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Objective Quantitative imaging evaluation was performed on the liver and spleen system lesions of patients with Gaucher disease after treatment. in order to deepen the understanding of Gaucher disease. Methods From August 1999 to August 2018, we registered, examined and treated children with Gaucher disease, and conducted quantitative imaging research on 40 children with Gaucher disease who were intensively followed up in Beijing Children's hospital, Capital Medical University until August 2018. At the same time, 34 normal volunteers were matched. All subjects were scanned with magnetic resonance imaging(MRI). The fat fraction(FF), iron content(R2*), standard apparent diffusion coefficient(sADC), slow apparent diffusion coefficient(D), fast apparent diffusion coefficient(D*) and perfusion fraction(f)of the liver and spleen were measured. The quantitative parameter values measured by patients with Gaucher disease and normal subjects were statistically analyzed by independent sample t-tests. Results The results showed that there was no significant difference in FF, R2*, sADC, D, D*, f of the liver and spleen, and liver elasticity was also within the normal range. However, the volume of liver and spleen in patients was significantly different from that in normal subjects. Conclusions After treatment, the volume of the liver and spleen in patients with Gaucher disease is greater than that of normal people, but other quantitative parameters are within the normal range, indicating that long-term enzyme replacement therapy can delay the progress of liver and spleen diseases to a certain extent. Quantitative imaging has a certain value in the evaluation of Gaucher disease.
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Objective@#To explore the imaging manifestations of thoracic CT in patients with Gaucher disease (GD) in order to improve the diagnostic ability.@*Methods@#Forty-three patients with GD were collected from May 2003 to October 2018 in Beijing Children′s Hospital, including 25 males and 18 females, aged from 10 to 34 years, with an average age of (21±6) years. All the patients underwent routine chest CT examinations, and analysis and description of pulmonary interstitial and parenchyma imaging manifestations were performed.@*Results@#Among the 43 GD patients, 20 patients presented with abnormal chest CT findings: 10 showed diffuse interlobular septa thickening, mainly distributed in the lower lobes of both lungs; 5 showed ground glass opacities in a single or multiple lobes of the lung. There were 2 cases with small nodules, which showed round-like nodules of different sizes. One case had pulmonary fibrosis, especially in the left upper lobe. Other manifestations included bullae in 3 cases,localized pleural thickening in 2 cases, pneumothorax in 1 case; pulmonary hypertension in 1 case and thymus enlargement in 12 cases. Most of the GD patients had pulmonary lesions between 10 and 14 years old. The signs of interlobular septa thickening and thymus enlargement were common, with 5 cases in each age group.@*Conclusions@#GD involves the lungs in half of the patients. The manifestations of the lungs are diverse, and most of them are diffuse interstitial lesions. The main signs are interlobular septal thickening and ground glass opacity, which are consistent with the pathology of Gaucher cell infiltration.But the signs are not specific, the diagnosis should be made in combination with the clinical information, and attention should be paid to the differentiation of lung infiltration caused by other diseases.
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Objective:To explore the imaging manifestations of thoracic CT in patients with Gaucher disease (GD) in order to improve the diagnostic ability.Methods:Forty-three patients with GD were collected from May 2003 to October 2018 in Beijing Children′s Hospital, including 25 males and 18 females, aged from 10 to 34 years, with an average age of (21±6) years. All the patients underwent routine chest CT examinations, and analysis and description of pulmonary interstitial and parenchyma imaging manifestations were performed.Results:Among the 43 GD patients, 20 patients presented with abnormal chest CT findings: 10 showed diffuse interlobular septa thickening, mainly distributed in the lower lobes of both lungs; 5 showed ground glass opacities in a single or multiple lobes of the lung. There were 2 cases with small nodules, which showed round-like nodules of different sizes. One case had pulmonary fibrosis, especially in the left upper lobe. Other manifestations included bullae in 3 cases,localized pleural thickening in 2 cases, pneumothorax in 1 case; pulmonary hypertension in 1 case and thymus enlargement in 12 cases. Most of the GD patients had pulmonary lesions between 10 and 14 years old. The signs of interlobular septa thickening and thymus enlargement were common, with 5 cases in each age group.Conclusions:GD involves the lungs in half of the patients. The manifestations of the lungs are diverse, and most of them are diffuse interstitial lesions. The main signs are interlobular septal thickening and ground glass opacity, which are consistent with the pathology of Gaucher cell infiltration.But the signs are not specific, the diagnosis should be made in combination with the clinical information, and attention should be paid to the differentiation of lung infiltration caused by other diseases.
