2.Oliguria and acute renal dysfunction in a six-month-old infant.
Ya-Jie CUI ; Chun-Lan SONG ; Yi-Bing CHENG
Chinese Journal of Contemporary Pediatrics 2017;19(2):203-207
The infant (a girl aged 6 months) was admitted to the hospital because of oliguria and acute renal dysfunction. The laboratory examination results showed serious metabolic acidosis and increased blood urea nitrogen and serum creatinine levels. The patient continued to be anuric after 10 days of treatment with continuous renal replacement therapy (CRRT). she died a day later. The family history showed that the patient's sister died of acute renal failure 6 months after birth. The genomic sequencing results showed AGXT mutation in the patient and confirmed the diagnosis of primary hyperoxaluria type 1 (PH1). Her parents were heterozygous carriers. PH1 should be considered when the children have abnormal renal function or recurrent renal calculi or have a family history of these symptoms. AGXT gene analysis is an important method for PH1 diagnosis.
Acute Kidney Injury
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etiology
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Female
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Humans
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Hyperoxaluria, Primary
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complications
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Infant
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Mutation
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Oliguria
;
etiology
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Transaminases
;
genetics
3.Paroxysmal muscle weakness, liver enlargement, and hypoglycemia in a boy.
Ya-Jie CUI ; Chun-Lan SONG ; Yi-Bing CHENG
Chinese Journal of Contemporary Pediatrics 2017;19(10):1104-1108
A boy aged 11 years was admitted due to intermittent weakness and difficulty in walking for 6 years, and hepatomegaly, glycopenia and unconsciousness for 4 years. The laboratory examinations showed severe metabolic acidosis, hypoglycemia, and abnormal liver function. CT scan showed marked liver enlargement with fat density shadow. The boy was given fluid infusion, correction of acidosis, intravenous injection of glucose, L-carnitine, compound vitamin B, and coenzyme Q10, but he was in a persistent coma and it was difficult to correct refractory metabolic acidosis and hypoglycemia. The boy died. Blood and urinary organic acid screening and gene detection confirmed that the boy had late-onset glutaric aciduria type II (GAIIc) caused by electron-transferring-flavoprotein dehydrogenase (ETFDH) gene defect. GAIIc is an inherited metabolic disease with a low incidence, resulting in a high misdiagnosis rate. GAIIc should be considered for children with recurrent weakness or reduced activity endurance, hypoglycemia, and marked liver enlargement with abnormal liver function. Urinary organic acid analysis and blood tandem mass spectrometry can help with the early diagnosis of GAIIc, and ETFDH gene analysis helps to make a confirmed diagnosis.
Child
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Hepatomegaly
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etiology
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Humans
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Hypoglycemia
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etiology
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Male
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Multiple Acyl Coenzyme A Dehydrogenase Deficiency
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diagnosis
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Muscle Weakness
;
etiology
4.Role of MiR-20a-3p in Axonal Repair in Rats with Spinal Cord Injury
Yan-jun ZHANG ; Zhi-jie WANG ; Tian-yi WANG ; Xue-ming CHEN ; Li-bin CUI ; Song-jie XU ; Xin YUAN ; Ya-dong LIU ; Peng ZHAO ; Qi-chao WU ;
Chinese Journal of Rehabilitation Theory and Practice 2019;25(3):307-313
Objective:To study the molecular biology mechanisms of Wistar rats after spinal cord injury, and find out key microRNAs. Methods:A total of 15 Wistar rats were divided into control group (
5.Myocardial protective effect of L-carnitine in children with hand, foot and mouth disease caused by Coxsackie A16 virus.
Ya-Jie CUI ; Chun-Lan SONG ; Fang CHEN ; Peng LI ; Yi-Bing CHENG
Chinese Journal of Contemporary Pediatrics 2017;19(8):908-912
OBJECTIVETo investigate the myocardial protective effect of L-carnitine in children with hand, foot and mouth disease (HFMD) caused by Coxsackie A16 virus and possible mechanisms.
METHODSA total of 60 HFMD children with abnormal myocardial enzyme after Coxsackie A16 virus infection were enrolled and randomly divided into L-carnitine group and fructose-1,6-diphosphate group (fructose group), with 30 children in each group. The two groups were given L-carnitine or fructose diphosphate in addition to antiviral and heat clearance treatment. Another 30 healthy children who underwent physical examination were enrolled as control group. The changes in myocardial zymogram, malondialdehyde (MDA), superoxide dismutase (SOD), and apoptosis factors sFas and sFasL after treatment were compared between groups.
