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Objective:To investigate the efficacy and safety of targeted therapy with Crizotinib for children with ALK gene mutation positive inflammatory myofibroblastic tumor (IMT). Methods:A retrospective analysis was performed on 4 children with ALK gene mutation positive IMT admitted to Shanghai Children′s Hospital from January 2019 to June 2021.Among them, 3 cases were given the targeted drug Crizotinib[280 mg/(m 2· time), q12h] orally, and 1 case was observed after complete tumor resection to analyze the efficacy and adverse drug reactions. Results:All 4 cases were male, aged from 2 years and 3 months to 11 years and 3 months.The tumors originated from the abdominal cavity in 2 cases, the right orbit in 1 case, and the right lung in 1 case.Pathological immunohistochemistry and fluorescence in situ hybridization were both positive for ALK gene mutation, and complete remission was achieved after comprehensive treatment.Among them, 3 patients were treated with oral Crizotinib, and 2 patients were tried to stop taking the drug for 1 year, relapsed 1 month later, and still achieved complete remission after the second treatment.The 4 cases were followed up for 8-30 months, and all survived.All the cases showed no abnormalities in blood image, liver and kidney function, myocardial enzyme profile, cardiac function, hearing and vision, and 2 cases showed prolonged Q-T interval in the course of Crizotinib treatment, which could be recovered by temporary withdrawal of drug, and no abnormality in electrocardiogram was found in continued drug use. Conclusions:Crizotinib was used to treat ALK mutation positive IMT, shrink tumor and consolidate postoperative treatment, which is a good choice for IMT in children with difficult surgical resection and refractory recurrence.
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Acute appendicitis is one of the most common acute abdominal diseases in pediatric surgery.Appendectomy is the standard of care for the treatment of acute appendicitis in children and its curative effect is definite.With the improvement of endoscopic techniques, laparoscopic appendectomy has become a mainstream operation in the recent years.However, individual therapy based on disease progression is attracting more and more attention, which has led to new questions.The management of these questions has not formed consensus at present, such as conclusion timing, operative methods, treatment of appendiceal abscess and effect of non-operative management.This review summarizes the treatment progress of acute appendicitis in children.
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Objective To investigate the efficacy of venous-arterial extracorporeal membrane oxygenation (VA-ECMO) in the treatment of refractory septic shock in children.Methods From January 2016 to December 2018,the clinical data of children with refractory septic shock (RSS) treated by VA-ECMO in Department of Critical Medicine Affiliated Children's Hospital of Shanghai Jiao Tong University were retrospectively analyzed.The patients with refractory septic shock (RSS) treated by VA-ECMO were compared with those with non-refractory septic shock (NRSS).Results There were 8 cases in the RSS-ECMO group and 6 cases in the NRSS-ECMO group.The sex,age,PRISM score,complication showed no significant difference in the two groups.The median time of ECMO in the RSS-ECMO group was 182 (141,216) h,and 5 patients were survived and were discharged from the hospital.The blood lactic acid and vasoactive drug index in the RSS-ECMO group was significantly higher than that in the NRSS-ECMO group (P＜0.05 or P＜0.01).The time of vasoactive drugs use and the ratio of combined continuous renal replacement therapy (CRRT) in the RSS-ECMO group were higher than those in the NRSS-ECMO group,but there was no significant difference (P ＞ 0.05).Atter ECMO establishment,the mean invasive arterial pressure increased significantly at 6 h,and lactic acid decreased significantly at 12 h after ECMO support.SCVO2 returned to normal at 24-h ECMO therapy.Conclusions The success rate of VA-ECMO treatment in children with refractory septic shock complicated with MODS is similar to that of children with non-refractory septic shock.The relationship between ECMO and hemodynamic indexes in sepsis should be further explored.
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Objective To study the clinical efficacy and safety of extracorporeal membrane oxygenation (ECMO) in critically ill neonates.Method From November 2016 to September 2018,the clinical data of 5 cases who received ECMO treatment in NICU of our hospital were retrospectively analyzed.The indication of ECMO was reversible respiratory failure irresponsive to conventional therapy.The treatment mode was V-A ECMO.Oxygenation index (OI),vasoactive-inotropic score,blood lactate before and 24 h after ECMO were recorded.Complications of ECMO were also studied.Paired t-test was used to compare the pre and post treatment parameters.Result Among the 5 cases,4 cases were male and 1 case was female.3 cases were diagnosed with meconium aspiration syndrome,2 cases pulmonary hypertension.OI[(9.5 ± 1.8) vs.(60.6 ± 19.4)],vasoactive-inotropic score[(19.5 ± 12.0) points vs.(204.0 ± 143.8) points]and blood lactate [(2.8 ± 1.5) mmol/L vs.(9.6 ± 3.6) mmol/L]) were all significantly decreased at 24 h after ECMO treatment (P ＜ 0.05).During follow-up,3 cases survived,2 cases died.All the 5 cases showed thrombocytopenia,3 cases developed renal failure and received continuous renal replacement therapy,1 case got intracranial hemorrhage.2 of the 3 survived cases developed neurological impairment and need long term follow-up and rehabilitation therapy.Conclusion ECMO treatment has remarkable effects on critically ill neonates and may actually save lives,but the risk of complications are quite high.
