A recently published prospective study marks a breakthrough for congenital olfactory disorders in children.The study provides the first long-term,three-year follow-up data,robustly demonstrating the durable efficacy a...A recently published prospective study marks a breakthrough for congenital olfactory disorders in children.The study provides the first long-term,three-year follow-up data,robustly demonstrating the durable efficacy and safety of autologous nasal epithelial stem cell transplantation.This work reveals immense therapeutic potential for a condition traditionally considered untreatable.However,this milestone achievement also presents new challenges.To translate this pioneering therapy from a single-center success to a global standard,multicenter,controlled clinical trials must be initiated immediately.Only through rigorous validation can we ensure its widespread adoption and ultimately bring hope to millions of children worldwide.展开更多
BACKGROUND Functional gastrointestinal disorders(FGIDs)in children present with chronic symptoms like abdominal pain,diarrhea,and constipation without identifiable structural abnormalities.These disorders are closely ...BACKGROUND Functional gastrointestinal disorders(FGIDs)in children present with chronic symptoms like abdominal pain,diarrhea,and constipation without identifiable structural abnormalities.These disorders are closely linked to gut-brain axis dysfunction,altered gut microbiota,and psychosocial stress,leading to psychia-tric comorbidities such as anxiety,depression,and behavioral issues.Under-standing this bidirectional relationship is crucial for developing effective,holistic management strategies that address physical and mental health.AIM To examine the psychiatric impacts of FGIDs in children,focusing on anxiety and depression and their association with other neurodevelopmental disorders of childhood,such as attention-deficit/hyperactivity disorder,emphasizing the role of the gut-brain axis,emotional dysregulation,and psychosocial stress.Key mechanisms explored include neurotransmitter dysregulation,microbiota imbalance,central sensitization,heightening stress reactivity,emotional dysregulation,and symptom perception.The review also evaluates the role of family dynamics and coping strategies in exacerbating FGID symptoms and contributing to psychiatric conditions.METHODS A narrative review was conducted using 328 studies sourced from PubMed,Scopus,and Google Scholar,covering research published over the past 20 years.Inclusion criteria focused on studies examining FGID diagnosis,gut-brain mechanisms,psychiatric comorbidities,and psychosocial factors in pediatric populations.FGIDs commonly affecting children,including functional constipation,abdominal pain,irritable bowel syndrome,gastroesophageal reflux,and cyclic vomiting syndrome,were analyzed concerning their psychological impacts.RESULTS The review highlights a strong connection between FGIDs and psychiatric symptoms,mediated by gut-brain axis dysfunction,dysregulated microbiota,and central sensitization.These physiological disruptions increase children’s vulnerability to anxiety and depression,while psychosocial factors-such as chronic stress,early-life trauma,maladaptive family dynamics,and ineffective coping strategies-intensify the cycle of gastrointestinal and emotional distress.CONCLUSION Effective management of FGIDs requires a biopsychosocial approach integrating medical,psychological,and dietary interventions.Parental education,early intervention,and multidisciplinary care coordination are critical in mitigating long-term psychological impacts and improving both gastrointestinal and mental health outcomes in children with FGIDs.展开更多
