Objective: To investigate the characteristics of breakthrough cases of varicella in Haishu District, Ningbo City, Zhejiang Province from 2017 to 2022, so as to provide the evidence for varicella prevention and control. Methods: Information on reported cases of varicella and vaccination in Haishu District from 2017 to 2022 were collected through the China Infectious Disease Reporting Management Information System and the Immunization Program Information Management System of Zhejiang Province. The breakthrough cases who had received 1 or 2 doses of varicella vaccine 42 days before the onset of disease were selected. The distribution of sex, age, region and the interval between onset and last immunization were descriptively analyzed. Results: A total of 1 563 varicella cases were reported from 2017 to 2022. There were 928 breakthrough cases (59.37%), of which 660 cases with 1-dose immunization history (42.23%) and 268 cases with 2-dose immunization history (17.15%). The proportion of 1-dose breakthrough cases in total annual cases showed a decreasing trend (P<0.05) from 2017 to 2022, while there was no significant tendency on the proportion of cases with 2-dose immunization history (P>0.05). There were 392 males (59.39%) and 268 females (40.61%) with 1-dose immunization history. There were 150 males (55.97%) and 118 females (44.03%) with 2-dose immunization history. Breakthrough cases with 1- and 2- dose immunization history had an median age of 13.00 (interquartile range, 4.00) and 9.00 (4.00) years, respectively, and the difference was statistically significant (P<0.05). The regional distribution of the breakthrough cases with 1- and 2- dose immunization history was mainly in the rural-urban fringe, with 319 cases (48.33%) and 137 cases (51.12%), respectively, and the difference was statistically significant (P<0.05). The median interval between onset and last immunization was 12.00 (interquartile range, 3.00) and 4.00 (3.00) years, respectively, and the difference was statistically significant (P<0.05). Conclusions The breakthrough cases of varicella in Haishu District from 2017 to 2022 were mainly cases with 1-dose immunization history, males, and residents in rural-urban fringe. The age was older and the interval between onset and the last immunization was longer in cases with 1-dose immunization history than in cases with 2-dose immunization history.

ObjectiveTo evaluate the clinical effect of Jiechang Buxu pills on chronic relapsing ulcerative colitis （syndrome of spleen-kidney Yang deficiency） and the repair effect on mucosal barrier injury. MethodA total of 108 patients were randomly assigned into observation （54 cases） and control （54 cases） groups. Both groups were treated with mesalazine enteric-coated tablets， 0.5 g/time， 3 times/d. In addition， the observation group received Jiechang Buxu pills， 10 g/time， 3 times/d， and the control group received simulant of Jiechang Buxu pills， 10 g/time， 3 times/d. Both groups were treated for 3 consecutive months. During monthly follow-up， clinical response， clinical remission rate， C-reactive protein （CRP） compliance rate， fecal calprotectin （FC） compliance rate， and endoscopic mucosal healing rate were compared. The early recurrence rate was recorded after 3 months of follow-up. The modified Mayo score， mucosal histological score， colonoscopic mucosal score， inflammatory bowel disease questionnaire （IBDQ） score， and syndrome score of spleen-kidney Yang deficiency were compared before and after treatment. The levels of hypoxia-inducible factor-1α （HIF-1α）， diamine oxidase （DAO）， D-lactic acid （D-LA）， interleukin（IL）-1β， IL-6， IL-17A， IL-22， tumor necrosis factor-α （TNF-α）， T helper 17 （Th17）， and regulatory T cells （Treg）， and Th17/Treg ratio were measured and calculated before and after treatment. ResultThe clinical response rate， clinical remission rate， and CRP compliance rate in the observation group were higher than those in the control group （P<0.05， P<0.01） 1， 2 and 3 months after treatment. The FC compliance rate in the observation group was higher than that in the control group （P<0.05） 2 and 3 months after treatment. After treatment， the observation group had higher endoscopic mucosal healing rate （P<0.01） and lower early recurrence rate （P<0.05） than the control group. In addition， the observation group had lower Mayo score， mucosal histological score， colonoscopic mucosal score， and syndrome score of spleen-kidney Yang deficiency （P<0.01）， higher IBDQ score （P<0.01）， lower levels of HIF-1α， DAO， D-LA， IL-1β， IL-6， IL-17A， TNF-α， Th17， and Th17/Treg and higher levels of IL-22 and Treg （P<0.01） than the control group. ConclusionJiechang Buxu pills combined with mesalazine enteric-coated tablets can improve the clinical response rate， clinical remission rate， CRP compliance rate， FC compliance rate， and endoscopic mucosal healing rate， reduce the early recurrence rate and disease activity， promote histological remission， and improve the quality of life in the patients with chronic relapsing ulcerative colitis （syndrome of spleen-kidney Yang deficiency）. The therapy may exert the therapeutic effect by inhibiting inflammation， inducing intestinal mucosal immune tolerance， and protecting the function of intestinal mucosal barrier.

