检索结果-南通市图书馆

Vitamin B_(12) Deficiency in Newborns and their Mothers_Novel Approaches to Early Detection,Treatment and Prevention of a Global Health Issue

引用

Current Medical Science 2020年第5期40卷 801-809页

作者： Gwendolyn Gramer Georg f.hoffmann University Hospital Heidelberg Center for Pediatric and Adolescent MedicineDivision of Neuropediatrics and Metabolic MedicineDietmar Hopp Metabolic CenterIm Neuenheimer Feld 6691 Heidelberg 69120Germany

Vitamin B 12 deficiency,mostly of maternal origin in newborns,is a well treatable condition but can cause severe neurologic *** women of childbearing age and pregnant women worldwide vitamin B12 deficiency has been reported with frequencies of 10%-50%.Children with vitam in B I2 deficiency are asym ptom atic at birth but may develop severe multisystemic symptoms,including irreversible developmental impairment in the second halfyear of *** detection of vitamin B12 deficiency allows for presymptomatic *** article provides an overview over the function of vitamin B12 and discusses causes and frequency of vitamin B12 deficiency in newborns,infants,and women of childbearing *** describes novel successful approaches to newborn screening(NBS)for vitamin B,2 deficiency and results of a pilot study which performed systematic NBS for vitamin B12 deficiency using so-called second-tier strategies by measuring homocysteine and methylmalonic acid in dried blood *** for diagnostics in mothers of children with vitamin B12 deficiency are described as well as results of systematic work-up in mothers and treatment and follow-up of children with vitamin B12 deficiency detected by *** options of vitamin B12 deficiency are presented including a newly developed standardized supplementation scheme with exclusively oral vitamin BI2 *** for preventive approaches to vitamin Bl2 deficiency for children and mothers are *** children worldwide could benefit from systematic inclusion of vitamin B12 deficiency into NBS *** addition,preventive approaches to maternal vitamin B12 deficiency should be implemented systematically during maternal care.

关键词： vitamin B_(12)deficiency prevention treatment mother and child health newborn screening maternal

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

High incidence of maternal vitamin B12 deficiency detected by newborn screening: first results from a study for the evaluation of 26 additional target disorders for the German newborn screening panel

引用

World Journal of Pediatrics 2018年第5期14卷 470-481页

作者： Gwendolyn Gramer Junmin fang-hoffmann Patrik feyh Glynis Klinke Peter Monostori Jürgen G.Okun Georg f.hoffmann Division of Neuropediatric and Metabolic Medicine Department of General PediatricsCenter for Pediatric and Adolescent MedicineUniversity Hospital HeidelbergIm Neuenheimer Feld 43069120 HeidelbergGermany

Background Newborn screening (NBS) in Germany currently includes 15 target disorders. Recent diagnostic improvements suggest an extension of the screening panel. Methods Since August 2016, a prospective study evaluating 26 additional target disorders (25 metabolic disorders and vitamin B12-deficiency) in addition to the German screening panel is performed at the Newborn Screening Center Heidel-berg. first-tier results from tandem-MS screening are complemented by second-tier strategies for 15 of the additional target disorders. NBS results of seven patients diagnosed symptomatically with one of the additional target disorders by selective screening since August 2016 are retrospectively evaluated. Results Over a 13-month period, 68,418 children participated in the study. Second-tier analyses were performed in 5.4%of samples. Only 59 (0.1%) of study participants had abnormal screening results for one of the additional target disorders. Target disorders from the study panel were confirmed in 12 children: 13-hydroxy-3-methylglutaryl coenzyme A (CoA)-lyase deficiency, 1 citrullinemia type I, 1 multiple acyl-CoA dehydrogenase-deficiency, 1 methylenetetrahydrofolate reductase-deficiency, and 8 children with maternal vitamin B12-deficiency. In addition, six of seven patients diagnosed symptomatically outside the study with one of the target disorders would have been identified by the study strategy in their NBS sample. Conclusions Within 13 months, the study 'Newborn Screening 2020' identified additional 12 children with treatable con-ditions while only marginally increasing the recall rate by 0.1%. Maternal vitamin B12-deficiency was the most frequent finding. Even more children could benefit from screening for the additional target disorders by extending the NBS panel for Germany and/or other countries.