RESULTSThere was no significant difference in treatment response between the L-carnitine group and the fructose group (P>0.05). One child in the fructose group progressed to critical HFMD, which was not observed in the L-carnitine group. Before treatment, the L-carnitine group and the fructose group had significantly higher indices of myocardial zymogram and levels of MDA, sFas, and sFasL and a significantly lower level of SOD than the control group (P<0.05), while there were no significant differences in these indices between the L-carnitine group and the fructose group (P>0.05). After treatment, the L-carnitine group and the fructose group had significant reductions in the indices of myocardial zymogram and levels of MDA, sFas, and sFasL and a significant increase in the level of SOD (P<0.05); the fructose group had a significantly higher level of creatine kinase (CK) than the control group and the L-carnitine group, and there were no significant differences in other myocardial enzyme indices, MDA, sFas, and sFasL between the L-carnitine group and the fructose group, as well as between the L-carnitine and fructose groups and the control group (P>0.05). SOD level was negatively correlated with aspartate aminotransferase, lactate dehydrogenase (LDH), CK, and creatine kinase-MB (CK-MB) (r=-0.437, -0.364, -0.397, and -0.519 respectively; P<0.05), and MDA level was positively correlated with LDH and CK-MB (r=0.382 and 0.411 respectively; P<0.05).
CONCLUSIONSL-carnitine exerts a good myocardial protective effect in children with HFMD caused by Coxsackie A16 virus, possibly by clearing oxygen radicals and inhibiting cardiomyocyte apoptosis.
Carnitine ; therapeutic use ; Child, Preschool ; Coxsackievirus Infections ; complications ; Female ; Hand, Foot and Mouth Disease ; drug therapy ; etiology ; metabolism ; Heart ; drug effects ; Humans ; Infant ; Male ; Malondialdehyde ; analysis ; Myocardium ; metabolism ; pathology ; Superoxide Dismutase ; metabolism
6.Expression, purification, characteristics and homology modeling of the HMGS from Streptococcus pneumoniae.
Ya-Li BEN ; Gu-Zhen CUI ; Chen LI ; Rui HAN ; Jie ZHANG ; Qing-Ye ZHANG ; Jian WAN ; De-Li LIU
Biomedical and Environmental Sciences 2009;22(3):229-236
OBJECTIVETo understand the molecular basis for a potential reaction mechanism and develop novel antibiotics with homology modeling for 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) synthase (HMGS).
METHODSThe genetic engineering technology and the composer module of SYBYL7.0 program were used, while the HMGS three-dimensional structure was analyzed by homology modeling.
RESULTSThe mvaS gene was cloned from Streptococcus pneumoniae and overexpressed in Escherichia coli from a pET28 vector. The expressed enzyme (about 46 kDa) was purified by affinity chromatography with a specific activity of 3.24 micromol/min/mg. Optimal conditions were pH 9.75 and 10 mmol/L MgCl2 at 37 degrees C. The V(max) and K(m) were 4.69 micromol/min/mg and 213 micromol/L respectively. The 3D model of S. pneumoniae HMGS was established based on structure template of HMGS of Enterococcus faecalis.
CONCLUSIONThe structure of HMGS will facilitate the structure-based design of alternative drugs to cholesterol-lowering therapies or to novel antibiotics to the Gram-positive cocci, whereas the recombinant HMGS will prove useful for drug development against a different enzyme in the mevalonate pathway.
Amino Acid Sequence ; Base Sequence ; Cloning, Molecular ; Gene Expression Regulation, Bacterial ; physiology ; Hydroxymethylglutaryl-CoA Synthase ; chemistry ; genetics ; metabolism ; Models, Molecular ; Molecular Sequence Data ; Mutation ; Protein Conformation ; Streptococcus pneumoniae ; enzymology ; genetics
7.Effects of yin qiaojiedu soft capsule on influenza virus load and M1 expression in mice.
