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1.
Wibke Johannis Jenny Blommer Andreas R. Klatt Joerg H. Renno Klaus Wielckens 《Biochemia medica : ?asopis Hrvatskoga dru?tva medicinskih biokemi?ara / HDMB》2012,22(3):373-379
Introduction:
Heavy chain diseases (HCD) are neoplastic proliferations of B cells which secrete truncated immunoglobulin heavy chains without associated light chains. Being rare and probably underdiagnosed diseases the aim of this report is to show an additional case of gamma heavy chain disease in a 48 year old female patient with rheumatoid arthritis focusing on the laboratory presentation.Materials and methods:
Laboratory work-up included agarose gel electrophoresis (AGE), capillary zone electrophoresis (CZE), immunofixation and nephelometrically determined immunoglobulin and immunoglobulin subclasses of the patient’s serum. Urine samples were also subjected to immunofixation and to a SDS-PAGE with consecutive immunoblot.Results:
Nephelometrically measured elevated IgG concentrations were noted in combination with a decreased gamma globulin region and an increased beta globulin region on AGE. A definite monoclonal spike was not identified on AGE but at least suspected on CZE; finally serum and urine immunofixation demonstrated a monoclonal gamma heavy chain devoid of any corresponding light chains confirming the diagnosis of HCD. Analysis of the gamma heavy chain (HC) with means of SDS-PAGE revealed proteins of 40 kD and 80 kD most likely presenting a truncated HC in its monomeric and dimeric form and possibly leading to the failure of IgG-subclass typing with the applied IgG subclass antisera.Conclusion:
This case report illustrates a new case of gamma HCD demonstrating variable laboratory manifestations and therefore the need for heightened awareness concerning this disease when confronted with abnormal and discrepant protein profiles in routinely applied laboratory tests. 相似文献2.
Nihar Ranjan Dash Biswajit Mohanty 《Indian journal of clinical biochemistry : IJCB》2012,27(1):100-102
Multiple myeloma is a group of B-cell disorders resulting in the secretion of a specific and unique monoclonal immunoglobulin
(M-protein). Protein electrophoresis is advised whenever multiple myeloma is suspected. The monoclonal protein migrates as
a single entity in the electric field and is detected by the non-specific protein stain as a more intensely stained band superimposed
on the usual protein pattern. The M-protein usually migrates in the gamma or beta region of the normal protein pattern; very
rarely it may appear in the α2 or even in α1 region. Here we have given an atypical case presentation where the patient with
multiple myeloma presented with two M-spike one each in α2 and β-globulin region on agarose gel protein electrophoresis with
hypoglobulinemia but with reversed A:G ratio. 相似文献
3.
T. Malati B. Yadagiri D. Murali Mohan Krishna V. Shantaram D. Raghunadharao K. Subbarao 《Indian journal of clinical biochemistry : IJCB》2001,16(1):52-59
In the present study, monoclonal gammapathy was identified in a total of 245 patients of plasma cell dyscrasias during period
of 1987 to 2000. The monoclonal band was identified in serum by agar gel electrophoresis in all the cases and in urine in
a few cases. Characterization of paraprotein (monoclonal immunoglobulin class and light chain type) was carried out by employing
immunoelectrophoresis and/or immunofixation electrophoresis using heavy chain specific gamma, alpha, mu, delta and epsilon
and light chain specific kappa (K), lambda (λ) antisera. Serum immunoglobulins Ig G, Ig A, and Ig M were estimated by immunoturbidometry.
Serum urea, creatinine, uric acid, alkaline phosphatase, total proteins, albumin, calcium and phosphorus were estimated by
using routine biochemical methods. Among the 245 cases, 73.1% monoclonal gammapathies were of secretory type and 7.3% were
non-secretory. Monoclonal gammapathies were associated with 80.4% of multiple myeloma, 8.9% of solitary plasmacytoma, 4.1%
of extra-medullary plasmacytoma, 3.3% of lymphoma and 2.9% of plasma cell leukemia. Classification of secretory monoclonal
immunoglobulin revealed monoclonal immunoglobulin Ig G in 74%, Ig A 15% and Ig M in 2.9% cases. 相似文献
4.
T. Malati 《Indian journal of clinical biochemistry : IJCB》2001,16(1):1-8
The neoplastic proliferation of single clones of plasma cells causes synthesis of very large amount of monoclonal immunoglobulins
consisting of only one type of heavy either the gamma, alpha, mu, delta or epsilon chain or only kappa or lambda light chains.
Each monoclonal immunolobulin differs idiotypically from each other. These monoclonal immunoglobulins are also called paraproteins
and are frequently associated with a broad heterogeneous group of plasma cell dyscrasias. Occasionally their presence is observed
in a few benign conditions and in old age. In the present review a detailed account of different types of monoclonal gammapathies
are described. 相似文献
5.
