Objective To study the potential of bFGF to promote human bone marrow derived MSCs to differentiate into cardiomyogenic cells, and its effect on proliferation of MSCs. Methods MSCs isolated from adult human bone marrow were cultured in four different systems. Group A: medium with 5-aza. Group B: medium with bFGF. Group C: medium with 5-aza+bFGF. Control group: medium alone. The morphological changes of MSCs were observed. Then immunocytochemistry staining against ?-actin,cTnT,and Connixin43 was performed. The expression of Nkx2.5, GATA-4 and cTnT was detected by semi-quantitative RT-PCR. The proliferation of MSCs in different groups was measured by MTT. Results The MSCs in both group A and C partially differentiated into myogenic cells and expressed proteins of ?-actin,cTnT,and connixin43. In group A and group C, the mRNA level of Nkx2.5,GATA-4 and cTnT was higher than that of control group. In group B, mRNA level of Nkx2.5 and GATA-4 was higher than that of control group. As compared with the control group, cell proliferation was faster in group B than in group A and C. And the proliferation was faster in group C than in group A. Conclusion bFGF can promote the proliferation of MSCs significantly. When combining with 5-aza, bFGF can promote MSCs to differentiate into cardiomyogenic cells more effectively.

Objective To investigate the influence of Siraitia Grosvenori and Rehmannia Glutinosa on the Hematopoietic stem cells proliferation and function .Methods Cells from the peripheral blood , spleen and bone marrow of mice were stained with indicated antibodies , and analyzed by flow cytometry .Mice were divided 3 groups:control group, Siraitia Grosvenori treatment group and Rehmannia Glutinosa treatment group .After 4.5Gy IR treatment, mice divided 4 groups: control group, 4.5Gy IR treatment and feed with normal food, 4.5Gy IR treatment and feed with Siraitia Grosvenori and 4.5Gy IR treatment and feed with Rehmannia Glutinosa for 1 month.Results Mice fed with Siraitia Grosvenori and Rehmannia Glutinosa decreased the percentage of B cells and increased the percentage of M cell .For HSCs, the number of HSCs was increased , especially the number of LT-HSCs.After 4.5Gy IR treatment, mice fed with Siraitia Grosvenori and Rehmannia Glutinosa increase the number of HSCs , and increased the percentage of M cells . Conclusion Siraitia Grosvenori and Rehmannia Glutinosa promote the hematopoietic stem cells and progenitor cells proliferation and function and recover the damage that caused by IR treatment .

Objective To investigate the clinical implications of portal vein bloodletting immediately before reperfusion during orthotopic liver transplantation(OLT).Methods Thirty-two patients with end-stage liver diseases undergoing non veno-venous OLT were divided into bloodletting group (n=21)and control group(n=11).During anhepatic phase,we maintained mean arterial pressure ＞70 mm Hg,cardiac index＞2.5 L·min-1·m-2 by infusion,norepinephrine and dopamine.Blood samples were taken at the time when portal vein was clamped(T1),the time when portal vein was unclamped (T2),10 minutes after neohepatic phase(T3),neohepatic phase 30 minutes(T4)for electrolytes,blood gas and plasma inflammatory cytokines.Hemodynamic and ventilation parameters were also recorded.Results There was no significant difference in mortality(X2=1.12,P＞0.05)and arrhythmia incidence (X2=1.73,P＞0.05)between the two groups.Serum calcium,magnesium were both significantly lower than normal.After anhepatic phase,potassium,tumor necrosis factor alpha,interleukin-6 in radial artery didn't alter significantly;Bloodletting had no effect on lactic acid.There was no significant difference in hemodynamic and ventilation parameters among four time periods.Conclusion Bloodletting seemed to have no effect on changes of internal environment.

380 mmHg) while the PaCO2 increased slightly at T5 and decreased gradually. The PCWP decreased slightly at T2, T3 , T4 and increased quickly at T5 , T6, levelling with T0 at T9. PVRI was on gradual increase beginning ahepatic stage and falling to the level of T0 at T7. The changes of PAP were similar with PCWP, the value at T6 being higher than that at T3, T4 significantly. Conclusion DO2 -directed hemodynamic management protects pulmonary function from damage during LTx.

Objective To study the influence of IL-33 on the Hematopoietic stem cells and progenitor cells . Methods Cells from the peripheral blood , spleen, thymus and bone marrow were stained with indicated antibodies and analyzed by flow cytometry . The LT-HSCs were sorted and culture using in vitro clonogenic assay . Results The percentage of B cells and T cells was decreased and the percentage of M cells was increased in the peripheral blood from IL -33 transgenic mice .Compared with the wildtype mice , the number of HSCs , MPPs and CLP was decreased;meanwhile the number of CMP and GMP was increased in the bone marrow from IL-33 transgenic mice .An in vitro clonogenic assay showed that LT-HSCs increased the ability to self-renew from IL-33 transgenic mice .And the percentage of S-G2-M stage hematopoietic stem cell was increased from IL-33 transgenic mice .Conclusion IL-33 increase the myeloid differentiation in hematopoietic stem cells .

Cytochrome P450 (CYP450) is a superfamily of heme-thiolate proteins, which is involved in the metabolism and activation of various endogenous and exogenous substances, including food, drugs and pollutants. Previous studies of the CYP450 genes mainly focused on their function in drug metabolism. However, in recent years, studies have found that CYP450 are also involved in the development and progression of various diseases, including Parkinson's disease (PD), cancer, cardiomyopathy and heart failure (HF). By far, the process and related mechanisms of CYP450 in the metabolism of endogenous substances in brain tissues has not been clarified yet. In this paper, we summarized the research progress of CYP450 genes involved in the metabolism of endogenous substances, in order to provide a new idea for the exploration of the functions of CYP450 genes expressed in the brain.

