Rotor syndrome is an autosomal recessive disorder of bilirubin metabolism， and it is difficult to diagnose and differentiate due to its extreme rarity and a lack of specific clinical manifestations. In recent years， the development of genetic testing technology has enabled the early diagnosis of atypical patients. Literature search shows that only 19 cases with detailed clinical and genetic data have been reported. This article reports a case of a pregnant woman with an increase in direct bilirubin during pregnancy who was diagnosed with Rotor syndrome based on a bi-allelic mutation in the SLCO1B1 and SLCO1B3 genes and delivered successfully at last， and a retrospective analysis was performed for related articles， in order to facilitate the early accurate diagnosis of patients with Rotor syndrome and guide medications from the perspective of genetic mechanisms.

Objective To summarize the clinical characteristics of children with drug-induced hypersensitivity syndrome(DIHS)complicated with herpesvirus reactivation,and to promote the early and accurate identification,diagnosis,and treatment of DIHS in children.Methods The medication history,clinical manifestations,treatment,and prognosis of 12 children confirmed DIHS complicated with herpesvirus reactivation in Guangzhou Women and Children's Medical Center between January 2018 and March 2023 were retrospectively analyzed.The changes in hematological parameters,inflammatory indexes,and hepatic and renal function within 5 d before the eruption,5 d,and 6-10 d after eruption were compared.Results Of the 12 patients,the male-to-female ratio was 5∶1,with a median age of 27(interquartile range 20.50-34.75)months.Two or more antibiotics were used at least two to six weeks before onset,with a combination of 3 or more antibiotics in 7 children,and a combined or sequential application of 2 antibiotics in 5 children.The antibiotics included cephalosporins(n=12),semisynthetic penicillins(n=5),vancomycin(n=4)and azithromycins(n=7).All 12 patients presented fever,rashes,and multiple organ involvement.The rashes were red maculopapules in the early stage and then gradually developed into massive fusion exceeding 50%of the whole body.Among them,seven children were accompanied by facial edema,and two had purplish-red facial rashes.11 children suffered from exfoliative dermatitis in the later stage.12 children presented obviously enlarged lymph nodes.Liver involvement was the most common(100%,simple increase of transaminase in four children,cholestasis in six children,and hepatic failure in two children),and lung involvement was found in nine children.Laboratory examination showed no significant increase in leukocytes or eosinophils within 5 d before the eruption,but low levels of atypical lymphocytes.After the eruption,leukocytes,eosinophils,and atypical lymphocytes increased progressively.Inflammatory indexes of hypersensitive C-reactive protein(CRP),procalcitonin(PCT)increased dramatically before and after the eruption.All the children received intravenous immunoglobulin(IVIG)and methylprednisolone,two children were given antiviral therapy,and nine children were treated with multiple plasma exchanges.After treatment,nine children were cured,one developed immune reconstitution syndrome,and two died of hepatic failure.Conclusions Antibiotics are common allergenic drugs for DIHS in children.Its clinical manifestations include fever and rashes,accompanied by multiple organ involvement,such as the liver and lung.When leukocytes,eosinophils,and atypical lymphocytes are progressively elevated after the eruption,DIHS should be highly suspected,herpesvirus activation should be monitored,medication history should be traced,and early active immunotherapy and antiviral therapy should be conducted if necessary.

Anti-seizure medications (ASMs) are the main therapy for epilepsy.There are many kinds of ASMs with complex mechanism of action, so it is difficult for pharmacists to examine prescriptions.This paper put forward some suggestions on the indications, dosage forms/routes of administration, appropriateness of usage and dosage, combined medication and drug interaction, long-term prescription review, individual differences in pathophysiology of children, and drug selection when complicated with common epilepsy, for the reference of doctors and pharmacists.

Objective To explore the relationship between non-high density lipoprotein cholesterol(non-HDL-C)level and leptomeningeal collateral circulation in patients with acute middle cerebral artery occlusion.Methods A total of 85 patients with first-onset acute cerebral infarction with middle cerebral artery M1 segment occlusion were enrolled.According to the results of DSA,LMC circulation was assessed by American Society of Interventional and Therapeutic Neuroradiology/Society of Interventional Radiology Collateral Circulation Assess-ment System.All patients were assigned to better LMC circulation group(score 2～4,n = 30)and worse LMC circulation group(score 0～1,n = 55),and the levels of non-HDL-C were compared between the two groups.Results The levels of LDL-C and non-HDL-C in worse LMC circulation group were significantly higher than those of the better LMC circulation group(P = 0.026,P = 0.010).non-HDL-C was an independent risk factor for the worse LMC circulation(OR = 3.019,95%CI:1.053～8.658,P = 0.04).LMC circulatory score of patients was negatively correlated with the levels of non-HDL-C level(r =-0.228,P = 0.036).The AUC of non-HDL-C predicted for the worse LMC circulation was 0.638(95%CI:0.521～0.755,P = 0.036).Conclusions non-HDL-C in patients with acute cerebral infarction was significantly related to worse LMC circulation,and was a risk factor for worse LMC circulation.It is suggested that the higher expression of non-HDL-C could be used to predict worse LMC circulation as a serological indicator.