We report a case of hemolytic anemia that was subsequently identified to be a case of α-thalassaemia harboring the common rightward 3.7 kb deletion/HbH. The diagnosis was based on sequential analyses using BioRad D10 HPLC, Alkaline gel electrophoresis, GPO α THAL-IC strips and the identification of the specific genetic lesion using an α Globin reverse dot blot hybridization assay. Supravital stain of RBCs helped in identifying classical HbH inclusions. In a background of a variable clinical presentation, lack of definitive hematological markers, and general under-diagnosis of α-thalassaemias we have used this case to highlight the features and sequence of techniques involved in identifying and characterizing an α-globin chain mutation, starting from a diffuse clinical history and presentation up to the identification of a specific genetic lesion involved. 相似文献
6.
Moushumi Lodh Binita Goswami Nikhil Gupta Surajeet K. Patra Alpana Saxena 《Indian journal of clinical biochemistry : IJCB》2012,27(4):410-413
Multiple myeloma is a disseminated malignancy of monoclonal plasma cells that accounts for 15 % of all hematological cancers. The present study was conducted to evaluate the role of inflammation and oxidant-antioxidant dynamics in the etiology of this disease. The study population comprised of 20 cases of multiple myeloma and 20 healthy controls. The parameters evaluated were serum malondialdehyde (MDA), superoxide dismutase (SOD) and ferritin levels. The serum MDA levels were 1.9 ± 0.96 nmol/ml in cases as compared to 0.98 ± 0.55 nmol/ml in the controls. Similarly, a statistically significant difference was noted in the SOD and ferritin levels between the cases and controls (93.2 ± 23.8 vs. 210.1 ± 190.5 U/ml and 285.8 ± 216.4 vs. 131.8 ± 30.1 ng/ml respectively). Our study highlights the imbalance in the oxidant-anti oxidant mechanism and the role of smoldering inflammation in the etiology of multiple myeloma. 相似文献
7.
8.
Vijay S. Bhat K. K. Dewan P. R. Krishnaswamy A. K. Mandal P. Balaram 《Indian journal of clinical biochemistry : IJCB》2010,25(1):99-104
Hemoglobin Q- India (alpha) 64 Asp → His is an alpha chain variant which is generally found in heterozygous state and presents
normal hematological blood picture. Here we report a rare case of HbQ-India with a thalassemic phenotype that has been analyzed
using a combination of mass spectrometry, gene sequencing and PCR analysis. This combined analyses revealed the HbQ variant
to be associated with a beta chain mutation, IVS 1-1 [G>T]. Though HbQ has earlier been reported with thalassemic trait using
different techniques, this is the first report of a compound α and β chain Hb heterozygous mutant involving HbQ and IVS1-1
being validated using Mass Spectrometry and Reverse dot blot hybridization. 相似文献
9.
快节奏的市场竞争加速了产品更新换代,为了提升竞争力,企业研发活动逐渐外部化,研发活动呈现产业化趋势.研发活动产业化的基础是,有一个专门从事研发活动的主体,这一新兴的“主体”在运作模式上,具有一些不同于其他企业主体的特征.研究借鉴国外研发主体运行机制,对于我国研发主体再造、发展研发产业具有重要意义.以MR＆D公司为例,作了这方面的探讨. 相似文献
10.
Udit Agrawal Ajay Meshram Jayant Vagha Kirti Swarnkar Kamlesh Palandurkar 《Indian journal of clinical biochemistry : IJCB》2012,27(4):417-421
Mucopolysaccharidoses (MPS) are a group of genetic diseases and its diagnosis is a challenging task due to multiple differential diagnosis. We had combined clinical findings, radiological and ophthalmological features. Biochemical test for urine glycosaminoglycans (GAG) was done for confirmation of diagnosis in the patient. The case of Sanfilippo disease was characterized by slowly progressive, severe CNS involvement with mild somatic disease. Radiological features were suggestive of Sanfilippo disease and urine GAG test for MPS was positive in the case. With the clinical features we had multiple differential diagnoses. The radiological investigations minimized the list and the biochemical test confirmed GAG in urine. In this case the combination of clinical, radiological and biochemical findings confirmed the diagnosis of Sanfilippo disease. 相似文献
11.
M. Shafi M. Zargar Z. A. Shah M. Salahuddin M. Siddiqi 《Indian journal of clinical biochemistry : IJCB》1998,13(2):106-110
Serum and urine samples from 513 patients clinically suspected of monoclonal gammopathies over a period of five years (1992–97)
were subjected to various immunological procedures viz, electrophoresis, immunoelectrophoresis and immunoglobulin estimations.
