It was a retrospective study. The case data of thirteen patients diagnosed with methylmalonic acidemia (MMA) combined with hemolytic uremic syndrome (HUS) hospitalized at the Children's Hospital Affiliated to Capital Institute of Pediatrics from January 2010 to December 2022 were analyzed, to explore clinical and genetic characteristics of MMA combined with HUS (MMA-HUS). The onset age of MMA was 18 days to 5 years old, with 11 patients of early onset and 2 patients of late onset, 4 patients of simple MMA and 9 patients of combined MMA, and 7 patients of secondary hypertension and 3 patients of secondary pulmonary arterial hypertension. Among 13 patients, 7 patients underwent genetic testing, with 1 patient of MMUT gene mutation and 6 patients of MMACHC gene mutation. The mutation sites were all from their parents and all were known pathogenic variants. Among them, 5 patients had MMACHC gene c.80A>G heterozygous variation, accompanied by cardiovascular involvement. After etiological and symptomatic treatment, 6 patients improved, 7 patients died of multiple organ failure, and all the deaths were early-onset MMA. This study shows that MMA-HUS is more common in early-onset MMA, with a severe condition and a high mortality rate. Its prognosis is related to multiple factors such as genotype, diagnosis and treatment timing. c.80A>G variation of MMACHC gene may be related to HUS and cardiovascular involvement.

Objective To investigate the incidence rate,clinical characteristics,and prognosis of neonatal stroke in Shenzhen,China.Methods Led by Shenzhen Children's Hospital,the Shenzhen Neonatal Data Collaboration Network organized 21 institutions to collect 36 cases of neonatal stroke from January 2020 to December 2022.The incidence,clinical characteristics,treatment,and prognosis of neonatal stroke in Shenzhen were analyzed.Results The incidence rate of neonatal stroke in 21 hospitals from 2020 to 2022 was 1/15 137,1/6 060,and 1/7 704,respectively.Ischemic stroke accounted for 75％(27/36);boys accounted for 64％(23/36).Among the 36 neonates,31(86％)had disease onset within 3 days after birth,and 19(53％)had convulsion as the initial presentation.Cerebral MRI showed that 22 neonates(61％)had left cerebral infarction and 13(36％)had basal ganglia infarction.Magnetic resonance angiography was performed for 12 neonates,among whom 9(75％)had involvement of the middle cerebral artery.Electroencephalography was performed for 29 neonates,with sharp waves in 21 neonates(72％)and seizures in 10 neonates(34％).Symptomatic/supportive treatment varied across different hospitals.Neonatal Behavioral Neurological Assessment was performed for 12 neonates(33％,12/36),with a mean score of(32±4)points.The prognosis of 27 neonates was followed up to around 12 months of age,with 44％(12/27)of the neonates having a good prognosis.Conclusions Ischemic stroke is the main type of neonatal stroke,often with convulsions as the initial presentation,involvement of the middle cerebral artery,sharp waves on electroencephalography,and a relatively low neurodevelopment score.Symptomatic/supportive treatment is the main treatment method,and some neonates tend to have a poor prognosis.

