1.Rapid Identification of Different Parts of Nardostachys jatamansi Based on HS-SPME-GC-MS and Ultra-fast Gas Phase Electronic Nose
Tao WANG ; Xiaoqin ZHAO ; Yang WEN ; Momeimei QU ; Min LI ; Jing WEI ; Xiaoming BAO ; Ying LI ; Yuan LIU ; Xiao LUO ; Wenbing LI
Chinese Journal of Experimental Traditional Medical Formulae 2025;31(2):182-191
		                        		
		                        			
		                        			ObjectiveTo establish a model that can quickly identify the aroma components in different parts of Nardostachys jatamansi, so as to provide a quality control basis for the market circulation and clinical use of N. jatamansi. MethodsHeadspace solid-phase microextraction-gas chromatography-mass spectrometry(HS-SPME-GC-MS) combined with Smart aroma database and National Institute of Standards and Technology(NIST) database were used to characterize the aroma components in different parts of N. jatamansi, and the aroma components were quantified according to relative response factor(RRF) and three internal standards, and the markers of aroma differences in different parts of N. jatamansi were identified by orthogonal partial least squares-discriminant analysis(OPLS-DA) and cluster thermal analysis based on variable importance in the projection(VIP) value >1 and P<0.01. The odor data of different parts of N. jatamansi were collected by Heracles Ⅱ Neo ultra-fast gas phase electronic nose, and the correlation between compound types of aroma components collected by the ultra-fast gas phase electronic nose and the detection results of HS-SPME-GC-MS was investigated by drawing odor fingerprints and odor response radargrams. Chromatographic peak information with distinguishing ability≥0.700 and peak area≥200 was selected as sensor data, and the rapid identification model of different parts of N. jatamansi was established by principal component analysis(PCA), discriminant factor alysis(DFA), soft independent modeling of class analogies(SIMCA) and statistical quality control analysis(SQCA). ResultsThe HS-SPME-GC-MS results showed that there were 28 common components in the underground and aboveground parts of N. jatamansi, of which 22 could be quantified and 12 significantly different components were screened out. Among these 12 components, the contents of five components(ethyl isovalerate, 2-pentylfuran, benzyl alcohol, nonanal and glacial acetic acid,) in the aboveground part of N. jatamansi were significantly higher than those in the underground part(P<0.01), the contents of β-ionone, patchouli alcohol, α-caryophyllene, linalyl butyrate, valencene, 1,8-cineole and p-cymene in the underground part of N. jatamansi were significantly higher than those in the aboveground part(P<0.01). Heracles Ⅱ Neo electronic nose results showed that the PCA discrimination index of the underground and aboveground parts of N. jatamansi was 82, and the contribution rates of the principal component factors were 99.94% and 99.89% when 2 and 3 principal components were extracted, respectively. The contribution rate of the discriminant factor 1 of the DFA model constructed on the basis of PCA was 100%, the validation score of the SIMCA model for discrimination of the two parts was 99, and SQCA could clearly distinguish different parts of N. jatamansi. ConclusionHS-SPME-GC-MS can clarify the differential markers of underground and aboveground parts of N. jatamansi. The four analytical models provided by Heracles Ⅱ Neo electronic nose(PCA, DFA, SIMCA and SQCA) can realize the rapid identification of different parts of N. jatamansi. Combining the two results, it is speculated that terpenes and carboxylic acids may be the main factors contributing to the difference in aroma between the underground and aboveground parts of N. jatamansi. 
		                        		
		                        		
		                        		
		                        	
2.Research progress on information needs of patients undergoing tumor immunotherapy
Wen TIAN ; Xiaoming GAO ; Haoyue YAO ; Dan WU
Chinese Journal of Modern Nursing 2024;30(25):3496-3500
		                        		
		                        			
		                        			This study aims to review the content of information needs, influencing factors, and current support status of patients undergoing tumor immunotherapy and to provide reference for developing targeted information support strategies for these patients.
		                        		
		                        		
		                        		
		                        	
3.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.
		                        		
