旋磁场对大鼠血清铜蓝蛋白活力的影响

用联大茴香胺法测定大白鼠血清中铜蓝蛋白的活力，大白鼠体外血清在旋磁场中曝磁３０分钟后，血清铜蓝蛋白活力增加，而大白鼠整体血清在旋磁场中曝磁３０分钟后，血清铜蓝蛋白活力无明显变化。     

慢性肝病141例与微量元素关系的研究

本文对141例慢性肝病(慢活肝30例,肝硬化111例)患者血、肝组织中微量元素锌、铜、铁的含量测定,并测定肝硬化患者血清铜兰蛋白含量及尿锌的排出量。结果血清中锌值均明显低于对照组(P<0.05),肝硬化较慢活肝更低,而铜与铜兰蛋白含量均明显高于对照组(P<0.01)。其中19例肝硬化患者作肝组织锌、铜测定,锌含量特别低,其均值比对照组低3倍(P<0.05),而铜含量则高于对照组1倍以上(P<0.05)。肝硬化患者24h尿锌排出量明显高于对照组(P<0.001)。通过对这些微量元素代谢变化的探讨,为慢性肝病治疗中纠正其代谢障碍提供了科学依据。     

结核性胸膜炎患者胸水,血清铜蓝蛋白测定及临床意义

探讨铜蓝蛋白与结核性胸膜炎患者有胸膜肥厚及粘连的关系。方法：用散射光比浊法对２１例结核性胸膜炎患者的胸水和血清ＣＰ水平进行检测，并以１５例性胸水患者及５０例正常人对作对照。     

Hereditary ceruloplasmin deficiency: A cerebro-retino-diabetic disease

Hereditary ceruloplasmin deficiency (HCD) is a recently recognized neurodegenerative disease of an autosomal recessive inheritance. This disease is caused by the defect of the ceruloplasmin gene. The initial manifestation of HCD is usually adult-onset diabetes mellitus. Neurological symptoms occur 10 to 20 years thereafter, between 40 and 60 years of age. These include dementia, abnormal involuntary movements, and cerebellar ataxia. Retinal degeneration is also present. The cardinal pathological change is a massive iron deposition to the brain, liver and pancreas. A marked loss of neurons is observed in the caudate nucleus and putamen of the brain. The number of insulin cells is decreased in the islets of the pancreas. In the clinical settings, it seems that HCD patients may exist among the population of adult-onset diabetic patients.     

Elevation of serum ceruloplasmin levels in brain tumours

Serum ceruloplasmin levels have been estimated in 80 patients with various intracranial space occupying lesions and in 30 controls. The ceruloplasmin levels were significantly increased in all brain tumours except in meningiomas. After therapy, the ceruloplasmin levels were still significantly increased when compared to controls and their respective preoperative values. However, the rise in levels of ceruloplasmin in malignant tumours compared to benign was statistically not significant. It is concluded that ceruloplasmin may have a role to play as an acute phase reactant protein in brain tumours.     

Catecholamine Stimulation of Copper Dependent Haemolysis: Protective Action of Superoxide Dismutase,Catalase, Hydroxyl Radical Scavengers and Serum Proteins (Ceruloplasmin,Albumin and Apotransferrin)

Abstract: Copper induced lysis of washed rat erythrocytes was stimulated by catecholamines, the order of effectiveness being: adrenaline > noradrenaline ∼ dopamine > L-DOPA. The degree of effectiveness is related to the rate of copper ion dependent oxidation of catecholamine, adrenaline being more rapidly oxidized than the other catecholamines investigated. Superoxide dismutase, catalase and different hydroxyl radical scavengers (mannitol, tris, formate and ethanol) markedly reduced the haemolytic effect of copper and catecholamine, suggesting the possibility that superoxide radicals and hydrogen peroxide, formed in the reaction system, cooperate in producing hydroxyl radicals, which are directly involved in the haemolytic action. The plasma proteins, ceruloplasmin, albumin and apotransferrin, also reduced the copper-catecholamine induced haemolysis. The protective action is probably not related to the copper binding ability of these proteins.     

