Pediatric

摘要： BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a severe and potentially deadly condition associated with extensive inflammation and immune activation.Cytokine adsorption may serve as a supportive treatment that can stabilize organ function in affected patients by reducing their circulating cytokines levels.To date,no descriptions of clinical experiences associated with the use of HA330-II column hemoadsorption for the treatment of children affected by HLH have been published.CASE SUMMARY We describe the case of an 11-year-old child with Epstein-Barr virus-associated HLH complicated by liver failure.She underwent HA330-II column hemoadsorption and chemotherapy and exhibited reductions in levels of inflammatory cytokines,including interleukin(IL),IL-6,IL-8,IL-10,and interferon-γ.The patient’s condition and laboratory parameters gradually improved with treatment.CONCLUSION Hemoadsorption may play an important role in cytokine storm elimination in children with HLH combined with liver failure and consequent multiple organ failure.

摘要： BACKGROUND Paediatric supraglottic airway devices(SGAs)are widely used in routine anaesthesia and serve as primary or back-up devices for difficult airway management.The inflatable Ambu laryngeal masks and non-inflatable i-gel are two improvements of SGAs based on classic laryngeal masks.The clinical performance and safety of these two devices in paediatric patients are still unclear and warrant further investigation.AIM To perform a systematic review and meta-analysis on the clinical performance and safety of Ambu laryngeal masks and i-gel in anaesthetised paediatric patients.METHODS MEDLINE,Embase,Web of Science and Cochrane Central Register of Controlled Trials were searched from inception dates to April 2020.We identified published randomised controlled trials(RCTs)in which the intervention involved the use of Ambu laryngeal masks and i-gel in anaesthetised paediatric patients(age<18 years).We assessed the oropharyngeal leak pressure(OLP)as the primary outcome.The secondary outcomes were insertion time,success rate of insertion on the first attempt,and incidence of adverse events.RESULTS After searching for all relevant trials published up to April 2020,data from seven RCTs with a total of 667 paediatric patients(323 and 344 participants in the i-gel and Ambu groups,respectively)were evaluated.The mean OLP in anaesthetised paediatric patients was lower in the Ambu group[21.82 cmH2O for Ambu vs 23.98 cmH2O for i-gel,P=0.003,95%confidence interval(CI):-3.58 to-0.75,I2=68%,Mantel-Haenszel random model].We did not find any clear evidence of differences between the devices in terms of insertion time,success rate of insertion,and incidence of adverse events except for blood staining(risk ratio 5.86,95%CI:1.76 to 19.46,P=0.004,I2=0,fixed-effect model).CONCLUSION The i-gel airway may provide a better seal and is therefore probably more suitable than the Ambu laryngeal mask airway in anaesthetised paediatric patients.However,the evidence is insufficient to allow making firm conclusions or to guide clinical practice,owing to the small number of relevant published studies.

摘要： BACKGROUND Biliary atresia(BA)is a rare pediatric disease.AIM To compare the outcomes of laparoscopic portoenterostomy(Lap-PE)with those of laparotomy(Open-PE)at a single institution.METHODS The surgical outcomes of PE were retrospectively analyzed for patients with a non-correctable type of BA from 2003 to 2020.RESULTS Throughout the assessment period,119 patients received PE for BA treatment,including 66 Open-PE and 53 Lap-PE cases.Although the operation duration was longer(medians:for Open-PE,242 min;for Lap-PE,341 min;P80%for both groups for the first half year post surgery,followed by a gradual decrease with time;there were no statistically significant differences in the native liver survival rates for any durations assessed.CONCLUSION Lap-PE could be a standard therapy for BA.

摘要： The diagnosis of ovarian torsion(OT)in girls is challenging compared to that of testicular torsion in boys for many reasons,but it behooves the medical community to continue to strive to better identify this uncommon but clinically important pathology^([1]) OT occurs when the ovary and associated structures rotate around its vascular pedicle,leading to eventually irreversible necrosis.^([2,3])Studies attempting to identify clinical features for early diagnosis have not reached a consensus on the best clinical predictors of this condition,making radiographic imaging of the ovaries even more crucial.^([4,5])

摘要： Vitamin D deficiency is widespread in children and is associated with increased fracture severity. Previous studies have shown mixed results on the impact of vitamin D supplementation on bone health parameters measured on dual-energy x-ray absorptiometry (DEXA) scan. This is the first longitudinal DEXA study in a pediatric fracture population. Pediatric fracture patients with vitamin D levels below 20 ng/mL were counseled to take vitamin D and calcium per a serum-based protocol. Patients underwent baseline DEXA within the initial 12 weeks post fracture and had follow-up scans at 6 and 12 months. 48 patients were enrolled, 32 patients completed two DEXA scans, and 19 completed three DEXA scans. There was a significant increase in lumbar spine BMC, TBLH BMD, and TBLH BMC between DEXA 1 and 2 (p 0.001). A positive trend in DEXA parameters is suggested between DEXA 1 and DEXA 3. Height adjusted z-scores (HAZ) were calculated which showed no statistical significance, p-values > 0.05. In this group, there are no significant changes in TBLH, BMC or BMD z-scores one year after fracturing despite vitamin D and calcium supplementation. This suggests that children returning to collision sports may be at continued risk for refracture and future studies are needed.

