1.A Case of Multidisciplinary Treatment for Inflammatory Myofibroblastic Tumor Complicated by ANCA-Associated Vasculitis
Shaoying WANG ; Linyi PENG ; Ke ZHENG ; Zhiwei WANG ; Dachun ZHAO ; Xia ZHANG ; Lin ZHAO ; Wenhui WANG ; Weiqing WANG ; Zhenzhen ZHU ; Jin XU ; Min SHEN
JOURNAL OF RARE DISEASES 2026;5(1):43-51
A 51-year-old male presented with nasal obstruction, followed by progressive hearing loss and blurred vision. Imaging identified space-occupying lesions in the paranasal sinuses, orbits, and paraspinal regions, while laboratory tests confirmed positive anti-proteinase 3 anti-neutrophil cytoplasmic antibody(PR3- ANCA) immunoglobulin G (IgG)and markedly elevated serum IgG4. Despite treatment with corticosteroids, immunosuppressants, and radiotherapy, the patient exhibited steroid dependency with relentless disease progression. Following multidisciplinary consultation, a diagnosis of inflammatory myofibroblastic tumor (IMT) coexisting with ANCA- associated vasculitis (AAV) was favored, though IgG4-related disease remained a critical differential. Ultimately, profound immunosuppression precipitated a severe herpesvirus infection, leading to disseminated intravascular coagulation and multiple organ dysfunction syndrome. This case underscores the rarity and diagnostic complexity of concurrent IMT and AAV, highlights the therapeutic dilemma of balancing primary disease control against fatal opportunistic infections, and emphasizes the critical role of multidisciplinary collaboration in the diagnosis and treatment of complex diseases.
2.Cardiac Damage in Patients with Primary Aldosteronism and Different Degrees of Obstructive Sleep Apnea Syndrome
Shaoying ZHENG ; Mingshuang ZHOU ; Xue LI
Acta Medicinae Universitatis Scientiae et Technologiae Huazhong 2025;54(2):240-246
Objective To investigate the characteristics of left ventricular hypertrophy in patients with primary aldosteron-ism(PA)accompanied by varying severity of obstructive sleep apnea(OSA),and to further identify potential factors contributing to ventricular hypertrophy in this population.Methods A retrospective analysis was performed on 308 patients with PA who received treatment at Kunming Medical University Affiliated Cardiovascular Hospital from January 2021 to June 2022.For com-parison,309 hospitalized patients diagnosed with essential hypertension(EH)during the same period were included as the control group.According to polysomnography findings,the patients were categorized into PA/EH with OSA group and PA/EH without OSA group.PA patients with OSA were further stratified into mild,moderate,and severe OSA subgroups based on the apnea-hypopnea index(AHI).Demographic characteristics,biochemical profiles,and echocardiographic parameters were compared across the groups.Results Compared to the EH groups with or without OSA,interventricular septal diastolic thickness(IVS-DT),left ventricular mass index(LVM),and left ventricular mass index(LVMI)was increased in PA with OSA group(all P<0.05).The body mass index(BMI)was increased in both the PA with OSA group and EH with OSA group compared to their counterparts without OSA(all P<0.05).Within the three subgroups of PA patients with OSA,left ventricular end-diastolic di-mension(LVEDD),IVSDT,left ventricular posterior wall diastolic thickness(LVPWDT),and LVM measurements were in-creased in severe OSA group compared to the mild OSA group(all P<0.05).Conclusion Aldosterone is an independent risk factor from OSA for left ventricular hypertrophy;In PA patients with OSA,cardiac impairment is severer in the moderate and severe OSA subgroups compared to those without OSA,and the severity of cardiac impairment escalates as OSA severity increa-ses.These findings highlight the necessity of screening for OSA in PA patients to stratify cardiac impairment risk and enable early therapeutic intervention.