Pediatric inflammatory bowel disease(PIBD)is a chronic inflammatory disorder of the gastrointestinal tract,with rising global incidence and prevalence.Over the past two decades,biologics have added to the therapeutic ...Pediatric inflammatory bowel disease(PIBD)is a chronic inflammatory disorder of the gastrointestinal tract,with rising global incidence and prevalence.Over the past two decades,biologics have added to the therapeutic armamentarium and revolutionized the approach to treatment of inflammatory bowel disease.The available biologics include monoclonal antibodies which target inflammatory cytokines(anti-tumor necrosis factor alpha,anti-interleukin 12/23)or recruitment of leucocytes to the gastrointestinal tract(anti-alpha4beta7 integrin)and small molecules(Janus kinase inhibitors,sphingosine 1-phosphate-inhibitors)which modify the proinflammatory signaling.Considering their potential disease-modifying ability,recent pediatric guidelines from the West have advocated upfront use of biologics in appropriate clinical scenarios as a top-down approach rather than the conventional step-up approach.Although real-world studies are available regarding the clinical efficacy of biologics in PIBD,there is paucity of long-term outcome and safety data in children.Also,little information is available about the best approach in the newly industrialized-developing countries where PIBD is rising but at the same time,infections are prevalent and resources are limited.In this review,we summarize the efficacy and safety profile of biologics and small molecule drugs and discuss the challenges in the management of PIBD,especially in the developing world,and future directions.展开更多
BACKGROUND Pediatric short bowel syndrome(SBS)poses management challenges,and teduglutide is a potential therapy.However,comprehensive data on its pediatric safety are lacking.AIM To evaluate the impact of teduglutide...BACKGROUND Pediatric short bowel syndrome(SBS)poses management challenges,and teduglutide is a potential therapy.However,comprehensive data on its pediatric safety are lacking.AIM To evaluate the impact of teduglutide on infection and gastrointestinal adverse events in pediatric SBS patients via systematic review and meta-analysis.METHODS Following PRISMA 2009 guidelines and PROSPERO registration,we searched PubMed,Web of Science,and EMBASE for randomized controlled trials(RCTs)(pediatric SBS patients≤18 years;teduglutide vs placebo/standard care).Two reviewers screened studies,extracted data,and assessed bias(ROB2).Metaanalyses used RevMan 5.4(Mantel-Haenszel method,random-effects if I^(2)≠0).Trial sequential analysis and GRADE were applied.RESULTS Three RCTs involving 115 pediatric patients were included.Pooled analysis revealed no statistically significant differences between the teduglutide and control groups for the primary outcome of infection events[RR=0.83;(95%CI:0.44-1.56);P=0.57;I^(2)=0%;2 studies,n=55].Similarly,no significant differences were found for secondary outcomes:Upper respiratory tract infection[RR=0.68;(95%CI:0.32-1.47);P=0.33;I^(2)=0%],catheter site infection[RR=1.86;(95%CI:0.23-14.78);P=0.56;I^(2)=0%],vomiting[RR=1.35;(95%CI:0.10-18.23);P=0.82;I^(2)=72%],abdominal pain[RR=2.47;(95%CI:0.50-12.16);P=0.27;I^(2)=0%],nausea[RR=1.31;(95%CI:0.24-7.22);P=0.75;I^(2)=0%],diarrhea[RR=1.02;(95%CI:0.23-4.43);P=0.98;I^(2)=0%],and abdominal distension[RR=1.49;(95%CI:0.18-12.35);P=0.71;I^(2)=0%].The overall certainty of evidence assessed by GRADE was moderate.CONCLUSION Teduglutide does not increase infection or gastrointestinal adverse event risk in pediatric SBS,but small sample sizes limit conclusions.Larger studies are needed.展开更多
Hepatitis A virus(HAV)infection remains a significant public health concern in many developing countries.The annual incidence of HAV infection is 1.5 million,though this figure may be underestimated owing to the infec...Hepatitis A virus(HAV)infection remains a significant public health concern in many developing countries.The annual incidence of HAV infection is 1.5 million,though this figure may be underestimated owing to the infection’s asymptomatic nature and the presence of milder disease variants.The clinical spectrum of HAV infection now ranges from asymptomatic infection to fulminant hepatitis.Despite the availability of safe and highly effective vaccines,HAV infections remain a major contributor to acute viral hepatitis worldwide.展开更多
This article examines the growing prevalence of pediatric obesity and its con-nection to metabolic dysfunction-associated steatotic liver disease(MASLD)in children and adolescents,focusing on the role of glucagon-like...This article examines the growing prevalence of pediatric obesity and its con-nection to metabolic dysfunction-associated steatotic liver disease(MASLD)in children and adolescents,focusing on the role of glucagon-like peptide-1 receptor agonists in treatment.Pediatric obesity and MASLD present significant long-term health risks,making early intervention crucial.The article reviews the patho-physiology of both pediatric obesity and MASLD,explores current therapeutic strategies,and discusses the emerging role of glucagon-like peptide-1 receptor agonists,such as liraglutide,semaglutide,exenatide,and dulaglutide,in managing obesity,as well as explores current limited pediatric literature on the use of these medications in MASLD.展开更多
