1.The interval of rescue treatment does not affect the efficacy and safety of Helicobacter pylori eradication: A prospective multicenter observational study.
Minjuan LIN ; Junnan HU ; Jing LIU ; Juan WANG ; Zhongxue HAN ; Xiaohong WANG ; Zhenzhen ZHAI ; Yanan YU ; Wenjie YUAN ; Wen ZHANG ; Zhi WANG ; Qingzhou KONG ; Boshen LIN ; Yuming DING ; Meng WAN ; Wenlin ZHANG ; Miao DUAN ; Shuyan ZENG ; Yueyue LI ; Xiuli ZUO ; Yanqing LI
Chinese Medical Journal 2025;138(12):1439-1446
BACKGROUND:
The effect of the interval between previous Helicobacter pylori (H. pylori) eradication and rescue treatment on therapeutic outcomes remains unknown. The aim of this study was to investigate the association between eradication rates and treatment interval durations in H. pylori infections.
METHODS:
This prospective observational study was conducted from December 2021 to February 2023 at six tertiary hospitals in Shandong, China. We recruited patients who were positive for H. pylori infection and required rescue treatment. Demographic information, previous times of eradication therapy, last eradication therapy date, and history of antibiotic use data were collected. The patients were divided into four groups based on the rescue treatment interval length: Group A, ≥4 weeks and ≤3 months; Group B, >3 and ≤6 months; Group C, >6 and ≤12 months; and Group D, >12 months. The primary outcome was the eradication rate of H. pylori . Drug compliance and adverse events (AEs) were also assessed. Pearson's χ2 test or Fisher's exact test was used to compare eradication rates between groups.
RESULTS:
A total of 670 patients were enrolled in this study. The intention-to-treat (ITT) eradication rates were 88.3% (158/179) in Group A, 89.6% (120/134) in Group B, 89.1% (123/138) in Group C, and 87.7% (192/219) in Group D. The per-protocol (PP) eradication rates were 92.9% (156/168) in Group A, 94.5% (120/127) in Group B, 94.5% (121/128) in Group C, and 93.6% (190/203) in Group D. There was no statistically significant difference in the eradication rates between groups in either the ITT ( P = 0.949) or PP analysis ( P = 0.921). No significant differences were observed in the incidence of AEs ( P = 0.934) or drug compliance ( P = 0.849) between groups.
CONCLUSION:
The interval duration of rescue treatment had no significant effect on H. pylori eradication rates or the incidence of AEs.
REGISTRATION
ClinicalTrials.gov , NCT05173493.
Humans
;
Helicobacter Infections/drug therapy*
;
Helicobacter pylori/pathogenicity*
;
Male
;
Female
;
Prospective Studies
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Middle Aged
;
Anti-Bacterial Agents/adverse effects*
;
Adult
;
Aged
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Treatment Outcome
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Proton Pump Inhibitors/therapeutic use*
2.Occupational health risk assessment of n-hexane based on four risk assessment models
Fei LI ; Lu KONG ; Boshen WANG ; Fanwei XU ; Na CAI ; Baoli ZHU
Journal of Environmental and Occupational Medicine 2024;41(6):655-660
Background N-hexane has been a widely used solvent in industrial production, but it is volatile at room temperature and can be accumulated in the body, and its prolonged occupational exposure may lead to serious chronic diseases in workers. Objective To use four risk assessment models to evaluate the health risk levels of n-hexane-exposed workers, discuss the applicability of the four models in the health risk assessment of n-hexane exposure, and make an important supplement to the health risk assessment of n-hexane in China. Methods In 2022, a total of 167 jobs (1724 workers) exposed to n-hexane in 85 manufacturing enterprises in Jiangsu Province were selected, and a cross-sectional study was conducted and included questionnaire surveys and evaluation of on-site air n-hexane of each job. Subsequently, the China’s classification standards of occupational hazards at workplaces (China model), U.S. Environmental Protection Agency (EPA) model, Singapore semi-quantitative risk assessment model (Singapore model), and the International Council on Mining and Metals (ICMM) model were applied to the quantitative, semi-quantitative, and qualitative assessments of the occupational health risk level of n-hexane-exposed workers. Results All job’s 8-h time-weighted average concentrations (CTWA) of n-hexane were within the national occupational exposure limits (OELs). The results of the China model graded all jobs as relatively harmless. The Singapore model graded all jobs as low risk, except that two monitoring sites of adhesive jobs were assessed as medium risk. The ICMM quantitative model evaluated all jobs as intolerable for n-hexane airborne exposure, while the matrix method evaluated all jobs as low risk. The U.S. EPA model identified five sites involving painting, printing, and adhesive jobs as high risk and the other jobs as low risk. Conclusion Inconsistent grading results are observed by using the four models for the occupational health risk assessment of n-hexane exposure, that is, harmless for all jobs by China model, while medium and high risks by Singapore model and U.S. EPA model. Therefore, we recommend to combine the Singapore model and the U.S. EPA model with the China model to assess the occupational risk of n-hexane-exposed workers by considering actual concentrations of exposure.