3.Cardiac Damage in Patients with Primary Aldosteronism and Different Degrees of Obstructive Sleep Apnea Syndrome
Shaoying ZHENG ; Mingshuang ZHOU ; Xue LI
Acta Medicinae Universitatis Scientiae et Technologiae Huazhong 2025;54(2):240-246
Objective To investigate the characteristics of left ventricular hypertrophy in patients with primary aldosteron-ism(PA)accompanied by varying severity of obstructive sleep apnea(OSA),and to further identify potential factors contributing to ventricular hypertrophy in this population.Methods A retrospective analysis was performed on 308 patients with PA who received treatment at Kunming Medical University Affiliated Cardiovascular Hospital from January 2021 to June 2022.For com-parison,309 hospitalized patients diagnosed with essential hypertension(EH)during the same period were included as the control group.According to polysomnography findings,the patients were categorized into PA/EH with OSA group and PA/EH without OSA group.PA patients with OSA were further stratified into mild,moderate,and severe OSA subgroups based on the apnea-hypopnea index(AHI).Demographic characteristics,biochemical profiles,and echocardiographic parameters were compared across the groups.Results Compared to the EH groups with or without OSA,interventricular septal diastolic thickness(IVS-DT),left ventricular mass index(LVM),and left ventricular mass index(LVMI)was increased in PA with OSA group(all P<0.05).The body mass index(BMI)was increased in both the PA with OSA group and EH with OSA group compared to their counterparts without OSA(all P<0.05).Within the three subgroups of PA patients with OSA,left ventricular end-diastolic di-mension(LVEDD),IVSDT,left ventricular posterior wall diastolic thickness(LVPWDT),and LVM measurements were in-creased in severe OSA group compared to the mild OSA group(all P<0.05).Conclusion Aldosterone is an independent risk factor from OSA for left ventricular hypertrophy;In PA patients with OSA,cardiac impairment is severer in the moderate and severe OSA subgroups compared to those without OSA,and the severity of cardiac impairment escalates as OSA severity increa-ses.These findings highlight the necessity of screening for OSA in PA patients to stratify cardiac impairment risk and enable early therapeutic intervention.
4.Correlation Analysis of Serum DKK-1 and LTBP2 Levels with Disease Activity and Prognosis in Patients with Connective Tissue Disease-related Interstitial Pneumonia
Yarao FENG ; Jinliang YANG ; Huan LUO ; Shaoying GUO ; Zhanfen REN ; Xuejun ZHENG
Journal of Modern Laboratory Medicine 2024;39(6):135-140
Objective To analyze the changes in the expression levels of serum Dickkopf-related protein 1(DKK-1)and latent transforming growth factor binding protein 2(LTBP2)in patients with connective tissue disease(CTD)related interstitial pneumonia(IP)of different disease activity levels before and after treatment.Methods A total of 121 CTD patients who visited the First Affiliated Hospital of Hebei North University from January 2022 to October 2023 were collected and separated into an observation group(CTD-related IP patients,n=62)and a reference group(CTD without IP patients,n=59)based on the incidence of IP.The observation group was separated into a stable phase group(n=26)and an acute exacerbation phase group(n=36)based on disease activity.Enzyme-linked immunosorbent assay(ELISA)detected DKK-1 and LTBP2 levels.Pearson or Spearman were used to analyze correlations between DKK-1 and LTBP2 levels with clinical data.Logistic regression was applied to analyze influencing factors of acute exacerbation in CTD-related IP patients.Results The serum levels of DKK-1(14.98±3.32 ng/ml)and LTBP2(32.64±4.01 ng/ml)in the observation group were higher than those in the reference group(2.21±0.67 ng/ml,8.73±2.15 ng/ml),the differences were statistically significant(t=28.983,57.518,all P<0.05).The proportions of patients with ground glass opacity(66.67%)and honeycomb opacity(52.78%),serum DKK-1(19.67±4.10 ng/ml),LTBP2(38.76±4.92 ng/ml)and C-reactive protein(CRP)(32.46±3.12 mg/L)in the acute exacerbation group were higher than those in the stable phase group(30.77%,23.08%,8.48±1.37 ng/ml,24.17±3.65 ng/ml,22.05±2.80 mg/L),the differences were statistically significant(t/x2=7.790,5.534,13.362,12.781,13.524,all P<0.05).The serum levels of DKK-1 and LTBP2 in patients with acute exacerbation of CTD-related IP after treatment were positively correlated with ground glass opacities,honeycomb opacities,CRP and different disease activity(r=0.526,0.518,0.513,0.548;0.499,0.514,0.520,0.561,all P<0.05).As the treatment time extended,the serum levels of DKK-1 and LTBP2 in CTD-related IP patients in the stable and acute exacerbation groups decreased,and the serum levels of DKK-1 and LTBP2 in the acute exacerbation group were higher than those in the stable group before treatment,1 month after treatment,and 3 months after treatment,the differences were statistically significant(t=13.355,13.206,15.913;12.781,12.263,11.161,all P<0.05).DKK-1[OR(95%CI):2.458(1.297~4.657)],LTBP2[OR(95%CI):2.739(1.567~4.789)]were independent risk factors for acute exacerbation of CTD related IP patients(all P<0.05).Conclusion The serum levels of DKK-1 and LTBP2 in CTD-related IP patients are increased,and closely related to disease activity.Both decrease after 3 months of treatment and can monitor the treatment efficacy of patients to a certain extent.
