OBJECTIVE:To systematically evaluate the efficacy and safety of glutathione (GSH) in preventing oxaliplatin-in-duced peripheral neurotoxicity(OIPN),and to provide evidence-based reference in the clinic. METHODS:Randomized controlled trials (RCTs) about therapeutic efficacy of GSH vs. placebo/no any measures (called placbo group) or other drug in preventing OIPN were retrieved from PubMed,EMBase,Cochrane library,CJFD,Wanfang database and VIP. Meta-analysis was performed with Rev Man 5.3 statistical software after data extraction and quality evaluation with Jadad scale. RESULTS:18 RCTs were in-cluded,involving 1200 patients. The results of Meta-analysis showed that:total incidence of oxaliplatin-induced chronic peripheral neurotoxicity (OICPN)[RR=0.71,95%CI(0.59,0.87),P<0.001] and the incidence of severe OICPN [RR=0.50,95%CI(0.42, 0.60),P<0.001] in GSH group were significantly lower than placebo group,with statistical significance;there was statistical signif-icance in the incidence of oxaliplatin-induced acute peripheral neurotoxicity(OIAPN)[RR=0.89,95%CI(0.72,1.09),P=0.25]. The incidence of severe OICPN in GSH group was significantly higher than mecobalamine group,with statistical significance [RR=2.06,95%CI(1.07,3.99),P=0.03]. There was no statistical significance in the incidence of OICPN[RR=1.38,95%CI(0.83,2.31), P=0.21] and severe OICPN [RR=1.91,95%CI(0.85,4.30),P=0.12] between GSH group and Ca+Mg mixture group. CONCLU-SIONS:GSH can effectively prevent the occurrence of OICPN,however,its therapeutic efficacy is equivalent to Ca+Mg mixture and inferior to mecobalamine in preventing severe OICPN.

Objective To explore the mechanism of the secondary deterioration of neurological symptoms in Wilson' s disease (WD) at early stage of treatment using D-penicillamine. Methods Forty non-treated WD patients, 32 of encephalic and 8 hepatic type respectively, were enrolled in the study. Their neural symptoms were scored using modified Young grade. Cerebrospinal fluid (CSF) copper, serum copper, urinary copper, neuron specific enolase (NSE) in CSF and the albumin ratio CSF/serum (AR) were measured at the same time. After 3 months of treatment with D-penicillamine, neural symptoms of patients were scored again. All dates were analyzed. Results After 3 months of treatment with D-penicillamine, 15 patients (46. 9%) developed a secondary deterioration in neurological symptoms. The concentration of copper and the NSE in CSF of patients whose neural symptom was increasingly deteriorated. The serum copper declined after treatment((0. 37± 0. 09) vs (0. 25 ± 0. 08) mg/L, t = 3. 17, P < 0. 05). The 24 hours urinary copper of patients whose symptoms had deteriorated was much lower than that of patients who had not. No significant change was found in AR ratio before and after the treatment (9. 53 ± 3.18vs12.24±3.17) in the worsened group (t=1.45, P>0. 05). Conclusions The degree of the injury in the neural system and the dose of penicillamine may affect the deterioration of the neural symptom.

This article was aimed to study the chemical constituents from the chemical split fractions of Mori Cortex. The compounds were isolated with Diaion HP-20, Toyopearl HW-40, Sephadex LH-20, MCI Gel CHP-20, Silica gel column chromatography and preparative HPLC. Structures of compounds were identified by physicochemical properties and spectral analysis. The results showed that 23 compounds were obtained. And their structures were identified. The 16 compounds were obtained from the 30% ethanol fraction as vanillic acid (1), 3,4-dimethoxyphenol (2), benzoic acid (3), syringic acid (4), kelampayoside A (5), p-hydroxyphenylpropionic acid (6), caffeic acid (7), hydroferulic acid (8), 6,7-dihydroxycoumarin (9), 5,7-dihydroxycoumarin (10), morin-7-O-β-D-glucopyranoside (11), liriodendrin (12), 2,3-trans-dihydromorin (13), 2,3-cis-dihydromorin (14), 2,3-trans-dihydroquercetin (15), 2,3-cis-dihydroquercetin (16). The 4 compounds were obtained from the 50% ethanol fraction as scopoletin (17), morin (18), kaempferol-7-O-β-D-glucopyranoside (19), umbelliferone (20). The 3 compounds were obtained from the 80% ethanol fraction as sanggenon R (21), cis-mulberroside A (22), resveratrol (23). It was concluded that compounds 2, 4-6, 11, 16, 19 were isolated from this plant for the first time.