KINKY HAIR SYNDROME

Abstract. Møllekær, A. M. (Department of Paediatrics and the Department of Neuropathology, århus University School of Medicine, århus, Denmark). Kinky Hair Syndrome. Acta Paediat Scand, 63289, 1974.–Two brothers with Kinky Hair Syndrome are described. Both of them had hypothermia. In the younger boy low levels of serum copper and ceruloplasrnin were demonstrated. At autopsy the most surprising finding was a demyelinating process in the brain. The fact that the younger boy showed signs of Kinky Hair syndrome at birth makes it difficult to accept an 'intestinal malabsorption of copper as the only underlying defect in the disease.     

食管、贲门癌患者手术前后血清铜蓝蛋白含量变化的研究

本文用放射免疫分析法测定了４５例食管、贲门癌患者手术前后血清铜蓝蛋白（ＳＣＰ）含量的变化，并与４４例正常人进行对照。结果表明：食管、贲门癌病人手术前后均显著高于对照组（Ｐ＜０．０１）；手术后病人ＳＣＰ呈递减趋势，７２ｈ后ＳＣＰ开始明显下降，与手术前对照（Ｐ＜０．０５）。并观察到术后有转移的患者ＳＣＰ持续保持高水平。病情稳定者，ＳＣＰ趋于正常。由此认为：ＳＣＰ可以作为食管、贲门癌的诊断、判断预后、有否转移和复发的一项重要辅助参考指标。     

Management Perspective of Wilson’s Disease: Early Diagnosis and Individualized Therapy

Wilson’s disease (WD) is an inherited disease caused by mutations in ATP7B and is characterized by the pathological accumulation of copper in the liver and brain. Common clinical manifestations of WD include a wide range of liver disease and neurological symptoms. In some patients, psychiatric symptoms may be the only manifestation at the time of diagnosis. The clinical features of WD are highly variable and can mimic any disease of internal medicine. Therefore, for unexplained medical diseases, the possibility of WD should not be ignored. Early diagnosis and treatment can improve the prognosis of WD patients and reduce disability and early death. Gene sequencing is becoming a valuable method to diagnose WD, and if possible, all WD patients and their siblings should be genetically sequenced. Copper chelators including D-penicillamine, trientine, and dimercaptosuccinic acid can significantly improve the liver injury and symptoms of WD patients but may have a limited effect on neurological symptoms. Zinc salts may be more appropriate for the treatment of asymptomatic patients or for the maintenance treatment of symptomatic patients. High-quality clinical trials for the drug treatment of WD are still lacking, therefore, individualized treatment options for patients are recommended. Individualized treatment can be determined based on the clinical features of the WD patients, efficacy and adverse effects of the drugs, and the experience of the physician. Liver transplantation is the only effective method to save patients with acute liver failure or with severe liver disease who fail drug treatment.     

Astrocytic ceruloplasmin expression,which is induced by IL-1beta and by traumatic brain injury,increases in the absence of the IL-1 type 1 receptor

IL-1alpha and IL-1beta are induced immediately after insults to the brain, and signaling through the type 1 IL-1 receptor is essential for a normal microglial and astroglial response to injury. To better understand which genes are induced in astrocytes by IL-1beta, we used the unbiased technique of differential display to analyze mouse astroglial gene expression after IL-1beta treatment. Two novel genes were induced, as well as the gene for ceruloplasmin, a ferroxidase with antioxidant properties. Ceruloplasmin was analyzed further by Northern and Western blot. RNA and protein levels of ceruloplasmin were increased when astrocytes were treated with IL-1beta. To determine whether the IL-1 type 1 receptor (IL-1R1) is essential for the injury-induced expression of ceruloplasmin, a Western blot analysis was performed after a traumatic brain injury on mice that were IL-1R1-deficient. Ceruloplasmin increased significantly above controls after injury; however, injury-induced levels of ceruloplasmin were lower in IL-1R1-deficient (2.7-fold increase) than in the wild-type animals (3.5-fold increase). These data indicate that while IL-1R1 deletion has a slight effect on ceruloplasmin expression, it is not essential for either the basal or the induced expression of ceruloplasmin in vivo. Since ceruloplasmin buffers free copper, oxidizes ferrous iron, and catalyzes the dismutation of free radicals, increased levels of ceruloplasmin likely protect neurons and glia from sustaining damage after injury. Furthermore, as the IL-1R1 has been proposed to be a target for achieving neuroprotection after injury, these data suggest that the protection afforded by ceruloplasmin will be retained even when the IL-1R1 is antagonized.