3.Incidence and risk factors of anemia among newly reported HIV/AIDS patients in Jiangsu Province in 2021
Zhi ZHANG ; Qi SUN ; Tao QIU ; Ping DING ; Boshen WANG ; Baoli ZHU
Chinese Journal of Schistosomiasis Control 2024;36(6):598-605
Objective To investigate the incidence of anemia and evaluate the immune status among newly reported HIV/AIDS patients in Jiangsu Province in 2021, and to identify the risk factors of anemia among patients living with HIV infections. Methods Newly reported HIV/AIDS patients in Jiangsu Province from January 1 to December 31, 2021 that were registered in China’s National AIDS Comprehensive Control Information Management System were enrolled. Subjects’ fresh whole blood samples were collected, and hemoglobin levels, CD4 and CD8 cell counts and HIV viral loads were measured. Anemia was defined according to hemoglobin levels, and the immunological parameters and HIV viral loads were compared between HIV-infected patients with and without anemia. The risk factors of anemia were identified among individuals living with HIV infections using univariate analysis and multivariate logistic regression analysis. In addition, subjects’ CD4 cell counts one year following antiretroviral therapy (ART) were retrieved from China’s National AIDS Comprehensive Control Information Management System, and compared between subjects with and without anemia. Results A total of 635 newly diagnosed HIV/AIDS patients were reported in Jiangsu Province in 2021, including 544 males (85.67%) and 91 females (14.33%), and with ages of 15 to 83 years, and the overall incidence of anemia was 5.51% (35/635) among the study subjects. Men, individuals at ages of 45 years and lower and workers had relatively higher hemoglobin levels, with median hemoglobin levels of 156 (interquartile range, 22), 154 (interquartile range, 23) g/L and 162 (interquartile range, 19) g/L, respectively. The median baseline HIV viral load was 40 500.00 (interquartile range, 119 735.00) copies/mL among HIV-infected individuals with anemia and 29 754.00 (69 183.00) copies/mL among those without anemia (Z = -0.91, P = 0.31), and the median baseline CD4 and CD8 cell counts were significantly lower among HIV-infected individuals with anemia [166 (interquartile range, 143) cells/μL and 755 (653) cells/μL] than those without anemia [308 (253) cells/μL and 892 (638) cells/μL] (Z = -4.36 and -2.37, both P values < 0.05). The median CD4 cell counts remained lower among HIV-infected individuals with anemia than those without anemia [296 (interquartile range, 229) cells/μL vs. 457 (interquartile range, 313) cells/μL; Z = -3.71, P < 0.05] one year following ART, and the proportions of moderate and severe immunosuppression were significantly higher among HIV-infected individuals with anemia (40.00% and 17.14%) than those without anemia (21.00% and 9.33%) (χ2 = 10.37 and 8.79, both P values < 0.01). Univariate analysis showed a higher detection rate of anemia among female HIV-infected individuals than among males [odds ratio (OR) = 4.528, 95% confidence interval (CI): (3.811, 5.245), P < 0.001], a higher rate among HIV-infected individuals at ages of 45 to < 60 years [OR = 3.415, 95% CI: (1.191, 9.788), P = 0.022] and 60 years and older [OR = 5.820, 95% CI: (2.013, 16.826), P < 0.001] than among those at ages of 15 to < 30 years, a higher rate among HIV-infected individuals through heterosexual transmission than among those through homogeneous transmission [OR = 3.015, 95% CI: (1.423, 6.387), P = 0.004], a lower rate among HIV-infected individuals with an educational level of college and above than among those with an educational level of primary school [OR = 0.103, 95% CI: (0.028, 0.386), P < 0.001], a higher rate among HIV-infected individuals with baseline CD4 cell counts of < 200 cells/μL than among those with baseline CD4 cell counts of 200 cells/μL