This article was aimed to study the chemical constituents of seeds of Descurainia Sophia (L.) Webb. ex Prantl., in order to lay the material foundation for further interpretation of seeds of D. Sophia and provide pharmacodynamic basis as well as the basis for attributing its nature and taste. The compounds were isolated and purified by Diaion HP-20, Toyopearl HW-40, MCI Gel CHP-20, ODS, Silica gel chromatography combining with Pre-HPLC. The structures were identified on the basis of spectral data and physicochemical properties. Twenty eight compounds were isolated and identified from 20% and 80% ethanol fraction. Thirteen compounds were identified from 20% ethanol fraction: kaempferol-3-O-β-D-glucopyranosyl-7-O-β-D-gentiobioside(1), quercetin-3-O-β-D-glucopyranosyl-7-O-β-D-gentiobioside (2), isorhamnetin-3-O-β-D-glucopyranosyl-7-O-β-D-gentiobioside (3), isorhamnetin-3,7-di-O-β-D-glucopyranoside (4), quercetin-3,7-di-O-β-D-glucopyranoside (5), kaempferol-3, 7-di-O-β-D-glucopyranoside (6), kaempferol-3-O-β-D-xylopyranosyl (1 → 2)-β-D-glucopyranoside (7), methyl sinapate (8), syringaldehyde (9), (S)-p- hydroxyphenyl lactate acid (10), (S)-2-hydroxy-phenylpropionic acid (11), scopoletin (12), sinapic acid (13). Fifteen compounds were identified from 80% ethanol fraction: isorhamnetin-3-O-β-D-glucopyranoside (14), quercetin-3-O-β-D-glucopyranoside (15), kaempferol-3-O-β-D-glucopyranoside (16), quercetin (17), kaempferol (18), isorhamnetin (19), syringic acids (20), quercetin-3-O-β-D-arabinopyranoside (21), quercetin-3-O-β-D-xylopyranoside (22), 6-O-[E]-Sinapoyl-(α- and β)-D-glucopyranoside (23), dimethyl (E, E)-4,4'-dihydroxy-3,3',5,5'-tetramethoxylign-7,7'-dien-9,9'-dioate (24), dimethylthomasidioate (25), 2-hydroxy-3-(1H-indol-3-yl) propanoic acid (26), 2-hydroxyl-3-(1H-indol-3-yl) propanoic acid methyl ester (27), 4'-O-methyl-dihydroquercetin (28). It was concluded that compounds 7-11 and 21-28 were isolated from seeds of D. sophia (L.) Webb. ex Prantl. for the first time.

Objective To investigate the clinical and CLB1 gene mutation characteristics of GM1 gangliosidosis patient. Methods The clinical data of one GM1 gangliosidosis patient from Children′s Hospital Affiliated to Zhengzhou University in March 2018 were reviewed and analyzed. The patient was diagnosed by gene detection and enzymatic activity. Results The patient is a 4 years and 1 month old boy, mainly presented psychomotor retrogression. His β?galactosidase activity was low (8.0 nmol·g-1·min-1). Two splice site mutations (c.458?2A(IVS4)>G and c.1068+5G(IVS10)>A) of patient′s CLB1 gene were screened by targeted next generation sequencing. The results of Sanger sequencing showed that the mutations are compound heterozygous and both are first reported. The mutation c.1068+5G(IVS10)>A was derived from patient′s mother, and the other one is de nove. Conclusion GM1 gangliosidosis is a rare neurodegenerative disease, which could be accurately diagnosed by the next generation sequencing and enzyme assay.

Objective: To investigate the clinical and CLB1 gene mutation characteristics of GM1 gangliosidosis patient. Methods: The clinical data of one GM1 gangliosidosis patient from Children′s Hospital Affiliated to Zhengzhou University in March 2018 were reviewed and analyzed. The patient was diagnosed by gene detection and enzymatic activity. Results: The patient is a 4 years and 1 month old boy, mainly presented psychomotor retrogression. His β-galactosidase activity was low (8.0 nmol·g^-1·min^-1). Two splice site mutations (c.458-2A(IVS4)>G and c.1068+5G(IVS10)>A) of patient′s CLB1 gene were screened by targeted next generation sequencing. The results of Sanger sequencing showed that the mutations are compound heterozygous and both are first reported. The mutation c.1068+5G(IVS10)>A was derived from patient′s mother, and the other one is de nove. Conclusion GM1 gangliosidosis is a rare neurodegenerative disease, which could be accurately diagnosed by the next generation sequencing and enzyme assay.

