用混合粘合剂碳糊电极测定丁螺环酮

用混合粘合剂碳糊电极测定丁螺环酮张正奇，曾鸽鸣，刘传桂，黎艳飞（湖南大学化学化工系，长沙４１００８２）碳糊电极无毒，制作方便，表面更新容易，应用电位范围广，在药物分析中已有应用［１～５］。我们在液体石腊中加入添加剂，组成混合粘合剂，可显著改善电极的检...     

Association between pancreatic cystadenocarcinoma, malignant liver cysts, and polycystic disease of the kidney

Polycystic kidney disease is an autosomal dominant disease that may be associated with cystic disease of the liver. In women, the cysts may develop early and be more troublesome than in men. Cystadenocarcinoma of the pancreas is uncommon, comprising 1% of primary pancreatic malignancies. This case report is the first to describe a familial association between polycystic kidney disease and cystadenocarcinoma of the pancreas and liver in the English medical literature. A patient with autosomal dominant polycystic kidney disease (ADPKD) and multiple hepatic cysts developed cystadenocarcinoma of the pancreas with multiple malignant liver cysts. The patient's mother, sister, and niece had ADPKD, and the patient's sister also died of pancreatic cystadenocarcinoma. We believe that the development of these two disease entities in which the primary pathology is cyst formation has a genetic association. (Gastroenterology 1997 Jun;112(6):2104-7)     

Hormone replacement therapy and vigilance: double-blind,placebo-controlled EEG-mapping studies with an estrogen-progestogen combination (Climodien,Lafamme) versus estrogen alone in menopausal syndrome patients

The aim of the double-blind, placebo-controlled study was to investigate the effects of a continuous combined estrogen-progestogen treatment (Climodien, Lafamme) as compared with estrogen alone on vigilance in insomniac postmenopausal syndrome patients, objectified by EEG mapping. METHODS: In a 3-arm, 2-month parallel group design phase, patients received a combination of estradiol valerate 2 mg and the novel progestogen dienogest 3 mg (Climodien 2/3) or estradiol valerate 2 mg alone or placebo. In a subsequent open-label phase, all patients received estradiol valerate 2 mg+dienogest 2 mg (Climodien 2/2). EEG mapping was carried out before and after the 2-month double-blind phase as well as after the 2-month open-label treatment. RESULTS: As compared with placebo, Climodien 2/3 induced a marked and highly significant increase in absolute power in all frequency bands, specifically in alpha-2 activity. Moreover, a significant increase in relative alpha-2 power, a decrease in relative delta and beta power as well as an acceleration of the dominant frequency and of the delta and alpha centroids suggested a marked improvement in vigilance. In contrast, under estradiol valerate 2 mg alone, only a slight augmentation of alpha and attenuation of relative delta and beta power occurred, suggesting only a slight vigilance improvement as compared with placebo. Thus, dienogest 2 mg increased the estrogen effect, which was also confirmed by a statistical evaluation of the differences between Climodien 2/3 and estradiol valerate alone (augmentation of alpha-2, attenuation of relative beta, acceleration of the dominant frequency). Moreover, Climodien 2/2 also markedly increased alpha-2 power, decreased relative beta-2 power and accelerated the alpha centroid. Finally, comparing Climodien 2/3 with Climodien 2/2, there was even a dose-efficacy relation. CONCLUSIONS: Estradiol valerate 2 mg improves vigilance slightly, thereby confirming previous findings. The additional administration of dienogest does not minimize the effect of estrogen, but on the contrary increases it, which makes the combination superior to both placebo and estradiol valerate alone. Vigilance improvement may be of great therapeutic benefit to menopausal syndrome patients at a time when increased adaptability is needed to adjust to increasing sexual, marital, occupational and social difficulties known to occur specifically in this period of life.     

Immunological parameters in girls with Turner syndrome

Disturbances in the immune system has been described in Turner syndrome, with an association to low levels of IgG and IgM and decreased levels of T- and B-lymphocytes. Also different autoimmune diseases have been connected to Turner syndrome (45, X), thyroiditis being the most common.     

A point mutation in the 5' splice site of the dystrophin gene first intron responsible for X-linked dilated cardiomyopathy

X-linked dilated cardiomyopathy (XLDC) is a familial heart disease presenting in young males as a rapidly progressive congestive heart failure, without clinical signs of skeletal myopathy. This condition has recently been linked to the dystrophin gene in some families and deletions encompassing the genomic region coding for the first muscle exon have been detected. In order to identify the defect responsible for this disease at the molecular level and to understand the reasons for the selective heart involvement, a family with a severe form of XLDC was studied. In the affected members, no deletions of the dystrophin gene were observed. Analysis of the muscle promoter, first exon and intron regions revealed the presence of a single point mutation at the first exon-intron boundary, inactivating the universally conserved 5' splice site consensus sequence of the first intron. This mutation introduced a new restriction site for MseI, which cosegregates with the disease in the analyzed family. Expression of the major dystrophin mRNA isoforms (from the muscle-, brain- and Purkinje cell-promoters) was completely abolished in the myocardium, while the brain- and Purkinje cell- (but not the muscle-) isoforms were detectable in the skeletal muscle. Immunocytochemical studies with anti- dystrophin antibodies showed that the protein was reduced in quantity but normally distributed in the skeletal muscle, while it was undetectable in the cardiac muscle. These findings indicate that expression of the muscle dystrophin isoform is critical for myocardial function and suggest that selective heart involvement in dystrophin- linked dilated cardiomyopathy is related to the absence, in the heart, of a compensatory expression of dystrophin from alternative promoters.