BACKGROUND Gastrointestinal diseases in young children are often anatomic or inflammatory in nature and can present with symptoms similar to those of Cow’s milk protein allergy(CMPA),complicating diagnosis.This case ...BACKGROUND Gastrointestinal diseases in young children are often anatomic or inflammatory in nature and can present with symptoms similar to those of Cow’s milk protein allergy(CMPA),complicating diagnosis.This case series highlights 3 pediatric patients initially misdiagnosed with CMPA,emphasizing the need for a thorough evaluation.CASE SUMMARY Case 1:A 3-year-old child with chronic abdominal distension and constipation was initially treated for CMPA and was later diagnosed with Hirschsprung disease through rectal biopsy.Surgical intervention involved a laparoscopic colostomy followed by a pull-through procedure,leading to a successful recovery.Case 2:A 2-month-old infant presented with greenish-yellow vomiting and abdominal distension.Initially misdiagnosed with CMPA,further investigation using barium studies revealed partial intestinal malrotation.The patient underwent a laparoscopic Ladd’s procedure and recovered well.Case 3:A 6-month-old infant with persistent vomiting and failure to thrive had been treated for CMPA.Detailed imaging studies indicated achalasia.The child underwent a Heller myotomy,which resulted in significant symptom improvement and weight gain CONCLUSION Thorough evaluation of gastrointestinal symptoms is necessary in children.A high suspicion for alternative diagnoses will prevent delays in accurate diagnosis and proper treatment,leading to improved outcomes.展开更多
The surgical treatment of severe scoliosis and kyphoscoliosis in the pediatric population is complicated and has high morbidity and mortality risks.Severe scoliosis has traditionally been defined by a coronal Cobb ang...The surgical treatment of severe scoliosis and kyphoscoliosis in the pediatric population is complicated and has high morbidity and mortality risks.Severe scoliosis has traditionally been defined by a coronal Cobb angle of greater than 90°or 100°.The usual corrective methods for these patients have been anterior or posterior release and osteotomies using a combined anterior-posterior or posterior-only approach.Many of these patients have pre-existing pulmonary compromise;therefore,an anterior approach is often not reasonable or possible.Acute correction of a deformity may also cause neurologic injury.Halo gravity traction(HGT)allows for progressive,gradual,and sustained correction of the spinal deformity in the coronal,sagittal,and axial planes,leading to a decrease in the amount of correction needed at definitive posterior fusion.This relates to decreased postoperative neurologic deficit and improved pulmonary function.Preoperative HGT has evolved to be the surgical adjunct in the treatment of severe spinal deformity.Indications for HGT,best protocols of application,and optimal duration of traction still lack uniformity;thus,a review of the literature remains relevant and necessary.This review summarizes the existing literature on HGT,including its indications,applications,duration of traction,and associated complications.展开更多
BACKGROUND Approval of teduglutide is an important addition to the limited treatment options for short bowel syndrome(SBS).However,real-world evidence on teduglutide therapy for SBS in Latin America is scarce.AIM To i...BACKGROUND Approval of teduglutide is an important addition to the limited treatment options for short bowel syndrome(SBS).However,real-world evidence on teduglutide therapy for SBS in Latin America is scarce.AIM To investigate the effectiveness and safety of teduglutide in clinical practice in Argentina with a 24-week follow-up.METHODS This non-interventional multicentre cohort study included consecutive patients(aged≥1 years)with SBS who were dependent on parenteral support(PS)and received≥1 dose of teduglutide according to currently approved