Klinefelter's Syndrome: Hypogonadism,abnormal carbohydrate metabolism. Is it a result of biotin deficiency?

The aetiology of Klinefelter's Syndrome is not known. The causative factor(s) must explain the hypogonadism, low androgen levels, the disordered carbohydrate metabolism and the commonly associated psychiatric conditions. A biotin deficient/dependent state can account for the above. A biotin deficient Klinefelter's Syndrome patients with the above is described. The possible role of biotin in the primary, secondary and tertiary prevention of Klinefelter's Syndrome needs further research.     

Membrane flow within the myelin sheath in IDPN neuropathy

This report describes some aspects of beta,beta'-iminodipropionitrile (IDPN) neuropathy in rats as observed by ultrastructural methods and X-ray diffraction. Light microscopy shows gross swelling of the axons in proximal lumbar spinal roots 8 days after intraperitoneal injection of IDPN. Mean axon cross-sectional area and mean axon perimeter increased to 280% and 160% of their control values, respectively. At the same time, myelin membrane packing was not visibly disturbed. In addition, X-ray diffraction patterns, recorded under physiological conditions, demonstrate that the myelin lipid bilayer thickness and widths of the aqueous spaces between bilayers did not change. Related observations are made on posterior tibial nerve (PNS myelin) and ventral spinal cord (CNS myelin). The various observations together are interpreted in terms of a fluid myelin membrane. It is proposed that the myelin membrane flows during axon swelling even though normal membrane-membrane contacts are maintained within the sheath. Membrane flow and slippage between membranes are explained in terms of a molecular model of the myelin multilayer.     

人皮质骨矿化基质中骨盐框架结构

目的：研究人皮质骨矿化基质中骨盐的框架结构及框架中骨微间隙。方法：应用透射电镜、场发射扫描电镜观察、电脑图像分析及能谱分析，分析无骨病成人长骨、扁骨２００例骨盐分布特征。结果：骨盐框架结构由微柱、微梁、微小梁、弓状梁、致密点、隔板和骨微间隙构成。骨微间隙由洞、内衬和壁组成，洞平均直径为８４．４±７５．６ｎｍ，与骨小管相比有显著差异（Ｐ值＜０．００１），平均密度为１１～１７个／μｍ２，与骨小管之比超过１０：１。骨盐分针形结晶和微颗粒结晶。结论：骨盐框架结构及骨微间隙是骨盐在人皮质骨矿化基质中的存在形式，可能与骨盐吸收、沉着有关。     

Uptake and intracellular distribution of idarubicin in secondary cultures of normal and neoplastic urothelium

This study analyzes the uptake and endocellular distribution of idarubicin (IDA) in normal and neoplastic urothelial secondary cultures in relation to the changes in concentration and time of exposure. The urothelial lines were isolated by Freshney's method from biopsy fragments taken from five patients with superficial bladder cancer. Pharmacological experiments were carried out on subcultures previously immunophenotypically characterized and did not exceed ten passages. The uptake and endocellular distribution of IDA was analyzed by densitometric image analysis on cells treated for 10, 20, 30 and 60 min and 2 h with scalar dosages from 10 ng/ml to 2430 ng/ml. Microscopic observations and densitometric analyzes revealed that in the cells treated with IDA, fluorescence was higher in the cytoplasm compared to the nucleus and increased with the change in dosage. Moreover, densitometric data showed that IDA uptake in the first 20 min was higher in the neoplastic cells, but after that period its behavior became heterogeneous at 30 and 60 min, while at 2 h there was an inversion of the trend. These results suggest that the in vitro cytotoxicity should be evaluated in order to verify whether the elevated uptake of IDA in the first 20 min of treatment is really correlated to a more elevated toxicity in the neoplastic cells with respect to the normal cells. This is presently under investigation.     

Organization of intensive care units in Europe: lessons from the EPIC study

Objective: To study differences related to intensive care unit (ICU) structure and patient demography between the various countries of Western Europe. Design: Application of data collected by the European Prevalence of Infection in Intensive Care (EPIC) study, a one-day prevalence study. Setting: Voluntary participation of all Western European ICUs. A total of 1417 ICUs responded. Patients: All patients, older than 10 years of age, occupying a bed in the participating ICUs over a 24-h period. 10 038 patient case reports were submitted. Results: The study revealed important differences. In particular, there seems to be a north/south divide with fewer ICU beds and more severely ill patients in the south. The United Kingdom seemed more similar to southern European countries than to the north. Conclusion: While there are similarities between European countries, large differences still remain and are important to identify to enable us to work together to create a more uniform system of intensive care, which will in turn give more effective and efficient patient care. Received: 7 February 1997 Accepted 8 July 1997     

Primary Aldosteronism

ABSTRACT A follow-up examination was performed one month to 20 years after adrenalectomy in 28 cases with surgically treated primary aldosteronism due to adrenal adenoma. The mean age at diagnosis was 45, and the mean duration of hypertension seven years. Severe hypertension with a diastolic blood pressure of 130 mmHg or more was observed in 35%. Postoperatively cerebrovascular catastrophe developed in two cases, both of which belonged to the group of patients with severe hypertension. Normalization of blood pressure was observed in 70% and in the remaining subjects the blood pressure was lower than at diagnosis. The blood pressure response to adrenalectomy appeared unpredictable in view of such parameters as the initial blood pressure, age at diagnosis, and duration of the hypertensive state. Toxicosis during pregnancy and metrorrhagia was observed with unexpectedly high frequency in this study population. Low ambulatory plasma renin activity was recorded at the follow-up in 15 out of 18 subjects studied in the absence of evidence of hyperaldosteronism.     

雌激素对老年早期大鼠脑心脏毛细血管结构的影响

目的　探讨老年前期大鼠应用雌激素后脑、心微血管结构的变化 ,了解雌激素对脑、心血管的影响 ,为临床合理应用雌激素替代治疗提供实验依据。方法　制作老年早期衰老大鼠模型 ,通过内源性碱性磷酸酶显示法、动脉插管墨汁灌注观察应用雌激素后大鼠脑额叶、海马、下丘脑 ,心脏毛细血管结构的变化。结果　苯甲酸雌二醇组 (estradiolben zoate ,EB)脑额叶皮层、海马、下丘脑、心肌等部位毛细血管面积均明显高于对照组 (P <0 .0 5 )。结论　老年前期雌性大鼠应用雌激素后可影响脑、心脏微血管结构 ,改善心、脑供血