1.Analysis of risk factors and severity prediction of acute pancreatitis induced by pegaspargase in children
Xiaorong LAI ; Lihua YU ; Lulu HUANG ; Danna LIN ; Li WU ; Yajie ZHANG ; Juan ZI ; Xu LIAO ; Yuting YUAN ; Lihua YANG
Chinese Journal of Applied Clinical Pediatrics 2024;39(3):170-175
Objective:To analyze the risk factors for asparaginase-associated pancreatitis (AAP) in children with acute lymphoblastic leukemia (ALL) after treatment with pegaspargase and evaluate the predictive value of pediatric sequential organ failure assessment (SOFA) score, pediatric acute pancreatitis severity (PAPS) score, Ranson′s score and pediatric Ministry of Health, Labour and Welfare of Japan (JPN) score for severe AAP.Methods:Cross-sectional study.The clinical data of 328 children with ALL who received pegaspargase treatment in the Department of Pediatric Hematology, Zhujiang Hospital, Southern Medical University from January 2014 to August 2021, as well as their clinical manifestations, laboratory examinations, and imaging examinations were collected.The SOFA score at the time of AAP diagnosis, PAPS score and Ranson′s score at 48 hours after AAP diagnosis, and JPN score at 72 hours after AAP diagnosis were calculated, and their predictive value for severe AAP was evaluated by the receiver operating characteristic (ROC) curve.Results:A total of 6.7%(22/328) of children had AAP, with the median age of 6.62 years.AAP most commonly occurred in the induced remission phase (16/22, 72.7%). Three AAP children were re-exposed to asparaginase, and 2 of them developed a second AAP.Among the 22 AAP children, 16 presented with mild symptoms, and 6 with severe symptoms.The 6 children with severe AAP were all transferred to the Pediatric Intensive Care Unit (PICU). There were no significant differences in gender, white blood cell count at first diagnosis, immunophenotype, risk stratification, and single dose of pegaspargase between the AAP and non-AAP groups.The age at diagnosis of ALL in the AAP group was significantly higher than that in the non-AAP group ( t=2.385, P=0.018). The number of overweight or obese children in the AAP group was also higher than that in the non-AAP group ( χ2=4.507, P=0.034). The areas under the ROC curve of children′s JPN score, SOFA score, Ranson′s score, and PAPS score in predicting severe AAP were 0.919, 0.844, 0.731, and 0.606, respectively.The JPN score ( t=4.174, P=0.001) and the SOFA score ( t=3.181, P=0.005) showed statistically significant differences between mild and severe AAP. Conclusions:AAP is a serious complication in the treatment of ALL with combined pegaspargase and chemotherapy.Older age and overweight or obesity may be the risk factors for AAP.Pediatric JPN and SOFA scores have predictive value for severe AAP.
2.A consensus on the management of allergy in kindergartens and primary schools
Chinese Journal of School Health 2023;44(2):167-172
Abstract
Allergic diseases can occur in all systems of the body, covering the whole life cycle, from children to adults and to old age, can be lifelong onset and even fatal in severe cases. Children account for the largest proportion of the victims of allergic disease, Children s allergies start from scratch, ranging from mild to severe, from less to more, from single to multiple systems and systemic performance, so the prevention and treatment of allergic diseases in children is of great importance, which can not only prevent high risk allergic conditions from developing into allergic diseases, but also further block the process of allergy. At present, there is no consensus on the management system of allergic children in kindergartens and primary schools. The "Consensus on Allergy Management and Prevention in Kindergartens and Primary Schools", which includes the organizational structure, system construction and management of allergic children, provides evidence informed recommendations for the long term comprehensive management of allergic children in kindergartens and primary schools, and provides a basis for the establishment of the prevention system for allergic children.
3.Liddle syndrome complicated with Gordon syndrome: A case report
Xiaorong PAN ; Xiang FANG ; Dongze LI ; Rong YANG ; Jia WU ; Yalin ZHANG ; Yi YAO ; Chenxi GAO ; Xiaoyang LIAO
Chinese Journal of Clinical Thoracic and Cardiovascular Surgery 2022;29(09):1236-1238
Liddle syndrome and Gordon syndrome are two rare single-gene inherited hypertension diseases. In patients≤40 years, the prevalence of Liddle syndrome is about 1% and Gordon syndrome is uncertain all over the word, for which is often misdiagnosed and mistreated. The therapies of those diseases are targeted at gene mutation sites, as well as combined with modified lifestyle, and can achieve satisfactory diseases control. This paper reports a patient who is diagnosed with Liddle syndrome and Gordon syndrome at the same time. We aimed to consolidate and improve the diagnosis and accurate treatment of those two diseases by sharing, studying and discussing together with clinical doctors.