Objective:To explore the efficacy and safety of crisaborole ointment (crisaborole) treatment in children with atopic dermatitis (AD).Methods:PubMed, Embase, Medline, CNKI, Wanfang Medicine, VIP, and Chinese Medical Journal Databases were searched (up to March 2022) and the literature on randomized controlled trials (RCTs) and cohort studies of crisaborole treatment in children with AD was collected. The quality of RCTs and cohort studies was evaluated using the modified Jadad scale and Newcastle-Ottawa scale, respectively. Data on effective rate and incidence of adverse event in children treated with crisaborole were extracted. The meta-analysis of single proportions was performed with Stata 15 software.Results:A total of 8 studies (4 RCTs and 4 prospective cohort studies, respectively) were entered in the analysis, including 1 855 children (aged from 3 months to 17 years) treated with crisaborole. The 8 studies were all of high quality. The meta-analysis of single proportions showed that the effective rate of children with AD treated with crisaborole for 14-28 days was 46% [95% confidence interval ( CI): 32%-61%], the incidence of adverse events during treatment was 28% (95 %CI: 19%-38%), and the incidence of treatment discontinuation due to adverse events was 1% (95 %CI: 0-2%). Most of the adverse events caused by crisaborole were local skin reactions, mainly characterized by local pain, itching, dermatitis, paresthesia, and local infection, etc. Seven of the 8 studies reported the pain at the application site, and the incidence of pain at the application site was 17% (95 %CI: 11%-24%). Most adverse events were mild to moderate and could be alleviated without treatment. Serious adverse events occurred in 8 patients (0.4%), 7 of which were unrelated to the treatment drugs. Conclusion:Crisaborole has good efficacy and safety in the treatment of mild to moderate AD in children.

Objective:To explore the efficacy and safety of crisaborole ointment (crisaborole) treatment in children with atopic dermatitis (AD).Methods:PubMed, Embase, Medline, CNKI, Wanfang Medicine, VIP, and Chinese Medical Journal Databases were searched (up to March 2022) and the literature on randomized controlled trials (RCTs) and cohort studies of crisaborole treatment in children with AD was collected. The quality of RCTs and cohort studies was evaluated using the modified Jadad scale and Newcastle-Ottawa scale, respectively. Data on effective rate and incidence of adverse event in children treated with crisaborole were extracted. The meta-analysis of single proportions was performed with Stata 15 software.Results:A total of 8 studies (4 RCTs and 4 prospective cohort studies, respectively) were entered in the analysis, including 1 855 children (aged from 3 months to 17 years) treated with crisaborole. The 8 studies were all of high quality. The meta-analysis of single proportions showed that the effective rate of children with AD treated with crisaborole for 14-28 days was 46% [95% confidence interval ( CI): 32%-61%], the incidence of adverse events during treatment was 28% (95 %CI: 19%-38%), and the incidence of treatment discontinuation due to adverse events was 1% (95 %CI: 0-2%). Most of the adverse events caused by crisaborole were local skin reactions, mainly characterized by local pain, itching, dermatitis, paresthesia, and local infection, etc. Seven of the 8 studies reported the pain at the application site, and the incidence of pain at the application site was 17% (95 %CI: 11%-24%). Most adverse events were mild to moderate and could be alleviated without treatment. Serious adverse events occurred in 8 patients (0.4%), 7 of which were unrelated to the treatment drugs. Conclusion:Crisaborole has good efficacy and safety in the treatment of mild to moderate AD in children.

ObjectiveTo develop the comprehensive geriatric assessment (CGA) nursing information management system and to explore its application value. MethodsCGA and mobile nursing information system were combined to design and develop the CGA nursing information management system. Totally 196 geriatric inpatients admitted in Geriatric Hospital of Nanjing Medical University from April 1st to June 30th, 2018 were selected using convenient sampling. These geriatric patients were managed by the well-trained nursing staff using the system, including CGA at admission and before discharge and continuous CGA. The changes of CGA at admission and discharge were used to assess the clinical application value of the CGA nursing information management system. ResultsThe consistency rate of the implementation of CGA nursing intervention with CGA nursing norms was 67%-91%. The geriatric patients' pain scores at discharge decreased compared with those at admission(P＜0.01), but there was no statistically significant difference in malnutrition risk, pressure ulcer, fall and ADL scores (P＞ 0.05). ConclusionsThe CGA nursing information management system is conductive to the implementation of CGA clinically, the monitoring of the effects of CGA nursing intervention, and the nursing quality control by nursing managers, which is worth promoting clinically and in communities.

