Changes in the frequency, phenotype, and function of mucosal-associated invariant T lymphocytes in peripheral blood of children with metabolic associated fatty liver disease / 临床肝胆病杂志

Objective To investigate the change in mucosal-associated invariant T (MAIT) lymphocytes in peripheral blood of children with metabolic associated fatty liver disease (MAFLD) and its clinical significance. Methods A total of 18 children with MAFLD who attended Hunan Children's Hospital from March to May, 2022, were enrolled as MAFLD group, and 20 normal children who attended the hospital during the same period of time were enrolled as control group. Peripheral blood samples were collected, and flow cytometry was used to measure the percentages of MAIT lymphocytes (CD3 + CDl61 + TCRVα7.2 + cells), different MAIT lymphocyte subsets (CD4 + CD8 - MAIT cells, CD4 - CD8 - MAIT lymphocytes, CD4 - CD8 + MAIT lymphocytes, and CD4 + CD8 + MAIT lymphocytes), and MAIT lymphocytes expressing PD-1, CD69, perforin, CD107α, CXCR3, CXCR6, and CCR6. The correlation of MAIT lymphocyte frequency with liver inflammation, fat content, and fibrosis degree was analyzed. The t -test was used for comparison of normally distributed continuous data between two groups, and the Mann-Whitney U test was used for comparison of non-normally distributed continuous data between two groups. The Spearman correlation analysis was used for correlation analysis. Results Compared with the control group, the MAFLD group had significant increases in the percentage of MAIT lymphocytes in peripheral blood, the percentages of MAIT cells expressing PD-1, CD69, CD107α, CXCR3, CXCR6 and CCR6, and the percentages of CD4 + CD8 - MAIT cells and CD4 + CD8 + MAIT lymphocytes among MAIT cell subsets (all P < 0.05), as well as a significant reduction in the percentage of CD4 - CD8 + MAIT cells among MAIT cell subsets ( P < 0.001). The percentages of CD4 + CD8 + MAIT lymphocytes and CD107α-positive MAIT lymphocytes were negatively correlated with alanine aminotransferase ( r =-0.474 and -0.550, P =0.047 and 0.018). Conclusion The migration of peripheral blood MAIT lymphocytes to the liver plays a protective role against liver inflammation in children with MAFLD.

Clinical features and genetic testing of a child with hepatic failure syndrome type 2 / 中华医学遗传学杂志

OBJECTIVE@#To explore the genetic basis for a child with infantile liver failure syndrome type 2 (ILFS type 2).@*METHODS@#Clinical features of the child were analyzed. Next generation sequencing was also carried out for him.@*RESULTS@#The child was found to harbor compound heterozygous variants of the NBAS gene, which included a novel nonsense c.2746A>T (p.R916X, 1456) variant in exon 24 and a missense c.3596G>A (p.C1199Y) mutation in exon 31, which has been associated with ILFS type 2. The two variants were respectively inherited from his father and mother.@*CONCLUSION@#The compound heterozygous variants of c.3596G>A and c.2746A>T of the NBAS gene probably underlay the ILFS type 2 in this child.

Analysis of etiology and prognosis of 120 children with pediatric acute liver failure / 中华实用儿科临床杂志

