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1.
We report a 20 month old female patient with diploid-triploid mixoploidy (46,XX/69,XXX) syndrome with hypothyroidism and precocious puberty. The triploid cell line was only expressed in the fibroblast culture and comprised the majority (95%) of the cells. Chromosome analysis of the fetal blood sample and peripheral blood sample were normal. The patient shows typical features of full triploidy (growth and severe mental retardation, cranial and facial dysmorphism, complete syndactyly of fingers 3/4, partial syndactyly of toes 2/3) and facial but no body asymmetry. At the age of 5 months central hypothyroidism and precocious puberty were diagnosed. Thin pigmented streaks were visible on the wrists and legs of the patient at the age of 16 months. This is the first patient reported so far with 46,XX/69,XXX mixoploidy suffering from hypothyroidism and precocious puberty.  相似文献   
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As hypersensitivity to natural rubber latex (NRL) has become an increasingly recognized problem in children, identification of all groups at risk seems important. In this study hypersensitivity to NRL was evaluated in 337 children with potential risk factors. We identified by questionnaire children's underlying diseases, history of surgical procedures, evidence of atopy and patient's history of NRL-specific reactions. Hypersensitivity to NRL and other allergens relevant in the Düsseldorf area was evaluated by skin prick test and specific IgE. In 9.2% of all children hypersensitivity to NRL was observed. Significant risk factors for hypersensitivity to NRL were, among the underlying diseases, spina bifida (odds ratio 29.2), hydrocephalus internus (10.1), gastrointestinal malformation (5.2) and atopy (2.2). Surgical procedures with significant risk were the implantation of a ventriculo-peritoneal shunt (15.7) and surgery of the gastrointestinal tract (3.1). Frequency of surgical procedures correlated (p < 0.001) with risk of hypersensitivity. Frequent surgery and atopy were found to have an additional effect on the risk of hypersensitivity. Information about previous NRL-specific reactions obtained by questionnaire was of little predictive value when performing multivariate analysis. For children at high risk for hypersensitivity to NRL preoperative evaluation and, in case of hypersensitivity, preventive measures seem to be advised.  相似文献   
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Estrogen and progesterone receptor analysis results were compared within and between six laboratories in Sweden using frozen breast cancer cytosol samples, and the same technique (enzyme immunoassay, Abbott Laboratories). The concordance in receptor status (positive vs. negative) was excellent (98.4% (571/580)). The discordant results were attributable to values near cut-off (n = 4) or outliers (n = 5), the latter probably being due to analytical errors. One laboratory reported significantly higher ER concentrations than the others; thus caution should be observed when comparing absolute values from different centers. For PgR there were similar differences between the laboratories. However, the intra- and inter-laboratory differences were small compared with the overall variability in ER and PgR content between different samples in a large database. The range of the median intra-laboratory coefficient of variation was 11-23% for ER and 12-19% for PgR, indicating that there is room for improvement in the quality of assay performance.  相似文献   
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The report deals with the development of procedures, evaluation of laser application and prevention of complications involved in intensive chemoradiation therapy of neoplasms of hemopoietic and lymphoid tissue in childhood. The clinical data on 548 patients, aged 2 months-15 years, with leukemia and solid tumor were evaluated. Laser application for treatment of different complications of chemoradiation therapy of pediatric leukemia proved a valuable innovation. It had a pronounced antiinflammatory and analgetic effect, promoted tissue regeneration processes and cut down the duration of complication treatment drastically.  相似文献   
6.
The effects of adenosine and sodium nitroprusside (SNP) on central hemodynamics and myocardial blood flow and metabolism were investigated postoperatively after elective coronary artery bypass (CABG) surgery in ten sedated and mechanically ventilated patients in the intensive care unit. During three consecutive 15-min periods, SNP (0.8 +/- 0.1 micrograms.kg-1 x min-1), adenosine (88.9 +/- 13.3 micrograms.kg-1 x min-1), and then again SNP (0.7 +/- 0.1 micrograms.kg-1 x min-1) were infused to control postoperative hypertension at a mean arterial pressure of approximately 80 mm Hg. Systemic and pulmonary hemodynamics and global (coronary sinus flow, CSF) as well as regional (great cardiac vein flow, GCVF) myocardial blood flow and metabolic variables were measured. During adenosine infusion, in comparison to SNP, heart rate was unchanged, stroke volume index and cardiac index increased (24% and 32%, respectively), and the systemic vascular resistance index decreased (-26%). Mean pulmonary arterial pressure (24%) as well as pulmonary capillary wedge pressure (27%) and central venous pressure (18%) were higher with adenosine compared to SNP. Adenosine also increased CSF and GCVF (108% and 103%, respectively) without altering the CSF/GCVF flow ratio compared to SNP. Furthermore, adenosine increased the coronary oxygen content (51%) and decreased the arterio-great cardiac vein oxygen content difference (-48%) without changing regional myocardial oxygen consumption, indicating a more pronounced hyperkinetic myocardial circulation compared to SNP. In addition, adenosine infusion decreased arterial PO2 (-11%) and increased the intrapulmonary shunt fraction (57%). The PR interval time of the electrocardiogram was prolonged (12%) and the ST segment was more depressed during adenosine infusion compared to SNP.(ABSTRACT TRUNCATED AT 250 WORDS)  相似文献   
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Thin films on aluminum-tungsten alloys were prepared by co-deposition of pure aluminum and pure tungsten, each sputtered by an independently controlled magnetron source, on glass and sapphire substrates. Completely amorphous films were obtained in the Al80W20-Al67W33 composition range. Passivity and corrosion behavior of amorphous Al-W alloys were investigated in 1 M deaerated hydrochloric acid solution using polarization and impedance spectroscopy measurements and have been correlated with the properties of pure alloy components. Tungsten and sputter-deposited Al-W thin films are inherently passive materials while aluminum undergoes pitting corrosion in hydrochloric acid solution. The passive film formed at the OCP on each alloy possesses excellent electric and dielectric properties comparable to those of the isolating film on tungsten. The absolute impedance increases with increasing tungsten content in the alloy. According to electrochemical polarization measurements, alloying Al with W in solid solution significantly enhances the material's resistance to pitting corrosion by shifting the breakdown potential above 2000 mV (Al67W33) and lowering the corrosion rate at the OCP by more than two orders of magnitude. The most likely mechanism explaining the passivity of amorphous Al-W alloys, the Solute Vacancy Interaction Model (SVIM), involves the formation of complexes between highly oxidized solute atoms (W+6) and mobile cation vacancies, which restrict the transport of Cl through the oxide film and inhibit its breakdown in hydrochloric acid solution. The role that film stress relaxation effects and microscopic defects in amorphous Al-W films, of the some composition, and deposited on various substrates play in their corrosion resistance is discussed.  相似文献   
10.
Supply of the main and doubling crews of cosmonauts with vitamins C, A, D, E, B1, B2, B6, PP, and B12, folic acid and carotinoids was studied during training and after the completion of short-term space flights (from 4 to 13 days). As the result of the investigation conducted during the intensive training, 1 month before the space flight some cases of certain vitamins deficiency were revealed. Administration of additional vitamins during 2 weeks before the flight led to a rise in the cosmonauts' supply with vitamins. The findings obtained after the short-term space flights evidence an intensive metabolization of vitamins and increased vitamin requirements during this period.  相似文献   
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