Objective:To analyze and summarize the efficacy and safety of thalidomide in the treatment of refractory systemic juvenile idiopathic arthritis（sJIA）.Methods:The clinical data of ten patients with refractory sJIA admitted to Department of Nephrology and Immunology in Children's Hospital of Hebei Province from January 2015 to March 2022 were collected，and the clinical manifestations，efficacy and safety of thalidomide in the treatment of refractory sJIA were analyzed retrospectively. Systemic juvenile arthritis disease activity score（sJADAS）was used to evaluate the efficacy of the treatment. Statistical analysis was performed by repeated measurements using general linear models.Results:Among the 10 children（4 males and 6 females）with refractory sJIA，the average age of onset was（7.5±3.3）years. Seven patients were complicated with macrophage activation syndrome at an early stage of disease.The average course of disease was（4.4±1.7）years，and the longest course of disease was 8.3 years. Before the application of thalidomide，all the 10 children experienced relapses（ranging from 2 to 10 times）. The indices of 10 children treated with thalidomide at 6 months and 12 months were compared with those before treatment. Peripheral blood leukocytes［（10.19±3.67）×10 9/L，（8.53±2.83）×10 9/L vs.（16.11±7.81）×10 9/L， F=7.918，11.084， P=0.020，0.009］，C-reactive protein［19.13（0.38，35.21）mg/L，8.05（0.10，18.00）mg/L vs. 59.34（24.20，131.90）mg/L， F=7.030，12.731， P=0.026，0.006］，sJADAS scores［6.00（1.50，12.50）scores，3.00（0，12.50）scores vs. 20.00（11.50，28.00）scores， F=14.710，17.870， P=0.004，0.002］were decreased significantly. The doses of prednisone［0.13（0，0.45）mg/（kg·d），0.02（0，0.06）mg/（kg·d）vs. 0.42（0.16，1.47）mg/（kg·d）， F=5.890，7.623， P=0.041，0.022］were significantly decreased.All the differences were statistically significant. Prednisone was successfully discontinued in 7 cases. Tocilizumab was gradually withdrawn in 3 cases，and tocilizumab administration interval was prolonged in 1 case. None of the 10 children had serious adverse reactions. Conclusion:Thalidomide is clinically effective in the treatment of sJIA，and can reduce the required dose of prednisone and prolong the tocilizumab free remission.

Objective:To compare the similarities and differences of macrophage activation syndrome（MAS）combined with systemic juvenile idiopathic arthritis（sJIA）versus with juvenile onset systemic lupus erythematosus（JSLE）.Methods:The clinical data of 48 children with MAS admitted to the Department of Nephrology and Immunology in Children's Hospital of Hebei Province from May 2015 to January 2023 were retrospectively analyzed. The patients were divided into sJIA-MAS and JSLE-MAS group，and the clinical manifestations，laboratory indicators and treatment of the two groups were compared.Results:Among the 48 children（14 males and 34 females）with MAS，the average age of onset was 9.5（3.0，11.8）years. There were 28 cases（11males and 17 females）of sJIA-MAS and 20 cases（3 males and 17 females）of JSLE- MAS. All the 48 children with MAS had fever and hyperferinemia，and the fever with sJIA-MAS was mostly continued fever or remittent fever. Respiratory tract infection was the most common trigger in sJIA-MAS［15 cases（53.6%）］，and disease activity was the most common trigger in JSLE-MAS［13 cases（65.0%）］.Additionally，viral infections（EB virus and cytomegalovirus）were also one of the triggers in MAS［sJIA：7 cases（25%），JSLE：4 cases（20%）］.Compared with JSLE-MAS，the number of days with fever［15.0（12.0，21.0）days vs. 6.0（4.0，9.5）days， Z=-3.812， P=0.001］and the length of hospital stay［29.0（26.3，39.8）days vs.26.0（19.3，30.8）days， Z=-1.958， P=0.049］were longer in sJIA. Compared with JSLE-MAS，ALT［（685.32±561.67）U/L vs.（139.61±124.44）U/L， t=4.973， P=0.001］，AST［784.00（235.25，1 251.25）U/L vs.189.50（53.25，374.08）U/L， Z=-3.283， P=0.001］，CRP［11.48（3.56，28.89）mg/L vs.1.91（0.53，8.98）mg/L， Z=-3.200， P=0.001］，ferritin［32 167.0（12 384.8，65 963.8）μg/L vs.2 003.5（922.5，11 430.0）μg/L， Z=-4.130， P=0.001］，ferritin max/ESR min［1 353.35（355.75，4 342.53）vs.91.92（34.94，291.53）， Z=-4.120， P=0.001］were higher in sJIA.The decrease of CRP was greater in sJIA［80.04（45.64，143.71）mg/L vs.10.20（6.27，25.64）mg/L， Z=-4.433， P=0.001］.Compared with sJIA-MAS，peripheral white blood cell counting［4.05（2.90，7.73）×10 9/L vs.1.56（1.15，3.47）×10 9/L， Z=-3.577， P=0.001］and platelet counting［（162.68±92.19）×10 9/L vs.（110.10±72.99）×10 9/L， t=2.118， P=0.040］were lower in JSLE-MAS. Kidney involvement was more common in JSLE-MAS［10 cases（50%）vs.0 cases（0%）， χ 2=17.684， P=0.001］.There was no significant difference in the incidence of sJIA-MAS and JSLE-MAS meeting the criteria of hemophagocytic lymphohistiocytosis［6 cases（21.4%）vs.5 cases（25.0%）， χ 2=0.084， P=0.772］. Conclusion:Compared with JSLE-MAS，sJIA-MAS is more dangerous and difficult to control，while JSLE-MAS involves more organs，among which the blood system and kidney are more common.

