The resistance to steroid remains a major trouble in the treatment of idiopathic nephrotic syn-drome,and the mechanisms are complicated. Recent studys have shown that multidrug resistance gene l and P-glycoprotein170,glucocorticoid receptor,renal pathology,gene mutation and complications are all closely related to the resistance to steroid. This review is focused on the mechanisms of steroid resistance of children′s idiopath-ic nephrotic syndrome.

Objective To provide reference and ideas for information service in hospital library by analyzing the influencing factors for its service satisfaction.Methods The satisfaction of hospitalized patients for information service was investigated.The characteristics of patients,their requirements for information service and the influencing factors for their satisfaction were analyzed by logistic regression analysis.Their satisfaction for information service was assessed according to the IPA method and analyzed using the SPSS19.0.P<0.05 was considered statistically significant.Results Health information was provided for 221 patients.No significant difference was found in sex,age,education level between the importance of information service and the influence of satisfaction for information service (P>0.05).The importance of information service and the satisfaction of patients for information service were rather high.IPA analysis showed that the patients considered high the importance of library-provided personal bibliography and departments-provided health pamphlets with a low satisfaction,which should thus be solved.Conclusion The satisfaction of patients for information service is good.However,the importance of library-provided personal bibliography and departments-provided health pamphlets is high and the satisfaction for them is low.It is thus necessary for librarians to improve their service and encourage more medical staff to participate in information service for patients.

The 10-month baby boy,with normal development,mainly due to sleep in frequent tongue bite nearly 4 months.Bitten his tongue after faring asleep,biting bleeding,bite pain awake.Many of his tongue ulcers,serious impact on children's lives,family companionship in suffering.History found in the supplementary week before the onset of the left frontal children hurt skin bruising.Electroencephalogram showed:Sleep of epileptiform discharges in the left frontal and central anterior temporal areas,but bite the tongue during sleep electroencephalogram synchronization no relevant abnormal discharge.The final diagnosis of traumatic epilepsy,frontal lobe epilepsy syndrome automatically lead to tongue bite tongue with traumatic ulcers.Oral Clonazepam 0.25 mg before sleep,the symptoms disappeared that night,nighttime sleep peacefully.His tongue ulceration has healed after a month.Readers are advised to take advantage of these key parts of the diagnostic process and diagnostic thinking or diagnostic procedures,combined with their own clinical practice,serious thinking,learning,summarized,and benefit from it.

Objective:To explore the changes in the diffusion tensor imaging (DTI) parameters of the pontocerebellar tract 3 months and one year after ischemic stroke and analyze the data′s potential for predicting long-term motor outcomes.Methods:Thirty patients with middle cerebral artery infarction were prospectively studied using DTI within 3 months and 1 year after the onset. A region of interest-based analysis was performed for the fractional anisotropy (FA) of the middle cerebellar peduncles (MCP) in the pontocerebellar fibers (PCF). Neurological functioning was evaluated using the National Institutes of Health Stroke Scale and the degree of paresis was assessed at the same time using paresis grading. At one year after the onset, balance function, functional prognosis and self-care ability were evaluated using the Brunel Balance Scale, the modified Rankine Scale and the Functional Independence Scale respectively.Results:The average FA on the healthy side 3 months and 1 year after onset was significantly less than on the affected side at each time point. The ratio of the FA of the affected side to that of the unaffected side (rFA) in the PCF correlated significantly with the average paresis scores of the lower and upper extremity and the total paresis score at each time point. Moreover, the rFA of the PCF was significantly correlated with the average functional independence score, the prognosis for the upper and lower extremities as well as motor functioning. The area ratio under the ROC curve of the PCF for predicting lower extremity motor outcome was 0.84, and the optimal threshold was 0.92 (sensitivity 71.4%, specificity 73.9%). The area ratio under the ROC curve was not a significant predictor of upper extremity motor outcome.Conclusion:The DTI parameter rFA of the PCF in patients with subacute middle cerebral artery infarction can help to predict the long-term recovery of motor ability. It can serve as an important reference index for predicting the long-term motor ability of the lower limbs.