摘要： As a result of the obesity epidemic,non-alcoholic fatty liver disease(NAFLD)represents a global medical concern in childhood with a closely related increased cardiometabolic risk.Knowledge on NAFLD pathophysiology has been largely expanded over the last decades.Besides the well-known key NAFLD genes(including the I148M variant of the PNPLA3 gene,the E167K allele of the TM6SF2,the GCKR gene,the MBOAT7-TMC4 rs641738 variant,and the rs72613567:TA variant in the HSD17B13 gene),an intriguing pathogenic role has also been demonstrated for the gut microbiota.More interestingly,evidence has added new factors involved in the“multiple hits”theory.In particular,omics determinants have been highlighted as potential innovative markers for NAFLD diagnosis and treatment.In fact,different branches of omics including metabolomics,lipidomics(in particular sphingolipids and ceramides),transcriptomics(including micro RNAs),epigenomics(such as DNA methylation),proteomics,and glycomics represent the most attractive pathogenic elements in NAFLD development,by providing insightful perspectives in this field.In this perspective,we aimed to provide a comprehensive overview of NAFLD pathophysiology in children,from the oldest pathogenic elements(including genetics)to the newest intriguing perspectives(such as omics branches).

摘要： BACKGROUND Liver transplantation(LT)has become an acceptable curative method for children with several liver diseases,especially irreversible acute liver failure and chronic liver diseases.King Chulalongkorn Memorial Hospital is one of Thailand’s largest liver transplant centers and is responsible for many pediatric cases.AIM To report the experience with pediatric LT and evaluate outcomes of livingrelated vs deceased-donor grafts.METHODS This evaluation included children who underwent LT between August 2004 and November 2019.Data were retrospectively reviewed,including demographics,diagnoses,laboratory values of donors and recipients,the pediatric end-stage liver disease(PELD)or model for end-stage liver disease(MELD)score,graft source,wait time,perioperative course,postoperative complications,and survival rates.Continuous data were reported using the median and interquartile range.The Mann–Whitney U-test was used to compare the wait time between the living-related and deceased-donor groups.The chi-square or Fisher's exact test were used to compare the frequencies of between-group complications.Survival rates were calculated using the Kaplan–Meier method.RESULTS Ninety-four operated pediatric liver transplant patients were identified(54%were females).The median age at transplantation was 1.2(0.8-3.8)years.The median PELD and MELD scores were 20(13-26.8)and 19.5(15.8-26.3),respectively.Most grafts(81.9%)were obtained from living-related donors.The median wait time for the living donors was significantly shorter compared with the deceased donors at 1.6(0.3-3.1)mo vs 11.2(2.1-33.3)mo(P=0.01).Most patients were diagnosed with biliary atresia(74.5%),and infection was the most common complication within 30 d posttransplantation(14.9%).Without a desensitization protocol,9%of transplants were ABOincompatible.Eight hepatitis B core antibodies(anti-HBc)-negative recipients received positive anti-HBc grafts without different observed complications.The overall survival rate was 93.6%and 90.3%at 1 and 5 years,respectively.No graft loss during follow-up was noted among survivors.CONCLUSION A significant number of pediatric LT cases were reported in Thailand.Based on relatively comparable outcomes,ABO-incompatible and HBc antibody-positive grafts may be considered in an organ shortage situation.

摘要： Cytomegalovirus (CMV) infection is a common complication of liver transplantationin children. The CMV serostatus of recipients and donors is theprimary risk factor, and prophylaxis or pre-emptive strategies are recommendedfor high-risk patients. Graft rejection, coinfection and Epstein-Bar virusreactivation, which can lead to post-transplant lymphoproliferative disease, areindirect effects of CMV infection. Assessment of CMV infection viral load shouldbe routinely performed upon clinical suspicion. However, tissue-invasive CMVdisease is not associated with CMV viraemia and requires confirmation by tissuepathology. Oral valganciclovir and intravenous ganciclovir are equivalenttreatments, and the duration of treatment depends on factors including CMV viralload, tissue pathology, and clinical response. Risk stratification by donor andrecipient status prior to transplantation and post-transplantation antiviralprophylaxis or pre-emptive therapy are recommended. Adult guidelines havebeen established but additional study of the effectiveness of the preventiveguidelines in children is needed. This review summarizes the burden, risk factors,clinical manifestations, laboratory evaluation, treatment, and prevention of CMVinfection in children after liver transplantation.

摘要： Children infected by severe acute respiratory syndrome coronavirus 2(SARSCoV-2)seem to have a better prognosis than adults.Nevertheless,pediatric solid organ transplantation(SOT)has been significantly affected by the unprecedented coronavirus disease 2019(COVID-19)pandemic during the pre-,peri-,and posttransplant period.Undoubtedly,immunosuppression constitutes a real challenge for transplant clinicians as increased immunosuppression may prolong disease recovery,while its decrease can contribute to more severe symptoms.To date,most pediatric SOT recipients infected by SARS-CoV-2 experience mild disease with only scarce reports of life-threatening complications.As a consequence,after an initial drop during the early phase of the pandemic,pediatric SOTs are now performed with the same frequency as during the pre-pandemic period.This review summarizes the currently available evidence regarding pediatric SOT during the COVID-19 pandemic.

摘要： This letter to editor discussing on the publication on severe acute respiratory syndrome coronavirus 2 pandemic and surgical diseases.Concerns on procedures are raised and discussed.