Purpose: This study identified risk factors for neurogenic lower urinary tract dysfunction (NLUTD) in patients with acute ischemic stroke (AIS) through multidimensional analysis of the medical records of patients, aiming to reduce the incidence of NLUTD, improve prognosis, and facilitate rehabilitation. Materials and Methods: In this case-control study, patients with AIS were recruited from two tertiary general hospitals in Shenzhen, China, from March 2021 to October 2023. Patients were divided into NLUTD and non-NLUTD groups based on the presence and absence of NLUTD, respectively. Comparative analysis was performed using the Mann–Whitney U and chi-square tests, with significant variables being included in logistic regression analysis. Results: Of the 652 participants enrolled in this study, 119 participants (18.3%) developed NLUTD. Bivariate analysis showed that 39 of 54 screened factors exhibited a significant correlation (p<0.05) with the incidence of NLUTD after AIS. Significant variables identified through logistic regression analysis included Glasgow coma scale (GCS) and National Institutes of Health Stroke Scale (NIHSS) scores, anemia, aphasia, pneumonia, brainstem involvement, multiple lesions, urine clarity (CLA), random venous blood glucose (GLU) and hemoglobin (HGB) levels, and white blood cell (WBC) count. Conclusions A total of 11 risk factors for NLUTD were identified in this study. This finding provides valuable guidance for reducing the incidence of NLUTD after AIS and improving the quality of life of patients.

The data of a child with sphingosine phosphate lyase insufficiency syndrome (SPLIS) admitted to Children′s Hospital of Hebei Province on February 4, 2020 were retrospectively analyzed.The child had edema, complicated with ichthyosis, adrenal calcification, and hearing loss from the early infancy.Laboratory examination results suggested a low albumin level, hypercholesterolemia, a high proteinuria level, abnormal liver and renal functions, and hyponatremia.The child gave up treatment and died at home.Whole Exome Sequencing (WES) results showed two hete-rozygous mutations of SGPL1 gene (chr10: 72604336, c.134G>A, p.W45X; chr10: 72629563, c.719G>T, p.S240I). SPLIS is inherited in an autosomal recessive manner.It starts in infancy, and affects the kidney, skin, endocrine, nervous and immune systems.It is suggested that SPLIS patients should take genetic examination.Early diagnosis, appropriate intervention, and vitamin B 6 treatment may relieve some symptoms of SPLIS patients.Adeno-associated virus mediated SGPL1 gene replacement therapy can be a novel cure of SPLIS and is worthy of investigation.