Laboratory investigations confirmed gammopathies in 10.33%. It was observed that overall age of incidence for monoclonal gammopathies
in both sexes was between 42–72 years with a male to female ratio of 1.4∶1. Predominant paraprotein detected was IgG type
(75.47%) followed by IgA (16.98%) and Bence Jones proteins (7.55%). Amongst positive patients, 64.16% were having kappa (k) type light chains and 35.84% lambda (δ) type light chains. 69.39% patients with serum M component (IgG and IgA) had Bence
Jones proteinuria. Densitometric scanning revealed that majority of IgG type paraprotein was found in the slow gamma globulin
region and majority of IgA type paraprotein was found equally distributed between beta and fast gamma globulin regions. Both
types had decreased albumin and alpha-2-globulin concentrations as compared to normal controls. Immunoglobulin levels in patients
with paraprotein had very high levels of serum IgG (6467.0 mg%) and IgA (2714.0 mg%) in respective types of monoclonal gammopathies;
the rest of immunoglobulin classes were either at normal or decreased levels. 相似文献
12.
Moushumi Lodh Rajarshi Mukhopadhyay Balwant Kumar 《Indian journal of clinical biochemistry : IJCB》2015,30(2):234-237
Hemoglobin is a tetramer formed of two alpha and two beta globin chains. On exposure to high levels of blood glucose, hemoglobin gets non-enzymatically glycated at different sites in the molecule. HbA1c is formed when glucose gets added on to the N-terminal valine residue of the beta chain of hemoglobin. The development of chronic vascular complications of diabetes such as retinopathy, nephropathy and cardiovascular disease is intimately linked to the level of glycemic control attained by the individual with diabetes. We report a case of convulsions and monoplegia admitted to emergency department, showing unusually high glycated hemoglobin but plasma glucose not as high. The patient was not a known diabetic and we could not find any of the other documented conditions that are known to elevate glycated hemoglobin to such disproportionately high levels. Screening for abnormal hemoglobins was negative in the patient. Oral hypoglycemic drug treatment over 3 months and withdrawal of other medications only marginally lowered glycated hemoglobin. 相似文献
13.
V. G. Justin S.D Tarey T. Venkatesh 《Indian journal of clinical biochemistry : IJCB》1999,14(2):249-251
Routine investigations of a 70 year old male led to provisional diagnosis of anemia. However further investigations suggested
the possibilities of carcinoma of stomach associated with pernicious anemia, multiple myeloma and megaloblastic anemia. Finally
serum protein electrophoresis supported by the clinical suspicion confirmed multiple myeloma. 相似文献
14.
Vijay S. Bhat Amit Kumar Mandal Bobby Mathew 《Indian journal of clinical biochemistry : IJCB》2012,27(4):414-416
Hemoglobin J-Rajappen (alpha)90 Lys → Thr is an alpha chain variant found in heterozygous state and presents normal hematological blood picture. Due to the ambiguity in results obtained while analyzing by HPLC and alkaline gel electrophoresis, we report this rare case of HbJ-Rajappen using non denaturing gel electrophoresis and matrix assisted laser desorption ionization mass spectrometry. Though HbJ-Rajappen has earlier been reported using different techniques, this is the first report being validated using mass spectrometry technique. 相似文献
15.
供应链敏捷性的以往研究忽视了供应链企业间竞合关系的存在,且集体横向竞合对供应链敏捷性的影响及其作用机制仍不清晰。本文基于动态能力理论、社会网络理论以及组织学习理论,深入探讨了集体横向竞合对公司供应链敏捷性的影响机制。采用Bootstrap检验方法对216份专业市场中大型企业的问卷开展实证研究发现：集体横向竞合能直接或间接地对供应链敏捷性产生正向且显著的影响。集体横向竞合与供应链敏捷性之间存在三条中介路径,其中网络嵌入性中介路径对供应链敏捷性的影响最大,其次是组织学习能力中介路径,而网络嵌入性与组织学习能力的链式中介路径对供应链敏捷性的影响最小,且网络嵌入性是集体横向竞合与组织学习能力之间的唯一中介。 相似文献
16.
基于创新链视角,探究高新技术企业认定对企业创新的影响及其作用机制。以沪深A股上市公司为样本,研究发现：高新技术企业认定对企业创新绩效的影响存在多重路径,创新投入和成果产出在其过程中分别发挥中介作用,且创新投入与成果产出的链式中介效应显著。进一步,从信号机制、杠杆机制和触发机制三方面,揭示高新技术企业认定影响企业创新过程的内在机制。结果显示：在创新投入阶段,高新技术企业认定的信号机制激励外源融资进入创新链,提升企业研发投入;在成果产出阶段,高新技术企业认定发挥杠杆作用,撬动企业寻求产学研合作,促进企业科技成果产出;而高新技术企业认定是否能触发企业创新绩效提升,认定质量是关键情境变量。根据研究结论,提出了相应政策建议。 相似文献
17.