		                        		
		                        		
		                        	
4.Evaluation of the efficacy and safety of intravenous infusion of ferric derisomaltose in the treatment of iron deficiency anemia: a single-center retrospective analysis
Shaoxue DING ; Yihui ZHAO ; Ting WANG ; Jing GUAN ; Limin XING ; Hong LIU ; Guojin WANG ; Xiaoming WANG ; Yuhong WU ; Wen QU ; Jia SONG ; Huaquan WANG ; Lijuan LI ; Zonghong SHAO ; Rong FU
Chinese Journal of Hematology 2024;45(2):178-183
		                        		
		                        			
		                        			Objective:To investigate the clinical efficacy and safety of ferric derisomaltose injection versus iron sucrose injection in the treatment of iron deficiency anemia (IDA) .Methods:A total of 120 patients with iron deficiency anemia admitted from June 2021 to March 2023 were given intravenous iron supplementation with ferric derisomaltose to assess the efficacy and safety of hemoglobin (HGB) elevation before and after treatment. Simultaneously, the clinical effects of iron supplementation with iron sucrose were compared to those of inpatient patients during the same period.Results:Baseline values were comparable in both groups. Within 12 weeks of treatment, the elevated HGB level in the ferric derisomaltose group was higher than that of the iron sucrose group, with a statistical difference at all time points, and the proportion of HGB increased over 20 g/L in the patients treated for 4 weeks was higher (98.7%, 75.9% ). During the treatment with ferric derisomaltose and iron sucrose, the proportion of mild adverse reactions in the ferric derisomaltose group was slightly lower than that of the iron sucrose group, and neither group experienced any serious adverse reactions. The patients responded well to the infusion treatment, with no reports of pain or pigmentation at the injection site.Conclusion:The treatment of IDA patients with ferric derisomaltose has a satisfactory curative effect, with the advantages of rapidity, accuracy, and safety. Therefore, it is worthy of widespread clinical use.
		                        		
		                        		
		                        		
		                        	
5.Efficacy and safety of eltrombopag in the treatment of primary immune thrombocytopenia: real-world data from a single medical center
Xifeng DONG ; Yalan LI ; Nianbin LI ; Weinan LIN ; Ting WANG ; Huaquan WANG ; Lijuan LI ; Wen QU ; Limin XING ; Hong LIU ; Yuhong WU ; Guojin WANG ; Jia SONG ; Jing GUAN ; Xiaoming WANG ; Zonghong SHAO ; Rong FU
Chinese Journal of Hematology 2024;45(3):271-276
		                        		