and higher [OR = 4.340, 95% CI: (2.165, 8.702), P < 0.001], and lower detection rates among HIV-infected individuals with CD4/CD8 cell ratios of 0.208 to < 0.326 [OR = 0.232, 95% CI: (0.076, 0.711), P = 0.011] and 0.516 and higher [OR = 0.292, 95% CI: (0.104, 0.818), P = 0.019] than among those with CD4/CD8 cell ratios of < 0.208. Multivariate logistic regression analysis identified woman [OR = 4.945, 95% CI: (3.944, 5.946), P = 0.002], and CD4 cell counts of < 200 cells/μL [OR = 3.597, 95% CI: (1.448, 8.937), P = 0.006] as risk factors of anemia among newly reported HIV/AIDS patients. Conclusions The incidence of anemia was low among newly reported HIV/AIDS patients in Jiangsu Province in 2021, and the immune status was poorer among HIV-infected individuals with anemia than those without anemia at baseline and one year following ART. Female and CD4 cell counts of < 200 cells/μL are risk factors of anemia among individuals living with HIV infections, and intensified surveillance, follow-up and precision interventions are recommended targeting female HIV-infected individuals and HIV-infected individuals with low CD4 cell counts.
4.Therapeutic Effect of Cang-ai Volatile Oil on High Altitude Rats with Cardiac Impairment Based on 7.0T Cardiovascular Magnetic Resonance
Boshen LIANG ; Bojun CHEN ; Hongke YIN ; Lei WANG ; Haotian CHEN ; Xin FANG ; Haichen LI ; Jinghang SUO ; Lei XIONG ; Yonghai ZHANG ; Fabao GAO
Chinese Journal of Medical Imaging 2024;32(9):865-872
Purpose To explore the cardioprotective effect of cang-ai volatile oil(CAVO)on rats with cardiac function impairment model under low-pressure and low-oxygen environment in Tibet Plateau based on 7.0T cardiovascular magnetic resonance(CMR)imaging.Materials and Methods Forty SD rats were randomly divided into the normal group,the high altitude model group,the CAVO-treated group and the rhodiola rosea-treated group,with 10 rats in each group.Except for the normal group,the rats in other groups were transferred from the plain(500 m above sea level)to the Tibet Plateau(4 250 m above sea level)for two months,and then administered with the corresponding drugs by gavage for 14 d.The left ventricle function was measured by using a 7.0T high-field strength CMR and myocardial strain was analysed by using tissue tracing technique.HE staining was used to observe the morphology of cardiomyocytes,Masson staining to observe interstitial fibrosis,wheat germ agglutinin staining to observe cardiomyocyte hypertrophy,and transmission electron microscopy to observe the morphological changes of mitochondria in each group.Serum levels of creatine kinase,creatine kinase isoenzyme,lactate dehydrogenase,cardiac troponin T,superoxide dismutase,malondialdehyde and glutathione peroxidase were detected.Intracellular reactive oxygen species levels were detected using flow cytometry.Results The left ventricular ejection fraction of rats in the CAVO-treated group was higher than that of the high altitude model group[(66.61±1.38)%vs.(60.94±3.21)%;t=3.969,P=0.032];meanwhile,the global circumferential strain of the left ventricle in the CAVO-treated group was higher than that of the high altitude model group(-25.68±1.30 vs.-22.84±1.17;t=3.967,P=0.003).HE,Masson and wheat germ agglutinin staining showed hypertrophy and necrosis as well as interstitial fibrosis and ultrastructural disruption of cardiomyocytes in the high altitude model group,which improved after CAVO treatment.The level of cardiac troponin T in the serum of rats with CAVO treatment group was significantly decreased compared with that of the high altitude model group[(314.03±20.05)pg/ml vs.(518.30±18.13)pg/ml;1=13.090,P=0.001].Conclusion CAVO treatment can reduce cardiac injury caused by low-pressure hypoxia in high altitude,and its effect can be detected dynamically and non-invasively by 7.0T high-field strength CMR.
