1.Advances in the treatment of infantile hemangiomas of the larynx.
Xing GAO ; Xiaojun ZHAN ; Qinglong GU
Journal of Clinical Otorhinolaryngology Head and Neck Surgery 2023;37(12):972-976
Infantile hemangiomas are relatively common soft tissue tumors in infants and young children, with a prevalence of about 4.5% in full-term newborns. Subglottic Hemangioma (SGH) is a relatively rare type of hemangioma, and its special location often causes respiratory distress and potentially life-threatening conditions in infants. Therefore, it is necessary for clinicians to make an accurate diagnosis and formulate a detailed treatment plan based on the clinical manifestations, the auxiliary examinations, the medical history and the vital signs evaluation of patients.This review describes the pathophysiological mechanism of infantile hemangioma and provides a detailed discussion on commonly used treatment methods in detail.
Child, Preschool
;
Humans
;
Infant
;
Infant, Newborn
;
Hemangioma/diagnosis*
;
Hemangioma, Capillary
;
Laryngeal Neoplasms/surgery*
;
Larynx/pathology*
;
Soft Tissue Neoplasms
2.Research Progress on Capillary Leak Syndrome Associated with Hematopoietic Stem Cell Transplantation--Review.
Journal of Experimental Hematology 2023;31(4):1237-1241
Capillary leak syndrome (CLS) is a clinical syndrome characterized by impairment of vascular endothelial barrier function, increased vascular permeability, and reversible systemic edema. It is one of the early fatal complications after hematopoietic stem cell transplantation. So far, the exact pathogenesis of CLS has not been elucidated, and the diagnostic criteria and treatment methods have not been unified. At present, it is believed that the fundamental cause of CLS is hypercytokinemia, and the core factor is high permeability of vascular endothelial cells. According to the clinical manifestations, the natural course of CLS can be divided into prodrome, leakage and recovery stages. As far as treatment is concerned, symptomatic and supportive treatment is dominant according to different characteristics of each stage. In this review, the pathogenesis, clinical manifestations, diagnosis and treatment of hematopoietic stem cell transplant-associated CLS were briefly summarized.
Humans
;
Capillary Leak Syndrome/diagnosis*
;
Endothelial Cells
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Hematopoietic Stem Cell Transplantation/adverse effects*
3.Carrier screening and prenatal diagnosis for thalassemia-associated mutations in Jiaxing area of Zhejiang.
Huling JIANG ; Chiyan ZHOU ; Li YANG ; Suping LI ; Xiaodan LIU
Chinese Journal of Medical Genetics 2023;40(3):295-300
OBJECTIVE:
To study the molecular epidemiology of thalassemia in Jiaxing area of Zhejiang province and provide a basis for prenatal diagnosis, genetic counseling and prevention and control of birth defects.
METHODS:
A total of 24 003 pregnant women who presented at the Jiaxing Maternal and Child Health Care Hospital from April 2017 to September 2021 were enrolled. Capillary hemoglobin electrophoresis in combination with routine blood test were used for primary screening for carriers of thalassemia-associated mutations, and those with positive results were subjected to fluorescence quantitative PCR assay. Prenatal diagnosis was provided for couples with a risk of giving birth to children with intermediate or severe thalassemia.
RESULTS:
Among the 24 003 pregnant women, 1 211 cases were suspected as carriers of thalassemia-associated mutations, among whom 443 (36.58%) were confirmed by genetic testing. Among these, carriers of α-, β- and α-complex β-globin gene mutations have accounted for 27.31% (121/443), 70.65% (313/443) and 2.04% (9/443), respectively. The result of prenatal diagnosis for an at-risk couple was --SEA/αCSα, and the fetus was predicted to have intermediate or severe thalassemia. Termination of the pregnancy was recommended.
CONCLUSION
Hemoglobin electrophoresis combined with routine blood test during pregnancy may be used as a preliminary screening measure for carriers of thalassemia-associated variants. Combined with genetic testing, this will be of great significance for the control of thalassemia in this region.
Female
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Humans
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Pregnancy
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Electrophoresis, Capillary
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Genetic Counseling
;
Genetic Testing
;
Mutation
;
Prenatal Diagnosis
;
Thalassemia/genetics*
6.Determination of five saponins in Xuesaitong Dropping Pills by micellar electrokinetic chromatography and evaluation method of between-batch consistency.
