A Chinese case of Wolman disease

1989 ◽  
Vol 12 (3) ◽  
pp. 328-328 ◽  
Author(s):  
T. -R. Wang ◽  
S. -M. Chuang
Keyword(s):  
Wolman Disease ◽  
Chinese Case

Lymphocyte-to-monocyte ratio in predicting the calcific aortic valve stenosis in a Chinese case–control study

2020 ◽  
Vol 14 (14) ◽  
pp. 1329-1339
Author(s):  
Xiaochun Ma ◽  
Huibo Ma ◽  
Yan Yun ◽  
Shanghao Chen ◽  
Xiaofeng Zhang ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Valve Stenosis ◽  
Case Control Study ◽  
Independent Predictor ◽  
Inverse Correlation ◽  
Case Control ◽  
Lymphocyte To Monocyte Ratio ◽  
Study Results ◽  
Chinese Case ◽  
Control Study

Aim: This study examined the role of lymphocyte-to-monocyte ratio (LMR), an inflammatory biomarker, in predicting the severity of calcific aortic valve stenosis (CAVS) in a Chinese case–control study. Results: The LMR significantly decreased in the patients with CAVS compared with healthy controls. An inverse correlation was observed between the severity of stenosis and LMR in the patients. Additionally, the LMR was identified in the multivariate analysis as an independent predictor of severe CAVS. Conclusion: This study provides evidence of an inverse correlation between the severity of CAVS and LMR. LMR could potentially be applied as an independent predictor of severe CAVS and could be incorporated into a novel predictive model.

scholarly journals Enzyme replacement therapy and hematopoietic stem cell transplant: a new paradigm of treatment in Wolman disease

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Jane E. Potter ◽  
Gemma Petts ◽  
Arunabha Ghosh ◽  
Fiona J. White ◽  
Jane L. Kinsella ◽  
...  
Keyword(s):  
Stem Cell ◽  
Multimodal Therapy ◽  
Hematopoietic Stem Cell Transplant ◽  
Stem Cell Transplant ◽  
Venous Access ◽  
Cell Transplant ◽  
New Paradigm ◽  
Hematopoietic Stem ◽  
Wolman Disease ◽  
Enzyme Replacement

Abstract Background Wolman disease is a rare, lysosomal storage disorder in which biallelic variants in the LIPA gene result in reduced or complete lack of lysosomal acid lipase. The accumulation of the substrates; cholesterol esters and triglycerides, significantly impacts cellular function. Untreated patients die within the first 12 months of life. Clinically, patients present severely malnourished, with diarrhoea and hepatosplenomegaly, many have an inflammatory phenotype, including with hemophagocytic lymphohistiocytosis (HLH). Hematopoietic stem cell transplant (HCT) had been historically the only treatment available but has a high procedure-related mortality because of disease progression and disease-associated morbidities. More recently, enzyme replacement therapy (ERT) with dietary substrate reduction (DSR) has significantly improved patient survival. However, ERT is life long, expensive and its utility is limited by anti-drug antibodies (ADA) and the need for central venous access. Results We describe five Wolman disease patients diagnosed in infancy that were treated at Royal Manchester Children's Hospital receiving ERT with DSR then HCT—multimodal therapy. In 3/5 an initial response to ERT was attenuated by ADA with associated clinical and laboratory features of deterioration. 1/5 developed anaphylaxis to ERT and the other patient died post HCT with ongoing HLH. All patients received allogeneic HCT. 4/5 patients are alive, and both disease phenotype and laboratory parameters are improved compared to when they were on ERT alone. The gastrointestinal symptoms are particularly improved after HCT, with reduced diarrhoea and vomiting. This allows gradual structured normalisation of diet with improved tolerance of dietary fat. Histologically there are reduced cholesterol clefts, fewer foamy macrophages and an improved villous structure. Disease biomarkers also show improvement with ERT, immunotherapy and HCT. Three patients have mixed chimerism after HCT, indicating a likely engraftment-defect in this condition. Conclusion We describe combined ERT, DSR and HCT, multimodal treatment for Wolman disease. ERT and DSR stabilises the sick infant and reduces the formerly described prohibitively high, transplant-associated mortality in this condition. HCT abrogates the problems of ERT, namely attenuating ADA, the need for continuing venous access, and continuing high cost drug treatment. HCT also brings improved efficacy, particularly evident in improved gastrointestinal function and histology. Multimodal therapy should be considered a new paradigm of treatment for Wolman disease patients where there is an attenuated response to ERT, and for all patients where there is a well-matched transplant donor, in order to improve long term gut function, tolerance of a normal diet and quality of life.

