1.Therapeutic effect and mechanism of modified Chaihu Shugan Powder on a mouse model of gallbladder cholesterol stone with liver depression syndrome
Zhaoyan LI ; Heying WANG ; Renjie LIANG ; Yichun WANG ; Huiping CHEN ; Li MIN
Journal of Clinical Hepatology 2026;42(2):387-399
ObjectiveTo investigate the mechanism of action of modified Chaihu Shugan Powder in the treatment of abnormal gallbladder relaxation in gallbladder cholesterol stone (CS) with liver depression syndrome, and to provide a basis for clinical medication. MethodsMice were given a high-fat lithogenic diet combined with chronic unpredictable mild stress (CUMS) to establish a model of CS. A total of 45 male C57BL/6 mice were randomly divided into blank group (6 mice fed a normal diet) and CS group (39 mice fed a high-fat lithogenic diet). After CS modeling, the CS group was further randomly divided into four subgroups of CS group, CS liver depression group, traditional Chinese medicine group (treated with modified Chaihu Shugan Powder), and Western medicine group (treated with ursodeoxycholic acid), with 9 mice in each group. All subgroups were fed with the high-fat lithogenic diet, and all mice except those in the CS group were given 21 days of CUMS for modeling. Samples were collected after intervention. The serum levels of cholecystokinin (CCK), liver function parameters, and blood lipid profiles were measured; HE staining was performed for liver and gallbladder tissue; qPCR and Western blot were used to measure the mRNA and protein expression levels of G protein-coupled bile acid receptor 1 (TGR5) and glucagon-likepeptide-1/2 (GLP-1/2) in the intestine and TGR5 and glucagon-like peptide-2 receptor (GLP-2R) in gallbladder; metabolomics methods were used to determine bile acid composition in intestinal contents. The independent-samples t-test was used for comparison of continuous data between two groups; a one-way analysis of variance was used for comparison between multiple groups, and the least significant difference t-test or the Games-Howell method was used for further comparison between two groups. ResultsCompared with the blank group, the CS group showed significant gallstone formation, bile turbidity, hepatic steatosis, abnormal gallbladder wall structure, and significant increases in anxiety- and depression-like behaviors based on behavioral tests; significant increases in the level of total cholesterol in bile and the serum levels of alanine aminotransferase, aspartate aminotransferase, and low-density lipoprotein and significant reductions in the level of total bile acid (TBA) in bile and the serum levels of CCK and high-density lipoprotein (HDL) (all P<0.05); significant increases in the mRNA expression levels of GLP-1/2 and TGR5 in the intestine and the protein expression levels of GLP-2R and TGR5 in the gallbladder and significant reductions in the mRNA expression levels of GLP-2R and TGR5 in the gallbladder (all P<0.05); significant changes in multiple bile acid components in intestinal contents (all P<0.05). Compared with the CS group, the CS liver depression group had further aggravation of pathological and behavioral manifestations, changes in bile acid composition, significant increases in the protein and mRNA expression levels of TGR5 and GLP-1/2 in the intestine, and significant increases in the protein and mRNA expression levels of TGR5 and GLP-2R in the gallbladder (all P<0.01). Compared with the CS liver depression group, both treatment groups had an improvement in gallbladder morphology, alleviation of stones and liver injury, and recovery of liver function and blood lipid levels, as well as significant reductions in the protein and mRNA expression levels of TGR5 and GLP-1/2 in the intestine and TGR5 and GLP-2R in the gallbladder (all P<0.05); the traditional Chinese medicine group showed significant increases in glycodeoxycholic acid (GDCA), tauro-α-muricholic acid (T-α-MCA), and taurochenodeoxycholic acid (TCDCA) (all P<0.05), while the Western medicine group showed significant increases in taurohyodeoxycholic acid, T-α-MCA, TCDCA, GDCA, and glycoursodeoxycholic acid (all P<0.05). Compared with the Western medicine group, the traditional Chinese medicine group had significantly greater behavioral improvements, significantly higher levels of TBA in bile and serum HDL (both P<0.01), significant reductions in the protein expression levels of TGR5 and GLP-1/2 in the intestine and TGR5 and GLP-2R in the gallbladder, and a significant reduction in the mRNA expression level of TGR5 in the intestine (all P<0.01), as well as a significant increase in tauroursodeoxycholic acid and significant reductions in glycoursodeoxycholic acid, taurohyodeoxycholic acid, TCDCA, and taurolithocholic acid (all P<0.05). ConclusionModified Chaihu Shugan Powder can improve liver function and abnormal gallbladder relaxation in CS with liver depression syndrome by regulating the bile acid-TGR5 axis, thereby exerting the therapeutic effect of soothing the liver, resolving depression, moving Qi, and promoting bile flow.
