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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.
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Objective To explore whether fluid-attenuated inversion recovery can be used to estimate the onset time of acute ischemic stroke (ALS) based on the analysis of signal strength through the fluid-attenuated inversion-recov?ery (FLAIR)and volume of interest (ROI) in ALS patients with known time of onset. Method Forty-seven AIS patients who met the inclusion criteria were recruited from Baotou Central Hospital, Department of Neurology from January 2011 to December 2012. The patients had stroke onset within 12 hours and completed MRI scan including diffusion-weighted imaging DWI, apparent diffusion and coefficient ADC FLAIR. Based on MRI findings, patients were divided into, three groups:0~180 min, 180~360 min and 360~720 min groups. Signal strength values of the DWI、FLAIR and ADC in ipsi?lateral and contralateral sides were measured. Result There was a significant difference in the FLAIR signal strength among these three groups.The FLAIR signal strength was significantly lower in 0~180 min and 180~360 min groups than in 360-720 min. FLAIR positive rate was 16.7%, 62.5%, and 70.6% in 0~180 min, 180~360 min and 360~729 min groups, respectively. Conclusion FLAIR positive rate gradually increases as the onset prolongs. Thus, lower FLAIR posi?tive rate indicates shorter onset time of AIS, which can be used to assist acute intravenous thrombolytic therapy.
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Objective To explore the clinical and genetic characteristics,protein expression of Wiskott-Aldrich syndrome (WAS).Methods 1.Clinical data of a Chinese sick boy patient who was treated in the First Affiliated Hospital of Guangxi Medical University was collected,and DNA samples were obtained from the patient and his mother,12 WAS gene exons were amplified by polymerase chain reaction (PCR) followed by direct sequencing,and then the protein expression was analyzed by Western blot.2.China National Knowledge Infrastructure (CNKI) was searched to identify the clinical data and clear genetic diagnosis of WAS literature from Jan.1991 to Oet.2013,combined with the case of WAS patient treated in this hospital,and a retrospective relationship analysis was made among WAS phenotype,genotype and protein phenotype in China.Results 1.The boy had a classical WAS phenotype,his clinical scores were 4.Sequencing revealed a nonsense mutation in exon 1,c.71C ＞ T (p.R13X).Western blot analysis revealed WASP-.The patient's mother was normal It's a de novo mutation in the patient.2.Other 53 cases of WAS patients had been reported,and they were all are male children,onset age from 1 day to 3 years.Forty-nine cases of typical WAS views,4 cases of X-linked thrombocytopenia (XLT).Immunological tests lack of specificity,mutant gene distribute in each exons except 4,5,6,9,12 and 1,3,6,7,8,9,11 introns.Protein detection was mostly negative.Conclusions Affected males who presented recurrent infections,persistent thrombocytopenia and eczema,should be considered to have the possibility of suffering from the WAS.Genetic diagnosis is the golden standard to diagnose the disease.And detection of protein expression can help define the relationship amone phenotype,genotype and protein phenotype.
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Objective To study the efficacy of the integration of Chinese and Western medicine in treating recurring NS. Methods Patients were deivided into tow groups,control group was treated with western medicine,experimental group was treated with integration of Chinese and Western medicine.We could understand that which curative effect would be better by contrast.Results The complete remission rate and the total remission rate in experimental group were 53.2% and 95% respectively,it was significantly higher than that in control group.In control group,The complete remission rate and the total remission rate were 30.0% and 70% respectively.The remission rate was significantly diffence com- pared with two groups(P