Objective:To investigate the etiology, prognosis and prognostic factors of pediatric acute liver failure(PALF), in order to provide the basis for clinical treatment of PALF.Methods:The clinical data of children with PALF hospitalized at Hunan Children′s Hospital from May 2008 to May 2018 were collected, and the causes and prognosis were analyzed.According to the prognosis, the patients were divided into the death group and the survival group, whose biochemical indexes were then compared.After that, the statistical analysis of different data were carried out by using t-test, Wilcoxon test and χ2 test separately. Results:In 120 PALF cases, there were 68 males and 52 females, and there were 36 infants, 34 toddlers, 22 preschoolers and 28 school-age children.Twenty cases (16.7%) were caused by sepsis, 19 cases (15.8%) by genetic metabolic diseases, 18 cases (15.0%) by poisoning, 12 cases (10.0%) by viral infection, 6 cases (5.0%) by drugs, 1 case (0.8%) by bile polyp, and 1 case (0.8%) by tumor disease.Besides, the etiology of 43 cases (35.9%) was unknown.Among the cases with known etiologies, genetic metabolic and infectious diseases were the main cause of disease in infants, toddler patients were mostly caused by infectious diseases and drug/toxicants, and drug/toxicants and hereditary metabolic diseases were the dominant cause of disease in school-age children and preschoolers.Mortality rate of children with PALF was 50.0%.Among them, the mortality of Epstein-Barr virus-associated hemophagocytic syndrome, sepsis, Citrin deficiency and Tyrosinemia was higher than that of other diseases.Compared with the survival group, the total bilirubin (TB)[159.00(73.05, 274.00) μmol/L vs.62.75(2.65, 221.75)μmol/L], direct bilirubin(DB)[83.00(41.43, 160.00) μmol/L vs.38.74(10.98, 128.75) μmol/L], prothrombin time (PT)[39.60(24.93, 62.60) s vs.24.65(21.43, 29.83) s], international standardized ratio (INR)[3.40(2.30, 6.74) vs.2.09(1.85, 2.84)], and blood ammonia (NH 3) levels [109.50(85.25, 149.75) μmol/L vs.80.00(60.25, 102.75) μmol/L] in the death group were significantly increased, and the diffe-rences were statistically significant(all P<0.05); while the levels of albumin[(28.72±5.88) g/L vs.(33.69±4.96) g/L], alanine aminotransferase (ALT) [586.50(223.25, 1 082.00) U/L vs.1 434.00(615.00, 3 334.50) U/L]and aspartate aminotransferase (AST) [827.50(545.00, 2 024.00) U/L vs.1 663.50(821.00, 4 886.75) U/L]in the death group were significantly decreased, and the differences were statistically significant(all P<0.05). However, the blood glucose and cholesterol levels in both groups had no statistically significant difference. Conclusion:The mortality of children with PALF is high, and different age groups have different etiologies.The increase of TB, DB, PT, INR, NH3 and the ratio of hepatic encephalopathy, and the decrease of albumin, AST and ALT suggest poor prognosis.

Effect of transcutaneous neuromuscular electrical stimulation on prevention of intensive care unit-acquired weakness in chronic obstructive pulmonary disease patients with mechanical ventilation / 中华危重病急救医学

Objective To evaluate the effect of transcutaneous neuromuscular electrical stimulation on prevention of intensive care unit-acquired weakness (ICU-AW) in chronic obstructive pulmonary disease (COPD) patients with mechanical ventilation. Methods A prospective randomized controlled study was conducted. Sixty COPD patients aged 18-85 years old who were accepted mechanical ventilation therapy admitted to general intensive care unit (ICU) of the First Affiliated Hospital of Hunan University of Medicine from October 2017 to October 2018 were enrolled. Patients were divided into control group (n = 30) and intervention group (n = 30) by random number table method. All patients were accepted routine treatment, and on this basis, the intervention group was applied transcutaneous neuromuscular electrical stimulation on the extremities (twice a day, 30 minutes each time) after 24 hours of admission until ICU discharge. The Medical Research Council muscle strength score (MRC-Score), grip strength, incidence of ICU-AW on the 7th day after admission and on the day of ICU discharge; modified Barthel index score on the day of ICU discharge; and duration of mechanical ventilation, the length of ICU stay, and the length of hospital stay were compared between the two groups. Results Twenty-nine and 27 patients in the control group and the intervention group respectively finally completed the study in dividually. There was no significant difference in gender, age, Barthel index score before 2 weeks of ICU admission, body mass index or acute physiology and chronic health evaluation Ⅱ(APACHEⅡ) in ICU between the two groups. There was no significant difference in the MRC-Score, grip strength or incidence of ICU-AW on the 7th day after ICU admission between the two groups. Compared to the control group, the MRC-Score, grip strength and Barthel index score in the intervention group were significantly increased [MRC-Score: 55.97±8.43 vs. 46.32±7.36, grip strength (kg): 33.46±11.62 vs. 27.42±9.64, Barthel index score:46.04±5.46 vs. 42.13±3.32, all P < 0.05], the incidence rate of ICU-AW was significantly decreased [7.4% (2/27) vs. 31.0% (9/29), P < 0.05], and duration of mechanical ventilation, the length of ICU stay, the length of hospital stay were significantly shortened [duration of mechanical ventilation (days): 5.12±2.01 vs. 7.24±4.35, the length of ICU stay (days): 8.34±2.36 vs. 10.45±2.62, the length of hospital stay (days): 13.21±2.21 vs. 15.38±3.67, all P < 0.05]. Conclusion Transcutaneous neuromuscular electrical stimulation can effectively improve the muscle strength of COPD patients with mechanical ventilation and reduce the incidence of ICU-AW.

