Objective:To preliminarily investigate the efficacy and safety of endoscopic retrograde cholangiopancreatography (ERCP) for recurrent acute pancreatitis and refractory pancreatic pseudocysts following asparaginase-associated pancreatitis (AAP) in children.Methods:A retrospective analysis was conducted on the clinical data of 19 pediatric patients who underwent ERCP for recurrent aute pancreatitis and refractory pancreatic pseudocysts following AAP at Shanghai Children's Medical Center between November 2018 and July 2024. Data primarily included ERCP procedure outcomes, ERCP-related complications. Pseudocyst size, frequency of pancreatitis episodes, and body mass index (BMI) before and after the ERCP intervention were compared.Results:A total of 30 ERCP procedures were performed in 19 children, with a technical success rate of 96.7% (29/30). Among the 13 children with recurrent acute pancreatitis, 11 remained symptom-free post-ERCP. One clild experienced 3 further episodes of acute mild pancreatitis following asparaginase administration, though the severity and duration of abdominal pain were reduced compared with pre-ERCP episodes. Another child developed 1 episode of aute mild pancreatitis after overeating. Both of them were resolved following medical treatment. Of the 12 children with pancreatic pseudocysts, complete resolution was observed in 8 cases, and significant reduction in size was achieved in 4 cases. All patients showed an increase in BMI postoperatively. A total of 10 procedure-related adverse events occurred, includings 5 post-ERCP pancreatitis (PEP), 5 postoperative infection, 2 hyperamylasemia, and 1 postoperative bleeding. Co-occurrence of PEP and infection was noted in 2 procedures, and PEP with bleeding in 1 procedure. All complications were managed successfully with conservative treatment.Conclusion:ERCP demonstrates favorable efficacy and acceptable safety for managing recurrent acute pancreatitis and refractory pancreatic pseudocysts following AAP in children.

Asparaginase plays a crucial role in the combined chemotherapy treatment for childhood haematological tumours.However，asparaginase-associated pancreatitis（AAP）is a major challenge for the continuation of asparaginase therapy，which seriously affects the chemotherapy process and results in a lower disease-free survival rate in children with haematological tumours.Currently，the pathogenesis of AAP is still unclear，and its treatment in the acute phase mainly includes nutritional support，fluid resuscitation，and drug therapy.There are no domestic and international guidelines for the prevention of AAP，and the reported preventive methods mainly include low-fat diets，octreotide，vitamin A，as well as galactose and pyruvate.Since prevention of AAP and effective treatment of AAP are important to ensure the safety and sequential application，this article reviews the treatment and prevention methods of AAP.

Objective To explore the differences in clinical characteristics of sudden sensorineural hearing loss(SSNHL)between patients with coronavirus disease 2019(COVID-19)and those without-COVID-19.Methods 31 SSNHL patients with COVID-19 who were hospitalized in a department during the COVID-19 epidemic period(from December 2022 to January 2023)were included as the study group,and 12 SSNHL patients without COVID-19 who received treatment during the non-COVID-19 epidemic period(from December 2021 to January 2022)were co-llected as the control group.Two groups of patients received standardized treatment,and their clinical characteristics and prognosis were compared.Clinical characteristics of SSNHL patients with COVID-19 during the COVID-19 epidemic period were analyzed.Results The time interval from patients developed COVID-19 to the onset of SSNHL in the study group was 3-30 days.The time interval from onset to consultation were 3(1,7)days and 5(4,6)days in the study group and the control group,respectively,with no significant difference(P＞0.05).The average age of patients in the study group was(44.16±13.54)years,which was higher than that of the control group(35.23±9.24)years,and the difference was statistically significant(P＜0.05).The hearing at damaged fre-quency of the study group improved by 6.5(0.5,24.5)dB after therapy,which was lower than that of the control group(36.0[27.0,38.0]dB),with statistically significant difference(P＜0.05).There was a negative correlation of the interval of consultation with the average improvement level of hearing at damaged frequency(r=-0.318,P=0.033).The longer the interval,the less the hearing improvement and the worse the therapeutic effect.Corre-lation analysis was further conducted on the time interval from development of COVID-19 to SSNHL onset and the time interval of consultation in patients in the study group,which showed no correlation(r=-0.337,P=0.059).There was no statistically significant difference in the types and degree of SSNHL between two groups of patients(both P＞0.05).After standardized treatment,the rate of ineffective patients in the study group was 56.25％,which was higher than that in the control group(15.38％),and the difference was statistically significant(P＜0.05).Conclusion After adjusting the COVID-19 prevention and control policies,there are more hospitalized COVID-19 patients with SSNHL,with a higher proportion in patients of older age,with poorer efficacy,and inef-fective treatment.The earlier the treatment for SSNHL patients,the better the effect can achieve.COVID-19 may be a potential inducement and/or etiological factor of SSNHL,and further research is needed.

