患结节性多动脉炎的婴儿表现有低钠性高血压综合征

高血压合并低血钠性脱水被称作低钠性高血压综合征(H H S),是一种罕见且严重的高血压并发症。在此,作者报道1例17月龄的女童,其有严重的低血钠性脱水、低血钾、多尿和肾性蛋白尿,同时有恶性高血压和全身性炎性疾病。典型结节性多动脉炎的诊断多是基于肾动脉造影时显示有小动脉瘤及非动脉瘤样改变如动脉中断、动脉狭窄以及肾图灌注缺损而确定。抗高血压药物治疗引起血压下降,使得H H S患者异常得到恢复。但是,4个月的免疫抑制治疗后,结节性多动脉炎被有效控制。结论:这种罕见的系统性结节性多动脉炎在年龄非常小的儿童身上发生,是主要的价…     

351例股骨颈骨折的远期疗效

关于股骨颈骨折报道众多,治疗方案各异。基于股骨颈骨折与老龄化成比例的增加,目前它不仅是医疗问题,也成为了社会问题。Ljubljana大学创伤外科医院于1986年～1987年两年间收治了824例股骨上端骨折患者,其中股骨颈骨折351例。     

Effect of water restrictions on the physiological parameters,psychological behavior and brain c-Fos expression in rat

1 Introduction Exposure to hostile stressors causes a series of coor- dinated responses in the body, such as alterations of neu- roendocrine secretion, immune reaction and behavioral manifestation to maintain homeostasis stability and sur-vival of the organisms. Stressors are divided into two main categories: physical, or systemic, and psychological, or emotional / processive. Each stressor might activate a spe- cific central pathway to induce a special neuroendocrine response, even cause stre…     

Recurrence of bronchioloalveolar carcinoma in donor lung after lung transplantation: microsatellite analysis demonstrates a recipient origin

Bronchioloalveolar carcinoma is a distinctive subtype of pulmonary adenocarcinoma, without effective therapy, although there have recently been some attempts to use lung transplantation. However, a high post-transplantation local recurrence rate is described with some controversy regarding the possible involved mechanisms, the main possibilities being the lymphatic spread and aerosolization. Presented herein is a case of a bilateral lung transplantation for a bilateral and pneumonic form of non-mucinous bronchioloalveolar carcinoma in a 43-year-old woman. The histological analysis of mediastinal lymph nodes during surgery did not show neoplastic cells. Thirty-five months after transplantation several nodular opacities in donor lungs were detected. Three pulmonary wedge resections were performed showing a non-mucinous bronchioloalveolar carcinoma with the same histological characteristics as the primary. Again, the mediastinal lymph nodes were tumor free. A complete microsatellites molecular analysis was performed to compare the primary and recurrent carcinoma using capillary electrophoresis, showing that the recurrent tumor was generated in a recipient cellular clone. The absence of lymph node metastasis and the molecular evidence of the recipient origin of the neoplasm supports the contamination of the new lungs at the time of implantation as being the reason for the high incidence of recurrence after lung transplantation in this kind of disease.     

Gene and cancer therapy--pseudorabies virus: a novel research and therapeutic tool?

The past decade has been marked by significant advances in the application of gene transfer into living cells of animals and humans, with the resulting catapulting of preclinical and basic scientific concepts into therapeutic trials. A variety of virus-mediated gene delivery techniques have proved to be superior to other methodologies. This article concisely reviews the current status of gene and tumor therapy, focusing on virus-based technologies, describes the molecular biology of neurotropic herpesviruses and the application of herpes simplex virus, a relative of pseudorabies virus (PRV) in gene transfer and cancer therapy protocols. Finally, it addresses the issue of whether PRV, a nonhuman pathogen, could serve as a suitable research and therapeutic tool as concerns genetic and tumor therapy.     

Prenatal detection of a 17p11.2 duplication resulting from a rare recombination event and novel PCR-based strategy for molecular identification of Charcot-Marie-Tooth disease type 1A

Charcot-Marie-Tooth disease, type 1A (CMT1A) is caused in most cases by a 1.5 Mb duplication on chromosome 17p11.2 arising after unequal crossing-over between repeated sequences called CMT1A-REPs, flanking the 1.5 Mb unit. A 3.2 kb recombination hot spot has been defined, resulting in a junction fragment between EcoRI (distal CMT1A-REP) and SacI (proximal CMT1A-REP). This was further reduced to a 1.7kb EcoRI-NsiI fragment, and recently to a 731 bp hot spot region within this fragment. We describe the CMT1A-REPs-based PCR method used to identify CMT1A duplications and report on a family case in which a 29-year-old pregnant woman requested prenatal diagnosis for two successive pregnancies because her husband was affected with CMT1A. Our method enabled us to characterise the duplication in both foetuses and demonstrate that it arose from a rare recombination event taking place outside the 1.7 kb region. Since our approach is simple and enables the entire set of duplications occurring after recombination in the enlarged 3.2kb region including the hot spot to be detected, we suggest it might be considered for use in primary screening for pre- and postnatal diagnosis of CMT1A.     