4.Cerebral venous sinus thrombosis caused by pegasparaginase: a clinical analysis of 8 cases
Yinyan YAN ; Hongyong LIU ; Lihua YU ; Danna LIN ; Lulu HUANG ; Xiaorong LAI ; Yajie ZHANG ; Juan ZI ; Li WU ; Xu LIAO ; Lihua YANG
Chinese Journal of Neuromedicine 2022;21(5):462-468
Objective:To investigate the risk factors and prognoses of cerebral venous sinus thrombosis (CVST) caused by pegasparaginase (PEG-Asp).Methods:A total of 252 children with acute lymphoblastic leukemia (ALL) were treated with PEG-Asp chemotherapy in our hospital from December 2016 to July 2021, including 8 children with CVST. The clinical manifestations, laboratory and imaging features, treatments and prognoses of these children with CVST caused by PEG-Asp were analyzed retrospectively.Results:(1) CVST occurred during induction chemotherapy in 4 children, during re-induction chemotherapy in 3 children, and during consolidation stage in one child. CVST occurred in two children who received PEG-ASP chemotherapy once, in one child who received PEG-Asp chemotherapy twice, and 5 children who received PEG-Asp chemotherapy more than twice. The median time between CVST occurrence and last treatment of PEG-Asp was 20.5 d. (2) The clinical manifestations included paroxysmal headache ( n=4), nausea or vomiting ( n=3), convulsions ( n=2) and persistent blurred vision ( n=1). (3) CVST appeared at the sigmoid sinus ( n=6), transverse sinus ( n=4) and superior sagittal sinus ( n=4), of which one child was complicated with hemorrhage in left frontal parietal and right parietal cortex, and one with reversible posterior encephalopathy syndrome; 8 children were not complicated with thrombus in other parts. (4) Some of the children were complicated with abnormal blood coagulation. When CVST occurred, fibrinogen level decreased in 3 children, anti-thrombin III level decreased in 2 children, and D-dimer level increased in 3 children. (5) Six children were treated with low molecular weight heparin (LMWH), of which, 4 were treated with rivasaban and one with warfarin sequentially. The total course of anticoagulation was 56 d. (6) The symptoms of 6 children disappeared after anticoagulation; Magnetic resonance venography (MRV) showed disappeared thrombus in 4 children and reduced thrombus range in 2 children. One child with intracranial hemorrhage did not use PEG-Asp anymore; 7 accepted PEG-Asp further during follow-up chemotherapy, of which one had CVST recurrence and the range of thrombus was reduced after anticoagulant therapy. Conclusions:When children with ALL develop unexplained neurological symptoms during PEG-Asp chemotherapy, CVST should be highly vigilant. Enhanced MRI and MRV should be performed for early diagnosis. Some children are complicated with abnormal blood coagulation, and LMWH, warfarin and rivasaban are effective. The prognosis is good and there are no sequelae. Most children accepted PEG-Asp again will not have CVST again.