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Objective To summarize the therapeutic effects of extracorporeal membrane oxygenation (ECMO) on critically ill children with severe cardiopulmonary failure.Methods The pediatric patients supported with ECMO admitted to pediatric intensive care unit (PICU) from December 2015 to August 2017 were enrolled in this study.The data of demographics of patients,diagnosis,indication for ECMO,the procedure of ECMO support,complications,and survival status were analyzed.Results A total of 17 pediatric patients including 9 male and 8 female with severe cardiopulmonary failure treated with ECMO were studied.The median of age was 24 (2,117) months,and the median of body weight was 12 (5,33) kg.The indications for initiation of ECMO were cardiovascular failure with poor response to conventional therapy and severe acute respiratory distress syndrome (ARDS) without any beneficial effect obtained from mechanical ventilation.The percutaneous cannulation was done under ultrasound guidance by a team of trained intensivists through right cervical vein and internal carotid artery resulting in veno-arterial extracorporeal membrane oxygenation support.The mean duration of ECMO support was 212.5 h with median 188.5 (3-924) h.All patients were treated with mechanical ventilation for prevention from pulmonary atelectasis.Of 17 patients,10 were coupled with continuous renal replacement therapy (CRRT) to keep fluid balance.As a result,11 children (64.7％) were successfully weaned from ECMO defined as survival for 24 h after ECMO,and 10 children (58.8％) were alive to discharge from hospital.One of 2 cardiac arrest cases treated with extracorporeal cardiopulmonary resuscitation (ECPR) was alive.During ECMO support,there were 41 adverse events happened including pressure ulcers (64.7％),thrombocytopenia (52.9％),bleeding (35.3％).Conclusions The survival rate at discharge was 58.8％ in pediatric patients with severe cardiopulmonary failure with poor response to conventional therapy.Our data suggest that ECMO support is an important rescue technique for pediatric critical illness.
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Objective To summarize the etiology and surgical treatment of obstructive infantile cholestasis.Methods Clinical data of 108 cases of obstructive infantile cholestasis was studied retrospectively from April 2009 to April 2014.Results Correct diagnosis was established in all 108 patients by laparoscopic biliary tract exploration and cholangiography.Among those,there were noncorrectable biliary atresia in 81 cases (75.0％),correctable biliary atresia in 5 cases (4.6％),inspissated bile syndrome in 8 cases (7.4％),infantile hepatitis syndrome in 6 cases (5.6％),choledochal cyst in 4 cases (3.7％),biliary hypoplasia in 2 cases (1.9％),1 case (0.9％) suffered from spontaneous bile duct perforation,1 case (0.9％) suffered from oppression of lymph nodes in hepatic portal.Patients of nocorrectable biliary atresia were treated with open Kasai portoenterostomy or laparoscopic Kasai portoenterostomy,correctable biliary atresia and choledochal cyst underwent laparoscopic cyst excision and Roux-Y hepaticojejunostomy,inspissated bile syndrome,infantile hepatitis syndrome and biliary hypoplasia were treated by laparoscopic cholecystostomy and biliary tract irrigation.The patient of spontaneous bile duct perforation was treated with laparoscopic common bile duct exploration and T-tube drainage,the lymph node was excised in patient with oppression of lymph nodes in hepatic portal.All infants were followed-up for 3 months to 48 months,the clearance of jaundice rate varied in patients with Kasai portoenterostomy,patients with non-Kasai portoenterostomy were all in good condition and there were no symptom recurrence.Conclusion Biliary atresia,inspissated bile syndrome,infantile hepatitis syndrome,choledochal cyst and biliary hypoplasia are the most common cause of surgery-related infantile cholestasis.Kasai portoenterostomy,hepaticojejunostomy and cholecystostomy and biliary tract irrigation are the main surgical method for surgery-related infantile cholestasis.