Objective:To understand the distribution characteristics of pulse oxygen saturation (SpO 2) in the high altitude areas. Methods:From September 2016 to December 2017, all healthy and full-term live births in the First Hospital of Lanzhou University and Dunhuang, Jiuquan, Jingning and Lintao branches were selected as the research objects.Critical congenital heart disease or pulmonary diseases were excluded in advance.SpO 2 in different parts of newborns within 12 hours of birth was measured.Statistical comparisons of SpO 2 in different parts of male and female neonates in the same area and SpO 2 in different parts of different regions were made.The number of SpO 2 and the distribution of percentile difference between the lower limbs and the right upper limb of newborns at different altitude areas were obtained. Results:There were no significant difference between male and female infants in SpO 2 value at any lo-wer extremity and right upper extremity and the difference value in the same area (all P>0.05). There were significant differences in the median ( M) values of SpO 2 and the M value of the difference between the two values of any lower limb and right upper limb at different altitudes (all P<0.05). The results showed that the M value of SpO 2 of the right upper limb ( M=96%) in Lanzhou (1 520 m above sea level) was lower than that in Dunhuang (1 138 m above sea level, M=97%) and Jiuquan (1 481 m above sea level, M=97%), and the M value of the difference between the two values ( M=2%) was higher than that in Dunhuang and Jiuquan ( M=1%) ( P<0.05). The M of SpO 2 of any lower limb ( M=96%) and right upper limb ( M=96%) in Jingning areas (1 668 m above sea level) were lower than that in Dunhuang and Jiuquan area (all P<0.05). The median value of SpO 2 of any lower limb ( M=96%) and right upper limb ( M=96%) in Lintao area (1 883 m above sea level) was lower than that in Dunhuang and Jiuquan areas, and the M value of the difference between the two values ( M=2%) was higher than that in Dunhuang and Jiuquan (all P< 0.05). When the altitude was lower than 1 500 m, the M value of SpO 2 was 97%, P3- P97 was 93%-100%; the M value of the difference between any lower limb and right upper limb was 1, and P3- P97 was 0-4.When the altitude was higher than 1 500 m, the M value of SpO 2 was 95%-96%, P3- P97 was 89%-100%; the M value of SpO 2 of an lower limb and right upper limb was 1%-2%, and P3- P97 was 0-9%. Conclusions:The SpO 2 number and percentage distribution of the difference between any lower limb and right upper limb of newborns at different altitudes are different.

Objective:In order to explore the impact of corona virus disease 2019(COVID-19)on the hospitalization of children with bronchiolitis and to improve clinicians′ understanding of the characteristics of bronchiolitis during the COVID-19 epidemic.Methods:This was a multicenter clinical study, and the data have been collected from 23 children′s medical centers in China.All the clinical data were retrospectively collected from children with bronchiolitis who were hospitalized at each study center from January 1, 2019 to December 31, 2021.The results included gender, age at hospitalization, length of stay, respiratory syncytial virus(RSV) test results, severity rating, ICU treatment, and the total number of children hospitalized with respiratory tract infection during the same period.The clinical data of children with bronchiolitis in 2019 before COVID-19 epidemic and in 2020、2021 during COVID-19 epidemic were statistically analyzed and compared.Results:According to a summary of data provided by 23 children′s medical centers, there were 4 909 cases of bronchiolitis in 2019, 2 654 cases in 2020, and 3 500 cases in 2021.Compared with 2019, the number of bronchiolitis cases decreased by 45.94% in 2020 and 28.70% in 2021.In 2019, 2020 and 2021, there were no significant differences in gender ratio, age, and duration of hospitalization.Compared with 2019, the ratio of bronchiolitis to the total number of hospitalizations for respiratory tract infection decreased significantly in 2020 and 2021( χ2=12.762, P<0.05; χ2=84.845, P<0.05).The proportion of moderate to severe bronchiolitis cases in both 2020 and 2021 was lower than that in 2019, and the difference was statistically significant ( χ2=4.054, P<0.05; χ2=8.109, P<0.05).There was no statistically significant difference in the proportion of bronchiolitis cases requiring ICU treatment between 2019, 2020, and 2021 ( χ2=1.914, P>0.05).In 2019, a total of 52.60%(2 582/4 909) of children with bronchiolitis underwent RSV pathogen testing, and among them, there were 708 cases with RSV positive, accounting for 28.00%.In 2020, 54.14%(1 437/2 654) of children with bronchiolitis underwent RSV pathogen testing, and there were 403 cases with RSV positive, accounting for 28.04%.In 2021, 66.80%(2 238/3 500) of children with bronchiolitis underwent RSV pathogen testing, and there were 935 cases with RSV positive, accounting for 41.78%.Compared with 2019 and 2020, the RSV positive rate in 2021 showed a significant increase( χ2=99.673, P<0.05; χ2=71.292, P<0.05). Conclusion:During the COVID-19 epidemic, the implementation of epidemic prevention and control measures reduced the hospitalization rate and severity of bronchiolitis, but did not reduce the positive rate of RSV detection.

OBJECTIVETo evaluate the therapeutic effect and safety of rapamycin in treatment of children with tuberous sclerosis complex (TSC) complicated with epilepsy.

METHODThis was an open-label, prospective, self-controlled study. From Sep. 2011 to Sep. 2013, 52 patients with the diagnosis of tuberous sclerosis complicated with epilepsy receiving rapamycin treatment for at least 24 weeks were enrolled.