5.A study of airborne pollen detection and allergic rhinitis visits in Cangzhou,China
Weiwei LIU ; Boshen WEN ; Xiaoming SU ; Yan HUANG ; Mengmeng SHEN ; Yujing JIA ; Bin WANG
Chinese Archives of Otolaryngology-Head and Neck Surgery 2024;31(10):652-656
OBJECTIVE To explore the distribution of airborne pollen in Cangzhou,to analyze the correlation between pollen characteristics,meteorological factors and the rate of allergic rhinitis visits,and to provide a reference basis for the precise prevention and treatment of allergic rhinitis in Cangzhou.METHODS The gravity deposition method was applied to collect daily airborne pollen from the monitoring sites in Cangzhou from March to October 2022 and record the pollen species and quantities.We also collected information on allergic rhinitis patients who visited the ENT Department of Cangzhou Central Hospital during the same period and meteorological data(daily maximum temperature,minimum temperature,maximum relative humidity,maximum precipitation,and maximum wind speed at ground level)during the same period,and analyzed the relationship between the number of AR patients'visits,the amount of pollen,and the meteorological factors.RESULTS 1.A total of 229 pollen exposures were collected from March to October 2022,totaling 19 368 pollen grains,of which 18 750 grains of pollen could be identified in 19 families and 618 grains were difficult to identify the families.2.There were two peaks of pollen during the period,in April and September respectively.Spring pollen to tree-based category,to the pine family,willow willow family,Xylariaceae(ash)most often;summer pollen volume has declined,but still tree-based category;fall pollen to herbaceous category,to the Asteraceae Artemisia,mulberry,Gramineae most often.3.The pollen concentration in Cangzhou City was negatively correlated with maximum humidity,maximum precipitation,maximum temperature,and minimum temperature.Maximum wind speed at ground level was positively correlated with pollen concentration.4.Pollen concentration was positively correlated with the number of visits.The correlation analysis between daily medical visits from June to October and air pollen concentration showed a significant positive correlation,while the results from March to May showed no significant correlation.CONCLUSION The peak of airborne pollen dispersal in April and September in Cangzhou.Spring and summer pollen is dominated by tree species,and the dominant pollen is Pinaceae,Willow genus of Populus,and Xylariaceae(ash genus);fall pollen is dominated by herbaceous species,and the dominant pollen is Artemisia,Asteraceae,Mulberry,and Gramineae.Meteorological factors are important factors affecting pollen concentration.Within a certain range,the lower the maximum humidity,the lowest maximum precipitation,the lower the highest or the lowest temperature,the higher the pollen concentration,and the pollen concentration is more affected by the lowest temperature.There is a correlation between pollen concentration and the number of AR patients'visits from June to October,which can be used as an environmental early warning indicator of the prevalence of AR.
