Childhood fibrous dysplasia presenting as blindness: a skull base approach for resection and immediate reconstruction

Fibrous dysplasia is an abnormal fibroosseous process of bone of unknown cause. The incidence of skull involvement varies, painless enlargement being the most common presenting symptom. Change in vision is a rare but recognized finding. We report a 3-year-old boy with extreme fibrous dysplasia involving the skull base, who presented with blindness. He underwent exposure osteotomies of the frontal bones and orbits to provide access for skull base tumor removal. The orbital roofs were reconstructed with microplate-fixed cranial grafts. One and one half years after tumor excision followed by immediate reconstruction, the boy retains facial symmetry, and his ocular function has not deteriorated.     

Transport of K in PVC matrix membranes containing valinomycin

We report impedance measurements on PVC matrix membranes which contain KBPh₄ with varying proportions of valinomycin. In agreement with our earlier measurements the value of the bulk membrane resistance (R_b) is much larger in the presence of valinomycin, indicating that the mobility of K⁺ is greatly reduced by the valinomycin. R_b shows a linear variation with valinomycin/K⁺ ratio between 0 and 1, but it is invariant at higher valinomycin/K⁺ ratios. Thus there is no evidence for a special transport mechanism for K⁺ in these membranes.     

汽车电子系统要求低静态电流

如今的汽车电子系统越来越复杂。同时，汽车环境对任何电子产品来说都是很大的挑战，因为汽车电子系统要求运行电压很宽，并且有很大的瞬态电压和温度变化。另外，性能要求也越来越高，需要多个供应电压以满足系统的不同要求。典型的     

单片同步降压型转换器LTC3416

许多微处理器和数字信号处理器(DSP)需要一个核心电源和一个在启动期间必须进行排序的输入／输出(I／O)电源。如果未经过适当的电源排序，可能发生闭锁或过流，从而损害微处理器的I／O端口或诸如存储器、逻辑电路、现场可编程门阵列(FPGA)或数据转换器之类支持设备的I／O端口。为了确保在主电源电压未被适当偏置之前I／O负载不被驱动，对核心电源电压和I／O电源电压进行跟踪是必要的。     

Hyperhomocysteinemia: detection, risk assessment, and treatment

OBJECTIVE: The aim of this study was to evaluate both the importance of the screening strategy and the familial aggregation characteristics of families with hypercholesterolemic children. PATIENTS AND METHODS: Ninety-one families (369 subjects) with one hypercholesterolemic child were studied. In addition to clinical and general biochemical evaluation, lipids including apo A-I and B-100 were examined. LDL was quantified under ultracentrifugation. RESULTS: Among the 91 children studied, 10 (10.99%) suffered heterozygous hypercholesterolemia, while 81 (89.01%) suffered polygenic hypercholesterolemia. Following a diet, polygenic children exhibited normal lipid parameters. In heterozygous children a decrease of 19% for total cholesterol, 19.9% for LDL-cholesterol and 16.3% for apo B were observed. When starting the study, 77.5% of the family members thought that they had normal serum lipid values. At the end of the study it was confirmed that only 28% were really normolipemic, indicating that 49.4% of the individual did not know that they were suffering dyslipemia. The study also showed that fathers exhibited the highest incidence of hypercholesterolemia (80.2%) followed by brothers (65.6%) and mothers (61.5%). Therefore, 69.4% of the individuals studied exhibited dyslipemia. CONCLUSIONS: The screening strategy allows one to diagnose a high percentage (almost 50%) of individuals suffering hypercholesterolemia in families with a child previously diagnosed of this pathology. Moreover, in these families there is a high degree of familiar aggregation of dyslipemia.     

Chances of cure are not compromised with sphincter-saving procedures for cancer of the lower third of the rectum

BACKGROUND: The goal of this study was to compare patterns of recurrence and long-term outcome after sphincter-saving procedures (SSPs) and abdominoperineal resection (APR) in patients with tumors located in the lower third of the rectum. METHODS: We reviewed the charts of 1001 patients operated on for primary rectal adenocarcinoma between 1980 and 1991. All patients with tumors located between 5 and 7 cm from the anal verge and treated with curative intent were included. RESULTS: Of the 261 patients who met our criteria, 162 had undergone SSP and 99 had undergone APR. The local recurrence rates for SSP and APR were 8% and 11%, respectively (p = 0.41), and the distant metastases rates were 23% and 28%, respectively (p = 0.35). Recurrence and distant metastases rates for SSP and APR, respectively, did not differ by TNM classification: state I, 10% versus 9% (p = 0.9); stage II, 25% versus 43% (p = 0.13); and stage III, 56% versus 57% (p = 0.92). Five-year disease-free survival rates for SSP and APR patients were 70.5% and 62.3%, respectively (p = 0.2). CONCLUSIONS: Tumors in the lower third of the rectum can be treated with sphincter-saving procedures without compromising the chance of cure.