RESULTOf the 52 children, 34 were male and 18 female. The median age at onset of epilepsy was 4.8 months (4 days-49 months), the median age for treatment with rapamycin was 27 months (4.5-172.5 months). Ten children had a family history of TSC. In 24 children TSC gene detection was carried out, among whom TSC1 mutation was detected in 4 cases and TSC2 mutation in 20. Before rapamycin therapy, 59.62%, (31/52) patients took more than 3 antiepileptic drugs, of whom 10 cases even took more than 5 kinds of antiepileptic drugs. Fifty-two patients received rapamycin treatment for 24 weeks, seizure free rate was 25.00% (13 cases), the total effective rate was 73.08% (38 cases); 31 cases received treatment for 48 weeks, seizure free 6 cases, total effective 23 cases; 17 cases accepted treatment for 72 weeks, seizure free 5 cases, total effective 13 cases; 12 cases received treatment for 96 weeks, seizure free 3 cases, total effective 9 cases. With the decrease of seizure attacks, use of antiepileptic drug types were reduced simultaneously, they had a negative correlation. Before rapamycin therapy, the average frequency of seizures was 70.27 times/d, the number of antiepileptic drug kinds was 1.30. After 24, 48, 72, 96 weeks' treatment, the average seizure frequency was reduced to 1.94-2.80 times /d and the antiepileptic drugs were reduced to 0.83-0.97 kinds. On every visit during the follow-up, blood and urine routine tests, liver and kidney function test showed no abnormality in the 52 cases. The drug dosage was 1 mg/(m(2)×d), average 0.7 mg/d (0.35-1.20 mg/d). Blood concentrations of rapamycin remained below 10 µg/L (average 6.5 µg/L). The main side effect was oral ulcer which happened in 23.08% (12/52). The oral ulcer would disappeared 2-3 days later. 17.31% (9/52 cases) had upper respiratory infection.

CONCLUSIONRapamycin was effective in children with tuberous sclerosis and epilepsy with few adverse reactions. The daily dose of rapamycin for children patients is 1 mg/m(2), which has a certain effect on seizures and a good safety profile.

Adolescent ; Anticonvulsants ; adverse effects ; therapeutic use ; Child ; Child, Preschool ; Epilepsy ; complications ; drug therapy ; Female ; Humans ; Infant ; Male ; Prospective Studies ; Seizures ; prevention & control ; Sirolimus ; adverse effects ; therapeutic use ; Treatment Outcome ; Tuberous Sclerosis ; complications ; genetics

OBJECTIVEThe authors sought to investigate the clinical features and characteristics of genetic mutation in patients with aspartylglucosaminuria.

METHODClinical data of two pediatric siblings in a family were analyzed retrospectively and relative literature was reviewed in order to study the clinical features, imaging and enzymatic characteristics and genetic mutations.

RESULTCase 1, the proband, male, he was hospitalized at 20 months of age because of fever and hepatosplenomegaly for nine days. This child was of moderate nutritional status and normal development. Blood tests showed hemoglobin 78.0 g/L, RBC3.18 × 10¹²/L, WBC 4.06 × 10⁹/L, neutrophils 0.236, lymphocytes 0.631, platelets 34 × 10⁹/L, C-reactive protein 17 mg/L. Blood biochemistry showed alanine aminotransferase 67.1 U/L, aspartate aminotransferase 74.1 U/L, serum albumin 32.8 g/L, direct bilirubin 10.5 µmol/L, lactate dehydrogenase 301.7 U/L. Bone marrow cytology showed reactive morphological changes in bone marrow cells. Atypical lymphocytes could be seen in both peripheral blood and bone marrow smears. Cranial MRI showed poor myelination. Aspartylglucosaminidase activity in peripheral leucocytes of the proband 5.7 nmol/(g × min) vs. normal control>26.6 nmol/(g × min). On his AGA gene and that of his parents, a heterozygous mutation site located in exon 3, c.392C>T (p.S131L), was identified as a novel mutation inherited from his father. The mutation from his mother has not been detected. The proband was not responsive to the anti-infectious medication, nutritional intervention and symptomatic treatment.He died one month after diagnosis.His elder brother, Case 2, showed fever, recurrent respiratory tract infection and progressive psychomotor regression with hepatosplenomegaly from the age of four years. Cranial MRI revealed extensive symmetrical leukodystrophy in bilateral cerebra, cerebellum and brainstem.He died at the age of six years.Related literature was summarized, and no Chinese AGU cases had been reported; 221 foreign cases were collected. The clinical and imaging characteristics were summarized. Delay in language development was one of the clinical symptoms that the majority of parents of AGU children first noted.

CONCLUSIONPatients with aspartylglucosaminuria lack of specific symptoms.For children with unexplained delayed speech and progressive mental retardation, the possibility of AGU should be considered, and efforts be made for enzymatic and genetic diagnosis. c.392C> T (p.S131L) was identified as a novel mutation of AGA gene.

Aspartylglucosaminuria ; diagnosis ; genetics ; pathology ; Aspartylglucosylaminase ; genetics ; metabolism ; Biomarkers ; blood ; Brain ; pathology ; Child, Preschool ; DNA Mutational Analysis ; Heterozygote ; Humans ; Infant ; Lysosomal Storage Diseases ; diagnosis ; genetics ; pathology ; Magnetic Resonance Imaging ; Male ; Mutation ; Pedigree ; Polymerase Chain Reaction