5.Clinical features and risk predictors of autism and intellectual disability in patients with Lennox-Gastaut syndrome
Na HE ; Bingmei LI ; Jie WANG ; Xiaorong LIU ; Bin LI ; Wenjun BIAN ; Siwen OU ; Zhiwei GAO ; Weiping LIAO ; Bin TANG
Chinese Journal of Neuromedicine 2019;18(4):381-386
Objective To investigate the co-occurrence incidence,clinical features and risk predictors of autism and intellectual disability in patients with Lennox-Gastaut syndrome (LGS).Methods Sixty-four patients with LGS were recruited in our Epilepsy Center from June 2012 to June 2018.Autism Behavior Checklist (ABC) and Childhood Autism Rating Scale (CARS) were performed to evaluate autism,while Chinese Wechsler Intelligence Scale for Children (C-WISC) and Gesell Developmental Scale were applied to estimate intelligence.The influences of different clinical factors in autism and intellectual disability were analyzed in patients with LGS.Results Among 64 patients with LGS,only three (4.7%) were diagnosed as having autism,and their average ABC and CARS scores were 80.0 and 40.0,respectively.The average ABC and CARS scores were 40.9±26.7 and 26.0±8.9 in thepatients with onset age<one year,which were significantly higher than those in other two groups,respectively (P<0.05).The average ABC and CARS scores in the patients accepted antiepileptic drugs (AEDs) ≥ 3 were 27.8±22.8 and 22.2±8.7,which were significantly higher than those in the patients accepted one or two kinds ofAEDs (P<0.05).In addition,the ABC and CARS scores showed significant differences in the groups with different seizure frequency and in the groups with or without symptomatic etiologies (P<0.05).Fifty patients (78.1%) presented different levels of intellectual disability;severe intellectual disability was the leading type,which accounted for 31.3% (20/64);12(18.8%),7(10.9%),and 11 (17.2%) patients were with mild,moderate or profound intellectual disability,respectively.As compared with patients without intellectual disability,patients with intellectual disability had younger onset age,higher proportion of slow background activity on EEG and higher proportion of symptomatic etiologies,with significant differences (P<0.05).Conclusion Patients are in higher risk of autism when they have earlier epilepsy onset age,higher frequency of epilepsy seizure attack,administration of AEDs ≥3 and symptomatic etiologies;early onset age is an independent risk predictor for intellectual disability of patients with LGS.
6.Identification and repositioning of catheter malposition during PICC insertion in 113 cancer patients
Lewen SHAO ; Xiaorong HU ; Aiyun JIN ; Qiaofen LIAO ; Weiming LU
Chinese Journal of Nursing 2018;53(4):454-456
This paper summarized identification and reposition strategies of catheter malposition in 113 cancer patients with peripherally inserted central catheters(PICCs) during catheterization.In the process of PICC catheterization,catheter malposition was identified by ultrasound and ECG in a real-time manner.A series of timely and effective reposition procedures were performed by internal jugular vein blocking,breathing combined with anterior segment wire withdrawal Overall,111 cases were successfully repositioned,the success rate was 98.23%,and 2 cases failed because of other reasons such as diseases.
7.Influence of SCN1A intronic mutations in mRNA splicing and relation of mRNA splicing changes with phenotype in febrile seizures related epilepsy
Lu YU ; Heng MENG ; Bin TANG ; Haiqing XU ; Xiuqu CAI ; Na HE ; Xiaorong LIU ; Bingmei LI ; Meimei GAO ; Yiwu SHI ; Yonghong YI ; Weiping LIAO
Chinese Journal of Neuromedicine 2018;17(8):757-764
Objective To study the influence of SCNIA intronic mutations in mRNA splicing in febrile seizures related epilepsy,and investigate the association between splicing changes and genotype-phenotype-inheritance pattern.Methods Molecular cloning of 5 SCN1A intronic mutations was performed in patients with partial epilepsy with antecedent febrile seizures plus (PEFS+) and Dravet syndrome (DS) through constructing mutant and wild-type plasmids of pTragetE2-3-4-5 and E24-25-26 by using Minigene splicing assay,and the in vitro expressions in HENK293 cells were detected.The mRNA splicing changes were analyzed qualitatively and quantitatively by reverse transcription (RT)-PCR and real time quantitative (q)-PCR.Results (1) Using RT-PCR,DS mutants presented a whole exon skipping without significant remain of normal mRNA transcripts,while PEFS+ mutants showed partial exon skipping or intronic insertion with coexistence of normal and aberrant mRNA transcripts.(2) Statistical differences were found between relative quantity (RQ) of aberrant and normal mRNA in PEFS+ mutant (c.473+5G>A:4.92%±1.05% and 6.10%±0.21%;c.473+5G>C:7.97%±1.12% and 3.94% ±1.25%) and that in DS mutant (c.602+1G>A:60.51%±1.81% and 0.060%±0.022%,P<0.05);similarly,there were statistical differences between relative RQ of normal and aberrant mRNA in PEFS+ mutant c.4853-25T>A (71.22%±11.92% and 7.38%±1.61%) and that in DS mutant c.4853-1G>C (0.08%±0.01% and 22.11%±2.83%,P<0.05).Conclusion The position and difference of splicing patterns of SCNIA intronic mutations are potential molecular pathogenesis for phenotypic difference of febrile seizures related epilepsy.