OBJECTIVE:To investigate the effects of UGT1A4142T>G polymorphism and blood concentration of valproic ac-id(VPA)on blood concentration of lamotrigine(LTG)in southern Chinese Han children with epilepsy,and to establish the predic-tion equation for quantitatively estimating blood concentration of LTG. METHODS:A total of 72 southern Chinese Han children with epilepsy selected from Guangzhou Women and Children's Medical Center during Jan. 2010-Sept. 2016 were given LTG+VPA. LC-MS/MS and enzyme amplified immunoassay were adopted to determine the blood concentration of LTG and VPA. RFLP-PCR was adopted to determine UGT1A4142T>G polymorphism. The relationships of age, gender, blood concentration of VPA, UGT1A4142T>G polymorphism and LTG concentration-to-dose-ratio (CDR) were also investigate. The prediction equation for blood concentration of LTG was established by multiple linear regression analysis. RESULTS:Age and blood concentration of VPA were positively related to CDR of LTG(r=0.225,0.300,P<0.05);there was no statistical significance in the influence of gender on CDR of LTG(P>0.05). UGT1A4 TT,TG and GG genotypes were detected in 39,29,and 4 cases respectively;the frequencies of each genotype were in line with the Har-dy-Weinberg balance(P>0.05). CDR of LTG of TT genotype was significantly lower than those of TG and GG genotype,with sta-tistical significance(P<0.05). Results of multiple linear regression analysis showed that the dose of LTG(x1),body weight(x2), blood concentration of VPA(x3)and UGT1A4142T>G polymorphism(x4)were all related to blood concentration of LTG(P<0.05). Using blood concentration of LTG(c)as dependent variable,above factors as independent variable,the regression equation was c=0.794+0.032x1-0.057x2+0.010x3+0.532x4(R2=0.616,P<0.05;UGT1A4 TT genotype was equal to 0,TG and GG genotype was equal to 1). There was a strong positive correlation between predicted blood concentration and measured ones(r=0.785,P=0.001). CONCLUSIONS:The dose of LTG,body weight,blood concentration of VPA and UGT1A4142T>G polymorphism may associated with blood concentration of LTG. Established prediction equation can provide reference for precise medication in south-ern Chinese Han children with epilepsy.

Objective To investigate the effect of age,gender,weight and UGT1A4142T>G gene poly-morphism on the efficacy of LTG in epileptic children treated with valproic acid ,and to determine the effective se-rum concentration of LTG in children with epilepsy in south China. Methods A total of 72 pediatric patients with epilepsy received LTG and VPA treatments were enrolled in this study. Patients were treated from January 2010 to September 2016 in Guangzhou women and childrens′medical center. Serum concentration of LTG was measured by using the liquid chromatography-tandem mass spectrometry. UGT1A4142T > G was genotyped by the polymerase chain reaction-restriction fragment length polymorphism method. The correlations between the efficacy of LTG and age,gender,weight were analyzed by chi-square test,non-parametric test and logistic regression analysis,respec-tively. Results The curative effect of patients who were younger and with lighter weight were relatively poor ,and men were better than women in the curative effect. UGT1A4142T > G was not related with LTG efficacy. When combined with VPA,the effective serum concentration of LTG in children with epilepsy was more than 2 g/mL. Conclusion There is a good correlation between age and LTG curative effect. The effective serum concentration of LTG in children with epilepsy,who were co-treated with VPA,was more than 2 g/mL. This study provides a refer-ence for the individual administration of children with epilepsy in south China.

Objective To determine whether the supplementation of ω-3 polyunsaturated fatty acids in parenteral nutrition (PN) confers treatment benefits to outcomes of septic patients.Methods The databases of MEDLINE,EMBASE,Cochrane central register of controlled trials,the CNKI and the Wanfang biomedicine were searched to identify prospective randomized controlled trials (RCTs) which compared the ω-3 polyunsaturated fatty acids supplemented with the standard PN on outcomes of adult patients with sepsis from January 1996 to June 2013.The methodological quality of the included studies was evaluated,and the Cochrane Collaboration RevMan 5.0 was used for data analysis.Results A total of 12 studies enrolling 721 patients were included.Significant reduction in 28-day mortality [relative risk (RR) 0.77,95％ confidence interval (95％CI) 0.59 to 0.99,P=0.04],short intensive care unit [ICU,weighted mean difference (WMD)=-3.10,95％CI-5.98 to-0.21,P=0.04] and hospital length of stay (WMD =-3.12,95％CI-4.65 to-1.60,P＜0.000 1) were observed in patients receiving ω-3 polyunsaturated fatty acids supplemented PN.There was no differences in days of mechanical ventilation between patients with or without adding ω-3 polyunsaturated fatty acids in PN (WMD =1.33,95％ CI-5.09 to 7.75,P=0.69).Conclusions Meta-analysis results demonstrated that PN supplemented with ω-3 polyunsaturated fatty acids was beneficial in improving the outcomes of patients with sepsis.However,this conclusion must be interpreted with caution due to the low quality of the enrolled trials.