2.Sub-committee of Anesthesiology of Guangzhou Integrated Traditional Chinese and Western Medicine Society.
Yi LU ; Cunzhi LIU ; Wujun GENG ; Xiaozhen ZHENG ; Jingdun XIE ; Guangfang ZHANG ; Chao LIU ; Yun LI ; Yan QU ; Lei CHEN ; Xizhao HUANG ; Hang TIAN ; Yuhui LI ; Hongxin LI ; Heying ZHONG ; Ronggui TAO ; Jie ZHONG ; Yue ZHUANG ; Junyang MA ; Yan HU ; Jian FANG ; Gaofeng ZHAO ; Jianbin XIAO ; Weifeng TU ; Jiaze SUN ; Yuting DUAN ; Bao WANG
Journal of Southern Medical University 2025;45(8):1800-1808
OBJECTIVES:
To explore the efficacy of DSA-guided intrathecal drug delivery system combined with Zi Wu Liu Zhu Acupoint Therapy for management of cancer pain and provide reference for its standardized clinical application. Methods and.
RESULTS:
Recommendations were formulated based on literature review and expert group discussion, and consensus was reached following expert consultation. The consensus recommendations are comprehensive, covering the entire treatment procedures from preoperative assessment and preparation, surgical operation process, postoperative management and traditional Chinese medicine treatment to individualized treatment planning. The study results showed that the treatment plans combining traditional Chinese with Western medicine effectively alleviated cancer pain, reduced the use of opioid drugs, and significantly improved the quality of life and enhanced immune function of the patients. Postoperative follow-up suggested good treatment tolerance among the patients without serious complications.
CONCLUSIONS
The formulated consensus is comprehensive and can provide reference for clinicians to use DSA-guided intrathecal drug delivery system combined with Zi Wu Liu Zhu Acupoint Therapy. The combined treatment has a high clinical value with a good safety profile for management of cancer pain.
Humans
;
Medicine, Chinese Traditional
;
Cancer Pain/therapy*
;
Drugs, Chinese Herbal/therapeutic use*
;
Drug Delivery Systems
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Pain Management/methods*
;
China
3.Clinical analysis of Haemophilus influenzae meningitis in children
Zhenzhen DOU ; Hong LI ; Qiang YE ; Wenbin LIU ; Lingyun GUO ; Bing HU ; Tianming CHEN ; Huili HU ; Xin GUO ; Heying CHEN ; Liang ZHU ; Haijuan XIAO ; Gang LIU
Chinese Journal of Pediatrics 2025;63(3):288-292
Objective:To analyze the clinical characteristics and outcomes of Haemophilus influenzae (Hi) meningitis in children. Methods:This is a retrospective case series study. This study included 34 Hi meningitis patients who admitted to Beijing Children′s Hospital, Capital Medical University, from January 1, 2010, to December 31, 2023. Data on clinical presentations, laboratory tests, hearing assessment and outcomes at discharge were collected. Patients were divided into 2 groups according to the outcome at discharge: favorable outcome group and unfavorable outcome group. Mann-Whitney U test and Fisher exact test was used to estimate the risk factors for an unfavorable outcome at discharge. Results:Thirty-four patients were enrolled. There were 16 males and 18 females. The age at onset ranged from 3 months to 12 years. Fever (34 patients (100%)), convulsions (17 patients (50%)), and coma (21 patients (62%)) were the common clinical presentations. Twenty-four patients (71%) developed complications. There were 15 patients (44%) needed treatment in the intensive care unit, 5 patients (15%) received intubation, and 4 patients (12%) had developed shock. The favorable outcome group included 23 patients, and the unfavorable outcome group included 11 patients. Female, patients with limb movement disability, dystonia, ventriculomegaly or hydrocephalus, and a concentration of glucose in the initial cerebrospinal fluid lower than 1 mmol/L were risk factors of unfavorable outcome at discharge (all P<0.05). Among the 23 patients (68%) in favorable outcomes group, 13 patients (57%) had data available on their long-term follow-up results, including 12 patients with favorable long-term outcomes and 1 patient with unfavorable long-term outcome. Among the 11 patients (32%) in unfavorable outcomegroup, 1 patient died, the other 10 