Neuroprotective modulation of the unfolded protein response in Marinesco-Sj?gren syndrome: PERK signaling inhibition and beyond

<正>Individuals with Marinesco-Sj?gren syndrome(MSS;OMIM 248800),a genetic disease of infancy,suffer various disabilities,including loss of motor coordination due to cerebellar degeneration,and skeletal muscle weakness.After a progressive phase,symptoms stabilize and patients live to old age.Therefore,any pharmacological treatment that delays or attenuates cerebellar degeneration and/or muscle pathology can significantly improve their quality of life.We recently found that inhibiting the protein kinase RNA-like endoplasmic reticulum kinase     

Yersinia enterocolitica causing suppurative arthritis of the shoulder.

Yersinia enterocolitica is a rare cause of extra-intestinal disease. A case in a 60 year old butcher with hepatic cirrhosis who developed suppurative arthritis of the shoulder caused by Y enterocolitica is presented. There was no evidence of gastrointestinal infection or septicaemia. The severity of the illness was associated with various factors, including the underlying disease, the type of occupation, and the difficulties encountered in defining the optimal therapeutic regimen.     

319例352眼眼外伤疗效观察

采用综合治疗，抗炎，散瞳，局部治疗与全身应用免疫抑制疗法，治疗３１９例３５２眼外伤获得了满意的疗效，眼球挫伤１０４例，眼球穿孔伤９３例，化学烧伤７１例，眼炸伤４６例。眼热烧伤５例，治疗包括及时清创胶粘或缝合，清除前房出血合理用皮质类固醇和免疫抑制剂等。眼球挫伤治愈率９７．１２％，穿透伤９３．１８％，热烧伤６６．６７％。３１９例眼外伤治愈率９４．６７％好转５．３３％，并对各种治疗方法进行了讨论。     

Unconjugated hyperbilirubinaemia in HBV carriers. Discriminant value of nicotinic acid (NA) test

Recently an unconjugated hyperbilirubinaemia, without any other abnormalities in liver function test, in 14.3% of HBV Japanese carriers has been noticed. Therefore, it would be possible to argue that the persistent infection of HBV in hepatocytes might play a role in an hypothetical metabolic derangement of bilirubin clearance. Twenty-five subjects in a group of 468 HBsAg+ patients (equal to 5.33%) presented an hyperbilirubinaemia. This percentage was not different from the 5.83% found in 3083 HBsAg- controls coming from the same institution. Therefore we could exclude that in our population the presence of HBV surface antigen itself would determine a statistically higher level of total bilirubin (TB) than in controls. The nicotinic acid (NA) loading test may reveal some bilirubin metabolic defects (i.e. Gilbert syndrome), even in subjects with normal basal values of TB. According to this background, we performed in 11 HBsAg+ males with basal TB higher than 17.1 mumol/l (1 mg%) (group A/1), 13 HBsAg+ males with basal TB lower or equal to 17.1 mumol/l (group A/2) and 14 HBsAg- normal controls matched for sex and age (group B) the NA test according to R?llinghoff et al. All the parameters calculated by the NA test resulted significantly different in the A/1 group compared with the B group, but not different from those found by several authors in the Gilbert's syndrome. On the contrary, no significant differences have been noticed between the latter group and the A/2 group.(ABSTRACT TRUNCATED AT 250 WORDS)     

Respiratory impairment and metal exposure in a group of 68 industrial welders

A group of 68 currently employed welders were examined who were exposed to welding fumes either for the whole or for at least half the working day and who used at least two welding processes, which were mainly manual metal arc welding and metal active gas welding, but also metal and tungsten inert gas welding, or oxyacetylene welding. The welders were classified into 3 groups according to the class of materials used, i.e.: 1) only mild and stainless steel; 2) mild and stainless steel and other metals; 3) only other metals. Chronic bronchitis symptoms were observed in 22% of the subjects; signs of suspected radiological fibrosis were present in 18%, initial signs of radiological fibrosis in 4%. Small airway disease was prevalent in the smokers (28 smokers versus 2 nonsmokers); one worker had an obstructive syndrome and another a restrictive syndrome (both were smokers). There was a good correlation between years of exposure and: 1) MEF25 in non-smokers; 2) urinary cobalt in the whole group. There was also a correlation between smoking and FEV1, MEF50, MEF25, PaO2 and urinary cadmium. Serum copper was higher in workers exposed full time than in those exposed part time. Urinary chromium and cobalt and serum copper were high in steel welders, lower in welders using steel and other metals and low in welders using other metals. Urinary chromium was similar in smokers and non-smokers. Serum zinc was found to be of little use for biological monitoring of welders. Urinary chromium was studied for one week in 10 full-time exposed steel welders. The difference between the chromium values at the end of the workshift and those at the beginning was small (low current exposure); the chromium values at the end of the workshift were not uniform in the whole group (different past exposure levels).     

Retrorenal colon: implications for percutaneous diskectomy

It has been recommended that computed tomography (CT) with the patient prone be performed in every patient undergoing percutaneous diskectomy; this would enable detection of a retrorenal location of the colon, which could interfere with the percutaneous procedure. In this evaluation of 346 prone CT studies, only one patient (0.29%) was found to have retrorenal or retropsoas bowel that would have been perforated at diskectomy. Because of this extremely low prevalence, the performance of prone CT in every patient undergoing percutaneous lumbar diskectomy is not believed to be necessary.