8.Therapeutic Observation of Filiform Fire-needle Therapy plus Mild Moxibustion for Flat Warts
Xue LIAO ; Xiaorong DUAN ; Cailian LI
Shanghai Journal of Acupuncture and Moxibustion 2017;36(5):584-587
Objective To observe the clinical efficacy of filiform fire-needle therapy plus mild moxibustion in treating flat warts.Method Ninety patients with flat warts were randomized into group A, group B and group C, 30 cases in each group. Group A was intervened by filiform fire-needle therapy plus mild moxibustion, group B was intervened by filiform fire-needle therapy alone and group C by mild moxibustion alone. The three groups all received treatment 3 times a week, 2 weeks as a treatment course, for 2 courses in total. The clinical efficacies of the three groups were compared.Result In group A, 12 subjects were recovered, 13 showed improved, and 5 cases failed; in group B, 7 subjects were recovered, 12 showed improved, and 11 failed; in group C, 5 cases showed recovered, 10 were improved, and 15 failed. The total effective rates were respectively 83.3% in group A, 63.3% in group B, and 50.0% in group C, and the total effective rate in group A was significantly higher than that in group B and C (P<0.05). Conclusion Filiform fire-needle therapy plus mild moxibustion can significantly help the skin rashes subside in treating flat warts, and the therapeutic efficacy is obviously superior to that of either of the two methods used alone.
9.Research progress of acute pain service:a literature review
Xiaorong LIU ; Dongxiu LIAO ; Yi SHEN ; Xianjing XIANG
Chinese Journal of Modern Nursing 2017;23(24):3172-3175
Acute pain is one of the most common clinical symptoms, and is the sensitive response to the stimulation caused by tissue damage. Also, it is a sign of impaired human health. Recently, acute pain service, which was developed in western countries, has been widely concerned across the whole world. It is worth to note that acute pain service has been applied in clinical settings in China over the past decade in different level hospitals. Acute pain service with high quality service can provide all patients with effective pain treatment, effective clinical monitoring and accurate evaluation. Meantime, the application of acute pain service plays a vital role either in the development of analgesia technology regulations or in the guidance of hospital training, clinical research and new technology research. This literature review aims to trace the national and international current status of acute pain service, and illustrate the potential development of acute pain service in the future.
10.Causes of misdiagnosis and mistreatment of Dravet syndrome with SCN1A mutations
Xiaorong LIU ; Jinxing LAI ; Liu LIU ; Lu YU ; Hui SUN ; Jianghong SHU ; Bingmei LI ; Weiping LIAO
The Journal of Practical Medicine 2016;32(11):1839-1843
Objective To analyze the causes of misdiagnosis and mistreatment of Dravet syndrome. Methods Patients with Dravet syndrome diagnosed according to clinical features and SCN1A gene mutation detection were recruited within recent 3 years. The patients were grouped into correct diagnosis-treatment group and misdiagnosis-mistreatment group according to whether the patients had ever been misdiagnosed and mistreated by sodium channel blockers. The clinical features were compared between two groups. Results Thirty-five cases with Dravet syndrome were collected and the rate of misdiagnosis reached 40%, Nine cases were misdiagnosed as symptomatic focal epilepsy, 4 as Lennox-Gastaut syndrome and 1 as Doose syndrome. The average age of onset in misdiagnosis-mistreatment group was (5.50 ± 3.56) months,and the age of confirmed diagnosis was (83.57 ± 105.62) months. The percentage of abnormal EEG, onset seizure with partial seizure, the seizure frequency within the first year from onset, onset with afebrile seizure, patients with status epilepticus or cluster seizures was higher in misdiagnosis-mistreatment group but it showed no significant statistical significance when compared with that of correct diagnosis-treatment group. The percentage of patients with mental retardation and focal neurological signs was significantly higher in misdiagnosis-mistreatment group (P=0.005 and 0.002, respectively). Conclusions Dravet syndrome is frequently misdiagnosed as symptomatic focal epilepsy. The appearance of focal neurological signs and mental retardation before confirmed diagnosis are important factors for misdiagnosis. Gene mutation screening will be helpful for differential diagnosis of Dravet syndrome.


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