patients (91%) had data available on their long-term outcomes. Eight patients had unfavorable long-term outcomes and 2 patients with favorable long-term outcomes. Patients who had unfavorable outcomes at discharge were at a greater risk of experiencing unfavorable long-term outcomes ( P=0.001). Conclusions:It is common for patients with Hi meningitis to have intracranial complications or develop into critical conditions. Patients who have limb movement disability, dystonia, ventriculomegaly or hydrocephalus, and who exhibit a concentration of glucose in the initial cerebrospinal fluid lower than 1 mmol/L tend to have unfavorable outcomes; they need to be assessed for sequelae.
4.Distribution of traditional Chinese medicine syndromes in intrahepatic cholestasis of pregnancy and its association with perinatal outcomes
Jin CHEN ; Dan YANG ; Qianrong LI ; Yan SANG ; Zhi YU ; Jiao XU ; Xuemei WANG ; Heying HUANG ; Xue TANG ; Lin ZHUANG ; Xiaoyin WANG
Journal of Clinical Hepatology 2025;41(11):2343-2350
ObjectiveTo investigate the distribution of traditional Chinese medicine (TCM) syndromes in intrahepatic cholestasis of pregnancy (ICP) and its association with perinatal outcomes, and to provide a basis for precise treatment based on TCM syndrome differentiation. MethodsA cross-sectional study was conducted among 275 patients with ICP who were admitted to The Affiliated Hospital of Chengdu University of Traditional Chinese Medicine from April 2023 to April 2025. A hierarchical cluster analysis was used to summarize TCM syndromes. The Kruskal-Wallis H test was used for comparison of non-normally distributed continuous data between groups, and the chi-square test was used for comparison of categorical data between groups. A multivariate Logistic regression analysis was used to identify the clinical features significantly associated with TCM syndrome. ResultsThe cluster analysis identified three core TCM syndromes among the 275 patients with ICP, i.e., liver-gallbladder damp-heat syndrome (45.8%), syndrome of blood deficiency generating wind (30.9%), and liver depression and spleen deficiency syndrome (23.3%). There was a significant difference in the distribution of TCM syndromes between different groups stratified by maternal age at delivery, parity, history of ICP recurrence, gestational weeks at disease onset, total bile acid (TBA), alanine aminotransferase (ALT), and comorbidity with gestational diabetes mellitus (GDM) (all P<0.05). The multivariate Logistic regression analysis showed that<34 gestational weeks at disease onset was significantly associated with all three syndromes (damp-heat: odds ratio [OR]=3.769, P<0.001; blood deficiency: OR=4.031, P<0.001; liver stagnation: OR=3.552, P<0.001). Liver-gallbladder damp-heat syndrome was associated with maternal age ≥35 years at disease onset (OR=2.048, P=0.014), parity ≥2 times (OR=1.921, P=0.034), history of ICP recurrence (OR=2.404, P=0.030), ALT ≥200 U/L (OR=2.051, P=0.018), comorbidity with GDM (OR=1.944, P=0.029), and TBA ≥40 μmol/L (OR=2.542, P=0.024). The syndrome of blood deficiency generating wind syndrome was associated with maternal age ≥35 years (OR=2.939, P=0.003), parity ≥2 time (OR=3.222, P=0.003), history of ICP recurrence (OR=3.809, P=0.010), ALT ≥200 U/L (OR=2.889, P=0.006), comorbidity with GDM (OR=3.711, P=0.001), and comorbidity with hypertensive disorders of pregnancy (OR=4.472, P=0.011). Liver depression and spleen deficiency syndrome was associated with TBA ≥40 μmol/L (OR=2.995, P=0.044). The analysis of perinatal outcomes showed that there were significant differences in mode of delivery, gestational weeks at the time of delivery, postpartum blood loss, and neonatal birth weight between the three groups with different TCM syndromes (all P<0.05). ConclusionLiver-gallbladder damp-heat syndrome, syndrome of blood deficiency generating wind, and liver depression and spleen deficiency syndrome are the main TCM syndrome types in ICP, and the distribution of TCM syndromes is closely associated with clinical factors and perinatal outcomes, which provides a basis for precise TCM syndrome differentiation and individualized treatment.