Objective:To investigate the clinical features and risk factors of second asparaginase-associated pancreatitis (AAP) in children with acute lymphoblastic leukemia (ALL).Methods:In this case-control study, the clinical data of ALL children who were diagnosed with pancreatitis and received asparaginase chemotherapy at Shanghai Children′s Medical Center, Shanghai Jiao Tong University School of Medicine from November 2013 to November 2023 were retrospectively analyzed.Multivariate Logistic regression was used to determine independent risk factors for the occurrence of second AAP.Results:A total of 78 children with AAP were included, of whom 32 were re-exposed to asparaginase.Ten of the 32 cases (31.25%) suffered second AAP, and they were rated at intermediate risk, with an age of (8.26±0.87) years.The number of asparaginase re-exposures before second AAP was (2.10±0.99) times.Second AAP developed during the intensification period in 2 cases and during the re-induction period in 8 cases.There were 3 cases (30.00%) of Grade 1 pancreatitis and 7 cases (70.00%) of Grade 2 pancreatitis.No patient died of second AAP.Two patients (20.00%) experienced AAP-related long-term complications, including chronic pancreatitis and insulin-dependent diabetes mellitus.After 2 children with no clinical data related to the first AAP were excluded, 30 re-exposed children were divided into a control group (22 cases) and a case group (8 cases) according to the presence or absence of second AAP.The univariate analysis showed that there were significant differences in activated partial thromboplastin time (APTT)[(29.80±5.25) s vs.(38.47±1.42) s, t=-0.45, P=0.023] and total bilirubin (TBIL)[18.40 (11.00, 25.53) μmol/L vs.24.30 (19.93, 46.48) μmol/L, Z=-0.32, P=0.020] between the 2 groups.The further multivariate Logistic regression analysis showed that after adjusting for age and immunophenotype, TBIL ( HR=1.115, P=0.048) was an independent risk factor for second AAP.Comparisons of the prognosis revealed that there were no significant differences in long-term complications ( P=0.168) and final survival ( P=0.716) between children with only once AAP (68 cases) and those with second AAP (10 cases). Conclusions:Second AAP mostly occurs after the second dose of asparaginase, and the re-induction period is the peak period of incidence of second AAP.Hyperbilirubinemia may be a risk factor of second AAP.Compared with once AAP, second AAP does not increase the mortality and the incidence of AAP-related long-term complications.

Objective To explore the differences in clinical characteristics of sudden sensorineural hearing loss(SSNHL)between patients with coronavirus disease 2019(COVID-19)and those without-COVID-19.Methods 31 SSNHL patients with COVID-19 who were hospitalized in a department during the COVID-19 epidemic period(from December 2022 to January 2023)were included as the study group,and 12 SSNHL patients without COVID-19 who received treatment during the non-COVID-19 epidemic period(from December 2021 to January 2022)were co-llected as the control group.Two groups of patients received standardized treatment,and their clinical characteristics and prognosis were compared.Clinical characteristics of SSNHL patients with COVID-19 during the COVID-19 epidemic period were analyzed.Results The time interval from patients developed COVID-19 to the onset of SSNHL in the study group was 3-30 days.The time interval from onset to consultation were 3(1,7)days and 5(4,6)days in the study group and the control group,respectively,with no significant difference(P＞0.05).The average age of patients in the study group was(44.16±13.54)years,which was higher than that of the control group(35.23±9.24)years,and the difference was statistically significant(P＜0.05).The hearing at damaged fre-quency of the study group improved by 6.5(0.5,24.5)dB after therapy,which was lower than that of the control group(36.0[27.0,38.0]dB),with statistically significant difference(P＜0.05).There was a negative correlation of the interval of consultation with the average improvement level of hearing at damaged frequency(r=-0.318,P=0.033).The longer the interval,the less the hearing improvement and the worse the therapeutic effect.Corre-lation analysis was further conducted on the time interval from development of COVID-19 to SSNHL onset and the time interval of consultation in patients in the study group,which showed no correlation(r=-0.337,P=0.059).There was no statistically significant difference in the types and degree of SSNHL between two groups of patients(both P＞0.05).After standardized treatment,the rate of ineffective patients in the study group was 56.25％,which was higher than that in the control group(15.38％),and the difference was statistically significant(P＜0.05).Conclusion After adjusting the COVID-19 prevention and control policies,there are more hospitalized COVID-19 patients with SSNHL,with a higher proportion in patients of older age,with poorer efficacy,and inef-fective treatment.The earlier the treatment for SSNHL patients,the better the effect can achieve.COVID-19 may be a potential inducement and/or etiological factor of SSNHL,and further research is needed.