		                        			
		                        			Objective:This study aimed at investigating the efficacy and safety of eltrombopag in the treatment of adult primary immune thrombocytopenia (ITP) and evaluated the factors influencing its efficacy and side effects.Methods:A total of 198 patients with adult ITP who were admitted to Tianjin Medical University General Hospital between January 2018 and March 2022 were retrospectively analyzed. The efficacy of each starting dose of eltrombopag was evaluated, and adverse events were analyzed. The factors influencing efficacy were investigated, including sex, age, adult ITP type, platelet antibodies, and combined drug treatments.Results:Of the 198 patients, 70 males and 128 females with a median age of 45 years (18-88 years) were included; 130 (65.7%) had newly diagnosed adult ITP, 25 (12.6%) had persistent adult ITP, and 43 (21.7%) had chronic adult ITP. The bleeding event scores at baseline were assessed; 84.3% had scores of<4 and 15.7% had scores of ≥4. The eltrombopag response rate (initial response) at 6 weeks was 78.8% (complete response [CR]: 49.0%; CR1: 14.6%; CR2: 15.2%). The median response time to eltrombopag was 7 (7, 14) days. The initial response rates to 25, 50, and 75 mg eltrombopag were 74.1%, 85.9%, and 60.0%, respectively ( P=0.031). The initial response rate to the 50 mg dose was significantly higher than that of the 25-mg and 75-mg doses. Two patients received 100 mg as the starting dose, and their initial response was 0. Regarding dose adjustment, 70.7% of the patients remained on the starting dose, 8.6% underwent dose adjustment to 50 mg, and 6.1% underwent dose adjustment to 75 mg. Another two patients underwent dose adjustment to 100 mg. After dose adjustment, the persistent response rates were 83.6%, 85.3%, and 85.7% for the 25-, 50-, and 75-mg doses, respectively, with no significant difference. After dose adjustment, the sustained efficacy rate for the 100-mg dose (4 patients) was 100.0%. After 6 weeks of treatment with eltrombopag, the overall bleeding score of patients with ITP decreased. The number of patients with a score of ≥4 decreased to 0, the number of patients with a score of<4 decreased, and there was no significant change in the number of patients with a score of 1-2. The most common adverse event associated with eltrombopag was impaired liver function (7.7%). No thrombosis events or other adverse events were observed. ITP type and number of megakaryocytes significantly affected the initial response to eltrombopag. The initial response rates to eltrombopag for newly diagnosed adult ITP, persistent adult ITP, and chronic adult ITP were 85.3%, 56.0%, and 76.2%, respectively ( P=0.003). For megakaryocytes, the initial response rates were 61.8%, 87.1%, and 84.3% ( P=0.009) for the decreased, normal, and increased megakaryocyte groups, respectively. Conclusion:Eltrombopag, as a second-line or higher treatment for adult ITP, has a rapid onset of action and good safety. The initial response rate is significantly higher with a dose of 50 mg than with a dose of 25 mg. Patients with newly diagnosed ITP and those with normal or increased megakaryocyte numbers have a higher initial response rate to eltrombopag.
		                        		
		                        		
		                        		
		                        	
6.A single-center analysis of pathogenic bacteria distribution and drug resistance in bacterial bloodstream infections among patients with hematological diseases
Mengting CHE ; Chaomeng WANG ; Hui LIU ; Haifang KONG ; Lijuan LI ; Jia SONG ; Huaquan WANG ; Guojin WANG ; Yuhong WU ; Jing GUAN ; Limin XING ; Wen QU ; Hong LIU ; Xiaoming WANG ; Zhidong HU ; Zonghong SHAO ; Rong FU
Chinese Journal of Hematology 2024;45(10):937-943
		                        		
		                        			
		                        			Objective:To analyze the distribution and drug resistance of pathogens of bacterial bloodstream infection in patients with hematological diseases in the Department of Hematology of Tianjin Medical University General Hospital, and to provide etiological data for clinical empirical anti-infection treatment.Methods:A retrospective analysis was conducted on the general clinical information, pathogenic bacteria and drug susceptibility test results of patients with hematological diseases diagnosed with bacterial bloodstream infection by menstrual blood culture in our center from January 2016 to December 2022.Results:Patients included 498 inpatients, with a total of 639 bacterial strains. Among the patients, 86.9% patients had malignancies, and 76.7% had agranulocytosis. Symptoms of concurrent infections, including those of the respiratory tract, oral mucosa, skin and soft tissues, and abdominal sources were observed in 68.3% patients. Gram-negative bacteria (G -) accounted for 79.0% of the isolated bacteria, and gram-positive bacteria (G +) accounted for 21.0%. The top five isolated pathogens were Klebsiella pneumoniae (22.5%), Escherichia coli (20.8%), Pseudomonas aeruginosa (15.0%), Enterococcus faecium (5.5%), and Stenotrophomonas maltophilum (5.0%). Escherichia coli exhibited a decreasing trend of resistance to quinolones, cephalosporins, and carbapenems. Klebsiella pneumoniae exhibited increasing rates of resistance to quinolones and cephalosporins between 2016 and 2018, but the rated decreased after 2019. The resistance rate to carbapenems exhibited by Pseudomonas aeruginosa was approximately 20%. Carbapenem-resistant strains of Pseudomonas aeruginosa strains were first detected in 2017, with a peak resistance rate of 35.7%, detected in 2019. A 60.0% resistance rate to methicillin was observed in methicillin-resistant coagulase-negative staphylococci (MRCNS), and one case of linezolid-resistant MRCNS was detected. Conclusions:Pathogenic bacteria of bacterial bloodstream infections were widely distributed in our center, and precautions are warranted against carbapenem resistant P. aeruginosa and Klebsiella pneumoniae.
		                        		