indications.RESULTS The study population(n=45)included 21 adult and 24 pediatric patients.The proportion of adult and pediatric patients who showed clinical response(defined as a≥20%reduction in weekly PS volume)after 24 weeks of treatment was 90.4%[95%confidence interval(CI):69.6%-98.8%]and 83.3%(95%CI:62.6%-95.2%),respectively.Overall,12 patients(26.6%;95%CI:14.6-41.9)were weaned from PS support at the 24-week assessment,6(28.5%;95%CI:11.5-52.1)in the adult cohort,and 6(25%;95%CI:9.7-46.7)in the pediatric cohort.Only baseline PS requirement was inversely associated with weaning from PS(P=0.025).The most frequently reported treatmentemergent adverse events(TEAEs)were mild to moderate abdominal pain and abdominal distension(16.6%;and 9.5%,respectively).None of the reported TEAEs led to treatment discontinuation.CONCLUSION This prospective real-world study demonstrated the effectiveness and safety of teduglutide in adult and pediatric patients with SBS in Argentina.The clinical response observed in both adults and pediatric patients was greater than that reported in phase 3 trials and was consistent with the results of other real-world studies.展开更多
Objective:To investigate the efficacy of a tripartite collaborative nursing intervention in enhancing resilience among families of pediatric leukemia patients.Methods:Based on Walsh’s family resilience theory and col...Objective:To investigate the efficacy of a tripartite collaborative nursing intervention in enhancing resilience among families of pediatric leukemia patients.Methods:Based on Walsh’s family resilience theory and collaborative nursing principles,and after reviewing a large amount of literature,a tripartite intervention was constructed,which was led by the nursing team,coordinated by medical social workers,and supported by volunteers.Caregivers of pediatric hematology inpatients at a tertiary-level Class A hospital were selected as research subjects.which were divided into a control group(n=30)and an experimental group(n=30)according to a randomized block design.The control group are treated with standard nursing care,which included health education,counseling,and psychological support from nurses.The experimental group,in addition,was provided with a tripartite collaborative nursing intervention for 3 months.Results:The family resilience level,social support,and family function scores of the experimental group were higher than those of the control group,and the differences were statistically significant(P≤0.05).Conclusion:The implementation of tripartite collaborative nursing intervention improves the resilience of families of children suffered from leukemia.展开更多
BACKGROUND Despite advances in treatment,the prognosis for patients with high-risk pediatric solid tumors remains dismal.Tandem autologous stem cell transplantation(ASCT)offers promise for improving outcomes in these ...BACKGROUND Despite advances in treatment,the prognosis for patients with high-risk pediatric solid tumors remains dismal.Tandem autologous stem cell transplantation(ASCT)offers promise for improving outcomes in these patients.This study aimed to examine the efficacy and prognostic factors of tandem ASCT in pediatric patients with high-risk solid tumors.AIM To determine the survival outcomes and prognostic factors in pediatric patients with high-risk solid tumors undergoing tandem ASCT.METHODS A total of 40 pediatric patients with high-risk solid tumors treated from March 2015 to August 2022 were included in this retrospective study.The diagnoses of the patients included neuroblastoma,germ cell tumors,atypical teratoid/rhabdoid tumor,medulloblastoma,and pineoblastoma.After induction chemotherapy,all patients received tandem ASCT and were allocated into two groups(group A and group B)based on high-dose chemotherapy regimens.Prognostic relevance was evaluated by examining patient characteristics,such as sex,age,lactate dehydrogenase levels,primary site,the number of metastatic sites,and bone marrow involvement.RESULTS The median follow-up duration since the first ASCT was 24 months(range:1-91 months),with 5-year overall survival(OS)and event-free survival(EFS)rates of 73%and 70%,respectively,for the entire cohort.The 3-year OS rates were 67%for group A and 87%for group B(P=0.29),with corresponding 3-year EFS rates of 67%and 79%(P=0.57).Among neuroblastoma patients,the 5-year OS and EFS were 69%and 63%(P=0.23).Univariable analysis revealed a notable association of age≥36 months and elevated lactate dehydrogenase level at diagnosis with poorer OS.Despite acute adverse effects,all patients demonstrated good tolerance to the treatment,with no occurrences of transplant-related mortality.CONCLUSION Tandem ASCT demonstrates promising survival outcomes for patients with high-risk solid tumors,particularly neuroblastoma,with manageable toxicity and no transplant-related mortality.展开更多
