Objective:To investigate the clinical phenotype and prognosis among different genotypes of progressive familial intrahepatic cholestasis(PFIC) by cases analysis.Methods:The PFIC cases diagnosed at Beijing Children′s Hospital from 2015 to 2022 were collected, and the clinical phenotypic characteristics, treatment and prognosis were compared and analyzed.Results:A total of 628 cases of cholestatic liver disease were diagnosed, and 26 cases of PFIC were found, accounting for 4.1%.The number of PFIC 2 were the most, 14(53.8%)cases; three(11.5%) cases were PFIC 1; five(19.2%)cases were PFIC 3; while two(7.7%) cases were PFIC 4 and PFIC 6, respectively, and there was no case of PFIC 5.Type 1, 2, 4, and 6 had early onset ages(2 days to 21 months), while type 3 had a wide range of onset ages(8 to 145 months). The symptoms included jaundice(96.2%), pruritus(42.3%), and mucosal bleeding(15.4%). All three cases of type 1 had extrahepatic manifestations of diarrhea and malnutrition.Two cases of type 3 were found to have end-stage liver disease.Cases of PFIC 3 had increased serum γ-glutamyltransferase(97.2-439.5 U/L), while those of other types were normal.The bile acids were all increased(10.1-599.6 μmol/L). Abdominal ultrasound mainly showed liver enlargement(80.8%)and enhanced echogenicity of liver parenchyma(73.1%), enlargement of the spleen(61.5%). Ultrasound liver elastography ranged from 6.3 kPa to 23.1 kPa, there were 21(80.8%) cases ≥9 kPa.Among 26 cases, one case was lost to follow-up, and 11 cases were effective by oral medication alone.Fourteen children were still suffering from relapse or progress after drug treatment: four cases received liver transplantation (three cases had a good prognosis and one case died), two cases received biliary drainage, six cases were still taking drugs orally, and two cases died without active intervention in disease progress.Conclusion:Type 2 is the most common type in PFIC.The onset of most cases is in infancy.Jaundice, pruritus and hepatosplenomegaly are common clinical manifestations, and extrahepatic manifestations can be seen in type 1 cases.Type 3 cases can start with end-stage liver disease.Bile acid of all cases are increased.Except for type 3, the serum γ-glutamyltransferase of cases are normal.Oral medication has certain effects on some cases, but more than half progress, and some need biliary diversion or liver transplantation.

Objective:To evaluate the clinical efficacy and safety of Infliximab (IFX) in pediatric Crohn′s disease (CD).Methods:The efficacy of IFX therapy in 30 patients suffering from active CD who were not completely improved with traditional medicine and enteral nutrition or had intolerance to the medicine in Beijing Children′s Hospital Affiliated to Capital Medical University from December 2014 to December 2019 were retrospectively analyzed.Pediatric Crohn′s Disease Activity Index (PCDAI), blood biochemistry indices, mucosal healing, nutritional status, and adverse reactions were compared and evaluated.Results:Thirty active CD cases, with 18 males and 12 females, were enrolled, and the average age was (8.63±4.76) years old.Three cases who didn′t complete 3 times of IFX injection and 1 case who lost to be followed up were excluded.A total of 26 cases of CD in active period were enrolled in this study on efficacy.The clinical remission and response rate of 26 cases were 61.5% and 84.6%, respectively, at 14-week of IFX therapy.The clinical remission and response rate of 21 cases were 71.4% and 85.7%, respectively, at 30-week.The clinical remission and response rate of 15 cases were 86.7% and 93.3%, respectively, at 54-week.At week 14 th, PCDAI score [(9.56±8.05) scores vs.(29.02±10.86) scores] decreased compared with before treatment ( t=7.339, P<0.05). The levels of erythrocyte sedimentation rate [(15.54±10.26) mm/1 h vs.(33.77±21.30) mm/1 h] and C-reactive protein [(4.79±12.94 ) mg/L vs.(16.33±23.43) mg/L] were obviously decreased, and the hemoglobin [(126.27±16.51) g/L vs.(110.58±16.45) g/L], hematocrit [(37.03±3.95)% vs.(33.52±4.32)%], and albumin levels [(42.30±3.03) g/L vs.(37.13±5.68) g/L] were remarkably increased compared with those before treatment ( t=3.932, 1.993, -3.398, -3.060, -4.009, all P<0.05). Height for age Z score and body mass index Z score were increased after IFX treatment, without statistically significant differences (all P>0.05). Conclusions:IFX therapy had good clinical efficacy in controlling inflammatorys and inducing clinical remission in pediatric CD.