5.Consensus on the use of DSA-guided intrathecal drug delivery system combined with Zi Wu Liu Zhu Acupoint Therapy for cancer pain management
Yi LU ; Cunzhi LIU ; Wujun GENG ; Xiaozhen ZHENG ; Jingdun XIE ; Guangfang ZHANG ; Chao LIU ; Yun LI ; Yan QU ; Lei CHEN ; Xizhao HUANG ; Hang TIAN ; Yuhui LI ; Hongxin LI ; Heying ZHONG ; Ronggui TAO ; Jie ZHONG ; Yue ZHUANG ; Junyang MA ; Yan HU ; Jian FANG ; Gaofeng ZHAO ; Jianbin XIAO ; Weifeng TU ; Jiaze SUN ; Yuting DUAN ; Bao WANG
Journal of Southern Medical University 2025;45(8):1800-1808
Objective To explore the efficacy of DSA-guided intrathecal drug delivery system combined with Zi Wu Liu Zhu Acupoint Therapy for management of cancer pain and provide reference for its standardized clinical application.Methods and Results Recommendations were formulated based on literature review and expert group discussion,and consensus was reached following expert consultation.The consensus recommendations are comprehensive,covering the entire treatment procedures from preoperative assessment and preparation,surgical operation process,postoperative management and traditional Chinese medicine treatment to individualized treatment planning.The study results showed that the treatment plans combining traditional Chinese with Western medicine effectively alleviated cancer pain,reduced the use of opioid drugs,and significantly improved the quality of life and enhanced immune function of the patients.Postoperative follow-up suggested good treatment tolerance among the patients without serious complications.Conclusion The formulated consensus is comprehensive and can provide reference for clinicians to use DSA-guided intrathecal drug delivery system combined with Zi Wu Liu Zhu Acupoint Therapy.The combined treatment has a high clinical value with a good safety profile for management of cancer pain.
6.Clinical analysis of Haemophilus influenzae meningitis in children
Zhenzhen DOU ; Hong LI ; Qiang YE ; Wenbin LIU ; Lingyun GUO ; Bing HU ; Tianming CHEN ; Huili HU ; Xin GUO ; Heying CHEN ; Liang ZHU ; Haijuan XIAO ; Gang LIU
Chinese Journal of Pediatrics 2025;63(3):288-292
Objective:To analyze the clinical characteristics and outcomes of Haemophilus influenzae (Hi) meningitis in children. Methods:This is a retrospective case series study. This study included 34 Hi meningitis patients who admitted to Beijing Children′s Hospital, Capital Medical University, from January 1, 2010, to December 31, 2023. Data on clinical presentations, laboratory tests, hearing assessment and outcomes at discharge were collected. Patients were divided into 2 groups according to the outcome at discharge: favorable outcome group and unfavorable outcome group. Mann-Whitney U test and Fisher exact test was used to estimate the risk factors for an unfavorable outcome at discharge. Results:Thirty-four patients were enrolled. There were 16 males and 18 females. The age at onset ranged from 3 months to 12 years. Fever (34 patients (100%)), convulsions (17 patients (50%)), and coma (21 patients (62%)) were the common clinical presentations. Twenty-four patients (71%) developed complications. There were 15 patients (44%) needed treatment in the intensive care unit, 5 patients (15%) received intubation, and 4 patients (12%) had developed shock. The favorable outcome group included 