A single center study on the clinical features and treatment of infectious mononucleosis in children / 中华实验和临床病毒学杂志

Objective@#To investigate the clinical features of children with infectious mononucleosis (IM), to compare the difference of therapeutic effects between general treatment to antiviral therapy for IM.@*Methods@#This prospective study analyzed the clinical data and laboratory test results of 201 cases with IM in our hospital from January 1, 2016 to December 31, 2016. The follow-up period was 6-12 months. The patients were divided into two groups according to the order of admission. The clinical symptoms and laboratory test results of the two groups were observed after hospitalization.@*Results@#Of the total of 201 patients, male to female is 1.72∶1; Age: 8 months to 13 years 6 months (average 4.8±2.8 years), The disease frequently occurred in summer and autumn, accounted for 64.18%.The major clinical manifestations was fever (97.51%), angina (79.10%), enlarged of lymph node(68.66%), eyelid swelling (67.16%), hepatomegaly (53.73%) and splenomegaly (46.77%). There was no statistical difference in duration of fever, improved angina time, lymph nodes(liver, spleen) enlargement recovery time, heterotypic lymphocytes normalization time, lymphocyte function normalization time.There was significant difference in reducing the serum/plasma or total blood EBV-DNA in the short term between antiviral group and general treatment group (P<0.01), but for the long-term reduction of EBV-DNA, there was no significant difference (P>0.05). The low affinity EBV-CA-IgG was converted to high affinity EBV-CA-IgG in 89.41% of patients after 3 months and in 98.68% of patients after 6 months. With the recovery of the disease, the positive rate of EBV-CA-IgM and EBV-EA-IgG decreased. But the positive rate of EBV-NA-IgG increased. Serum/plasma EBV-DNA was completely overcast after 3 months, but after 12 months, the patient’s total blood EBV-DNA positive rate was still 71.11%.@*Conclusions@#In general IM patients, there was no significant difference between antiviral therapy and general treatment, so antiviral therapy is not generally recommended. EBV DNA in the whole blood of patients with IM will continue to be positive more than 6-12 months, indicating that to observe changes of IM should not use whole blood samples for EBV DNA testing.

Clinical application of alpha fetoprotein combined with abnormal prothrombin in detecting primary liver cancer / 中国基层医药

Objective To investigate the clinical value of serum alpha fetoprotein ( AFP) combined with abnormal prothrombin(PIVKA-Ⅱ) in the detection of primary liver cancer.Methods From May 2017 to October 2017,80 cases of primary liver cancer in Department of Hepatobiliary Surgery of Deyang People 's Hospital,100 cases of non HCC patients ( including chronic liver disease ,cirrhosis,metastatic liver cancer ) were selected,and another 100 healthy people were selected as control group.The levels of serum AFP and PIVKA -Ⅱ were detected by SIEMENS XP automatic immunoassay analyzer and lumipulseg 1200 automatic immune analyzer respectively.All data were analyzed by SPSS17.0 analysis software.Results Comparison between primary liver cancer and non -primary liver cancer:the levels of serum AFP and PIVKA -Ⅱ(F＝1.763;F＝1.788,sig＝0.000,P＜0.05).Comparison between non-primary liver cancer and normal group:the levels of serum AFP and PIVKA -Ⅱ(F＝0.896;F＝0.346,sig＝0.000,P＜0.05).Serum AFP had a sensitivity of 75.0%and a specificity of 71.5%in the diagnosis of primary liver cancer, while PIVKA -Ⅱ had a sensitivity of 82.0% and a specificity of 78.7%.The diagnosis sensitivity and specificity of AFP combined with PIVKA -Ⅱreached to 92.0% and 95.0%.The sensitivity of AFP combined with PIVKA-Ⅱin the diagnosis of primary liver cancer had statistically significant differences compared with AFP and PIVKA-Ⅱalone(χ2＝4.112,P＝0.008;χ2＝2.991,P＝0.010).Conclusion Combined detection of serum AFP and PIVKA-II has important clinical significance in the diagnosis of primary liver cancer .

Effect of oropharyngeal healthy exercise combined with guided education in the treatment of dysphagia in patients with Alzheimer's disease / 现代临床护理

Objective To explore the effect of oropharyngeal healthy exercise combined with guided education in the treatment of dysphagia in patients with Alzheimer's disease (AD). Methods 90 AD inpatients in the geriatric psychiatric department of Guangzhou Huiai Hospital during May 2014 and August 2015 were equally randomized into the study group and the control group with a random digit table:the former group was treated with routine oral muscles training and the latter with oropharyngeal healthy exercises combined with guided education plus routine muscles training. The comparison was done between the groups in terms of the total curative effectiveness 4 weeks following the intervention. Result The total effective rate in the study group was significantly higher than that in the control group (P<0.01). Conclusion The oropharyngeal healthy exercise combined with guided education can effectively promote the recovery of swallowing function in AD patients.