Objective:To summarize and analyze the clinical features of food protein-induced enterocolitis syndrome (FPIES).Methods:The medical history and follow-up data of 5 children with FPIES diagnosed in Department of Gastroenterology, Beijing Children′s Hospital of Capital Medical University from July 2018 to September 2019 were collected, and their clinical characteristics were summarized and analyzed.Results:Five children with FPIES were all infants, including 3 females and 2 males.Before the onset of the disease, the cases visited multiple departments and the average number of visits before diagnosis was 3.There were 4 cases of milk protein allergy and 1 case of egg white allergy.The patients had acute vomiting [5 cases (100%)], diarrhea [4 cases (80%)], early shock symptoms [5 cases (100%)], transient fever [2 cases (40%)]. Hematogenous leukocytes were increased in 3 cases (60%), C-reactive protein was increased in 1 case (20%), faecal leukocytes(+ )[2 cases (40%)], occult blood (+ ) [1 case (20%)]. Four cases were tested for food allergen specific IgE, of which 2 cases (40%) were positive for milk protein.After avoiding allergens, 3 patients (60%) needed intravenous rehydration treatment and 2 cases (40%) received oral rehydration treatment.The above 5 cases recovered quickly.Three patients (60%) used antibiotics.Four cases (80%) of the first-degree relatives of FPIES had a clear history of allergy.Families of children with FPIES had low awareness of the disease before the diagnosis was made, and the allergens were strictly avoided according to the doctor′s instructions after the diagnosis was made.Similar allergic reactions did not occur again, and complementary foods were gradua-lly added under the guidance of the doctor.Two patients had multiple food allergies.The body weight and length of 2 children with growth retardation were catching up with each other.Conclusions:FPIES is a serious food allergy related gastrointestinal disease which is easy to be misdiagnosed clinically.The diagnosis requires a combination of the family and personal allergy history, diet records, the characteristic performance of disease onset, the effect of diet avoi-dance and the necessary differential diagnosis.The long-term management and monitoring after diagnosis is also very important.

Objective:To investigate endoscopic and clinical characteristics of intestinal Behcet′s disease (BD) in children.Methods:General information, clinical manifestations, laboratory tests, imaging examinations and endoscopic characteristics of 14 children with intestinal BD treated in the Department of Gastroenterology, Beijing Children′s Hospital Affiliated to Capital Medical University from January 2016 to March 2020 were retrospectively analyzed.Results:Fourteen children with intestinal BD were recruited, involving 5 males and 9 females with the age of 1 month to 12 years and 11 months [(6.68±3.73) years old], and a median disease course of 6 months.All of them had gastrointestinal symptoms, including 11 cases with abdominal pain, 7 cases with diarrhea and 2 cases with vomiting.Complications of children with intestinal BD included gastrointestinal bleeding, intestinal perforation and stenosis.Extragastrointestinal symptoms included recurrent oral aphthosis in all the 14 cases, fever in 11 cases, skin lesions in 9 cases, vascular manifestations in 8 cases, positive pathergy test in 6 cases, joint manifestations in 5 cases, neurological manifestations in 1 case, and malnutrition in 6 cases.Observed by the endoscopy, lesions were mainly distributed in the distal ileum [61.5%(8/13 cases)]and ileocecal part[53.8%(7/13 cases)], with the main manifestation of ulcer.There were 3 cases with single ulcer and 10 cases with multiple ulcers, including 7 cases with deep and large ulcers.Pathological examinations of endoscopic lesions showed that the main features were mucosa chronic nonspecific inflammation, mucosa chronic active inflammation with ulcer and vasculitis.Conclusions:Clinical manifestations of intestinal BD vary a lot and are non-specific.Some children with intestinal BD may develop severe complications.Endoscopic lesions of intestinal BD have certain characteristics, which contribute to the diagnosis.Gastroenterologists need to be fully aware of intestinal BD.