已有研究侧重于探索供应商如何为制造企业提升研发创新,但忽视了非研发创新的内在价值。事实上,非研发创新同样是提升企业创新绩效的重要途径,鲜有研究探明供应链情境下非研发创新的结构与前因。基于社会资本理论,破解如何提升研发创新与非研发创新,并探明供应商为制造商带来更多的研发创新还是非研发创新。选取538份有效问卷,采用结构方程模型验证,实证结果表明：（1）制造商——供应商社会资本各维度均正向影响研发创新与非研发创新;（2）相对于研发创新,结构资本与认知资本更能提升制造企业非研发创新;而相对于非研发创新,关系资本更能提升制造企业研发创新;（3）社会资本各维度对非研发创新的各维度（产品或工艺改进、模仿创新、技术引进与市场创新）均产生正向影响。研究结论厘清了制造企业制造商——供应商社会资本不同维度提升研发创新/非研发创新的差异性影响,为制造企业提升研发创新/非研发创新提供理论解释与实践启示。 相似文献
18.
Emma Whittle Elzahn de Waal Tony Huynh Oliver Treacy Adam Morton 《Biochemia medica : ?asopis Hrvatskoga dru?tva medicinskih biokemi?ara / HDMB》2021,31(1)
We describe a case of severe hypervitaminosis D and mild hypercalcaemia in a 68-year-old woman who presented with fatigue and weight loss. Her 25-hydroxy vitamin D (25OHD) was > 400 nmol/L (50-150) and corrected serum calcium was 2.83 mmol/L (2.1-2.6). Her intact parathyroid hormone (PTH) was 4.9 pmol/L (2.0-9.5). Further investigation revealed an IgM kappa paraprotein, and a bone marrow aspirate confirmed a diagnosis of lymphoplasmacytic lymphoma/Waldenstrom’s macroglobulinemia (LPL/WM). As the vitamin D level was discordant with the patient’s other results and presentation, the presence of an assay interferent was suspected. A 1-in-2 dilution of the sample returned a 25OHD result of 84 nmol/L in keeping with the presence of an interferent. Testing for rheumatoid factor was negative. The sample was treated with an antibody blocking reagent (Scantibodies) and results were not consistent with heterophile antibody interference. The sample was then analysed using liquid chromatography tandem mass spectrometry (LC-MS/MS), which returned a 25OHD result of 82 nmol/L. Testing on an alternative immunoassay platform produced a 25OHD result of 75 nmol/L. Reapeted testing on the original platform following reduction of the monoclonal paraprotein with chemotherapy, returned a result of 64 nmol/L. The patient’s mild hypercalcaemia persisted following resolution of the monoclonal paraprotein, in keeping with a diagnosis of primary hyperparathyroidism. This case highlights the potential for paraproteins to cause assay interference, and the importance of considering interference when results are incongruous with the clinical presentation. 相似文献
19.
Theoretical, empirical and anecdotal evidence suggests that there are more violations of sustainability principles in supply chains in developing countries than in developed countries. Recent research has demonstrated that blockchain can play an important role in promoting supply chain sustainability. In this paper we argue that blockchain’s characteristics are especially important for enforcing sustainability standards in developing countries. We analyze multiple case studies of blockchain projects implemented in supply chains in developing countries to assess product quality, environmental accounting and social impact measurement. We have developed seven propositions, which describe how blockchain can help address a number of challenges various stakeholders face in promoting sustainable supply chains in developing countries. The challenges that the propositions deal with include those associated with an unfavorable institutional environment, high costs, technological limitations, unequal power distribution among supply chain partners and porosity and opacity of value delivery networks. 相似文献
20.
基于非竞争型投入占用产出模型,提出了一个分析高技术产业国内价值链与全球价值链相互关联的新框架,即以改进的显示性比较优势指数加权的国内完全增加值衡量全球价值链地位,以国内完全消耗系数的部门构成衡量国内价值链状况,两者通过国内投入结构相互影响。通过历史时期的纵向比较与跨国的横行比较、以及实证分析发现,国内价值链的高级化会明显地促进全球价值链地位的提升：(1)高端型和生产型投入对全球价值链地位提升具有明显的促进作用;(2)资源型投入越多反而不利于一国产业全球价值链地位的提升;(3)中国已逐步由资源型投入为主转到以生产型为主导,但高端型投入的严重低下,将是制约中国高技术产业全球价值链升级的瓶颈。最后,基于实证分析提出了针对中国高技术产业发展的政策建议。 相似文献