Objective:To explore the risk factors for the occurrence of thrombocytopenia (TCP) within 2 weeks after pediatric liver transplantation (LT) and examine the relationship between the occurrence of TCP and prognosis.Methods:From January 2021 to November 2021, clinical data were retrospectively reviewed for 162 pediatric LT recipients aged under 4 years at Organ Transplantation Center of Tianjin First Central Hospital.Based upon the lowest value of platelet count at Week 2 post-operation, they were assigned into two groups of TCP (n=90) and non-TCP (n=72). General preoperative profiles, intraoperative findings, postoperative complications, types of commonly used antibiotics, anticoagulant dosing and prognosis of two groups were compared.Univariate and multivariate analyses were utilized for examining the independent risk factors for TCP.Receiver operating characteristic (ROC) curve was plotted for examining the cut-off value of independent risk factors for diagnosing TCP.Results:Among them, 90 (55.56%) developed TCP within 2 weeks post-operation and 25(15.43%) developed TCP at Day 1 post-operation.The median preoperative platelet count was 178×10 9/L and the lowest value was 65×10 9/L at Day 3(1-4) post-operation with a declining rate of 63.5% and platelet count of recipient normalized at Day 6(4-7.25) post-operation.The results of univariate analysis showed statistically significant inter-group differences in operative duration[(574.43±80.53)min vs.(526.75±72.42)min], intraoperative blood loss[400(300, 550)ml vs.320(300, 400)ml], red blood cell transfusion[2(2, 3)U vs.2(1.5, 2.0)U], preoperative platelet count[178.5(141.75, 242.5)×10 9/L vs.257 (209.75, 357)×10 9/L], postoperative infection rate[27.8%(25/90)vs.13.9%(10/72)] and dosing rates of piperacillin sodium and tazobactam sodium[8.9%(8/90)vs.25.0%(18/72)] ( P<0.05). Multivariate Logistic regression analysis revealed statistically significant inter-group differences in operative duration( P=0.008), red blood cell transfusion( P=0.01), preoperative platelet count( P<0.01) and postoperative infection rate ( P=0.02). The results of ROC curve analysis showed that the cut-off values of operative duration, red blood cell transfusion and preoperative platelet count were 535 min, 2.75 U and 183.5×10 9/L respectively.Length of ICU stay was higher in TCP group than that in non-TCP group, and the difference was statistically significant [4(3, 5) vs.3(3, 4) day, P=0.006]. Conclusions:LT children aged under 4 years with intraoperative red blood cell transfusion>2.75 U, operative duration>535 min and preoperative platelet count<183.5×10 9/L are more likely to develop post-transplantation TCP.And occurrence of TCP prolongs the length of ICU stay in pediatric recipients.

Autosomal recessive congenital ichthyosis caused by CERS3 mutations is extremely rare in clinical practice. We recently identified a family of autosomal recessive congenital ichthyosis and performed multigene exome sequencing for hereditary skin diseases to identify causative genes. Mutation analysis revealed compound heterozygous mutations of c.746A>G(from the mother) and exon12 deletion(from the father)in CERS3 were detected in the proband, which were verified by Sanger sequencing and co-segregated with the ichthyosis phenotype in the proband and her parents. These mutations were both reported for the first time. For the treatment, the proband received an oral acitretin capsules of 20 mg once daily. After 3-month follow up, the patient's lesion improved significantly.

Liver failure(LF) is a rapidly progressing critical illness with a high mortality rate in clinical practices.Artificial liver therapy has been a major cure for LF.However, for LF infants and toddlers, therapeutic options for artificial liver have remained limited due to a great lack of weight-matched blood purification supplies.In recent years, our center has applied double plasma molecular adsorption plus plasmapheresis for managing LF children during perioperative period of liver transplantation.Treatment is smooth with an excellent efficacy of removing toxins.Here our institutional experiences were summarzied.