23 patients, and the unfavorable outcome group included 11 patients. Female, patients with limb movement disability, dystonia, ventriculomegaly or hydrocephalus, and a concentration of glucose in the initial cerebrospinal fluid lower than 1 mmol/L were risk factors of unfavorable outcome at discharge (all P<0.05). Among the 23 patients (68%) in favorable outcomes group, 13 patients (57%) had data available on their long-term follow-up results, including 12 patients with favorable long-term outcomes and 1 patient with unfavorable long-term outcome. Among the 11 patients (32%) in unfavorable outcomegroup, 1 patient died, the other 10 patients (91%) had data available on their long-term outcomes. Eight patients had unfavorable long-term outcomes and 2 patients with favorable long-term outcomes. Patients who had unfavorable outcomes at discharge were at a greater risk of experiencing unfavorable long-term outcomes ( P=0.001). Conclusions:It is common for patients with Hi meningitis to have intracranial complications or develop into critical conditions. Patients who have limb movement disability, dystonia, ventriculomegaly or hydrocephalus, and who exhibit a concentration of glucose in the initial cerebrospinal fluid lower than 1 mmol/L tend to have unfavorable outcomes; they need to be assessed for sequelae.
7.A national multi-center prospective study on the perioperative practice of enhanced recovery after surgery for choledochal cysts in children
Ming YUE ; Jiexiong FENG ; Yan′an LI ; Yuanmei LIU ; Zhigang GAO ; Qi CHEN ; Hongwei XI ; Qiang YIN ; Chengji ZHAO ; Yuzuo BAI ; Wanfu LI ; Libin ZHU ; Weibing TANG ; Hongqiang BIAN ; Huizhong NIU ; Zhiheng GUO ; Heying YANG
Chinese Journal of General Surgery 2024;39(11):827-832
Objective:To evaluate the safety and efficacy of enhanced recovery after surgery(ERAS) in the perioperative period of congenital choledochal cysts in children.Methods:This is a multicenter prospective randomized controlled study. The clinical data of 273 pediatric congenital choledochal cysts(CCC) patients who underwent surgery at 14 medical centers with complete follow-up data were collected through the medical data analysis platform. Among them, 123 cases in ERAS group were managed perioperatively in strict accordance with ERAS mode, and 150 cases in conventional group were managed according to traditional mode. The length of hospital stay,time to first farting, time to complete feeding, the incidence of complications, cost and readmission rate within 30 days,stress indexes and liver function were compared between the two groups.Results:Compared with the conventional group, median time to start farting (2.0 d vs. 3.0 d, P<0.001), median time to complete feeding (5.0 d vs. 7.0 d, P<0.001), median postoperative hospitalization time (6.0 d vs. 9.0 d, P<0.001),the median total length of stay(13.0 d vs. 15.0 d, P<0.001) were shorter,the median hospitalization cost (37,000 yuan vs.43,000 yuan P<0.001) was lower, and stress indexes recovered quickly. The incidence of postoperative hospital stay and readimission rate within 30 d were not statistically different between the two groups. Conclusion:It is safe and feasible to implement ERAS for children with CCC in the perioperative period, which can reduce stress response, speed up recovery,and save medical costs.