Shu-Nan LI ; Yi-Zhe HOU ; Le PENG ; Pian LI ; Xiang CAI ; Zheng LI ; Wen-Long LI
China Journal of Chinese Materia Medica 2021;46(22):5832-5838
The present study determined five saponins in Xuesaitong Dropping Pills(XDP) by micellar electrokinetic chromatography(MEKC), and evaluated between-batch consistency by MEKC fingerprints and similarity analysis. A background buffer was composed of 20 mmol·L~(-1) sodium tetraborate-20 mmol·L~(-1) boric acid solution(pH 8.5), 55 mmol·L~(-1) sodium dodecyl sulfate(SDS), 23 mmol·L~(-1) β-cyclodextrin, and 13% isopropyl alcohol. All separations were performed at 25 ℃,20 kV and the detection wavelength was set at 203 nm. The separation channel was a fused silica capillary with a dimension of 75 μm I.D. and a total length of 50.2 cm(effective length of 40.0 cm). The contents of notoginsenoside R_1, and ginsenosides Rg_1, Re, Rb_1, Rd were determined with their quality control ranges set. The fingerprints of XDP were established and the between-batch consistency was evaluated by similarity analysis. The contents of five saponins from the 19 batches of XDP were stable in the fixed ranges. Statistical analysis was carried out on the results of multiple batches of samples, and the specific quality control ranges were recommended as follows: notoginsenoside R_1 21.92-34.16 mg·g~(-1), ginsenosides Rg_1 83.54-131.78 mg·g~(-1), ginsenosides Re 13.58-19.82 mg·g~(-1), ginsenosides Rb_1 89.40-129.90 mg·g~(-1), and ginsenosides Rd 22.34-35.67 mg·g~(-1). Eleven characteristic peaks were identified in the fingerprints. Five peaks, notoginsenoside R_1 and ginsenosides Rg_1, Re, Rb_1, Rd, were identified with reference standards. The similarities of the 19 batches of samples were all above 0.988, indicating good between-batch consistency. This method is green and simple, and can be used for the quantitative determination and quality evaluation of XDP. It can also provide references for the quality control of other Chinese medicinal dripping pills.
Chromatography, Micellar Electrokinetic Capillary
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Drugs, Chinese Herbal
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Micelles
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Quality Control
;
Saponins
7.β-Thalassemia among Newborns in Wuhan Region and Its Influence Factors.
Bin ZHOU ; Qian XIONG ; Wen-Qian CAI ; Xi-Jiang HU ; Shan-Shan SHEN
Journal of Experimental Hematology 2021;29(1):198-202
OBJECTIVE:
To investigate the screening of β-thalassemia among newborns in Wuhan region, so as to explore the influencing factors of Hb A in dried blood spot.
METHODS:
Concentrations of Hb A,Hb A2,Hb F in the dried blood spots collected from 99 275 neonates in Wuhan region were analyzed by Sebia capillary electrophoresis. The screening result of β-thalassemia was interpretated accroding to the ratio of each group, the suspicious β-thalassemia newborns were recalled and the gene of thalassemia in those newborns was checked.
RESULTS:
Among 99 275 newborns, 1 408 positive patients were found, and the positive rate of screening was 1.41%. A total of 350 patients with gene mutation were found among 709 β-thalassemia suspicious patients. There were significantly statistical differences of positive predictive value among Hb A levels in different groups and there were also significantly statistical differences of positive predictive values among gestational weeks in different groups. No significantly statistical differences were observed among different genetic defects and phenotypes of heterozygous β-thalassemia in Hb A concentrations. Postnatal day and gestational age were significantly and positively associated with Hb A concentrations.
CONCLUSION
The capillary electrophoresis is an effective screening method for β-thalassemia of full-term neonate. Postnatal day and gestational age is associated with the pencentage of Hb A.
Electrophoresis, Capillary
;
Humans
;
Infant, Newborn
;
Mass Screening
;
Mutation
;
Thalassemia
;
beta-Thalassemia/genetics*
8.Prognostic value of the extravascular lung water and pulmonary vascular permeability indices in severe adult respiratory distress syndrome managed with extracorporeal membrane oxygenation.