Objective To compare the preparation efficiency of mouse pox and mouse hepatitis antibodies between two substrains of BALB/c and Big-BALB/c (B-BALB/c) mice, and to provide a theoretical basis and reference for the selection of laboratory animals in the preparation of monoclonal antibodies inducedin vivo through hybridoma.Methods Individuals weighing more than 5% of the weight of normal animals at 4 weeks of age (the criterion for late selection is more than 10%) were selected from a population of conventionally bred BALB/c mice and bred individually, and a subline of B-BALB/c mice was prepared after 10 generations of selection. A total of 40 BALB/c mice and 40 B-BALB/c mice aged 10 to 11 weeks, half male and half female, were selected and inoculated with the mousepox monoclonal antibody hybridoma cell line G23 or the murine hepatitis monoclonal antibody hybridoma cell line Y15 pre-treated with liquid paraffin, respectively. Mice ascites containing monoclonal antibodies were obtained by in vivo induction. The antibody titer was tested by indirect ELISA. The mice were grouped based on the sub-strains, gender and inoculation type of hybridoma to analyze the ascites production, antibody titer and antibody production, and to evaluate the antibody preparation efficiency of the two BALB/c mouse sub-strains.ResultsAfter 10 generations of breeding, the body weight of 10-week-old male and female B-BALB/c mice increased by 22.3% and 12.8%, respectively, compared with BALB/c mice of the same age. Compared with BALB/c mice, B-BALB/c mice had better tolerance and adaptation to secondary ascites collection. Compared with BALB/c mice, the ascites production and antibody titer during the preparation of antibodies in B-BALB/c mice were significantly increased, especially in the hybridoma cell line G23 vaccination group (both P＜0.000 1) . After inoculation with the hybridoma cell lines G23 or Y15, the average antibody production of B-BALB/c mice (14.99×10⁴ U and 33.22×10⁴ U) was higher than that of BALB/c mice (5.33×10⁴ U and 19.31×10⁴ U) (both P＜0.01). After inoculation with hybridoma cell line G23, the average antibody production per unit body weight of B-BALB/c mice (0.55×10⁴ U/g) was higher than that of BALB/c mice (0.23×10⁴ U/g) (P＜0.000 1). And the antibody production per unit body weight of female B-BALB/c or BALB/c mice was higher than that of male B-BALB/c or BALB/c mice (bothP＜0.01).Conclusion B-BALB/c mice can be used as an alternative to BALB/c mice in the in vivo induction of monoclonal antibody preparation, which can achieve the purpose of reducing the number of experimental animals used, lowering the labor cost, and improving the efficiency of antibody preparation.

Objective:To investigate the clinical application of continuous renal replacement therapy (CRRT) in infants with acute kidney injury (AKI) after liver transplantation.Methods:A retrospective study was conducted on infants with AKI after liver transplantation in Tianjin First Center Hospital from January 1, 2019 to June 1, 2021. Infants with AKI within 1 year after liver transplantation were divided into CRRT group and non-CRRT group according to whether CRRT was performed. The preoperative and intraoperative condition, the postoperative complications were compared, the risk factors of CRRT for AKI infants, the clinical characteristics of CRRT were analyzed, and the prognosis between CRRT group and non-CRRT group were compared.Results:① A total of 512 cases of pediatric liver transplantation were performed. A total of 189 cases (36.9%) developed AKI within 1 year after surgery, including 18 cases in CRRT group and 171 cases in non-CRRT group. ② There was no significant difference in preoperative conditions between the two groups. The duration of liver transplantation (hours: 8.8±1.5 vs. 7.5±1.3) and intraoperative blood loss [mL: 370 (220-800) vs. 310 (200-400)] in CRRT group were significantly higher than those in non-CRRT group. CRRT group had significantly higher incidence of postoperative complication [unplanned operation: 8 cases (44.4%) vs. 14 cases (8.2%), primary nonfunction: 1 case (5.6%) vs. 0 case (0%), retransplantation: 3 cases (16.7%) vs. 0 case (0%), hepatic artery thrombosis: 3 cases (16.7%) vs. 4 cases (2.3%), intestinal fistula: 2 cases (11.1%) vs. 2 cases (1.2%)] than non-CRRT group (all P < 0.05). ③ The average start time of CRRT was 10 (1-240) days. The per capita frequency of CRRT treatment was 3.3 (1.0-14.0) times. The average duration of each CRRT treatment was 10.1 (6.0-19.3) hours, the average reduction rate of serum creatinine (SCr) was 25.6% (13.5%-45.0%) after CRRT. ④ In CRRT group, 5 patients died, the 1-year and 2-year survival rates were both 72.22%. In non-CRRT group, 6 patients died, the 1-year and 2-year survival rates were 97.1% and 96.5%, respectively. There were significant differences in 1-year and 2-year survival rates between the two groups (both P < 0.01). Conclusions:The incidence of AKI after pediatric liver transplantation was high, and most infants treated with CRRT were associated with serious surgical complications. CRRT was a powerful means to remove inflammatory factors and maintain the stability of circulation and internal environment, which could improve the multi-organ dysfunction effectively.