Background: Despite strengthened efforts on human immunodeficiency virus (HIV) prevention and control, new HIV infections continue to increase among men who have sex with men (MSM) in China. Pre-exposure prophylaxis (PrEP), a highly effective HIV-prevention tool, has recently been included in China's Action Plan of HIV Prevention and Control. To promote future PrEP implementation, this study aims to identify the barriers and facilitators in PrEP use intention among MSM in China.Methods: In 2018, a cross-sectional survey was conducted among 300 MSM in Nanjing, Jiangsu Province. Questions on demographics, sexual behavior (including a seven-item high-risk behavior index), PrEP use intention, PrEP-related awareness and accessibility, and a seven-item public HIV stigma scale were included in the questionnaire. Bivariate and multivariate logistic regression analyses were conducted to identify factors associated with PrEP use intention. Results: Overall, 44.67％ of the participants had more than two male sexual partners and 5.56％ had HIV-positive sexual partners. Only 57.00％ had heard of PrEP and only four (1.33％) participants had used PrEP. However, 75.34％ expressed the willingness to use oral PrEP if its efficacy was assured. The beliefs that "PrEP can prevent HIV if taken as prescribed" (adjusted odds ratio (AOR) = 4.84, P < 0.001) and "PrEP can be scaled up in the community" (AOR = 3.24, P < 0.001) were positively associated with oral PrEP use intention. Concerns of side effects were negatively associated with oral PrEP use intention (AOR = 0.32, P = 0.006). Further, 77.00％ of the participants would choose injectable or implanted PrEP instead of oral PrEP. One of the PrEP stigma items, "Not certain if doctors can prescribe PrEP if I go to the clinic" was positively associated with the intention to use injectable or implanted PrEP (AOR = 3.03). The items "Heard of PrEP" (AOR = 2.74) and "PrEP can prevent HIV if taken as prescribed" (AOR = 2.65) were also positively related to the intention to use injectable or implanted PrEP. The most common concerns regarding PrEP use were efficacy (44.67％) and side effects (38.67％). The most common reasons for using injectable and implanted PrEP were adherence (81.94％ and 77.86％, respectively) and privacy concerns (56.48％ and 55.00％, respectively). Affordable price and coverage by health insurance were the driving factors for PrEP use. Conclusion: Given the low level of awareness of PrEP-related information in China, it's necessary to provide interventions for high-risk individuals and communities, in order to increase their awareness and knowledge of PrEP. Furthermore, additional alternatives to HIV prevention, such as long-acting injectable or implanted PrEP, should be investigated to reduce the risk of HIV infection in at-risk MSM. Even if this programme could be approved by the National Medical Products Administration of China and implemented, a supportive social environment for MSM is essential during its implementing.