Onset of action of pimecrolimus cream 1% in the treatment of atopic eczema in infants

BACKGROUND: Data on the efficacy of pimecrolimus cream 1% within the first days of treatment are scarce, as in previous studies, the first postbaseline assessment was performed only after 1 week. OBJECTIVE: We sought to investigate the onset of action of pimecrolimus cream 1% in infants with mild to very severe atopic eczema. METHODS: We used pimecrolimus cream 1% (n = 129) or vehicle cream (n = 66) administered in a double-blind manner for 4 weeks and then open-label pimecrolimus cream 1% for 12 weeks, with a 4-week follow-up period. RESULTS: Pimecrolimus cream 1% reduced the mean Eczema Area and Severity Index at 4 weeks by 71.5% compared with an increase of 19.4% with vehicle ( P < .001). The reduction in the Eczema Area and Severity Index with pimecrolimus cream 1% was significant at day 4 (38.5% vs 17.6% increase with vehicle). Significant improvements in caregivers' assessments of pruritus and sleep loss were observed with pimecrolimus cream 1% by day 2 ( P < .03) and day 3 ( P = .002), respectively, compared with vehicle. Responses to pimecrolimus cream 1% were sustained during the open-label phase, and pimecrolimus cream 1% was well tolerated. Symptoms of atopic eczema returned gradually after discontinuation. CONCLUSION: Pimecrolimus cream 1% was well tolerated and effective in patients with mild to very severe atopic eczema, with rapid onset of action and no disease rebound after discontinuation.     

P53 overexpression as an indicator of overall survival and response to treatment in osteosarcomas

The p53 gene located at chromosome 17pl3 is found to be altered (allelic loss or other mutation) in multiple human cancers, including osteosarcomas. The mutated gene produces a protein with a prolonged half-life thus rendering it detectable by conventional immunohistochemistry. We examined the correlation between p53 expression and clinical prognosis as well as response to therapy. Twentyone patients with previously untreated and histologically verified highly malignant osteosarcoma were used for this study. Biopsy material taken both prior to the start of COSS 91 protocol and at the time of surgery (ten weeks later) was examined for alterations in p53 protein expression and drug resistance. Two patients who had strong (+++) p53 protein expression and three others who became positive during the chemotherapy had significantly worse prognosis (all of them died within one year) than those who showed no p53 expression both at biopsy and after chemotherapy (all 11 patients are alive, average follow-up time: 3.5 years). All patients who showed any kind of positive p53 protein expression on initial biopsy were non-respon-ders to chemotherapy. In contrast, 69% (9 out of 13) of those who exhibited no p53 expression on initial biopsy were responders or intermediate responders to chemotherapy. We concluded that p53 expression may be a useful prognostic factor in osteosarcomas. The direct correlation between p53 positive expression and resistance to therapy can help in identifying patients who are in need of a more vigorous or different chemotherapeutical protocol.     

Passive expiratory flow-volume curve is not an accurate method to measure the respiratory time constant in rabbits

BACKGROUND: In mechanically ventilated patients, inspiration is forced by an externally applied positive pressure whereas expiration remains passive and depends on the time constant of the total respiratory system (tau), which constitutes an important determinant of mechanical ventilation. The end-inspiratory occlusion technique is one of the easiest methods to obtain tau values in ventilated patients, especially infants, but its accuracy is not well established. The aim of this study was to compare in anesthetized, paralyzed rabbits tau values given by the end-inspiratory occlusion technique (tau(rs)) to tau values obtained by references methods for measurements (i.e. the product of static lung compliance by airway resistance: tau(ref)) during carbachol-induced bronchospasm eliciting marked modifications of the respiratory mechanics. METHODS: Comparisons were made in the basal state and after carbachol-induced bronchoconstriction in seven New Zealand adult rabbits. This procedure resulted in a wide range of expiratory time constants. A pneumotachograph was used to measure expiratory flow and volume before and after end-inspiratory occlusion. The slope of the flow volume curve gave tau(rs). Then tau(rs) was compared with tau(ref) (which ranged from 0.30 to 1.96 s). RESULTS: Statistical analysis revealed a weak correlation between the two methods, and a size-dependent bias of tau(rs) measurements. CONCLUSIONS: The end-inspiratory occlusion technique leads to a systemic bias in measurements of respiratory time constant, especially when the resistance of the respiratory system is elevated.