Jin WEI ; Lei HUANG ; Lei XU ; Xiao-Min HU ; Xin-Jing GAO ; Zhi-Bo LI ; Da-Wei DUAN ; Peng WU ; Yu-Heng LANG ; Wen-Qing GAO ; Ying-Wu LIU ; Meng NING ; Tong LI
Chinese Medical Journal 2020;133(20):2501-2503
9.Analysis of 34 800 cases of Abnormal Hemoglobinopathy in Couples of Child-bearing Age in Chongqing Area.
Chun-Li LI ; Mei YANG ; Qiu-Hong LI
Journal of Experimental Hematology 2020;28(4):1316-1320
OBJECTIVE:
To explore the abnormal hemoglobinopathy in couples of child-bearing age in Chongqing.
METHODS:
A total of 34 800 subjects of child-bearing age were screened for thalassemia by using capillary electrophoresis from January 2015 to September 2018. PCR-flow cytometry fluorescence hybridization assay was used to detect the common thalassemia gene deletions and mutations.
RESULTS:
8 kinds of abnormal hemoglobinopathy were detected in 200 cases from 34 800 subjects of child-bearing age, the detection rate was 0.57% in couples of child-bearing age in Chongqing: Among 200 cases of abnormal hemoglobin pathy, Hb E was found in 90 cases (accounting for 45.0%), and Hb D in 25 cases (accounting for 12.5%). Hb NewYork was found in 25 cases (accounting for 12.5%). HbJ-bangkok was found in 25 cases (accounting for 12.5%), and Hb Q-Thailand in 16 cases (accounting for 8.0%). Hb Hope was detected in 15 cases (accounting for 7.5%). Hb S was detected in 3 cases (accounting for 1.5%). Hb Hasharon was detected in 1 case (accounting for 0.5%). The mean corpuscular volume (MCV) and mean corpuscular hemoglobin (MCH) of Hb E and Hb Q-Thailand were lower than normal reference intervals.
CONCLUSION
The detection rate of abnormal hemoglobinopathy in Chongqing is higher than the average level in China. Capillary electrophoresis can effectively screen abnormal hemoglobinopathy, which is great significant for aristogenesis and improvement of population quality.
Child
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China
;
Electrophoresis, Capillary
;
Hemoglobinopathies
;
Hemoglobins, Abnormal
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Humans
;
Thailand
;
Thalassemia
10.Development and Application of Automatic Analysis and Surveillance Platform for Chrimerism in Donors and Recipients after Allo-HSCT.
Jian-Cheng FANG ; Fang WANG ; Li-Li YUAN ; Mian-Mian WANG ; Ting-Ting LI ; Yi-Hang YANG ; Yang LIU ; Xiao-Li MA ; Xue CHEN ; Yang ZHANG ; Dai-Jing NIE ; Jia-Qi CHEN ; Hong-Xing LIU
Journal of Experimental Hematology 2020;28(3):1012-1018
OBJECTIVE:
To develop an automated chimeric analysis and reporting platform based on short tandem repeat (STR) and capillary electrophoresis methods for allogeneic hematopoietic stem cell transplantation (allo-HSCT) so as to improve work efficiency.
METHODS:
Apache, MySQL, PHP and HTML5 were used to build the database and interface. The STR locus geno typing and chimeric analysis logic and flow were set up on the basis of STR rules and capillary electrophoresis. STR genotyping and 194 times of chimeric testing data of 100 patients after allo-HSCT were used to test the platform for automatic STR locus genotyping, chimeric calculation and report generation.
RESULTS:
The established platform could realize the functions of STR locus customization, STR genotype determination, automatic chimeric analysis, and detection information database management, which can automatically generate an integrated report including multiple sequential chimeric results and trend graphs for the same patient and can be accessed and used simultaneously by different users through different browser interfaces. The results of automated analysis by the platform are completely consistent with that of manual analysis by experienced technicians, and the possibility of manual analysis error is reduced through automation. The time required for automatic analysis using this platform is approximately 1/6-1/5 of manual analysis.
CONCLUSION
The automatic analysis platform built in this study is operation stable and reliable in analysis results, which can improve work efficiency and report connotation, thus worthing popularized and applicable.
Electrophoresis, Capillary
;
Genotype
;
Hematopoietic Stem Cell Transplantation
;
Humans
;
Microsatellite Repeats
;
Tissue Donors

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