5.Listeria monocytogenes meningitis in 17 immunocompetent children
Bing HU ; Hongyan ZHENG ; Tianming CHEN ; Xin GUO ; Huili HU ; Shaoying LI ; Kaihu YAO ; Gang LIU
Chinese Journal of Applied Clinical Pediatrics 2018;33(22):1735-1738
Objective To summarize the clinical characteristics and treatment of 17 children with Listeria monocytogenes(LM) meningitis (LMM).Methods Case histories (including clinical features,laboratory examination,treatment,prognosis) of 17 LMM children who were hospitalized at the Department of Infectious Disease of Beijing Children's Hospital from January 1,2013 to December 31,2017 were analyzed retrospectively.The age ranged from 7 months to 10 years,with an average of 3 years and 5 months.Among them,1 case < 1 year old,1-3 years old was most common(10 cases,accounted for 59%),2 cases >3-<6 years old,and 4 cases≥6 years old.Related literatures were summarized.Results All of 17 patients were diagnosed by a positive cerebrospinal fluid culture of LM.All patients had fever.Other symptoms included seizures,headache and vomiting were found during the course of disease;infectious symptoms were relatively mild.The symptoms presented 7 to 50 days before admission.None of the patients was known to have immune deficiencies or any other underlying diseases.Five cases underwent strain typing,all resulting from strain type l/2a.All patients used cephalosporin antibiotics before the diagnosis.After the diagnosis was confirmed,sensitive antibiotics were used according to the drug sensitivity test,including Penicillin,Meropenem,Vancomycin,Linezolid,and Sulfamethoxazole-trimethoprim (SMZ),etc.Out of the 17 patients,2 case had hydrocephalus,of which 1 cases had clinical symptoms,and underwent surgery for a ventriculoperitoneal shunt.All patients were followed up for 1 year,with good prognosis and no neurological sequela.Conclusions LMM is rare in children,especially in children with no immune deficiencies.LMM in children can present with hydrocephalus.Ampicillin remains the first choice of treatment,while meropenem,SMZ and Linezolid can be used as substitution drugs.
6.Central nervous system infection caused by Exophiala dermatitidis in a case and literature review.
Bing HU ; Shaoying LI ; Huili HU ; Tianming CHEN ; Xin GUO ; Zhixiao ZHANG ; Fang DONG ; Zheng LI ; Quan WANG ; Kaihu YAO ; Gang LIU
Chinese Journal of Pediatrics 2014;52(8):620-624
OBJECTIVETo summarize the clinical features, imaging characteristics, diagnosis and treatment of a case with central nervous system infection caused by Exophiala dermatitidis, as well as to review the related literature.
METHODAssociated literature and clinical data of an 8-year-old boy who was diagnosed as central nervous system infection caused by Exophiala dermatitidis in Beijing Children's Hospital Affiliated to Capital Medical University and hospitalized twice from 2012 to 2014 were analyzed retrospectively.
RESULTThe boy was 8 years old with the chief complaint of dizziness for 2 months, intermittent fever for 1 month accompanied with spasm twice. He was diagnosed as bile ducts space-occupying lesions 2 years ago, when the pathological diagnosis was fungal infection. The boy was treated with irregular anti-fungal therapy. Then the boy developed nervous symptoms, impaired consciousness and abnormal physical activity that developed gradually. After hospitalization the cerebral MRI of the boy showed space-occupying lesions accompanied with edema of surrounding area. Filamentous fungi was found by brain biopsy, which was culture positive for Exophiala dermatitidis. After diagnosis the boy was treated with amphotericin B (AMB), voriconazole and 5-Fu, as well as symptomatic treatment. The state of the boy was improved gradually. Two months later, the boy could communicate with others normally and move personally. The lesions and edema seen on the MRI was decreased moderately. Accordingly, the boy was treated with oral voriconazole maintenance treatment for about 1 year and 4 months after discharge. During this period, the state of him was stable without symptoms. The lesions shown by MRI did not disappear but decreased on regular examination. However, recently the disease of the boy progressed again, with dizziness, neck pain, headache and progressive nervous symptoms (intermittent spasm, inability to cough, and impaired consciousness). The boy died at last, even with the active treatment at the second hospitalization. Exophiala dermatitidis was culture-positive again in his CSF, and was confirmed by PCR successfully.