Objective:To investigate the clinical features and risk factors of second asparaginase-associated pancreatitis (AAP) in children with acute lymphoblastic leukemia (ALL).Methods:In this case-control study, the clinical data of ALL children who were diagnosed with pancreatitis and received asparaginase chemotherapy at Shanghai Children′s Medical Center, Shanghai Jiao Tong University School of Medicine from November 2013 to November 2023 were retrospectively analyzed.Multivariate Logistic regression was used to determine independent risk factors for the occurrence of second AAP.Results:A total of 78 children with AAP were included, of whom 32 were re-exposed to asparaginase.Ten of the 32 cases (31.25%) suffered second AAP, and they were rated at intermediate risk, with an age of (8.26±0.87) years.The number of asparaginase re-exposures before second AAP was (2.10±0.99) times.Second AAP developed during the intensification period in 2 cases and during the re-induction period in 8 cases.There were 3 cases (30.00%) of Grade 1 pancreatitis and 7 cases (70.00%) of Grade 2 pancreatitis.No patient died of second AAP.Two patients (20.00%) experienced AAP-related long-term complications, including chronic pancreatitis and insulin-dependent diabetes mellitus.After 2 children with no clinical data related to the first AAP were excluded, 30 re-exposed children were divided into a control group (22 cases) and a case group (8 cases) according to the presence or absence of second AAP.The univariate analysis showed that there were significant differences in activated partial thromboplastin time (APTT)[(29.80±5.25) s vs.(38.47±1.42) s, t=-0.45, P=0.023] and total bilirubin (TBIL)[18.40 (11.00, 25.53) μmol/L vs.24.30 (19.93, 46.48) μmol/L, Z=-0.32, P=0.020] between the 2 groups.The further multivariate Logistic regression analysis showed that after adjusting for age and immunophenotype, TBIL ( HR=1.115, P=0.048) was an independent risk factor for second AAP.Comparisons of the prognosis revealed that there were no significant differences in long-term complications ( P=0.168) and final survival ( P=0.716) between children with only once AAP (68 cases) and those with second AAP (10 cases). Conclusions:Second AAP mostly occurs after the second dose of asparaginase, and the re-induction period is the peak period of incidence of second AAP.Hyperbilirubinemia may be a risk factor of second AAP.Compared with once AAP, second AAP does not increase the mortality and the incidence of AAP-related long-term complications.

Objective:To preliminarily investigate the efficacy and safety of endoscopic retrograde cholangiopancreatography (ERCP) for recurrent acute pancreatitis and refractory pancreatic pseudocysts following asparaginase-associated pancreatitis (AAP) in children.Methods:A retrospective analysis was conducted on the clinical data of 19 pediatric patients who underwent ERCP for recurrent aute pancreatitis and refractory pancreatic pseudocysts following AAP at Shanghai Children's Medical Center between November 2018 and July 2024. Data primarily included ERCP procedure outcomes, ERCP-related complications. Pseudocyst size, frequency of pancreatitis episodes, and body mass index (BMI) before and after the ERCP intervention were compared.Results:A total of 30 ERCP procedures were performed in 19 children, with a technical success rate of 96.7% (29/30). Among the 13 children with recurrent acute pancreatitis, 11 remained symptom-free post-ERCP. One clild experienced 3 further episodes of acute mild pancreatitis following asparaginase administration, though the severity and duration of abdominal pain were reduced compared with pre-ERCP episodes. Another child developed 1 episode of aute mild pancreatitis after overeating. Both of them were resolved following medical treatment. Of the 12 children with pancreatic pseudocysts, complete resolution was observed in 8 cases, and significant reduction in size was achieved in 4 cases. All patients showed an increase in BMI postoperatively. A total of 10 procedure-related adverse events occurred, includings 5 post-ERCP pancreatitis (PEP), 5 postoperative infection, 2 hyperamylasemia, and 1 postoperative bleeding. Co-occurrence of PEP and infection was noted in 2 procedures, and PEP with bleeding in 1 procedure. All complications were managed successfully with conservative treatment.Conclusion:ERCP demonstrates favorable efficacy and acceptable safety for managing recurrent acute pancreatitis and refractory pancreatic pseudocysts following AAP in children.