6.Basic Research on the Microstructure of Rat Bones in the High-Altitude Environment of Qinghai-Tibet Plateau
Sisi ZHAO ; Zhetao WANG ; Hongke YIN ; Chunhua WANG ; Jinghang SUO ; Boshen LIANG ; Lei WANG ; Fabao GAO
Journal of Sichuan University (Medical Sciences) 2024;55(6):1469-1476
Objective To establish a hypobaric hypoxia rat model in a real high-altitude environment,to investigate the effects of the real high-altitude environment on rat bone mass and bone microstructure using multiple methods such as Micro CT,blood biochemistry,and pathology,and to explore the potential mechanisms involved.Methods Sprague Dawley(SD)rats were transported to the Yushu Plateau Laboratory(at 4250 m above sea level)in Qinghai Province and kept there for 4,or 8,or 18 months.These groups were designated as H-4,H-8,and H-18,respectively.Upon completion of the high-altitude exposure,these animals were transported to the Molecular Imaging Laboratory,West China Hospital,Sichuan University(at 500 m above sea level)in Chengdu for relevant testing and comparison with the control animals raised in a low-altitude environment for the same durations(designated L-4,L-8,and L-18).The tests performed included blood biochemistry,Micro CT imaging,and pathological assessments such as ELISA,Western blot,and HE and TRAP staining.Results Compared with that of the control group,the body mass of rats in the H-4 and H-18 groups decreased significantly(H-4 group vs.L-4 group:[513.75±35.10]g vs.[649.18±60.03]g,P<0.01;H-18 group vs.L-18 group:[535.58±66.65]g vs.[670.86±44.96]g,P<0.01).The serum Ca2+concentration was higher in the H-8 group and H-18 group compared to that in the control group(H-8 group vs.L-8 group:[2.48±0.09]mmol/L vs.[2.38±0.07]mmol/L,P<0.05;H-18 group vs.L-18 group:[2.55±0.11]mmol/L vs.[2.13±0.27]mmol/L,P<0.05).No statistically significant difference was observed in the concentration of P3+.Bone metabolism indicator cross-linked carboxy-terminal telopeptide of type Ⅰ collagen(CTX-Ⅰ)was significantly increased in all high-altitude groups compared to the low-altitude groups(H-4 group vs.L-4 group:[1.44±0.08]ng/mL vs.[0.70±0.13]ng/mL,P<0.01;H-8 group vs.L-8 group:[1.52±0.10]ng/mL vs.[0.75±0.10]ng/mL,P<0.01;H-18 group vs.L-18 group:[2.70±0.13]ng/mL vs.[1.94±0.15]ng/mL,P<0.01).In addition,CT results showed a decrease in bone volume fraction of trabecular bone in the three high-altitude groups(H-4 group vs.L-4 group:[7.48±2.35]%vs.[10.40±2.93]%,P<0.05;H-8 group vs.L-8 group:[7.17±2.68]%vs.[10.09±2.95]%,P<0.05;H-18 group vs.L-18 group:[2.90±2.91]%vs.[8.68±4.11]%,P<0.01),and increased trabecular separation in the three high-altitude groups(H-4 group vs.L-4 group:[0.70±0.12]mm vs.[0.60±0.06]mm,P<0.05;H-8 group vs.L-8 group:[0.68±0.07]mm vs.[0.59±0.05]mm,P<0.01;H-18 group vs.L-18 group:[0.80±0.09]mm vs.[0.70±0.09]mm,P<0.05).TRAP staining showed an increase in osteoclasts in the H-4 and H-18 groups.Western blot results indicated an increase in the expression of receptor activator of nuclear factor-κB ligand(RANKL)and hypoxia inducible factor-1α(HIF-1α)in high-altitude environment,while the expression of osteoprotegerin(OPG)was inhibited.Conclusion The impact of high-altitude environment on rat femurs is characterized primarily by a reduction in trabecular bone mass and damage to bone microstructure.