Ying LIU ; Yu-Jing SHI ; Han SHI ; Ju-Ying ZHONG ; Fang-Zhou LIU ; Ying-Jie GAO ; Ya-Hong JIN ; Shan-Shan GUO ; Xiao-Lan CUI
Acta Pharmaceutica Sinica 2011;46(6):650-655
This study is to investigate the treatment of YinQiaojiedu soft capsule for influenza virus A/PR8/34 (H1N1) infection. The model of pneumonia was established by dropping influenza virus into the nose of normal mice, and the lung index and death rate were observed. Real time RT-PCR and Western blotting technique were used to detect the virus load and the relative expression of M1 protein in lungs of mice on the 1st, 3rd, 5th and 7th day after infection. The results showed that YinQiaojiedu soft capsule in 1 g x kg(-1) and 0.5 g x kg(-1) dose groups can decrease the lung index significantly on the 3rd, 5th and 7th day after being infected (P < 0.05, P < 0.01), and the number of death in the two groups of animals decreased significantly. YinQiaojiedu soft capsule in 1 g x kg(-1) dose group can decreased virus load at each time point, and lower it in 0.5 g x kg(-1) dose group at the 3rd, 5th and 7th day (P < 0.05, P < 0.01). YinQiaojiedu soft capsule can decrease the relative expression of M1 protein in lungs of mice, 1 g x kg(-1) and 0.5 g x kg(-1) dose groups are significantly lower in expression of M1 protein compared with model group at the 3rd and 7th day (P < 0.05, P < 0.01). It can be concluded that YinQiaojiedu soft capsule exerts antiviral effects against influenza virus by downregulating expression of virus load and M1 protein.
Animals
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Antiviral Agents
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administration & dosage
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pharmacology
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Capsules
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Drugs, Chinese Herbal
;
administration & dosage
;
pharmacology
;
Female
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Influenza A Virus, H1N1 Subtype
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Lung
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metabolism
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virology
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Male
;
Mice
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Mice, Inbred ICR
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Orthomyxoviridae Infections
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drug therapy
;
metabolism
;
virology
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Pneumonia
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metabolism
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virology
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Viral Load
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drug effects
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Viral Matrix Proteins
;
metabolism
8.Effects of jin chai antiviral capsule on IFITM3 expression in mice.
Ju-Ying ZHONG ; Xiao-Lan CUI ; Yu-Jing SHI ; Shan-Shan GUO ; Ying LIU ; Fang-Zhou LIU ; Ying-Jie GAO ; Ya-Hong JIN ; Hong-Xin CAO
Acta Pharmaceutica Sinica 2012;47(7):904-908
This study is to investigate the treatment of Jin Chai antiviral capsule for influenza virus FM1/47 (H1N1) infection. The model of pneumonia was established by dropping influenza virus into the nose of normal mice, real-time PCR and Western blot technique were used to detect the virus load and the interferoninducible transmembrane protein3 (IFITM3) in lung of mice at the 1st day, 3rd day, 5th day and 7th day after affected. The results showed that Jin Chai antiviral capsule in large, middle, small dose groups can decrease virus load significantly at each time point, after being affected (P<0.05, P<0.01), Jin Chai antiviral capsule can increase the interferoninducible transmembrane protein3 in lung of mice, large dose groups are significantly higher in expression of IFITM3 compared with model group at each time point (P<0.05, P<0.01). Middle dose groups are significantly higher in expression of IFITM3 compared with model group at the 3th day and the 5th day (P<0.05), small dose groups are significantly higher in expression of IFITM3 compared with model group at the 3th day (P<0.05). It can be concluded that Jin Chai antiviral capsule exerts antiviral effects against influenzavirus by raised expression of IFITM3.
Animals
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Antiviral Agents
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administration & dosage
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pharmacology
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Capsules
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Dose-Response Relationship, Drug
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Drug Combinations
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Drugs, Chinese Herbal
;
administration & dosage
;
pharmacology
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Female
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Influenza A Virus, H1N1 Subtype
;
drug effects
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Lung
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metabolism
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Male
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Membrane Proteins
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metabolism
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Mice
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Mice, Inbred ICR
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Orthomyxoviridae Infections
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metabolism
;
virology
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Plants, Medicinal
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chemistry
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Pneumonia
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metabolism
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virology
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Viral Load
;
drug effects
9.Pharmacodynamic experiment of the antivirus effect of Houttuynia cordata injection on influenza virus in mice.