		                        		
		                        		
		                        	
7.The effect of water improvement on iodine nutritional status and thyroid function of pregnant women in areas with high water iodine level
Kexin ZHANG ; Yuangui CHENG ; Wen WU ; Dingyan ZHANG ; Wen JIANG ; Xiaoming WANG ; Shuyao DONG ; Wanqi ZHANG
Chinese Journal of Endemiology 2023;42(6):447-452
		                        		
		                        			
		                        			Objective:To investigate the impact of switching to low iodine drinking water in areas with high water iodine levels on the iodine nutritional status and thyroid function of pregnant women.Methods:A cross-sectional survey was conducted in Gaoqing County, Zibo City, Shandong Province. Pregnant women who underwent prenatal examinations at the Obstetrics Clinic of Gaoqing County People's Hospital from 2019 to 2021 were selected as the survey subjects. With reference to the Criteria for the Classification of Water Source High Iodine Areas and High Iodine Disease Areas (GB/T 19380-2016), pregnant women with drinking water iodine > 100 μg/L were considered as the high water iodine group and ≤100 μg/L was the non-high water iodine group. Basic information, one random urine sample, fasting blood sample, 24-hour urine sample and drinking water sample of pregnant women were collected, and thyroid ultrasound examination was performed on pregnant women. Urinary iodine (UI) concentration (UIC) and drinking water iodine concentration (WIC) were measured by inductively coupled plasma mass spectrometry, and 24-hour urinary iodine excretion (UIE) and daily iodine intake (TII) of pregnant women were calculated. Serum thyroid hormone (TSH), free triiodothyronine (FT 3), free thyroxine (FT 4), thyroid peroxidase antibody (TPOAb) and anti-thyroid autoantibodies (TgAb) were determined by automatic chemiluminescence immunoassay. Creatinine (CR) was determined using deproteinized endpoint microplate method and UI/CR was calculate. Results:A total of 797 pregnant women were included, and the UIC was 150.2 (88.1, 281.3) μg/L, the iodine nutrition was generally at an appropriate level. Among them, 584 pregnant women in the non-high water iodine group had a UIC of 120.9 (74.9, 191.5) μg/L, which was at the iodine deficiency level; 213 pregnant women in the high water iodine group had a UIC of 321.1 (201.9, 569.1) μg/L, which was at the iodine super-appropriate level; the differences in WIC, UIC, UIE, TII, and UI/CR between the two groups were statistically significant ( Z = 21.63, 13.34, 15.14, 15.14, 11.81, P < 0.001). After stratification by different gestational periods, the differences were statistically significant when comparing WIC and TSH in pregnant women in the non-high water iodine group and UI/CR in pregnant women in the high water iodine group by gestational period ( H = 59.13, 7.30, 13.60, P < 0.05). A total of 744 pregnant women were tested for thyroid function, and 128 cases of TSH > 2.5 mU/L, 15 cases of hypothyroxemia, and 19 cases of subclinical hypothyroidism were detected, with detection rates of 17.2%, 2.0%, and 2.6%, respectively. The differences were statistically significant when comparing TSH and TPOAb levels and the proportion of pregnant women with TSH > 2.5 mU/L in the high water iodine and non-high water iodine groups ( Z = 3.04, - 2.17, χ 2 = 6.94, P = 0.002, 0.030, 0.008). The thyroid glands of pregnant women were examined in 720 cases, and 30 cases of goiter and 150 cases of thyroid nodules were detected, with detection rates of 4.2% and 20.8%, respectively. The median thyroid volume was 8.92 ml in the high water iodine group and 8.60 ml in the non-high water iodine group, which were both within the normal range, with no statistically significant difference between the groups ( Z = - 0.75, P = 0.455). Conclusions:After changing to low iodine water, the overall iodine nutrition of pregnant women in Gaoqing County is now at an appropriate level, and the reduction of water iodine effectively reduces the risk of TSH abnormalities in local pregnant women. However, pregnant women in the non-high water iodine group are iodine deficiency, and pregnant women in the high water iodine group are at iodine super-appropriate, and the iodine nutrition level of pregnant women in this area is highly variable, which causes the "illusion" that the overall iodine level of local pregnant women is suitable.
		                        		