Objective:To detect causative gene mutations in 1 patient with ADULT syndrome mainly presenting with ectodermal dysplasia.Methods:Clinical data were collected from a proband with ADULT syndrome, and genomic DNA was extracted from peripheral blood samples obtained from the proband and his parents. Exome sequencing was performed in the proband by using targeted panels for hereditary skin diseases to determine mutation sites, and then the candidate mutation sites were verified by Sanger sequencing in the family members.Results:The 22-year-old male patient presented with sparse and thin hair, scattered facial freckles, missing permanent teeth, cloudy corneas, palmoplantar erythema and keratosis, nail/toenail dystrophy, and nipple dysplasia. Genetic testing of the peripheral blood genomic DNA of the proband revealed a heterozygous mutation (c.1040G>T) in exon 8 of the TP63 gene, resulting in an amino acid change at position 347 (p.C347F) . The mutation was not detected in his father or mother with normal phenotypes, suggesting the cosegregation of the gene mutation with the disease phenotype in the family.Conclusion:The de novo heterozygous missense mutation in the TP63 gene may be the causative mutation in the proband, and combined with clinical manifestations, the proband was diagnosed with ADULT syndrome without finger/toe deformities.

Objective To summarize the experience of diagnosis and treatment of Takotsubo syndrome (TTS) after liver transplantation. Methods Clinical data of one TTS patient after liver transplantation was retrospectively analyzed. Clinical features, diagnosis and treatment strategies were summarized, and literature review was conducted. Results A 43-year-old female patient successfully underwent split liver transplantation due to primary biliary cirrhosis for 8 years. At postoperative 3 d, the patient developed anxiety, irritation, dyspnea, disorientation, hypotension, N-terminal pro-brain natriuretic peptide (NT-proBNP) of > 35 000 pg/mL, creatine kinase isoenzyme (CK-MB) of 5.9 U/L and troponin I (TnI) of 1.78 μg/L. Electrocardiogram indicated the signs of sinus rhythm. Echocardiography indicated diffuse weakening of the left ventricular wall motion and spherical dilatation of the apex, accompanied with moderate and severe regurgitation of the mitral valve and tricuspid valve. The left ventricular ejection fraction (LVEF) declined to 23%, whereas no abnormal segmental motion of ventricular wall or corresponding electrocardiogram changes were observed. The possibility of acute coronary syndrome was excluded. The InterTAK diagnostic score was 73. The diagnosis of TTS after liver transplantation was considered. Metoprolol, coenzyme Q₁₀, recombinant human brain natriuretic peptide, deacetyl lanatoside and lorazepam were given. Echocardiography at postoperative 10 d showed that the left ventricular function was significantly improved and the LVEF recovered to 50%. The patient was discharged 40 d after liver transplantation. The liver function was recovered well. During postoperative follow-up, she was given with metoprolol till the submission date, and no recurrence was reported. Conclusions TTS after liver transplantation is rare in clinical practice. It is difficult to make the diagnosis. The condition of TTS is severe and clinical prognosis is poor. Prompt diagnosis and interventions should be implemented.

Humans ; Femoral Artery/surgery* ; Hip Dislocation, Congenital ; Hip Joint ; Treatment Outcome