Objective: To investigate the antibiotic resistance of Helicobacter pylori(Hp) isolates cultured from endoscopic gastric mucosal samples and influencing factors for antibiotic resistance in children. Methods: From April 2013 to February 2016, Hp cultured from mucosa samples of the gastric antrum and the body of stomach was investigated in 246 patients with ¹³C breath test positive examined by gastroscopy.Resistance to Amoxicillin, Clarithromycin, Metronidazole, Tetracycline was tested for Hp by using E-test.The clinical data were collected from the patients, and the relationship among age, gender, endoscopic diagnosis, histological performance, eradication number factors and antibiotic resistance rate were analyzed. Results: Of 246 specimens, 174 cases (70.7%) were positive.The overall antibiotic resistance rates of isolates obtained were 96.55% (168/174 cases), 57.47% (100/174 cases), 4.02% (7/174 cases), 1.15%(2/174 cases) with respect to Clarithromycin, Metronidazole, Tetracycline and Amoxicillin.An antibiotic resistance rate was 43.10% (75/174 strains), among which the Clarithromycin resistance rate was 93.33% (70/75 strains), and the Metronidazole resistance rate was 6.67% (5/75 strains). Double resistance rate was of 54.02%(94/174 strains), among which the resistances rate of Clarithromycin+ Metronidazole was 52.30%(91/94 strains), and the resistances rate of Clarithromycin+ Tetracycline was 1.72%(3/94 strains). Triple resistance rate of Clarithromycin+ Metronidazole+ Tetracycline was 1.15%(2/174 strains). Quadruple resistance rate of Clarithromycin+ Metronidazole+ Amoxicillin+ Tetracycline was 1.15%(2/174 strains). Factor analysis showed that the resistance rate of Clarithromycin in children who had failed in eradication therapy [98.7%(148/150 cases)] was higher than that in children who had not undergone eradication therapy [83.3%(20/24 cases)], and the difference was statistically significant(χ²=14.610, P<0.05). There was no significant difference in the relationship between Hp resistance to Metronidazole and Clarithromycin and the age, gender, endoscopic diagnosis and histological manifestations(all P>0.05). Conclusions Hp resistance rate to Clarithromycin, Metronidazole is very high in children, but it is relatively low to Amoxicillin and Tetracycline, and multiple antibiotic resistance is at high prevalence.

Eosinophilic gastroenteritis is a relatively rare chronic gastrointestinal inflammatory disease in children,characterized by gastrointestinal eosinophil infiltration.Clinical manifestations due to infiltration of different sites and levels were diverse,endoscopic performance is often nonspecific,the diagnosis of eosinophilic gastroenteritis is mainly based on gastrointestinal symptoms,a large number of eosinophil histological infiltration,and exclude other diseases that cause eosinophil infiltration in gastrointestinal tissue.Treatments include diet therapy,glucocorticoids,anti-allergy drugs,immunomodulatory therapies.Although diet therapy and glucocorticoid treatment improve clinical symptoms in most children,long-term maintenance therapy and follow-up are often required to prevent recurrence.In children with serious complications,such as perforation and obstruction,may require surgery and other treatments.

Objective To evaluate CYP2C19 genotypes distribution in children with Helicobacter pylori (Hp) infection and to know its correlation with gender,age,severity and frequency of clinical symptoms,pathological classification of gastric antral mucosa.Methods Antral mucosas of 214 Hp infection patients who were hospitalized at Beijing Children's Hospital of Capital Medical University from July 2013 to December 2015 were collected.Genotypes were determined by PCR-sequence specific primer method,which were classified as homozygous extensive metabolizer (HomEM),heterozygous extensive metabolizer (HetEM) and poor metabolizer (PM).The differences in CYP2C19 genotype distribution in gender,age,severity and frequency of clinical symptoms,pathological classification of gastric antral mucosa were analyzed.Results (1) Among 214 Hp infected children,the percentage of HomEM was 48.1％ (103/214 cases),HetEM was 46.3％ (99/214 cases),and PM was 5.6％ (12/214 cases).PM in the CYP2C19 infection patients was lower than that in the normal Han nationality in China,and the difference was statistically significant (P ＜ 0.05).(2)Among the 214 Hp infected patients,there were 124 males and 90 females,and their median age was 9 years and 9 months(ranged from 2 years and 8 months to 17 years and 11 months).There was no statistical difference in gender and age of CYP2C19 genotypes(all P ＞ 0.05).(3) There was no statistical difference in severity and frequency of clinical symptoms of CYP2C19 genotypes(all P ＞0.05).(4) In HomEM group,according to pathological classification of gastric mucosa,there were 14 cases of mild injury,36 cases of moderate injury and 53 cases of severe injury,respectively.In HetEM group,there were 17 cases of mild injury,29 cases of moderate injury and 53 cases of severe injury,respectively.In PM group,there were 2 cases of mild injury,3 cases of moderate injury and 7 cases of severe injury,respectively.There was no statistical difference in the pathological degree of inflammation changes in gastric antral mucosa of CYP2C19 genotypes(all P ＞0.05).(5)Thirty-five cases didn't receive treatment,78 cases received Hp eradication one time and failed,101 cases received no less than 2 times of unsuccessful Hp eradication.The number of Hp unsuccessful eradications were positively correlated with the degree of pathological inflammation changes (r =0.219,P ＜ 0.01).There was obvious difference between the number of Hp unsuccessful eradication and the pathological degree of inflammation changes in gastric antral mucosa (x2 =12.414,P ＜ 0.05).Conclusions There was no statistical difference in CYP2C19 genotypes distribution as for different gender,age,severity and frequency of clinical symptoms,pathological classification of gastric antral mucosa and CYP2C19 genotypes.The number of Hp unsuccessful eradication was positively correlated with the degree of pathological inflammation changes.