		                        		
		                        		
		                        	
8.Iodine nutritional status of children and pregnant women in 37 coastal counties (cities and districts) of Shandong Province in 2021
Wenming ZHANG ; Xu ZHANG ; Wen JIANG ; Na LIANG ; Xiaoming WANG
Chinese Journal of Endemiology 2023;42(6):467-472
		                        		
		                        			
		                        			Objective:To learn about the prevalence of iodized salt in coastal counties (cities and districts, hereinafter referred to as counties) of Shandong Province, evaluate the iodine nutritional status of children and pregnant women, so as to provide data support for timely targeted prevention and control measures and scientific adjustment of intervention strategies.Methods:In 2021, in 37 coastal counties of Shandong Province, one township was selected from each of the five directions of the east, west, south, north, and middle of each county, one primary school was selected from each township, 40 non-boarding students aged 6 - 12 years old were selected from each primary school. In addition, 20 pregnant women were selected from each township. Children's and pregnant women's household salt samples and urine samples were collected to detect salt iodine and urinary iodine contents. The children from 19 counties selected from the 37 coastal counties of Shandong Province were examined by B-mode ultrasound, and the goiter rate of children was calculated.Results:Totally 7 736 household edible salt samples from the children were tested, and the coverage rate of iodized salt, iodized salt qualified rate and qualified iodized salt consumption rate was 74.3% (5 746/7 736), 74.3% (4 267/5 746) and 55.2% (4 267/7 736), respectively. A total of 7 740 urine samples were collected from the children, the median urinary iodine was 174.0 μg/L. A total of 4 344 children were selected for thyroid examination, and the goiter rate was 3.0% (131/4 344). Totally 4 513 household edible salt samples from the pregnant women were tested, and the coverage rate of iodized salt, iodized salt qualified rate and qualified iodized salt consumption rate was 83.7% (3 779/4 513), 77.6% (2 933/3 779) and 65.0% (2 933/4 513), respectively. A total of 7 740 urine samples were collected from the pregnant women, the median urinary iodine was 133.5 μg/L.Conclusions:Children in 37 coastal counties of Shandong Province are generally at the appropriate level of iodine, but pregnant women still have the risk of iodine deficiency. Comprehensive prevention and control measures for iodine deficiency disorders need to be continuously implemented to consolidate and deepen the achievements of iodine deficiency prevention and control.
		                        		
		                        		
		                        		
		                        	
9.Role and clinical significance of MUC4 gene mutations in thrombotic events in patients with classic paroxysmal nocturnal hemoglobinuria
Yingying CHEN ; Hui LIU ; Liyan LI ; Lijuan LI ; Huaquan WANG ; Jia SONG ; Yuhong WU ; Jing GUAN ; Limin XING ; Guojin WANG ; Wen QU ; Hong LIU ; Xiaoming WANG ; Zonghong SHAO ; Rong FU
Chinese Journal of Hematology 2023;44(7):561-566
		                        		