关键词： Metabolic disorders Newborn screening Second-tier Vitamin B12 deficiency

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

D2工具钢在气淬和回火过程中的各向异性畸变(英文)

引用

金属热处理 2004年第5期29卷 60-65页

作者：魏建飞 O.Kessler f.hoffmann P.Mayr 德国材料科学研究所

分别使用铸造试样及两种不同锻造程度的锻件试样 ,对高合金冷作模具钢D2在气淬和回火过程中的畸变行为进行了研究。试验结果表明 ,铸造试样的畸变是等向性的 ,而锻造试样的畸变显然呈各向异性。各向异性畸变程度随奥氏体化温度的升高而... 详细信息

分别使用铸造试样及两种不同锻造程度的锻件试样 ,对高合金冷作模具钢D2在气淬和回火过程中的畸变行为进行了研究。试验结果表明 ,铸造试样的畸变是等向性的 ,而锻造试样的畸变显然呈各向异性。各向异性畸变程度随奥氏体化温度的升高而增加。在本试验范围内 ,不同的锻造程度对各向异性的畸变程度影响不大。回火后各向异性畸变程度有所降低。使用测膨胀方法及金相检验探讨了产生各向异性畸变的可能机制。

关键词：工具钢气淬回火各向异性畸变冷作模具钢

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

“畸变工程”——生产过程中的畸变控制(英文)

引用

金属热处理 2004年第8期29卷 61-66页

作者： f.hoffmann O.Kessler Th.Luebben P.Mayr Stiftung Institut für Werkstofftechnik BremenGermanyStiftung Institut für WerkstofftechnikBremenGermanyStiftung Institut für WerkstofftechnikBremenGermanyStiftung Institut für WerkstofftechnikBremenGermany

直到最近为止 ,人们认为淬火是造成零件畸变的一个主要工艺过程 ,特别是淬火的具体操作过程。而今 ,众所周知淬火过程只不过是释放了零件在整个加工过程的一系列操作中积累起来的畸变潜能。然而 ,迄今为止 ,还没有一个以系统的方式来研... 详细信息

直到最近为止 ,人们认为淬火是造成零件畸变的一个主要工艺过程 ,特别是淬火的具体操作过程。而今 ,众所周知淬火过程只不过是释放了零件在整个加工过程的一系列操作中积累起来的畸变潜能。然而 ,迄今为止 ,还没有一个以系统的方式来研究和精确测定整个过程以及每一个加工步骤对零件的几何尺寸、成分偏析、显微组织和残余应力分布的影响。从 2 0 0 1年开始 ,由Deutscheforschungsgemeinschaft(德国研究基金 )在不来梅大学成立了“畸变工程”(SfB 5 70 )合作研究中心。

关键词：淬火成分偏析显微组织残余应力 “畸变工程” 畸变控制热处理

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Newborn screening for remethylation disorders and vitamin deficiency-evaluation of new strategies in cohorts from Qatar and Germany

引用

World Journal of Pediatrics 2017年第2期13卷 136-143页

作者： Gwendolyn Gramer Ghassan Abdoh Tawfeg Ben-Omran Noora Shahbeck Rehab Ali Laila Mahmoud Junmin fang-hoffmann Georg f.hoffmann Hilal Al Rifai Jürgen G.Okun University Hospital Heidelberg Center for Pediatric and Adolescent MedicineDivision of Neuropediatrics and Metabolic MedicineHeidelbergGermany Department of Pediatrics Hamad Medical CorporationDohaQatar

Background:Newborn screening is a precondition for early diagnosis and successful treatment of remethylation disorders and classical homocystinuria (cystathionine-β-synthase deficiency).Newborn screening for classical homocystinuria using total homocysteine measurement in dried blood spots has been very successfully performed for many years for newborns from ***:A new optimized newborn screening strategy for remethylation disorders and homocystinuria was developed and evaluated for newborns from Qatar using total homocysteine measurement as first-tier and methionine,methionine-phenylalanine-ratio and propionylcarnitine as *** cnt-offs were also retrospectively evaluated in newborn screening samples of 12 patients with remethylation disorders and vitamin B12 deficiency from Qatar and ***:Over a 12 months period,the proposed strategy led to a decrease in the recall rate in homocysteine screening for Qatar from 1.09% to 0.68%,while allowing for additional systematic inclusion of remethylation disorders and vitamin B12 deficiency into the screening panel for *** the evaluated period the applied strategy would have detected all patients with classical homocystinuria identified by the previous strategy and in addition 5 children with maternal nutritional vitamin B12 deficiency and one patient with an isolated remethylation *** retrospective evaluation of newborn screening samples of 12 patients from Germany and Qatar with remethlyation disorders or vitamin B12 deficiency showed that all of these patients would have been detected by the cutoffs used in the proposed new *** addition,an adapted strategy for Germany using methionine,methionine-phenylalanine-ratio and propionylcarnitine as first-tier,and homocysteine as a second-tier test was also positively evaluated ***:The proposed strategy for samples from Qatar allows inclusion of remethylation disorders and vitamin B12 deficiency

关键词： classical homocystinuria newborn screening remethylation disorders second-tier vitamin B12 deficiency

来源：

维普期刊数据库评论