Liver pathology and gene analysis in children with Shwachman-Diamond syndrome / 临床儿科杂志

Objective To analyze the gene abnormality and liver pathology in Shwachman-Diamond syndrome (SDS) in a child. Method The clinical data of one child with SDS were analyzed retrospectively. Result A male patient was 1 month old at onset with neutrophil decrease as the first manifestation, accompanied by anemia, elevated transaminase and repeated infection. Exocrine pancreatic dysfunction was atypical. Pathological examination of liver biopsy showed slight damage of liver cells under light microscope. The blood samples of child and parents were collected, and homozygous mutations of SBDS (NM_016038.2) Intron2 c.258+2T>C p.? were detected by two generation gene sequencing. And these mutations were from both his parents. Conclusion Gene testing is helpful in diagnosing SDS and we suggest that liver biopsy should be performed if condition allows.

Genetic analysis and literature review of Crigler-Najjar syndrome typeⅠ / 临床儿科杂志

ObjectiveTo analyze the Uridine diphosphoglucuronyl transferase 1 A1 gene (UGT1A1) mutation in non-he-molytic indirect hyperbilirubinemia.MethodsA female patient was diagnosed with type I Crigler-Najjar syndrome (CNS-I) after excluding hemolysis and hypothyroidism. Phototherapy treatment is effective while oral phenobarbital was not. UGT1A1 were ampliifed by polymerase chain reaction and DNA was sequenced.ResultsThe patient was compound heterozygote of c.1070A>G p. (Gln357Arg) and 1091C>T p. (pro364Leu) and diagnosed with CNS-I. Heterozygotes of the mutation were found in her parents.ConclusionsIn patients highly suspected of CNS, the genetic tests should be carried out as soon as possible.

Progress in the reprogramming of induced pluripotent stem cells with small molecules / 国际儿科学杂志

Induced pluripotent stem cells can differentiate into a variety of cell types,which promote the development of human disease model,drug toxicity screening and sources of autologous cells.However,there have been many problems in the induced pluripotent stem cells reprogramming,such as safety and low efficiency.Small molecules are considered as a promising method to improve the reprogramming processes of induced pluripotent stem cell,and more and more small molecules have been identified to maintain stem cell self-renewal,providing a new approach to produce the desired reprogramming cells.

Effect of Astragaloside Ⅳ on hematopoietic growth factor expression in rat bone marrow mesenchymal stem cells / 中国组织工程研究

BACKGROUND:Astragaloside Ⅳ is a major component of Huangqi,promoting proliferation and differentiation of bone marrow mesenchymal stem cells;however,the mechanism has been less reported yet.OBJECTIVE:To explore the effect of Astragaloside Ⅳ on expression of multiple hematopoietic growth factors in bone marrow mesenchymal stem cells.METHODS:Bone marrow mesenchymal stem cells were isolated from adult Wistar rats by using the method of adhesive culture and clone,and they were then plated on 96-well plate and separately incubated with 100 uL Astragaloside Ⅳ(25,50,100,200 g/L)for 72 hours.The cells in the control group were cultured with an equal volume of DMEM-LG culture liuquid.Indirect immunofluorescence was used to detect the biological activity,MTT method was used to evaluate the effect of Astragaloside Ⅳ on proliferation and differentiation of bone marrow mesenchymal stem cells,and RT-PCR method was used to measure the expression of hematopoietic growth factors in bone marrow mesenchymal stem cells.RESULTS AND CONCLUSION:The 3~(rd)-passage bone marrow mesenchymal stem cells highly expressed CD44 but lowly expressed CD45.As compared with control group,Astragaloside Ⅳ promoted proliferation of bone marrow mesenchymal stem cells in a time/dosage-dependent manner,in particular,the 200 g/L Astragaloside IV and 72-hour intervention(P＜ 0.05).SCF expression was significantly increased in the drug group compared with control group(P ＜ 0.01);however,TPO,GM-CSF,and TGF-β1 expressions were not changed significantly(P ＞ 0.05).Moreover,interleukin-3 expression was not found in the bone marrow mesenchymal stem cells.Astragaloside Ⅳ promoted in vitro proliferation of bone marrow mesenchymal stem cells,possibly involving in SCF secretion.