Fang-zhou LIU ; Han SHI ; Yu-jing SHI ; Ying LIU ; Ya-hong JIN ; Ying-jie GAO ; Shan-shan GUO ; Xiao-lan CUI
Acta Pharmaceutica Sinica 2010;45(3):399-402
It is to investigate the effect of two kinds of Houttuynia Cordata Injection on preventing and treating H1N1 influenza virus infection in mice. Pneumonia model was set up by intranasal infection of the normal and immunocompromised mice with influenza virus FM1 and PR8. The two injections were administered before and after the administration of virus, separately, and the lung index was observed. The results showed that the two preparations have obvious therapeutic effect on normal mice infected with influenza virus FM1 and PR8. And to FM1, the new injection's effect is better at small dosage. The results also showed that the two preparations have obvious prophylactic effect on immunodepressed mice infected with influenza virus FM1 and PR8. And to PR8, the old injection's effect is better at small dosage. Houttuynia Cordata Injection can improve the mice pneumonia caused by influenza virus H1N1 and decrease the lung index markedly. It has a remarkable preventive and therapeutic effect on H1N1 influenza virus in mice.
Animals
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Antiviral Agents
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administration & dosage
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therapeutic use
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Drugs, Chinese Herbal
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administration & dosage
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isolation & purification
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therapeutic use
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Female
;
Houttuynia
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chemistry
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Immunocompromised Host
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Influenza A Virus, H1N1 Subtype
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drug effects
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immunology
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Injections
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Male
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Mice
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Mice, Inbred ICR
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Orthomyxoviridae Infections
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complications
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drug therapy
;
immunology
;
prevention & control
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Plants, Medicinal
;
chemistry
;
Pneumonia, Viral
;
drug therapy
;
etiology
;
prevention & control
;
Random Allocation
10.The expression of platelet-derived growth factor (PDGF) receptor-beta and its correlation with extracellular matrix in hepatic tissue in hepatic fibrosis rats.
Cui-hua LU ; Yue-xiang CHEN ; Zhong-bing ZHANG ; Wei-fen XIE ; Jie-fei HUANG ; Run-zhou NI ; Ya-jun GUO ; Li-xin WEI ; You-xin JIN
Chinese Journal of Hepatology 2004;12(11):663-665
OBJECTIVETo investigate the expression of PDGF receptor-beta and its correlation with extracellular matrix in hepatic tissue during hepatic fibrosis.
METHODSThe model of hepatic fibrosis in rats was induced by carbon tetrachloride. PDGF receptor-beta subunit, collagen I, collagen III and a-SMA in hepatic tissues of these rats were examined using immunohistochemistry. The correlation between PDGF receptor-beta subunit and collagen I, III was analyzed using SAS software after the results of immunohistochemistry were semi-quantified.
RESULTSPDGF receptor-beta subunit and a-SMA were not detected in normal controls. Collagen I and III were distributed in the portal tracts and beneath the endothelia of the central veins and of the Disse spaces. Two weeks after CCl4 injection, the PDGF receptor-beta and a-SMA were detected, and the expression of collagen I and III increased. At the end of 4 and 6 weeks, the above four proteins were further increased. Two weeks after CCl4 injection, PDGF receptor-beta had no apparent correlation with collagen I and III. However, PDGF receptor-beta had a significant correlation with collagen I and III 2 weeks later, and the correlation coefficient was 0.74 and 0.60 respectively at 4 weeks, and 0.83 and 0.67 respectively at 6 weeks. PDGF receptor-beta had a significant correlation with a-SMA during the whole process of hepatic fibrosis and the correlation coefficient was 0.62, 0.69 and 0.81, respectively at the time of 2, 4 and 6 weeks after CCl4 injection.
CONCLUSIONThe PDGF receptor-beta was overexpressed during the process of hepatic fibrosis development, and it significantly correlated with collagen I and collagen III.
Animals ; Carbon Tetrachloride ; Carbon Tetrachloride Poisoning ; Collagen Type I ; biosynthesis ; genetics ; Collagen Type III ; biosynthesis ; genetics ; Extracellular Matrix ; metabolism ; Liver ; metabolism ; Liver Cirrhosis, Experimental ; chemically induced ; metabolism ; Male ; Rats ; Rats, Sprague-Dawley ; Receptor, Platelet-Derived Growth Factor beta ; biosynthesis ; genetics