This article reported a rare case of harlequin ichthyosis which was indicated with multiple structural abnormalities by prenatal ultrasound and diagnosed by trio-based whole-exome sequencing (Trio-WES). Prenatal diagnosis was performed because the ultrasound at 24 +4 gestational weeks revealed the fetus presenting with eclabium, flattened nose, short mandible, small auricle and abnormal posture of the toes. Copy number variation sequencing (CNV-seq) showed no chromosome aneuploidy or pathogenic copy number variants over 100 kb in the fetal or parental samples. Trio-WES showed that the fetus carried two heterozygous mutations, c.2593-1G>A and c.7444C>T in ABCA12. Sanger sequencing confirmed that c.2593-1G>A, a previously unreported variant, was paternally inherited and c.7444C>T was maternally inherited. Both parents had normal phenotype. The fetus was finally diagnosed with harlequin ichthyosis. After prenatal counseling, the parents made an informed choice to terminate the pregnancy at 28 +4 gestational weeks. The stillborn fetus showed multiple malformations The variants in this case expand the spectrum of variants in ABCA12 gene.

Objective:To explore the path of standardized training and quality supervision for geriatric nursing assistants basing on the three-way linkage of "hospital, government and market".Methods:From January 2019 to December 2020, we conducted standardized training and management for geriatric nursing assistants in 24 nursing institutions for the aged in Mianyang, Sichuan Province in batches. Through the three-way linkage of "hospital, government and market", the whole-process management mode of "standardized training of geriatric nursing assistants, one-to-many tracking service for three months after the training, and service quality supervision of nursing institutions for the aged" has been established. The theoretical and operational achievements of nursing staff before and after training, the service quality of nursing home and the incidence of adverse events before and after the implementation of the project were observed and compared.Results:From 2019 to 2020, a total of 224 geriatric nursing assistants were trained in five sessions. There were statistical differences in the theoretical and operational scores before and after the training ( t=-72.494, -26.150; P<0.01) . 13 nursing institutions participating in the training in 2019 were selected to compare the service quality before and after the project implementation, the difference was statistically significant ( t=-14.575, P<0.01) , and the incidence of nursing adverse events decreased (χ 2=-7.913, P<0.01) . Conclusions:The mode of geriatric nursing assistants training and quality supervision based on the three-way linkage of "hospital, government, market" is conducive to giving full play to the administrative function of the government and the professional function of the hospital, effectively integrating resources and improving the quality of geriatric nursing service in the region.

OBJECTIVE:To prospectively study the effects of trimetazidine on myocardial remodeling and oxidative stress in patients with hypertensive heart disease (HHD),in order to provide reference for clinical treatment of HHD.METHODS:Eighty-two HHD patients were selected from our hospital during Jan.2014-Jul.2016,and they were divided into control group and observation group by sortition randomization method,with 41 cases in each group.Control group received routine HHD chemical drug (antihypertensive drugs,lipid-lowering drugs,hypoglycemic agents and antiplatelet drugs,etc.) therapy.Observation group was additionally given trimetazidine 20 mg,tid,on the basis of control group.Both groups were treated for 3 months.Before treatment and after 3 months of treatment,SBP,DBP,New York Heart Association (NYHA) cardiac function grade,LVEF,LVESD,LVEDD,LVMI and the levels of GSH-Px,SOD,MDA and ROS were compared between 2 group.The occurrence of ADR was observed in 2 groups.RESULTS:Before treatment,there was no statistical significance in each index between 2 groups (P＞0.05).After 3 months of treatment,SBP and DBP of 2 groups were decreased significantly compared to before treatment (P＜0.01);there was no statistical significance between 2 groups (P＞0.05).There was no statistical significance in NYHA cardiac function grade compared to before treatment or between 2 groups (P＞0.05).LVESD,LVEDD and LVMI of observation group were decreased significantly compared to before treatment (P＜0.05);LVEF was increased significantly compared to before treatment (P＜0.05);LVEDD and LVMI of observation group were significantly lower than control group (P＜0.05).Compared to before treatment,SOD level of control group was decreased significantly,while the levels of GSH-Px and SOD in observation group were increased significantly;MDA and ROS of observation group were significantly lower than those of control group,with statistical significance (P＜0.05).No obvious ADR was found in 2 groups.CONCLUSIONS:Trimetazidine can improve cayocardial remodeling and reduce oxidative stress level of HHD patients with good safety.