Aim To investigate whether nicorandil (Nic)protects H9c2 cardiac cells against high glucose (HG)-induced injury and inflammation by inhibiting nuclear factor-κB (NF-κB )/cyclooxygenase-2 (COX-2 )pathway.Methods Cell viability was measured by cell counter kit-8 (CCK-8)assay.The expression lev-els of NF-κB,COX-2 and cleaved caspase-3 were de-termined by Western blot.The activity of lactate dehy-drogenase (LDH)in the culture medium was measured with commercial kits.The intracellular level of reactive oxygen species (ROS)was detected by 2′,7′-dichlor-fluorescein-diacetate (DCFH-DA)staining followed by photofluorography.The number of apoptotic cells was observed by Hoechst 33258 nuclear staining followed by photofluorography.Mitochondrial membrane poten-tial (MMP)was examined by rhodamine 123 staining followed by photofluorography.The secretion levels of interleukin-1β(IL-1β) and tumor necrosis factor-α(TNF-α) were detected by ELISA.Results After H9 c2 cardiac cells were treated with 35 mmol · L-1 glucose (high glucose,HG)for 24 h,the cell viability was significantly decreased .Pre-treatment of the cells with 20~100 μmol·L-1 Nic for 60 min or 50 μmol· L-1 Nic for 30~120 min before exposure to HG signif-icantly attenuated the decrease in viability induced by HG.On the other hand,HG increased the expression levels of phosphorated (p)-NF-κB p65 and cyclooxy-genase-2 (COX-2 )in H9c2 cardiac cells.Pre-treat-ment of the cells with 50 μmol·L-1 Nic for 60 min at-tenuated the up-regulation of p-NF-κB p65 and COX-2 expression levels induced by HG.Furthermore,HG induced considerable injuries and inflammatory re-sponse,leading to increases in LDH activity,ROS generation,MMP loss,the number of apoptotic cells, the expression of cleaved caspase-3 as well as the se-cretion levels of IL-1βand TNF-α.Pre-treatment of the cells with 50 μmol·L-1 Nic for 60 min before HG exposure,or co-treatment of the cells with 100 μmol· L-1 PDTC (an inhibitor of NF-κB)or 10 μmol·L-1 NS-398 (an inhibitor of COX-2)and HG for 24 h ob-viously reduced the above injuries and inflammatory re-sponse induced by HG. Conclusion Nic protects H9 c2 cardiac cells against HG-induced injury and in-flammation by inhibiting NF-κB/COX-2 pathway.

Objective To investigate inhibitory effect of Telmisartan on carotid arterial intima-media thickness in patients with essential hypertension. Methods 104 patients with mild or mid essential hypertension were randomly divided into three groups, which include telmisartan group( telmisartan 80 mg/d PO), ramipril group( ramipril 5 mg/d PO), and control group( other anti-hypertension agents). Blood pressure was monitored during treatment. Carotid arterial intima-media thickness(CIMT) were measured in all patients at beginning and patients who had the 12 months course. Results There were 91 patients who had the 12 months course,which include telmisartan group33 cases,ramipril group28 cases, and control 30 cases. The CIMT was significantly decreased in telmisartan group and ramipril group ( all P < 0. 05 ), and not changed in control while blood pressure fall effectively. In ramipril, group5 cases were ceased the course because of severe cough. Conclusion Both telmisartan and ramipril could decrease blood pressure and CIMT effectively, and there were less side effects in telmisartan group.