		                        			
		                        			Objective:This study aimed to investigate the role and clinical significance of MUC4 gene mutations in thrombotic events in patients with classic paroxysmal nocturnal hemoglobinuria (PNH) patients.Methods:A retrospective analysis was conducted on the clinical data and gene sequencing results of 45 patients with classic PNH admitted to the Department of Hematology, Tianjin Medical University General Hospital, from June 2018 to February 2022. MUC4 gene mutations in patients with classic PNH were summarized, and the risk factors for thrombotic events in these patients were analyzed. Additionally, the effects of MUC4 gene mutations on the cumulative incidence and survival of thrombotic events in patients with classic PNH were determined.Results:The detection rate of MUC4 gene mutations in patients with classic PNH who experienced thrombotic events (thrombotic group) was 68.8% (11/16), which was significantly higher than that in the non-thrombotic group [10.3% (3/29) ] ( P<0.001). All mutations occurred in exon 2. MUC4 mutation ( OR=20.815, P=0.010) was identified as an independent risk factor for thrombotic events in patients with classic PNH. The cumulative incidence of thrombotic events was 78.6% (11/14) in the MUC4 gene mutation group (mutation group) and 16.1% (5/31) in the non-mutation group, showing a statistically significant difference between the two groups ( P<0.001). Survival analysis showed a lower overall survival (OS) rate in the thrombotic group compared with that in the non-thrombotic group [ (34.4±25.2) % vs. (62.7±19.3) % ] ( P=0.045). The OS rate of patients was (41.7±29.9) % in the mutation group and (59.1±18.3) % in the non-mutation group ( P=0.487) . Conclusion:MUC4 gene mutations are associated with an increased incidence of thrombotic events in classic PNH patients, highlighting their role as independent risk factors for thrombosis in this population. These mutations can be considered a novel predictive factor that aids in evaluating the risk of thrombosis in patients with classic PNH.
		                        		
		                        		
		                        		
		                        	
10.Analysis of clinical characteristics of diagnosis and treatment of 18 patients with Caroli syndrome
Wen SHI ; Xiaoming HUANG ; Fengdan WANG ; Yunlu FENG ; Yang JIAO
Chinese Journal of Digestion 2022;42(1):25-29
		                        		
		                        			
		                        			Objective:To summarize and analyze the clinical, laboratory and imaging characteristics of patients with Caroli syndrome (CS), so as to deepen the understanding of the disease and explore the possible methods for improving early diagnosis.Methods:From January 2008 to June 2021, the clinical data of 18 CS patients who were hospitalized in Peking Union Medical College Hospital and diagnosed by pathology or by clinical and imaging features were collected. The general data, clinical manifestations, laboratory examination (white blood cell count, hemoglobin, etc.), imaging features (ultrasonography, computed tomography (CT), magnetic resonance imaging (MRI)), and diagnosis and treatment of the patients were retrospectively analyzed. Descriptive method was used for statistical analysis.Results:The median age of 18 CS patients was 18 years old (ranged from 1 to 39 years old); there were 10 male and 8 female patients with a male-to-female ratio of 5∶4. The median time from symptom onset to diagnosis was 24 months (ranged from 1 month to 28 years). At the time of diagnosis, 15 patients had already had portal hypertension-related complications, and 8 patients had biliary infections. The common symptoms included abdominal distension (6 cases), fever with or without abdominal pain (5 cases), and loss of appetite (3 cases). The common abnormal laboratory findings included peripheral white blood cell count, hemoglobin level and platelet count below the normal reference value range, alanine aminotransferase and bilirubin level above the normal reference value range, and 4 patients had positive autoantibodies. Four patients were clearly diagnosed according to the pathology of liver biopsy, 14 patients were clearly diagnosed by imaging. Among which the diagnostic rate of abdominal ultrasonography for CS was 4/18, CT was 11/15, and MRI or magnetic resonance cholangiopancreatography was 12/16. The typical features of abdominal ultrasonography were liver cysts with splenomegaly, typical manifestation of CT was intrahepatic bile duct dilatation with " central dot sign", and MRI typically manifested as multiple cystic dilatations of intrahepatic bile ducts. Among the 18 patients with CS, 1 case underwent right hepatectomy, 3 cases were waiting for liver transplantation, and the other 14 patients chose symptomatic treatment due to economic reasons.Conclusions:The clinical manifestations and laboratory findings of CS patients lack specificity, and the diagnosis of most patients is delayed. The lack of understanding of the disease by clinical and imaging doctors may be one of the reasons affecting the early diagnosis of CS patients. The findings of splenomegaly and liver cysts by abdominal ultrasound may provide clues for the diagnosis of CS for clinical and imaging doctors.
		                        		
		                        		
		                        		
		                        	
            
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