Objective To study the effects of Chrysanthemum Flos on blood pressure of spontaneously hypertensive rats(SHR)and evaluate its antioxidant activity in vitro and in vivo.Methods SHR were randomly divided into model,control and experimental-L,-H groups with 10 rats per group,and 10 WKY rats as blank group.Experimental-H,-L groups were given 2.10 and 0.525 g·kg-1 Chrysanthemum Flos extract by gavage;control group received 5.25 mg·kg-1 losartan by gavage;blank and model groups were given the same volume of 0.9％NaCl solution by gavage.Rats in each group were gavaged once a day for 8 weeks.After 8 weeks of continuous intragastric administration,the systolic blood pressure(SBP)and diastolic blood pressure(DBP)were observed.The contents of catalase(CAT),superoxide dismutase(SOD),glutathione peroxidase(GSH)and malondialdehyde(MDA)in serum were measured with kit colorimetry method.The in vitro free radical scavenging rates of Chrysanthemum Flos extract were detected by 1,1-diphenyl-2-picrylhydrazyl(DPPH)and 2,2'-amino-di(2-ethyl-benzothiazoline sulphonic acid-6)ammonium salt(ABTS)methods.Results The SBP of blank,model,control and experimental-H,-L groups were(132.00±2.45),(204.00±4.55),(171.00±2.16),(181.00±3.74)and(184.67±4.78)mmHg;the DBP were(73.33±4.03),(175.67±3.40),(120.33±0.94),(125.33±2.87)and(125.67±2.36)mmHg;the contents of serum CAT were(9.24±3.99),(8.40±2.98),(9.24±2.42),(8.59±2.70)and(8.49±1.47)U·mL-1;the contents of serum SOD were(122.40±12.30),(75.30±28.37),(125.39±31.35),(110.92±26.14)and(103.37±22.31)U·mL-1;the contents of serum GSH were(117.93±10.18),(78.29±23.68),(118.57±26.08),(109.89±20.52)and(98.73±14.71)U·mL-1;the contents of serum MDA were(8.36±2.08),(8.45±3.38),(8.22±3.04),(7.09±3.21)and(7.24±3.32)nmol·L 1,respectively.Compared with model group,the differences of above indicators in control group and experimental-H,-L groups were statistically significant(P＜0.05,P＜0.01).Chrysanthemum Flos extract showed certain free radical scavenging ability in vitro.The highest scavenging rates of DPPH and ABTS were 90.29％and 92.67％,respectively.Conclusion Chrysanthemum Flos extract had good antihypertensive activity.The antioxidation ability might be its antihypertensive mechanisms.

This study aimed to explore the mechanism of Qilongtian Capsules in treating acute lung injury(ALI) based on network pharmacology prediction and in vitro experimental validation. Firstly, UPLC-Q-TOF-MS/MS was used to analyze the main chemical components of Qilongtian Capsules, and related databases were used to obtain its action targets and ALI disease targets. STRING database was used to build a protein-protein interaction(PPI) network. Metascape database was used to conduct enrichment analysis of Gene Ontology(GO) and Kyoto Encyclopedia of Genes and Genomes(KEGG). AutoDock software was used to perform molecular docking verification on the main active components and key targets. Then, the RAW264.7 cells were stimulated with lipopolysaccharide(LPS) for in vitro experiments. Cell viability was measured by MTT and ROS level was measured by DCFH-DA. NO content was measured by Griess assay, and IL-1β, IL-6, and TNF-α mRNA expression was detected by RT-PCR. The predicted targets were preliminarily verified by investigating the effect of Qilongtian Capsules on downstream cytokines. Eighty-four compounds were identified by UPLC-Q-TOF-MS/MS. Through database retrieval, 44 active components with 589 target genes were screened out. There were 560 ALI disease targets, and 65 intersection targets. PPI network topology analysis revealed 10 core targets related to ALI, including STAT3, JUN, VEGFA, CASP3, and MMP9. KEGG enrichment analysis showed that Qilongtian Capsules mainly exerted an anti-ALI effect by regulating cancer pathway, AGE-RAGE, MAPK, and JAK-STAT signaling pathways. The results of molecular docking showed that the main active components in Qilongtian Capsules, including crenulatin, ginsenoside F_1, ginsenoside Rb_1, ginsenoside Rd, ginsenoside Rg_1, ginsenoside Rg_3, notoginsenoside Fe, notoginsenoside G, notoginsenoside R_1, notoginsenoside R_2, and notoginsenoside R_3, had good binding affinities with the corresponding protein targets STAT3, JUN, VEGFA, CASP3, and MMP9. Cellular experiments showed that Qilongtian Capsules at 0.1, 0.25, and 0.5 mg·mL~(-1) reduced the release of NO, while Qilongtian Capsules at 0.25 and 0.5 mg·mL~(-1) reduced ROS production, down-regulated mRNA expression of IL-1β, IL-6, TNF-α, and inhibited the inflammatory cascade. In summary, Qilongtian Capsules may exert therapeutic effects on ALI through multiple components and targets.
Humans ; Tumor Necrosis Factor-alpha ; Ginsenosides ; Caspase 3 ; Matrix Metalloproteinase 9 ; Interleukin-6 ; Molecular Docking Simulation ; Network Pharmacology ; Reactive Oxygen Species ; Tandem Mass Spectrometry ; Acute Lung Injury/genetics* ; Capsules ; RNA, Messenger ; Drugs, Chinese Herbal/pharmacology*