Can tourism help to revive shrinking cities? An examination of Chinese case

2021 ◽  
pp. 135481662110022
Author(s):  
Taotao Deng ◽  
Shuai Liu ◽  
Yukun Hu
Keyword(s):  
Panel Data ◽  
Population Growth ◽  
Urban Economy ◽  
Tourism Industry ◽  
Shrinking Cities ◽  
Rapid Urbanization ◽  
Positive Effects ◽  
Population Loss ◽  
Chinese Case ◽  
Economic Boom

Urban shrinkage has become a global phenomenon. Although China is still experiencing rapid urbanization, population losses arise in an increasing number of cities. As a booming industry, tourism is expected as a mean to create jobs and curb population loss. Can tourism industry contribute to revive the shrinking cities? Based on panel data of 54 shrinking cities in China, this article explores effects of tourism development on shrinking cities. The results show that there is no evidence that tourism has a significant impact on the population of the shrinking cities. However, tourism shows positive effects on these cities in terms of many aspects, including urban economy, employment, investment, and consumption. This indicates that tourism can revive shrinking cities by economic boom, rather than population growth.

scholarly journals Understanding Social Security Development: Lessons From the Chinese Case

SAGE Open ◽  
2021 ◽  
Vol 11 (3) ◽  
pp. 215824402110326
Author(s):  
Guan Huang ◽  
Zhuang Cai
Keyword(s):  
Social Security ◽  
Political Legitimacy ◽  
Political Context ◽  
Common Mechanism ◽  
Social Security Systems ◽  
Security Systems ◽  
Security Research ◽  
Chinese Case ◽  
Thematic Approach ◽  
Convergence And Divergence

Understanding the development of social security systems constitutes the ultimate goal of social security research. This review traces and compares two schools of thought regarding social security development: the convergence and divergence schools. Using a thematic approach, this article first categorizes extant studies into one of these two schools and then identifies the broadly accepted mechanism of social security development by comparing them. After reviewing the extant research and its theoretical underpinnings, this article applies Mill’s methods of agreement and difference to show how the Chinese case contributes to and challenges our understanding of social security development. By discussing the assumptions of current research on social security development in light of the Chinese case, this article illuminates how political legitimacy serves as a common mechanism of social security development regardless of political context or structure.

scholarly journals Coil-Assisted Retrograde Transvenous Obliteration for Gastric Varices in a Chinese Case

2021 ◽  
Author(s):  
Peng-Xu Ding ◽  
Chao Liu ◽  
Xin-Wei Han ◽  
Edward Wolfgang Lee
Keyword(s):  
Gastric Varices ◽  
Chinese Case

Regional innovation environment and innovation efficiency: the Chinese case

2015 ◽  
Vol 28 (4) ◽  
pp. 396-410 ◽  
Author(s):  
Shuai Wang ◽  
Jin Fan ◽  
Dingtao Zhao ◽  
Shanyong Wang
Keyword(s):  
Regional Innovation ◽  
Chinese Case

scholarly journals Infection rate of Eperythrozoon spp. in Chinese population: a systematic review and meta-analysis since the first Chinese case reported in 1991

2012 ◽  
Vol 12 (1) ◽  
Author(s):  
De-Sheng Huang ◽  
Peng Guan ◽  
Wei Wu ◽  
Tie-Feng Shen ◽  
He-Ling Liu ◽  
...  
Keyword(s):  
Systematic Review ◽  
Infection Rate ◽  
Chinese Population ◽  
Meta Analysis ◽  
Chinese Case

Primary multiple miliary osteoma cutis: an unusual Chinese case

2013 ◽  
Vol 53 (1) ◽  
pp. 73-75 ◽  
Author(s):  
Hui-Jun Ma ◽  
Chi-Yu Jia ◽  
Yang Yang ◽  
Lian-Jin Song ◽  
Rong Hu ◽  
...  
Keyword(s):  
Osteoma Cutis ◽  
Chinese Case

Topics, Aims, and Constraints in English Teacher Research: A Chinese Case Study

2009 ◽  
Vol 43 (1) ◽  
pp. 113-125 ◽  
Author(s):  
ALI SHEHADEH ◽  
JOHN LEVIS ◽  
GARY BARKHUIZEN
Keyword(s):  
Teacher Research ◽  
English Teacher ◽  
Chinese Case
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