cases. VCP immunoreactivity was present in Lewy bodies in Parkinson's disease and dementia with Lewy bodies, and neuronal nuclear inclusions in five polyglutamine diseases and intranuclear inclusion body disease, as well as in Marinesco bodies in aged control subjects. However, other neuronal and glial cytoplasmic inclusions in tauopathies and TDP‐43 proteinopathies were unstained. These findings suggest that VCP may have common mechanisms in the formation or degradation of cytoplasmic and nuclear inclusions of neurons, but not of glial cells, in several neurodegenerative conditions.     

Spinal cord injury following aortic arch replacement

Surgery Today - Postoperative spinal cord injury is a devastating complication after aortic arch replacement. The purpose of this study was to determine the predictors of this complication. A group...     

Redo cardiac surgery for active prosthetic valve endocarditis associated with hereditary hemorrhagic telangiectasia: report of a case

Hereditary hemorrhagic telangiectasia (HHT) is caused by an autosomal dominant gene and characterized by multiple arteriovenous malformations in several organs, leading to bleeding or shunting. These patients often suffer severe infections and heart failure, which should be managed in the perioperative period, when open heart surgery is indicated. We report a case of successful aortic root replacement for active prosthetic valve endocarditis and ventricular septal perforation in a patient with HHT, who had severe heart failure.     

Histological analysis of appendices removed during interval appendectomy after conservative management of pediatric patients with acute appendicitis with an inflammatory mass or abscess

Background/purpose

To clarify the role of interval appendectomy (IA) in pediatric patients with acute appendicitis with an appendiceal inflammatory mass or abscess, we histologically analyzed the appendices removed during IA.

Patients and methods

We treated 355 consecutive pediatric patients with acute appendicitis and reviewed the admission charts of patients who started conservative management (CM). The histology of the appendix removed during IA was also examined. The relationships among the clinical features, appendicolith formation at the time of IA and histological findings were analyzed by stepwise regression analyses.

Results

(1) CM was started in 48 patients (13.5 %). Recurrence or a remaining abscess was observed in nine patients (18.8 %). (2) Histopathological changes, particularly foreign body reaction with fibrosis and infiltration of inflammatory cells, were observed in about half of the specimens. (3) In a stepwise regression analysis, the presence of an appendicolith at IA was correlated with an appendicolith at diagnosis, foreign body reaction in the appendix and a decrease in the inflammatory reaction at diagnosis.

Conclusion

More than half the patients had strong histopathological changes in the appendix, suggesting a high possibility of recurrence. The presence of appendicolith formation at IA, which is a risk factor for recurrence, was influenced by the presence of an appendicolith at diagnosis, foreign body reaction in the appendix and the inflammatory status of patients at diagnosis. These clinical findings are indications for IA.     

Microsurgical technique used in right anterior segmentectomy and pancreatoduodenectomy with reconstruction of the right posterior hepatic artery for widespread bile duct cancer involving the hepatic hilus

A microsurgical technique was used in performing anterior hepatic segmentectomy and pancreatoduodenectomy with reconstruction of the posterior hepatic artery in a 64-year-old man with widespread bile duct cancer from the intrapancreatic bile duct over the hepatic hilus. The anterior hepatic artery was obviously involved and the posterior hepatic artery just behind common hepatic duct was very close to the cancer. Microsurgical anastomosis between the remnant gastroduodenal artery and the posterior hepatic artery at the hepatic hilus made it possible to preserve the posterior segment of the liver and to perform a curative resection of the cancer. The patient had pyrexia because of suprahepatic abscess after the operation, but the abscess drained spontaneously. Postoperative arteriogram showed neither obstruction nor kinking of the reconstructed artery. He was discharged 2 months after surgery and has been enjoying a normal quality of life for 10 months since, with no signs of recurrence. It is suggested that a microsurgical technique is useful for performing an accurate anastomosis with good patency that allows not only a safe but also a highly curative operation for advanced bile duct cancer.     

Relationships between polymorphisms of the human serum paraoxonase gene and insulin sensitivity in Japanese patients with type 2 diabetes

Human serum paraoxonase (PON1), which is associated with HDL, is an esterase and has been shown to reduce the susceptibility of LDL to lipid peroxidation. The objective of the study was to determine whether genetic polymorphisms of the PON1 gene are associated with insulin sensitivity. Forty-eight Japanese patients with type 2 diabetes were recruited, and euglycemic hyperinsulinemic clamp was performed to assess insulin sensitivity. The PON1 promoter polymorphism C(-108)T was determined by direct sequencing, and the coding region polymorphism Q192R was determined by polymerase chain reaction and digestion of the amplified fragments. No association was observed between the Q192R polymorphism and the glucose infusion rate (GIR), whereas GIR increased with the following order of genotypes: -108TT < -108CT < and -108CC (4.2+/-1.6, 5.1+/-2.5, and 6.9+/-2.5 mg kg(-1) min(-1), respectively; P<0.02, ANCOVA). Stepwise regression analysis revealed that the C(-108)T polymorphism significantly contributed to the GIR. It has been reported that oxidative stress attenuates insulin signaling in vitro. The PON1 promoter polymorphism C(-108)T may influence insulin sensitivity by modulating serum antioxidant capacity.