This study aimed to investigate the effect of serum containing ginseng and Moutan Cortex on human umbilical vein endothelial cells(HUVEC) injured with hydrogen peroxide(H_2O_2). HUVEC injured with H_2O_2 were divided into 6 groups, namely blank group, model group, ginsenoside(TGG) group, total glucosides of Moutan Cortex(TGM) group, paeonol(P) group and TGG+TGM+P group. After 24 hours of co-culture with H_2O_2, the activities of succinate dehydrogenase(SDH) and Ca~(2+)-Mg~(2+)-ATP were detected by microenzyme labeling. The apoptosis rate, intracellular Ca~(2+) concentration, reactive oxygen species(ROS) and mitochondrial membrane potential(JC-1) were detected by flow cytometry. The expressions of mitochondrial apoptosis pathway-related proteins Bax, Bcl-2, cytochrome C, caspase-3 and caspase-9 were detected by Western blot. The results showed that H_2O_2 could significantly damage HUVEC, decrease the activity of SDH and Ca~(2+)-Mg~(2+)-ATP(P<0.01), while could increase the apoptosis+necrosis rate, JC-1 decline rate, ROS increase rate and Ca~(2+) concentration increase rate(P<0.01). Serum containing ginseng and Moutan Cortex could increase the activities of SDH and Ca~(2+)-Mg~(2+)-ATP to different degrees, decrease the apoptosis+necrosis rate, JC-1 decline rate, ROS increase rate and Ca~(2+) concentration increase rate(P<0.05 or P<0.01), and down-regulate the protein expressions of Bax, caspase-3, caspase-9, cytochrome C, and up-regulate the protein expression of Bcl-2. The results showed that serum containing ginseng and Moutan Cortex has a protective effect on vascular endothelial cell injury induced by ROS, and its mechanism may be related to the improvement of mitochondrial function and the inhibition of the activation of mitochondrial apoptosis pathway.
Apoptosis ; Drugs, Chinese Herbal/pharmacology* ; Human Umbilical Vein Endothelial Cells ; Humans ; Paeonia ; Panax ; Reactive Oxygen Species

OBJECTIVE: To evaluate the clinical features of preterm infants with a birth weight less than 1 500 g undergoing different intensities of resuscitation. METHODS: A retrospective analysis was performed for the preterm infants with a birth weight less than 1 500 g and a gestational age less than 32 weeks who were treated in the neonatal intensive care unit of 20 hospitals in Jiangsu, China from January 2018 to December 2019. According to the intensity of resuscitation in the delivery room, the infants were divided into three groups:non-tracheal intubation ( RESULTS: Compared with the non-tracheal intubation group, the tracheal intubation and ECPR groups had significantly lower rates of cesarean section and use of antenatal corticosteroid ( CONCLUSIONS For preterm infants with a birth weight less than 1 500 g, the higher intensity of resuscitation in the delivery room is related to lower rate of antenatal corticosteroid therapy, lower gestational age, and lower birth weight. The infants undergoing tracheal intubation or ECRP in the delivery room have an increased incidence rate of adverse clinical outcomes. This suggests that it is important to improve the quality of perinatal management and delivery room resuscitation to improve the prognosis of the infants.
Birth Weight ; Cesarean Section ; China ; Female ; Gestational Age ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Pregnancy ; Retrospective Studies