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Objective To discuss the clinical manifestations,imaging features and prognosis of children with mild encephalitis/encephalopathy with a reversible splenial lesion(MERS).Methods Twenty-five patients with MERS admitted to Beijing Children′s Hospital,Capital Medical University,between November 2013 and March 2016 were enrolled and their clinical and imaging data were retrospectively analyzed.Ages of onset of these 25 cases were from 6 months to 13 years old.Because of different clinical manifestations in different onset ages,these 25 cases were divided into 2 groups:≤6 years old group (20 cases),with the onset age of 6 months to 3 years and 9 months old(average 2 years and 2 months);>6 years old group(5 cases),with the onset age of 9 years 3 months to 13 years old (average 10 years and 10 months).Results Nineteen cases among the 25 patients had infection history before onset,including 10 cases of digestive tract infection(all were ≤6 years old children),9 cases of respiratory tract infection(6 children ≤6 years old and 3 children >6 years old).The main clinical manifestations included convulsion (18/25 cases,72.0%),fever (17/25 cases,68.0%),vomiting (11/25 cases,44.0%),and disturbance of consciousness (11/25 cases,44.0%).The main clinical manifestation of ≤6 years old group was convulsion (18/20 cases,90.0%),while the main clinical manifestations of the>6 years old group were fever(3/5 cases,60.0%),headache and dizziness(2/5 cases,40.0%),and none of the patients in >6 years old group had convulsion.Eight cases had liver function injury,myocardial enzymes increased in 10 cases,and hyponatremia occurred in 9 cases.Magnetic resonance imaging (MRI) showed 21 cases were type Ⅰ MERS(only involving corpus callosum),and 4 cases of type Ⅱ MERS which involved corpus callosum as well as deep brain white matter,subcortical white matter (centrum semiovale).MRI lesions disappeared after 8-56 days (average 16.5 days) of anti-infection and reducing intracranial pressure treatment.Conclusion MERS is more common in ≤6 years old children,and digestive tract infection is common in ≤6 years old children,while respiratory tract infection is common in >6 years old children.The symptoms in children are mainly manifested as fever,convulsion,vomiting,conscious disturbance,and so on.Infection and hyponatremia are the main causes of MERS in children.MRI is the first choice of imaging examination methods.
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Objective To study the characteristics of immune function,Epstein-Barr virus(EBV) antibodies and EBV-DNA in children with different clinical types of EBV infection,which provide basis for prevention and treatment of EBV infection.Methods Clinical data of 103 patients suffering from EBV infection were retrospectively analyzed in Xijing Hospital of the Fourth Military Medical University.A total of 103 children were divided into infectious mononualeosis(IM) group(n=68),chronic active Epstein-Barr virus infection(CAEBV) group(n=13) and Epstein-Barr virus-related hemophagocytic lymphohistiocytosis(EBV-HLH) group(n=22).The changes of EBV antibodies,EBV-DNA,immunoglobulin levels,lymphocyte subpopulation and complement series were detected and compared among the three groups.A total of 26 healthy children at the same stage were enrolled as a control group,immunoglobulin levels,lymphocyte subpopulation and complement series were detected in control group,then compared with the rest of the three groups.Results The levels of C3 and C4 in CAEBV group and EBV-HLH group were significantly decreased than those in the control group(P0.05).CD8+T cells in IM group significantly increased than those in the rest of the three groups(P<0.05,respectively).T cells,CD8+T cells,CD4+ T cells,CD4+/CD8+ ratios,NK cells,B cells of EBV-HLH group significantly decreased than those in the rest of the three groups(P<0.05).The positive rates of EBV antibodies in CAEBV group and IM group were significantly higher than those in EBV-HLH group(P<0.05).EBV-DNA in EBV-HLH group were significantly higher than those of CAEBV group and IM group(P<0.05).Conclusion EBV-DNA levels in the serum are positively correlated with disease types and severity,the pathogenesis of IM,CAEBV and EBV-HLH induced by EBV infection are associated with immune dysfunction.Dynamic monitoring of EBV load and cell immune function can reflect disease status and progress risk.
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Objective To study the clinical characteristics of chronic active Epstein-Barr virus (EBV) infection (CAEBV) in children and to provide a basis for the diagnosis and treatment of CAEBV.Methods Clinical data,laboratory serology,pathological examination,treatment and follow-up results of 10 cases with CAEBV infection who were treated in Xijing Hospital of the Fourth Military Medical University from January 2008 to January 2016 were analyzed retrospectively.Results CAEBV major manifestations were continuous or intermittent fever,hepatomegaly,splenomegaly and lymphadenopathy,and others,including general fatigue,cough,hematemesis,diarrhea,skin rash,jaundice,sore throat,muscle joint pain,and so on.And with liver dysfunction,hematologic abnormality,and so on.All patients in anti-EB virus capsid antigen IgG (EBVCA-IgG)antibodies and EBEA-IgG antibodies had positive,while all patients in EBVCA-IgM antibodies had negative.The median load of EBV-DNA detected by real-time polymerase chain reaction(PCR) in the peripheral blood was 7.15× 105 copies/ml.Six of 10 cases CAEBV patients presented a poor clinical course,1 case died from intracranial hemorrhage,2 cases from respiratory failure,1 case from gastrointestinal bleeding,1 case from liver failure,1 case from severe multiple pathogens infection,rest 3 cases showed an improvement and 1 cases had a recurrence.Conclusion CAEBV infection has varieties of clinical features,with poor prognosis and high mortality.If the patients had unexplained fever,hepatomegaly,splenomegaly and lymphadenectasis,we should be timely detect virology and histopathological to diagnosis as early as possible.