Objective:To evaluate the efficacy and safety of Huangqi Guizhi Wuwu Decoction for preventing oxaliplatin-induced peripheral neurotoxicity(OIPN).Methods:PubMed, ScienceDirect, EMbase, VIP, CNKI and WanFang Data were searched to collect the randomized controlled trials(RCTs) of Huangqi Guizhi Wuwu Decoction for OIPN from the date of establishment until July 2019. Two reviewers independently evaluated the quality of literature and extracted data. RevMan5.3 software was used for Meta-analysis.Results:Fifteen RCTs (849 patients) were included. The results of Meta-analyses showed statistically significant differences in favor of Huangqi Guizhi Wuwu Decoction group compared with control group for all incidence of OIPN [ RR=0.57, 95% CI(0.40, 0.81), P=0.002] and incidence of serious OIPN [ RR=0.35, 95% CI(0.25, 0.48), P<0.000 01]. No differences were observed in disease control rates between two groups. There was statistically significant differences between Huangqi Guizhi Wuwu Decoction group and mecobalamine group for all incidence of OIPN [ RR=0.51, 95% CI(0.39, 0.66), P<0.000 01] and incidence of serious OIPN [ RR=0.37, 95% CI(0.19, 0.70), P=0.002]. Conclusions:Huangqi Guizhi Wuwu Decoction is more safety and effective than mecobalamine on the prevention of OIPN and did not affect the efficacy of chemotherapy. So Huangqi Guzhi Wuwu decoction can be widely extended to clinical applications.

Objective:To report a case of tocilizumab successfully used in a child with febrile infection-associated epilepsy syndrome (FIRES), and to provide a new idea for the treatment of FIRES in children.Methods:The diagnosis and treatment of 1 case of FIRES admitted in Children′s Hospital Affiliated to Zhengzhou University on February 15, 2021 were described, and the prognosis and follow-up of the child were evaluated. At the same time, the literatures on tocilizumab in the treatment of children′s FIRES were reviewed.Results:A 5-year-old case of FIRES was reported. The child was extremely refractory to immunotherapy and anti-seizure medicines, anesthetics and ketogenic diet. So he was treated with tocilizumab (each time 4 mg/kg) at the 36th day and 43rd day, and epileptic seizures were controlled 10 days after the 2nd doses of tocilizumab. During a follow-up of 10 months, his epileptic seizures were controlled and the cognitive behavior and speech function were well recovered. At present, only 3 cases of FIRES in children have been reported all over the world. All the seizures were well controlled and no obvious adverse reactions were observed.Conclusions:FIRES is a rare refractory epilepsy syndrome, resistant to many kinds of anti-seizure medicines or even anesthetic agents, which is difficult to treat and has poor prognosis. Preliminary trials have shown that tocilizumab is effective and well tolerated in children with FIRES. It may be a potential therapeutic modality for children with FIRES.

Objective:To explore the treatment of the patients with severe phenotype of mucopolysaccharidosis (MPS) type ⅣA by analysing the clinical feature and diagnosis.Methods:Two pediatric patients diagnosed as MPS ⅣA in severe form were enrolled in Children′s Hospital Affiliated to Zhengzhou University from August 2021 to April 2022.Two children from 2 pedigrees with the main manifestations of short stature and bone deformities were retrospectively included.The clinical manifestations, biochemical indexes, and bone imaging findings were retrospectively analyzed.Peripheral blood leukocytes were collected and subjected to the N-acetylgalactosamine-6-sulfatase (GALNS) assay and genetic sequencing.Gene analysis of amniotic fluid cells at the 18 th week of the second pregnancy of the mother of case 2 was performed for prenatal diagnosis.Allogeneic hematopoietic stem cell transplantation (allo-HSCT) was performed in both patients and to explore the treatment of patients with MPS ⅣA. Results:Both cases presented clinical manifestations of short stature, joint laxity, pectus carinatum, and genu valgus.X-ray examination revealed the decreased bone mineral density, ulnar deviation of the radial epiphysis, kyphosis and scoliosis.The respiratory and skeletal systems were affected in both patients, and the optic nerve was suspiciously affected. GALNS gene analysis showed that there were 2 missense mutations of c. 1019G>A (p.G340D) and c. 706C>G (p.H236D) in case 1, and 2 missense mutations of c. 425A>G (p.H142R) and c. 463G>A (p.G155R) were detected in case 2.Mutations in both cases were inherited from their fathers and mothers, which were all newly discovered that have not been reported.Only the c. 463G>A mutation was detected in the amniotic fluid cells of the mother of case 2.It is confirmed that case 2 was the carrier of MPS ⅣA, whose gene mutation was from the mother, and case 2 did not suffer the same disease as the proband.Both cases were treated with allo-HSCT with full donor chimerism and no severe transplant complications were reported.Their GALNS activity was within the normal range, and the scores of activities of daily living were higher than those before transplantation. Conclusions:The MPS ⅣA patients with severe phenotype is a rare autosomal recessive disease caused by GALNS mutations that is difficult to diagnose and poor prognosis.Early detection, diagnosis, and effective treatment contribute to improve the long-term quality of life.The allo-HSCT is an effective therapeutic strategy for MPS ⅣA.