Objective: To unveil the efficacy of Shaolin internal qigong exercise in treating capsulitis of the shoulder (CS) and explore objective outcome measures by observing the changes in the surface electromyography (sEMG) signals of shoulder muscle groups after regular practice of Shaolin internal qigong exercise in CS patients. Methods: Sixty CS patients were randomized into two groups by the random number table method, with 30 cases in each group. Patients in the qigong group practiced Shaolin internal qigong exercise on a regular basis, while patients in the electroacupuncture (EA) group received EA treatment. Before and after treatment, the sEMG signals of six muscles, i.e. biceps brachii, triceps brachii, deltoid, pectoralis major, latissimus dorsi and trapezius muscles, of the affected side were recorded at 45° abduction of the shoulder, 60° forward flexion and 90° internal rotation with the elbow flexed during maximal isometric contraction, and the integrated electromyography (iEMG) of each muscle was calculated. Results: The total effective rate was 93.3％ in the qigong group, higher than 83.3％ in the EA group (P<0.05). Intra-group comparison showed that the iEMG of biceps brachii, triceps brachii, pectoralis major and deltoid muscles in the qigong group increased significantly after intervention at 45° abduction of the shoulder, 60° forward flexion and 90° internal rotation with the elbow flexed (all P<0.05), and the iEMG of trapezius and latissimus dorsi muscles decreased (both P<0.05); in the EA group, the iEMG of biceps brachii, pectoralis major and deltoid muscles increased significantly during contraction (all P<0.05), while the iEMG of triceps brachii, trapezius and latissimus dorsi muscles had no significant changes (all P>0.05). After intervention, there were significant differences in the iEMG of most of muscles between the two groups (all P<0.05), except for the iEMG of deltoid muscle at 45° of abduction of the shoulder joint during isometric contraction (P>0.05). Conclusion: Shaolin internal qigong exercise can effectively increase the motion intensity of the biceps brachii, triceps brachii, pectoralis major and deltoid muscles and reduce the compensation of the latissimus dorsi and trapezius muscles in CS patients; compared with EA, it produces a better result in improving the coordination and stability in shoulder joint movements.

Objective To systematically evaluate the clinical effect and safety of repaglinide and metformin for treating diabetes mellitus (MD) of secondary failure of sulfonylurea (SFS).Methods The randomized controlled trials (RCT) at home and abroad on the comparison of effect and safety of repaglinide and metformin in treating MD with SFS were retrieved.The modified Jadad scale was employed to evaluate the literature quality.The RevMan5.3.1 software was used for conducting the meta analysis.Results A total of 10 RCT were included.The meta-analysis results showed that compared with metformin for treating MA with SFS,repaglinide decreased the fasting blood glucose effectively (MD=-2.30,95 ％ CI:-2.53--2.06,P＜0.01),increased the fasting C-peptide (MD=0.06,95％CI:0.02-0.11,P=0.01),reduced the postprandial 2 h blood glucose (MD=-2.17,95 ％CI:-2.44-1.89,P＜0.01) and decreased glycosylated hemoglobin (MD=-2.60,95％CI:-3.21--2.00,P＜0.01) as well as the adverse reactions (RR=0.05,95％CI:0.02-0.09,P＜0.01).However,there was no statistical difference in fasting insulin between the repaglinide and metformin groups (MD=0.18,95％CI:-0.18-0.54,P=0.32).Conclusion Currently evidences suggest that repaglinide is superior to metformin for treating MD with SFS.

OBJECTIVETo explore the clinical effect and mechanism of long snake moxibustion combined with functional exercise for treatment of ankylosing spondylitis in early stage.

METHODSSeventy-eight cases of ankylosing spondylitis in early stage were randomly divided into an observation group and a control group, 39 cases in each group. The observation group was treated with long snake moxibustion from Dazhui (GV 14) to Yaoshu (GV 2) combined with functional exercise; the control group was treated with oral administration of Sulfasalazine. The effects of the two groups were observed, and contents of erythrocyte sedimentation rate (ESR), tumor necrosis factor alpha (TNF-alpha), intercellular adhesion molecule-1 (ICAM-1) were tested before and after treatment.

RESULTSThe total effective rate of 89.7% (35/39) in the observation group, was superior to that of 76.9% (30/39) in the control group (P<0.05). The contents of ESR, TNF-alpha, and ICAM-1 showed downtrend after treatment in the both groups (P<0.05, P<0.01), and the observation group decreased more obviously (all P<0.01).

CONCLUSIONLong snake moxibustion at Governor Vessel combined with functional exercise for treatment of ankylosing spondylitis in early stage, can significantly improve clinical symptoms, decrease the level of serum inflammatory cytokines, and the effect is superior to that of Sulfasalazine oral administration.

Acupuncture Points ; Adolescent ; Adult ; Combined Modality Therapy ; Cytokines ; blood ; Exercise Therapy ; Humans ; Male ; Middle Aged ; Moxibustion ; Spondylitis, Ankylosing ; blood ; immunology ; therapy ; Young Adult