7.Therapeutic Effect of Cang-Ai Volatile Oil on High-Altitude Rats With Cardiac Hypertrophy Through Modulation of Oxidative Stress Response
Boshen LIANG ; Hongke YIN ; Lei WANG ; Haotian CHEN ; Xin FANG ; Sisi ZHAO ; Qi ZHU ; Lei XIONG ; Jinghang SUO ; Baijun CHEN ; Fabao GAO
Journal of Sichuan University (Medical Sciences) 2024;55(6):1485-1493
Objective To explore the therapeutic effect of Cang-ai volatile oil(CAVO)on rats with myocardial hypertrophy(MH)exposed to the hypobaric hypoxic environment of the Qinghai-Tibet Plateau using 7.0-tesla(7.0T)cardiac magnetic resonance imaging(CMR).Methods A total of 50 male specific pathogen-free(SPF)Sprague-Dawley(SD)rats were randomly assigned to a low-altitude control(CON)group,hypobaric hypoxia(HH)group,myocardial hypertrophy modeling(MH)group,MH modeling plus CAVO treatment(MH+CAVO)group,and MH modeling plus benadryl hydrochloride treatment(MH+RX)group,with 10 rats in each group.Except for the CON group,the rats in all the groups were kept and fed in the standard way for 8 weeks in a high-altitude environment(at 4250 m above sea level),and then given the corresponding treatment drugs by gastric gavage.Afterwards,7.0T high field strength CMR was used to measure left ventricular(LV)function and myocardial strain.Hematoxylin-eosin(HE)staining and Masson staining were performed to observe myocardial interstitial fibrosis.Wheat germ agglutinin(WGA)staining was performed to analyze the cross-sectional area of cardiomyocytes.Transmission electron microscopy was used to observe the ultrastructural changes of the myocardium.Serum levels of cardiac troponin T(cTnT),superoxide dismutase(SOD),malondialdehyde(MDA),and glutathione peroxidase(GSH-PX)were measured by ELISA.Results Compared with those of the control group,the MH group had significantly lower left ventricular global circumferential strain(LVGCS)at(-18.85±1.67)%and left ventricular global longitudinal strain(LVGLS)at(-20.39±1.48)%(P<0.05).However,the MH+CAVO group had significantly higher LVGCS at(-22.10±1.08)%and LVGLS at(-24.60±1.72)%compared with those of the MH group(both P<0.05),indicating that CAVO treatment improved LV function.The MH group had a decreased level of serum glutathione peroxidase(GSH-Px)in comparison with the CON group([1173.49±27.10]U/mL vs.[300.83±47.25]U/mL,P<0.01),a decreased SOD level in comparison with the CON group([302.27±3.65]U/mL vs.[105.96±4.03]U/mL,P<0.01),and an increased level of serum malondialdehyde(MDA)in comparison with the CON group([57.91±1.13]μmol/L vs.[6.65±2.99]μmol/L,P<0.01),suggesting that the antioxidant capacity of rats in the MH group was decreased.After CAVO intervention,rats in the MH+CAVO group exhibited an increase in the serum levels of SOD at(278.51±5.97)U/mL and GSH-Px at(961.82±17.56)U/mL,as well as a decrease in MDA at(17.79±1.33)μmol/L(all P<0.05).Conclusion CAVO can effectively improve cardiac function in rats with cardiac hypertrophy exposed to high-altitude environment by modulating oxidative stress and ameliorating cardiac hypertrophy.
8.Exercise performance reduction and preventive measures in highland sports
Boshen LI ; Jun YANG ; Peng WANG ; Xiaolin LI ; Maoxing LI ; Yuxuan ZHANG
Journal of Central South University(Medical Sciences) 2023;48(10):1464-1478
The plateau is a special environment with low pressure,low oxygen,low temperature,and high ultraviolet radiation.The exercise performance of people on the plateau is generally reduced,which seriously affects the life and health of people living in the plateau and entering the plateau.In recent years,the prevention and treatment of injury caused by high altitude hypoxia has attracted wide attention.It has shown that the higher the altitude with the longer the duration of exercise,the faster the stationing,the greater the impact on people's sports performance.Rapid entry into the plateau and long-term stay in the plateau have an impact on people's explosive power,endurance and fine operation.Advances in medical technology enable various prevention methods to be used to acclimate to high altitude environments.However,in vitro intervention methods are costly,easy to rebound and possess limited effects.Therefore,drug prevention and treatment is obviously a more economical choice.Chemical drugs increase the efficiency of high altitude exercise by improving the ischemic and hypoxic symptoms of the heart and brain,increasing lung ventilation and arterial oxygenation capacity,and accelerating the elimination of adverse product accumulation after exercise.Single Chinese medicine,Chinese patent medicine,and compound preparations can improve exercise performance by promoting body metabolism,improving muscle endurance,enhancing immunity,and other mechanisms.Traditional Chinese medicine has unique advantage and application prospect in improving plateau sports performance damage.