OBJECTIVE: To explore the genetic basis for a child with optic atrophy and global developmental delay. METHODS: A child who had presented at the Guangzhou Women and Children's Medical Center in January 2022 was selected as the study subject. Clinical data were collected. Whole exome sequencing (WES) was carried out for the child. Candidate variant was validated by Sanger sequencing and bioinformatic analysis. RESULTS: The child, a nine-month-old female, had manifested dysopia and global developmental delay. Genetic testing revealed that she has harbored a de novo c.425G>C (p.Arg142Pro) variant of the NR2F1 gene, which has been associated with Bosch-Boonstra-Schaaf syndrome. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the variant was classified as pathogenic (PS2+PM1+PM2_Supporting+PM5+PP3+PP4). CONCLUSION The c.425G>C (p.Arg142Pro) variant of the NR2F1 gene probably underlay the pathogenesis in this child. Above finding has enriched the genotypic and phenotypic spectrum of the NR2F1 gene.
Female ; Humans ; Infant ; Computational Biology ; COUP Transcription Factor I/genetics* ; Genetic Testing ; Genomics ; Genotype ; Optic Atrophy/genetics*

Objective: To investigate the clinical characteristics, treatment and prognosis of relapsed demyeli-nating disease (RDD) associated with myelin oligodendrocyte glycoprotein antibodies (MOG abs) children in southern China. Methods: Children with RDD associated with MOG abs at Department of Neurology in Guangzhou Women and Children′s Medical Center from January 2015 to December 2018 were retrospectively analyzed.The annualized relapse rates (ARRs) and expand disability status scale (EDSS) were used to assess the recurrence frequency and neurological dysfunction respectively. Results: Ten children were included with the age of (6.4±3.6) years old, and male to female ratio was 4∶6.(1)Clinical phenotype: all children had 24 episodes during follow-up, with acute disseminated encephalomyelitis (ADEM)(7/10 cases) and neuromyelitis optica spectrum disorders (NMOSD)(3/10 cases) on the first episode.Among 14 recurrent episodes, ADEM (9/14 times) was the most common, followed by optic neuritis(ON)(3/14 times)and brainstem encephalitis (2/14 times). By the final follow-up, the final diagnosis was multiphasic disseminated encephalomyelitis(MDEM)(6/10 cases), NMOSD(3/10 cases), ADEM-ON(1/10 case), respectively.(2)Laboratory examination: all the children had positive serum MOG abs in the acute stage.The serum MOG abs titer high group(≥1∶640)(6 cases)on the first episode complicated ON (3 cases) and long segment myelitis (3 cases) more common than those of low group(1∶320)(4 cases). (3)Imaging changes: 25 times of bain magnetic resonance imaging (MRI) were performed in the acute stage, MRI changes mostly involved the cortex and subcortical white matter.Four cases had abnormal spinal cord MRI.(4)Treatment and prognosis: intravenous methylprednone (IVMP) combined with intravenous immunoglobulin (IVIG) were administrated in acute stage.Rituximab (2/10 cases), mycophenolate mofetil (4/10 cases), IVIG (2/10 cases) monthly and low dose prednisone orally (2/10 cases) were given respectively in maintains stage.ARRs decreased from 1.4 to 0 and EDSS score improved significantly after these treatments above.Seven cases had residual neurological dysfunction with 3 cases of NMOSD, 3 cases of MDEM and 1 case of ADEM-ON, including motor dysfunction, learning disability and inattention, symptomatic epilepsy and visual impairment. Conclusions ADEM is the most common form of RDD associated with MOG abs in children.Those with high serum MOG abs titer on the first episode are prone to have ON or long segment myelitis.Immunomodification therapy is effective in the relapsed patients, residual neurological sequelae were related to the type of repeated demyelination.

OBJECTIVE: To explore the genetic basis for a child featuring global developmental disorder with epilepsy. METHODS: A child who had presented at Guangzhou Women and Children's Medical Center in July 2022 was selected as the study subject. Clinical data was collected. Potential variant was detected by whole exome sequencing (WES). Candidate variant was validated by Sanger sequencing and bioinformatic analysis. RESULTS: The child, a three-year-old ethnic Zhuang Chinese girl, had presented with global developmental disorder and epilepsy, for which rehabilitation therapy was ineffective. Genetic testing revealed that she has harbored a homozygous c.821T>C (p.Leu274Pro) missense variant of the PIGW gene, for which both of her parents and sister were heterozygous carriers. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the variant was classified as variant of uncertain significance. CONCLUSION The homozygous c.821T>C (p.Leu274Pro) variant of the PIGW gene probably underlay the onset of disease in this child. Above finding has enriched the mutational spectrum of the PIGW gene.
Child, Preschool ; Female ; Humans ; Computational Biology ; Developmental Disabilities ; Epilepsy/genetics* ; Genetic Testing ; Homozygote

With the development of nuclear energy technology and the use of depleted uranium weapons, the uranium exposed population is gradually expanding and the health effects of uranium exposure are of increasing concern. The toxicity of uranium to kidney, a sensitive organ for uranium to enter the body to produce effects, cannot be ignored. As of now, the effects of uranium exposure on the kidney are still not well understood, the threshold of uranium-induced kidney injury has been controversial, and there is a lack of sensitive and specific biomarkers for the diagnosis of early kindey damage, especially in the context of chronic uranium exposure. For these reasons, this paper reviewed the results of research on dose-effect relationships and biomarkers of uranium-induced kidney injury and provided an outlook on future research directions, with the aim of providing a basis for subsequent study on animal experiments and population health effects related to uranium exposure.