CONCLUSIONThe central nervous system infection caused by Exophiala dermatitidis is rare. Clinical features of this disease were similar to those of other fungal CNS infection, cerebral MRI of which could show the similar lumpy lesions. Diagnosis of the disease should be based on pathology and culture.
Amphotericin B ; administration & dosage ; Antifungal Agents ; administration & dosage ; Brain ; diagnostic imaging ; microbiology ; pathology ; Central Nervous System Infections ; diagnosis ; drug therapy ; microbiology ; Cerebrospinal Fluid ; microbiology ; Child ; Drug Therapy, Combination ; Exophiala ; isolation & purification ; Fatal Outcome ; Fluorouracil ; administration & dosage ; Humans ; Magnetic Resonance Imaging ; Male ; Mycoses ; diagnosis ; drug therapy ; microbiology ; Radiography ; Voriconazole ; administration & dosage
7.Casticin inhibits proliferation and invasion of breast cancer MCF-7 cells
Jihong HUANG ; Shaoying CHEN ; Jian ZHENG
Cancer Research and Clinic 2012;24(8):524-526
Objective To investigate the effects of casticin on proliferation and invasion on human breast cancer MCF-7 cells.Methods MTT assay and invasion assay were performed in MCF-7 cells treated with casticin,respectively.The expression of Bax,Caspase-3,MMP-2 and MMP-9 was analyzed by RT-PCR and Western blot.Results Casticin inhibited MCF-7 cells proliferation and invasion in a dose-dependent manner (P < 0.05).Additionally,Bax and Caspase-3 expression was increased in MCF-7 cells treated with casticin,whereas MMP-2 and MMP-9 were down-regulated.Conclusion Casticin may play a critical role in inhibiting breast tumor cell proliferation and invasion,reinforcing its potential in clinical application.
8.Laboratory detection on severe acute respiratory syndrome
Jicheng HUANG ; Zhuoyue WAN ; Qiuxia CHEN ; Hui LI ; Kui ZHENG ; Huanying ZHENG ; Xinge YAN ; Xin ZHANG ; Ling FAN ; Jie LI ; Xiaoling DENG ; Huiqiong ZHOU ; Ping HUANG ; Limei DIAO ; Haojie ZHONG ; Wanli ZHANG ; Shaoying XIE ; Jingdiao CHENG ; Jian WANG ; Jinyan LIN ; Feng DENG
Chinese Journal of Laboratory Medicine 2003;0(10):-
Objective To provide scientific evidence to identify and confirm severe acute respiratory syndrome (SARS) by laboratory detection.Methods Multiple clinical specimens were collected serially and systematically from the 4 suspected SARS patients, which occurred between Dec.2003 to Jan.2004 in Guangdong Province. The samples were tested by serologic and molecular methods.Results IgM or IgG antibodies against SARS-CoV were detectable after 6—8 days of the onset in four patients. The four-fold or greater rising in antibodies was clearly detected in three of the four patients, while the fourth patient’s seroconversion was from negative to positive. The results analysed by enzyme-linked immunosorbent assay( ELISA), immunoflourescence assay (IFA), and neutralization test were highly correlated. SARS-CoV RNA was just detected in 3 throat swab specimens from case 1 by real-time PCR. M, N and S genes were amplified by reverse transcriptase polymerase chain reaction (RT-PCR) from the positive samples. Sequencing results showed that they were SARS-CoV gene segments, and most closely matched SARS-CoV gene sequences were isolated from civet cats in Guangdong Province. Nevertheless, SARS-CoV was not isolated from any samples of the 4 patients.Conclusion Based on these results, the 4 reported cases were laboratorily confirmed as SARS cases.

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