8.Characteristics and recovery of hearing loss in 573 patients with bacterial meningitis
Jifeng SHI ; Haihong LIU ; Zhenzhen DOU ; Lingyun GUO ; Wenya FENG ; Yi ZHOU ; Ying LI ; Xin JIN ; Huili HU ; Zhipeng ZHENG ; Bing LIU ; Bing HU ; Tianming CHEN ; Xin GUO ; Heying CHEN ; Gang LIU
Chinese Journal of Pediatrics 2021;59(8):633-639
Objective:To analyze the characteristics and prognosis of hearing loss in children with bacterial meningitis.Methods:This was a single-center retrospective cohort study. Patients diagnosed with bacterial meningitis who were hospitalized in Beijing Children′s Hospital between 2010 and 2016 and older than 28 days and younger than 18 years at symptom onset were included in this study ( n=573). All clinical information including hearing assessment results during hospitalization were reviewed. All patients with hearing loss were followed up to repeat their hearing test and assess their hearing condition with parents′ evaluation of aural and (or) oral performance of children (PEACH). Patients were grouped according to their hearing assessment results, and Logistic regression analysis was used to analyze the risk factors for hearing loss in patients with bacterial meningitis. Results:Five hundred and seventy-three patients were enrolled in this study, including 347 males and 226 females. The onset age ranged from 29 days to 15.8 years. Two hundred and forty-six patients had identified causative pathogens, among whom 92 cases (37.4%) were pneumococcal meningitis cases. Hearing loss was found in 160 cases (27.9%) during hospitalization, involving 240 ears. Permanent hearing loss was found in 20 cases (16.9%), involving 32 ears. In the patients with permanent hearing loss, 87.5% (28/32) of ears were identified as severe or profound hearing loss during hospitalization. Logistic regression analysis showed that dystonia, the protein concentration level in cerebrospinal fluid>1 g/L, glucose concentration level lower than 1 mmol/L and subdural effusion were independent risk factors for hearing loss ( OR=2.426 (1.450-4.059), 1.865 (1.186-2.932), 1.544 (1.002-2.381) and 1.904 (1.291-2.809)). Conclusions:Hearing loss is a common sequela of bacterial meningitis in children. Most patients have transient hearing loss, but patients with severe or profound hearing impairment have a higher risk of developing permanent hearing loss.
9.Study on the Optimization of the Extraction Technology of Shengmaiyin Polysaccharide and Its Regulation Effects on Intest- inal Function of Spleen Deficiency Model Rats
Yu YOU ; Lin LUO ; Zhejie CHEN ; Meisi LIN ; Heying XU ; Bo XIONG ; Chaomei FU ; Tian XIE
China Pharmacy 2019;30(4):493-498
OBJECTIVE: To optimize the extraction technology of Shengmaiyin polysaccharide, and to investigate the regulation effects of Shengmaiyin and its polysaccharide on intestinal function of spleen deficiency model rats. METHODS: The contents of polysaccharide were determined by phenol-sulfuric acid method, and the extraction rate of polysaccharide was calculated. Using extraction rate of Shengmaiyin polysaccharide as investigation index, singel factor and orthogonal tests were used to optimize material-liquid ratio, extraction time, extraction temperature and extraction times of Shengmaiyin polysaccharide. Validation test was also conducted. Totally 80 male SD rats were randomly divided into blank group, model group, Shengmaiyin low-dose, medium-dose and high-dose groups (350, 700, 1 400 g/L, by crude drug), Shengmaiyin polysaccharide low-dose, medium-dose and high-dose groups (24.5, 49, 98 g/L, by crude drug), with 10 rats in each group. Except for blank group, other groups were given Rheum palmatum water decoction 10 mL/kg to induce spleen deficiency model, once a day, for consecutive 15 d. Since the 16th day, blank group and model group were given isovolumic water intragastrically, while other groups were given corresponding drugs, once a day, for consecutive 10 d. The general status of rats and body weights were recorded in each group. The serum contents of D-xylose, gastrin (GAS) and vasoactive intestinal peptide (VIP) were detected by phloroglucinol method or ELISA. RESULTS: The optimal extraction technology of Shengmaiyin polysaccharide was material-liquid ratio 1 ∶ 10(g/mL), extraction time 45 min, extraction temperature 80 ℃, extracting for 1 time. Results of validation test showed that extraction rates of the polysaccharide in 3 times were 7.43%, 7.64%, 7.80% (RSD=1.01%, n=3). After modeling, except for blank group, other groups suffered from loose stools, thin body and reduced food intake, and the body weight and serum level of D-xylose were decreased significantly compared with blank group (P<0.01). After last medication, above symptoms of administration groups were improved to different extents. Except for model group, body weight and serum contents of D-xylose in other groups were increased significantly than those before modeling or before medication (P<0.05 or P<0.01). Compared with blank group, body weight and serum content of GAS were decreased significantly in model group, while serum content of VIP was increased significantly (P<0.01). Compared with model group, body weight of Shengmaiyin medium-dose group and Shengmaiyin polysaccharide low-dose and high-dose groups, serum contents of D-xylose and GAS in Shengmaiyin medium-dose and high-dose groups and Shengmaiyin polysaccharide low-dose and medium-dose groups were increased significantly, while serum contents of VIP in Shengmaiyin groups and Shengmaiyin polysaccharide low-dose and medium-dose groups were all decreased significantly (P<0.05 or P<0.01). CONCLUSIONS: The optimized extraction technology of Shengmaiyin polysaccharide is stable and feasible. Shengmaiyin and its polysaccharide contribute to the recovery of intestinal function of spleen deficiency model rat, the effects of which may be associated with the secretion regulation of GAS and VIP.