Objective:To assess the occurrence, prognosis and possible early risk factors of jaundice in polytrauma patients.Methods:This study was a single-center, prospective study. Polytrauma patients (age>18 years) admitted to Tongji Trauma Center from October 2020 to January 2023 were enrolled. The patients with liver, biliary tract or pancreatic traumatic injury, previously suffered from chronic liver disease were excluded. The clinical characteristics of patients, laboratory test results, imaging examination results, Injury Severity Score (ISS), Glasgow Coma Score and APACHEⅡ score were collected. The incidence of jaundice, the classification of jaundice or the severity of jaundice after multiple injuries, the mortality rate of polytrauma patients with jaundice, and the early independent risk factors of jaundice in polytrauma were analyzed. The differences between the groups were compared by Student’s t test or χ2 test. The independent risk factors of jaundice were analyzed by Logistic regression analyzed. Results:A total of 742 polytrauma patients were included, 34.09% polytrauma patients were accompanied by jaundice, and the ratio of both moderate and severe jaundice were as high as 32.41%. The main type of jaundice was intrahepatic cholestatic jaundice (47.03%). The mortality rate of polytrauma patients accompanied by jaundice was significantly higher than that of polytrauma patients without jaundice (12.25% vs. 3.47%, P<0.001). Logistic regression analysis showed that ISS score ( OR=3.405, 95% CI: 1.962-7.438, P=0.026), plasma lactate ( OR=2.216, 95% CI: 1.203-4.862, P=0.017), interleukin-6 levels ( OR=2.431, 95% CI: 1.424-3.793, P=0.007), the overall duration of parenteral nutrition ( OR=3.011, 95% CI: 1.624-5.041, P=0.022), and the total duration of mechanical ventilation ( OR=3.572, 95% CI: 1.497-4.601, P=0.031) were the early independent risk factors for jaundice in patients after polytrauma. Conclusions:Polytrauma patients are prone to developing jaundice after injury, which is more harmful, especially for intrahepatic cholestatic jaundice after injury. Early identification and early intervention of risk factors associated with jaundice after injury should be strengthened.

Objective To investigate the molecular typing,virulence,and drug resistance features of bacterial strains in a simultane-ous outbreak of paratyphoid fever A and B,and then provide evidence for the prevention and treatment of the simultaneous transmission of different types of paratyphoid fever.Methods The clinical data of 31 patients confirmed as paratyphoid fever in the Hospital of Xin-jiang Production and Construction Corps from September 2018 to November 2018 were retrospectively analyzed.The isolated strains were performed serotyping and drug sensitivity tests.The molecular typing and the detection of virulence and drug resistance genes were carried out by multiplex PCR,pulsed-field gel electrophoresis(PFGE),and multilocus sequence typing(MLST).Results A total of 32 strains of Salmonella paratyphi were isolated from 31 patients,with 19 strains classified as type A and 13 as type B.The intermedi-ate rates of all strains against ciprofloxacin were 100%.The molecular typing and serotyping results of 11 representative strains were consistent.The PFGE fingerprints of Salmonella paratyphi A and B were also consistent.The MLST of Salmonella paratyphi A was ST85,and that of Salmonella paratyphi B was ST86.All strains carried virulence island SPI1-SPI5 representative genes such as invA,sitC,sseL,sifA,mgtC,siiE,and sopB,and regulatory gene phoP.Salmonella paratyphi A also carried cytolethal distending toxin(CDT)genes with trimeric structure such as cdtB,pltA,and pltB.The virulence plasmid genes such as pefA,prot6E,and spvB were all negative.Conclusion The simultaneous transmission of Salmonella paratyphi A and B has the characteristics of high pathogenicity and poor sensitivity to ciprofloxacin,which should be highly concerned by clinical and laboratory personnel.

Objective:To analyze the clinical characteristics of three patients with Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome.Methods:Three patients with IPEX syndrome diagnosed at the Children′s Hospital of Fudan University from January 24, 2013 to July 29, 2019 were selected as the study subjects. Their clinical features, laboratory investigations and results of genetic testing were summarized. Treatment and prognosis were also explored.Results:All of the three children had developed the disorder during infancy. One child had initial features including diabetes and diabetic ketoacidosis, whilst the other two had initiated by diarrhea. All patients had gastrointestinal involvement, and one was diagnosed as very early onset inflammatory bowel disease by colonoscopy and biopsy. Two children also had endocrine glands involvement. One child had manifested type 1 diabetes and positivity for thyroglobulin and thyroid peroxidase antibodies, though his thyroid function had remained normal. Another one had hypothyroidism and was treated by levothyroxine. Genetic testing revealed that all children had harbored missense variants of the FOXP3 gene, including c. 1222G>A (p.V408M), c. 767T>C (p.M256T) and c. 1021A>G (p.T341A). The clinical symptoms of one patient were alleviated following allogeneic hematopoietic stem cell transplantation. One patient was stable after treatment with infliximab plus insulin, and one child had died of refractory septic shock and multiple organ dysfunction syndrome at 3 months old. Conclusion:FOXP3 gene variant-associated IPEX syndrome may have very early onset and diverse clinical manifestations. For male patients with infantile onset chronic diarrhea, multiple endocrine or multiple system involvement, genetic testing is recommended, which may facilitate early diagnosis, treatment and genetic counseling.