9.Synchronous double primary malignant tumours of the tongue and lung: a case report and literature review
ZHANG Qianyu ; YANG Peixin ; CHEN Boshen ; WANG lin ; HUANG Zhiquan
Journal of Prevention and Treatment for Stomatological Diseases 2020;28(9):586-589
Objective:
To investigate the diagnosis and treatment of synchronous double primary malignant tumours of the tongue and lung.
Methods:
A case of adenoid cystic carcinoma(ACC) and lung adenocarcinoma with double primary malignancy was retrospectively analyzed.
Results:
The tumor of patient′s tongue base gradually grew. MRI showed multiple enlarged lymph nodes on both sides of neck. CT of the chest showed obvious lesions in the anterior basal segment of the right lower lobe. The pathological biopsy of the tongue mass identified ACC, and pathological biopsy of the lung mass identified lung adenocarcinoma. The tongue and lung tumors were both surgically resected, and the tongue defect was repaired at the same time. No residue was found after surgery, and no recurrence was found during the follow-up period. The aesthetic and functional restoration of the lingual region was good.
Conclusion
There are few cases of adenoid cystic carcinoma and lung adenocarcinoma with double primary malignancies, and the related diagnosis and treatment are very difficult; the simultaneous removal of double primary malignant tumors may achieve good prognosis.
10.Targeted therapy of pyogenic sterile arthritis, pyoderma gangrenosum, and acne syndrome (PAPA): a case report and literature review
Weiling LIANG ; Hanguang LI ; Gong ZHONG ; Boshen WANG ; Jianda MA ; Jianliang CHEN ; Huawei MAO ; Lau YU?LUNG ; Pamela LEE
Chinese Journal of Pediatrics 2020;58(12):977-981
Objective:To analyze the clinical course and targeted therapy of pyogenic sterile arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome.Methods:The clinical history of a 6-year-old boy with PAPA syndrome, who was admitted to Hong Kong University Shenzhen Hospital in September 2017, was reviewed. His genetic diagnosis was confirmed by whole exome sequencing. The response to targeted therapy was evaluated by comparing the inflammatory markers (erythrocyte sedimentation rate (ESR) and C reactive protein (CRP) and serum cytokines (interleukin (IL)-1, IL-6 and tumor necrosis factor-α (TNF-α)) before and after biological agents treatment. For literature review, "PAPA syndrome" and"PSTPIP1 gene"were used as keywords to retrieve papers published from January 1997 to December 2019 from Pubmed, Wanfang and CNKI database.Results:The patient was a 6-year-old boy, admitted to the hospital due to recurrent joint swelling and pain for more than 4 years. Before treatment, the CRP (256 mg/L), ESR (105 mm/1 h) and cytokines including serum TNF-α (7.43 ng/L), IL-1 (<5 ng/L), IL-6 (301 ng/L) were significantly elevated. Culture of the joint effusion was negative, but the IL-6 level was above 1 000 ng/L. MRI showed osteomyelitis at the lower end of the right femur. Gene detection found a heterozygous variation of PSTPIP1 gene (c.748G>A, p.E250K). Arthralgia once alleviated after the initiation of tocilizumab and infliximab, but recurred after 1 year of treatment. Thereafter, the anti-IL-1 receptor antagonist (Anakinra) was commenced, followed by a significant improvement of the arthralgia, and a complete remission during the follow-up. Besides, the level of CRP, ESR, serum TNF-α, IL-1 and IL-6 were all decreased to normal on the last followed up in December 2019. Literature review found 29 articles and 87 patients in total. The initial symptoms included those of arthritis ( n=58), pyoderma gangrenosum ( n=33), and acne ( n=24). Among all the cases, 13 genotypes were confirmed, and 47 variations involved amino acid p.E250. Steroid and/or biological agents were used in most patients. Conclusions:PAPA syndrome should be suspected in children with recurrent pyogenic sterile arthritis, and an early diagnosis could be achieved by genetic test. Targeted treatment with biological agent may control the symptoms effectively. Biological agents can control symptoms of this disorder effectively.


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