10. Clinical and pathological features of 13 children with Epstein-Barr virus-positive lymphoproliferative disease
Tianming CHEN ; Zhijuan DENG ; Bing HU ; Huili HU ; Heying CHEN ; Shaoying LI ; Gang LIU
Chinese Journal of Pediatrics 2018;56(10):759-764
Objective:
To summarize the clinical characteristics, virological and histopathological features, clinical outcome of Epstein-Barr virus-positive lymphoproliferative disease (EBV+LPD) in children.
Methods:
The clinical and follow-up data of 13 children histopathologically diagnosed as EBV+LPD in the Department of Infectious Disease of Beijing Children's Hospital between January 2011 and December 2016 were summarized.
Results:
Of the 13 patients, 5 were males and 8 females. The median age of disease onset was 6.0 years (range 1.3 to 15.0 years). The median duration between disease onset and diagnosis was 3 months (range 1 to 24 months). All the 13 patients had fever, 9 cases had hepatosplenomegaly and lymphoadenopathy, 4 cases had only lymphoadenopathy, 7 cases had reduced peripheral blood cells, 7 cases had lung involvement, 3 cases had central nervous system involvement, 3 cases had cardiac involvement, 3 cases had intestinal involvement, 2 cases had skin involvement and 1 case had abdominal mass. All the 13 patients underwent whole blood EBV-DNA PCR examination and the copies ranged from 1×108/L to 1×1011/L. Pathology of lymph node confirmed 6 cases, skin pathology confirmed 2 cases, lung pathology, ileum mucosa pathology, liver pathology, abdominal mass pathology and bone marrow pathology confirmed 1 case each. Among 13 patients, 9 cases presented with EBV-positive T cell lymphoproliferative disease(EBV+ T-LPD), 2 cases with hydroa vacciniforme (HV) and 2 cases with EBV-positive diffuse large B-cell lymphoma (EBV+ DLBCL) . All the patients were followed up for 2 days to 65 months after discharge. Among 9 cases of EBV+T-LPD, 1 case died in a short time, 1 case died after evolved to T-cell lymphoma, 2 cases recovered after hematopoietic stem cell transplantation, 1 case recovered after the chemotherapy of hemophagocytic lymphohistiocytosis(HLH) 2004 protocol and 4 cases were stable now. Of 2 cases of HV patients, 1 case died after evolved to HV like lymphoma and the other still have symptoms. Among 2 cases of EBV+ DLBCL, 1 case died shortly after discharge and the other was still stable after chemotherapy.
Conclusions
Chronic recurrent fever, lymphadenopathy and hepatosplenomegaly are the most common clinical manifestations in children with EBV+LPD. Involvement of lung, central nervous system, intestinal tract, skin and other organs are also involved frequently. For children with chronic fever of unknown cause and accompanied by lymphadenopathy and (or) hepatosplenomegaly, EBV + LPD should be considered highly when the whole blood EBV-DNA load continues to increase significantly, early biopsy of the proliferative lesion should be performed to make a definite diagnosis. The prognosis of EBV + LPD is poor, and some evolve to lymphoma, hematopoietic stem cell transplantation is an effective way to treat this disease.

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