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Objective To investigate the clinical characteristics of childhood purulent meningitis (PM)and the risk factors for its adverse outcome.Methods One hundred and nine children with PM were retrospective ana-lyzed,who were admitted to pediatric department in Xijing Hospital of the Fourth Military Medical University from Ja-nuary 2008 to July 201 6.They were divided into 5 age groups,the clinical features were compared among the different age groups.According to Glasgow prognostic score,all cases were then divided into 2 groups,the favorable outcome group and the adverse outcome group.All factors including normal information,disease history,clinical manifestations and laboratory examinations were compared between 2 groups.Results There were 72.5% (79 /1 09 cases)of the pa-tients younger than 3 years old.PMwas prone to spring and winter,and most children with PMhad preceding infection. The major clinical manifestations of PM were fever,convulsions and intracranial hypertension.The clinical manifesta-tions of PMwere different in different age groups,and convulsions were more commonly seen in less than 3 years old children,while headache,vomiting and meningeal stimulation had higher proportion in more than 3 years old children. The single factor analysis showed that there were repeated convulsions after admission (≥3 times),the cerebrospinal fluid (CSF)glucose(≤1 .5 mmol/L),CSF protein(≥1 g/L),CSF /blood glucose ratio and complications were signifi-cantly different between 2 groups(all P 500 ×1 06 /L,blood and CSF cultivate positive rate, co -infection,brain CT/MRI abnormality,electroencephalogram abnormality,treatment and duration of seizure more than 5 minutes were not significantly different(all P >0.05).Multivariate analysis showed that there were repeated convulsions after admission (≥3 times)(OR =27.84,P =0.048),CSF protein(≥1 g/L)(OR =28.44,P =0.027) and low CSF /blood glucose ratio (OR =22.1 5,P =0.041 )were independent risk factors for poor prognosis of PM. Conclusion PMhappens mostly in infantile period,with different clinical manifestations at different ages.The inde-pendent risk factors for poor prognosis were repeated convulsions after admission (≥3 times),CSF protein(≥1 g/L) and low CSF /blood glucose ratio.It indicates that if the high risk factors could be identified early,and then intervened immediately and followed up timely,it will be beneficial to improve the long -term prognosis.
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nal abnormalities of pediatric heart diseases.
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Objective: To observe effect of combination of TCM-WM with high colonic lavement for delaying renal failure. Methods: 98 cases of renal failure at compensatory phase were randomly divided into a treatment group treated with high colonic lavement, twice each week. 2 hours each time, and a control group treated with oral administration of coated aldehyde oxystarch,10g each time, thrice each day, 3 months constituting one course. Results: After lavenment. symptoms were improved, deceaseing velosity of the clearance rate of endogenous creatinine in blood and urine slowed lowered, renal survival rate of kidney increased, and the interval of dialasis and other replacement therapy obviously postponed in the treatment group as compared with that before treatment and the control group. Conclusion: The high colonic lavement has obvious therapeutiv effect in delaying renal failure velosity with a smaller stimulation to the stomach and smal intestine. It is an effective way for conservation treatment of renal failure.
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Objective To study and compare the clinical and MR imaging characteristics of common intracranial ring enhanced lesions.Methods MR imaging studies of 65 intracranial ring enhanced lesions were reviewed retrospectively. The lesions included 17 glioblastomas, 17 metastasises, 12 brain abscesses, 8 acoustic neurinomas, 7 craniopharyngiomas, 3 neurocysticercosises, 1 malignant lymphoma. All cases were confirmed by surgery and pathology. Results The ring enhancement of tumors presented heterogenous thickness of thick walls and irregular forms. Among them, the highly malignant tumors appeared very obviously heterogeneous thickness of the walls, especially the inner walls were uneven and coarse. While the ring enhancement of the non-tumor lesions usually were of homogeneous thickness of the walls, regular shapes and smooth inner walls. Conclusion The different patterns of intracranial ring enhanced lesions on MR can supply important diagnostic information. Combining the patients' history, age, location and the information of magnetic resonance imaging, we may make correct diagnosis.