Self-Mutilation in Neurodegeneration with Brain Iron Accumulation

Neurodegeneration with brain iron accumulation (NBIA) is the term applied to a heterogeneous group of disorders resulting in iron deposition in the basal ganglia. Well-known phenotypic features are progressive regression with extra pyramidal involvement and a variable course. A 10-year-old child born to consanguineous parents presented with progressive generalized opisthotonic dystonia, retrocollis, oromandibular dyskinesias, apraxia for swallowing, optic atrophy and severe self-mutilation of lips. MR imaging showed brain iron accumulation. Other causes of self-mutilation were excluded. Early infantile onset, ophisthotonic dystonia with oromandibular dyskinesias and characteristic MR images are suggestive of NBIA. There is only one case reported in the literature of self-mutilation in this condition.     

Iron Deficiency Anemia Associated With Acid-Modifying Medications: Two Cases and Literature Review

Iron deficiency anemia is often listed among potential adverse effects of gastric acid-suppressive medications, given that gastric acidity promotes intestinal absorption of nonheme iron. Additionally, the antacid calcium carbonate can inhibit iron absorption. However, there is little direct clinical evidence that proton-pump inhibitors, histamine-2 receptor antagonists, or calcium carbonate cause iron deficiency anemia. Most case reports have had substantial limitations (e.g., minimal follow-up and presence of other causes of iron deficiency), and retrospective cohort studies have lacked sufficient patient-specific detail to make strong causal inferences. We present 2 cases—both with detailed, prospective 10-year follow-up—in which combinations of proton-pump inhibitors, histamine-2 receptor antagonists and calcium carbonate were clearly associated with development of iron deficiency anemia. Overt iron-deficiency anemia is probably uncommon in patients who use acid-modifying medications and who have no other conditions that predispose to iron deficiency. Nevertheless, clinicians should be aware of this potential complication, given widespread use of these agents.     

血色病肝脏铁过度沉着症1例

1.病例资料：患者，男，48岁。以“乏力、多饮、多尿、多食，伴消瘦4个月，加重1周”入院。既往有乙型肝炎和地中海贫血病史30年，无输血史，无血色病家族史，曾行“脾脏切除”治疗。查体：慢性贫血面容，全身皮肤黄染有散在色素沉着，巩膜黄染，双手肝掌，男性乳房发育；肼右肋下3cm，剑突下5cm，质硬；腹部未触及肿块。     

Iron deficiency down-regulates the Akt/TSC1-TSC2/mammalian Target of Rapamycin signaling pathway in rats and in COS-1 cells

Iron deficiency (ID) is one of the most commonly known forms of nutritional deficiencies. Low body iron is thought to induce neurologic defects but may also play a protective role against cancer development by cell growth arrest. Thus, ID may affect cellular pathways controlling cell growth and proliferation, the mechanism of which is still not fully understood. The serine/threonine protein kinase Akt and its downstream target, the mammalian Target of Rapamycin (mTOR), is known to play a crucial role in the regulation of cell growth and survival. Therefore, we hypothesized that Akt/mTOR pathway could be influenced by ID. Three-week-old male Wistar-strain rats were divided into 3 groups and the 2 groups had free access to a control diet (C group) or an iron-deficient diet (D group). The third group (PF group) were pair-fed the control diet to the mean intake of the D group. After 4 weeks, rats were killed and their brains were sampled. In separate experiments, COS-1 cells were cultured with or without the iron chelator deferoxamine. Western blots of brain samples and COS-1 lysates were used to analyze the expression and phosphorylation state of Akt, TSC2, mTOR, and S6 kinase proteins implicated in the Akt/mTOR pathway. Using 2 different ID models, we show for the first time that iron deficiency depresses Akt activity in rats and in COS-1 cells, leading to a decrease in mTOR activity.     

铁缺乏时淋巴细胞增殖反应改变的实验研究

本研究采用体外细胞培养，３Ｈ－Ｔｄｒ掺入，原子吸收光谱分析等技术方法，观察了低浓度Ｆｅ－ｃｉｔ时淋巴细胞内铁浓度变化及经ＣｏｎＡ、ＰＨＡ诱导的细胞增殖反应能力改变，结果显示：铁缺乏时（第２、３、４组）ＳＩ降低，与正常组（第１组）比较相差非常显著（Ｐ＜０．０１），ＳＩ与细胞内铁浓度的变化呈显著正相关（Ｐ＜０．０５，ｒ１＝０．９４６６，ｒ２＝０．９３３９），说明铁缺乏时淋巴细胞增殖反应能力受抑。     

黑蔓不同部位微量元素的测定

为了了解吉林省长白县山区所产卫矛科雷公藤属植物黑蔓的药理作用,扩大它的药用部位和应用范围,我们从无机元素与中药药效有密切关系的角度出发,实验测出黑蔓含有Ba、Si、Cu、Fe、Zn、Sn、Co、Mn、Mg、Ca、Se等无机元素,本文重点对其中人体必需的微量元素Cu、Fe、Zn、Se作了定量分析。     

昆明城区、大理农村7月～7岁儿童铁缺乏调查分析

目的 :了解昆明城区及大理农村 7月～ 7岁儿童目前的铁缺乏水平 ,进一步展开对儿童铁缺乏症的防治 ,降低儿童营养性缺铁性贫血的患病率 .方法 :采用抽样调查的方法对两地 7月～ 7岁儿童共 96 9名进行血清铁蛋白 (SF)、锌原卟啉 (ZPP)、血红蛋白 (Hb)实验室检查 .结果 :7月～ 7岁儿童的总铁缺乏检出率为 2 5 1% ,铁减少检出率为 19 0 % ,贫血患病率为 6 1% ;男女童患病率在 1岁以前差别较大 ,1岁以后差别不明显 ;各年龄组患病率以 7月～ 1岁组最高 ;早产儿、低出生体重儿童患病率相对较高 ;铁缺乏的儿童营养不良的患病率高于正常儿童 .结论 :儿童缺铁性贫血仍要引起重视 .在防治中要抓重点人群 ,除改变膳食结构和饮食习惯 ,对目标人群要适当补充铁剂预防     

“维生素AD、铁营养强化奶的研制”研究报告

目的:研制维生素AD、铁营养强化奶,用于群体有效防治维生素AD、铁缺乏.方法:应用微胶囊技术科学配方成维生素AD、铁营养粉,添加到鲜牛奶中.结果:工业化生产维生素AD、铁营养强化奶.结论:该产品取得了很好的社会效益和经济效益.     

催化分光光度法测定人发中微量铁

拟定了一简单实用的测定人发中铁的催化分光光度法。在实验条件下,人发中常见的数10种微量元素均不干扰铁的测定,回收率达86～106%。