Objective To evaluate the efficacy and safety of tigecycline-based treatment approach on severe infection of patients with hematological diseases. Methods The clinical data of 64 patients who were treated with tigecycline-based treatment approach for severe infection were retrospectively reviewed. The curative effect was evaluated, meanwhile the drug side effects were observed. Results A total of 51 strains of bacteria were isolated from 64 patients, including 12 extended-spectrum β-lactamase(ESBL)and 15 multi-drug resistant strains and the total effective rate was 59.4%(38/64). Five patients diagnosed as carbapenem resistant infection and were treated with the addition dose of tigecycline and 3 patients relieved. Main adverse events were nausea, vomiting, diarrhea and hepatic dysfunction, but all events were slight. Conclusions Tigecycline-based treatment approach has a good clinical efficacy in treating severe infection of patients with hematological diseases, and the side effect is few.Tigecycline-based treatment approach could be used as a new choice for patients non-responding favorably to conventional anti-infective treatment or multiple resistant bacteria.

Objective To study the clinical features of congenital glucose-galactose malabsorption (CGGM),and to improve the understanding of CGGM.Method Clinical manifestations and treatment process of one patient with CGGM in our hospital were retrospectively analyzed.From 1966 to 2016 May,Chinese medical database and PUBMED were searched using Malabsorption syndrome,dehydration,hypernatremia , diarrhea , newborn , carbohydrate metabolism ,andglucose/galactose malabsorption as key words.The clinical features of CGGM reported in literatures were summarized.Result The patient in our hospital was a full-term female infant naturally delivered.The onset of the disease was on the 9th day after birth,and the clinical manifestations included severe diarrhea,severe dehydration,hypernatremia,metabolic acidosis and malnutrition.After intravenous infusion and symptomatic treatment,dehydration,hypernatremia and metabolic acidosis were corrected.However,there was no improvement of diarrhea characterized with watery and acidic stools,and neither was weight gain.Glucose loading test was negative,and fructose loading test was positive.Diarrhea was improved markedly using diagnostic carbohydrate-free formula,so CGGM was diagnosed clinically.SLC5A1 homozygous IVS7-2 A ＞ G mutation was detected which confirmed the diagnosis of CGGM.With carbohydrate-free formula feeding,the body weight of the infant was increased.Followed up for 2 months now,her body length and body weight were at P25 and P22 on growth curve respectively,and no obvious neurological sequela was observed.Our literature review revealed 7 reports including 48 cases of CGGM children.Literature review showed that:most children with CGGM (79.2％) had the onset within 7 days of life;main clinical features included diarrhea (100％),dehydration (100％),and malnutrition (54.2％);22.9％ of patients with carbohydrate-free formula and 27.1％ with fructose matrix formula were fed well;no death was detected,77.1％ had normal weight gain,and 91.7％ had normal development of the nervous system.Conclusion CGGM is rare.The symptoms include severe watery and acidic stools with onset during neonatal period.CGGM is associated with severe complications such as hypertonic dehydration and hypernatremia.The diagnosis is established based upon typical clinical manifestations,sugar loading test and SLC5A1 gene detection.Carbohydrate-free formula feeding is effective.

Uihgur medicine is not only an important component of medicine for ethnic minorities in China,but an vital supplement to modern medicine.With the development of modern science,in recent years good progerss has been made in basic sciences and applied research of Uighur medicine.According to the property of Uighur materia medica,medicine can be divided into hot,cold,wet,dry,dry-hot,wet-hot,wet-cold,and dry-cold types,and according to strength of medicinal properties,this medicine is divided into four classes.Most of the drugs of levels 3 and 4 are credited with toxicity.This article summarizes the property and toxicity of medicines of levels 3 and 4 which are includel in Pharmaceutical Standards-Uighur Medicine (1998) edited by the former State Ministry of Health of the People's Republic of China.