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1.
Although intellectual impairment is common in patients with myotonic dystrophy, this aspect of the disease has received relatively little research attention. We examined 41 patients with myotonic dystrophy using objective neuropsychological procedures and magnetic resonance imaging. Ten patients (24%) had severe and generalized intellectual dysfunction, while lesser or no cognitive impairment characterized the remaining patients. Degree of intellectual impairment was not related to neuromuscular status or sex. Patients with severe intellectual disturbance had significantly earlier onset of both myotonia and weakness and were more likely to inherit the disease from their mother. Magnetic resonance imaging findings indicated that while degree of cerebral atrophy was not related to severity of intellectual impairment, skull thickness, focal white matter lesions, and anterior temporal lobe abnormalities were significantly more common in patients with severely disturbed intellect. This study reports a number of previously unreported cerebral magnetic resonance imaging findings associated with intellectual impairment in myotonic dystrophy, but the etiology of these changes awaits neuropathologic examination.  相似文献   
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1. The somatostatin octapeptide-analogue, octreotide, is absorbed as intact peptide from the gastrointestinal (GI) tract. 2. In situ absorption experiments in rats confirmed our recent intubation studies in human volunteers demonstrating that the peptide has preferential absorption sites in the small intestine. Absorption of octreotide was higher in the jejunum than in the duodenum or the ileum. 3. Experiments with bile-duct cannulated rats demonstrated that the absorption of octreotide decreased in the presence of bile, reflecting a negative influence of biliary components on the absorption of the peptide. 4. Uptake experiments using rat jejunal brush border membranes were performed to analyse the absorption mechanisms. The transport of octreotide into jejunal brush border membranes was significantly higher than the uptake into membrane vesicles isolated from rat ileum. When initial uptake (0-15s) rates into the membrane vesicles were calculated as a function of the peptide concentration, a saturable component could be observed, indicative of transport mechanisms different from simple diffusion.  相似文献   
4.
At birth the human newborn is immature and has not yet acquired fully competent organ functions. During lactation especially the digestive tract and the immune system gain competence. Lactation is regulated by a complex interaction of numerous hormonal factors. Most important are probably prolactin and oxytocin—two hormones produced and secreted in the pituitary. The production and secretion of these two hormones is again regulated by several factors in the central nervous system and these mechanisms can be disturbed by a variety of exogenous factors such as drugs and any other substances interacting with dopamine metabolism in the brain. It is important to know these physiological interactions to be able to understand the regulation of lactation and to be able to assess which factors could interfere with this process.  相似文献   
5.
Mouse monoclonal antibodies were raised against recombinant Salmonella typhi 36-kDa porin monomer. Specificities of 16 monoclonal antibodies were analyzed as reactivity patterns in dot immunobinding and Western blot (immunoblot) assays using isolated outer membrane proteins of gram-negative bacteria and cloned purified S. typhi porin monomers and trimers. Four monoclonal antibodies were specific for Salmonella spp.  相似文献   
6.
Spontaneous rupture of the Achilles tendon, which is associated with underlying systemic disease and concurrent oral corticosteroid therapy, is a relatively rare occurrence, with very few cases reported in the English medical literature to date. Patients with this affliction frequently present weeks to months after the rupture occurs because there is no succinct traumatic event. The neglected rupture renders surgical repair more difficult secondary to the retraction of the triceps surae muscle group and the concomitant increase in width of the defect that results. The authors present a case report of a patient with a neglected spontaneous rupture of the Achilles tendon who had been on oral corticosteroid therapy to treat the manifestations of systemic lupus erythematosus. A gastrocnemius recession was performed in a "V to Y" fashion to gain needed lengthening, and an end-to-end anastomosis was also performed. The plantaris tendon was then used to help reinforce the anastomosed site.  相似文献   
7.
In situ forming parenteral drug delivery systems: an overview.   总被引:7,自引:0,他引:7  
Biodegradable injectable in situ forming drug delivery systems represent an attractive alternative to microspheres and implants as parenteral depot systems. Their importance will grow as numerous proteins will lose their patent protection in the near future. These devices may offer attractive opportunities for protein delivery and could possibly extend the patent life of protein drugs. The controlled release of bioactive macromolecules via (semi-) solid in situ forming systems has a number of advantages, such as ease of administration, less complicated fabrication, and less stressful manufacturing conditions for sensitive drug molecules. For these reasons, a number of polymeric drug delivery systems with the ability to form a drug reservoir at the injection site are under investigation. Here, we review various strategies used for the preparation of in situ forming parenteral drug depots and their potential benefits/draw-backs, especially with regard to the delivery of protein drug candidates.  相似文献   
8.
Autoimmune neutropenia: clinical and laboratory studies in 143 patients   总被引:3,自引:0,他引:3  
Summary Clinical and laboratory data of 143 patients with primary or secondary autoimmune neutropenia (AIN) were evaluated. Primary AIN was found predominantly in children below 3 years, whereas secondary AIN was more frequent in patients 40–60 years of age. Female patients with primary AIN were slightly more prevalent (54%) than male patients (46%). The peripheral blood count showed normal or diminished leukocyte counts with median absolute neutrophil counts of 250 cells/l. In 38% of the patients neutropenia was accompanied by monocytosis. Bone marrow examination revealed in 95% a normo- or hypercellular marrow with a marked reduction of mature neutrophils in 56% of the specimens. Twenty-three percent of the sera showed specificity for the NA1 antigen. Patients were usually affected by benign bacterial infections of the skin and of the upper respiratory tract, as well as by recurrent otitis media. Infections were treated symptomatically, and only six patients required continuous administration of antibiotics. Remission of neutropenia during treatment occurred in three of six patients treated with intravenous immunoglobulin G and in three of four patients who received steroid therapy. Except for one patient neutropenia relapsed after discontinuation of therapy. During a follow-up of 6–36 months, spontaneous remission has been observed in four patients.This work was supported by theDeutsche Forschungsgemeinschaft (Mu 277/9-7)  相似文献   
9.

Objective:

To present the clinical management of a ganglion cyst presenting on the dorsolateral aspect of the foot.

Clinical Features:

A 45-year-old female cyclist complaining of ganglion cyst following training period.

Intervention and Outcome:

Patient was treated with high-frequency electroacupuncture in four consecutive sessions over four weeks, and reported resolution of the cyst following therapeutic intervention.

Conclusions:

Ganglion cysts of the foot are relatively rare connective tissue tumours with variable treatment approaches. Electroacupuncture may be a novel and non-invasive conservative approach for the treatment of ganglion cysts. Further evaluation of the efficacy of such treatment is warranted.  相似文献   
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