The significance of gut microbiota(GM)in human health is being increasingly researched.An imbalance in GM composition,known as dysbiosis,is linked to various and other health issues.In addition,antibiotics are the pri...The significance of gut microbiota(GM)in human health is being increasingly researched.An imbalance in GM composition,known as dysbiosis,is linked to various and other health issues.In addition,antibiotics are the primary and most significant factors leading to major changes in the composition and function of the GM,which may result in colonization by antimicrobial-resistant(AMR)pathogens.Therefore,alternative antibiotic strategies for combating AMR pathogens are urgently needed.This narrative review highlights current knowledge regarding various pertinent strategies for decolonizing bacterial pathogens from GM and emphasizes decolonization therapies’critical role in pediatric surgical disorders.Strategies such as decontamination of the digestive tract utilizing antibiotics,the use of probiotics,and particularly fecal microbiota transplantation have introduced new options for clinical treatment.These treatments show the potential to restore GM balance and have demonstrated advantages for intestinal disorders related to pediatric surgery,including inflammatory bowel disease,neonatal necrotizing enterocolitis,Hirschsprung-associated enterocolitis,and short bowel syndrome.Despite GM therapeutics,recent strategies are still in their developmental phase and exhibit challenges that need further research.Thus,potential future directions for GMtargeted decolonization therapies are under consideration.Innovative alternative strategies to combat AMR though GM modulation in disorders related to pediatric surgery appear to be promising and should continue to be prioritized for further research and development.展开更多
Background:Precision medicine is an emerging approach for treating pediatric cancer due to its ability to target tumor-specific genetic drivers rather than provide broad and aggressive treatments.The study aimed to ou...Background:Precision medicine is an emerging approach for treating pediatric cancer due to its ability to target tumor-specific genetic drivers rather than provide broad and aggressive treatments.The study aimed to outline the establishment and impact of a Precision Medicine Clinic(PMC)in the setting of pediatric oncology,with the objective of offering targeted treatment options within the institution and creating a scalable model for adoption by other healthcare systems to achieve a wider impact.Methods:Recognizing this need for an individualized approach to treating patients,Cook Children’s Medical Center(CCMC)established a multidisciplinary molecular tumor board in 2019,followed by the launch of an official PMC in 2021.Before this,there was no dedicated place to discuss and evaluate genetic sequencing results.Results:In 2022 and 2023,the PMC discussed 69 patients with a wide variety of oncologic diagnoses.Through the clinic’s efforts,133 genetic variants across 101 genes have been identified,spanning oncogenic pathways related to cell cycling,DNA processing,and cell signaling.Of the sequenced patients,four have received targeted therapy according to recommendations from the PMC.Conclusion:While the PMC continues to evaluate patients and their long-term outcomes,the continually growing PMC at CCMC represents the beginning of the advancement of treating pediatric oncology patients through the interpretation of genetic sequencing results,making actionable targeted treatment recommendations,and continuing to follow the patient’s course of care over time.This additionally provides a framework for starting a PMC that can be adapted for specific clinical needs and implemented broadly.展开更多
Background:Psychosocial functioning and quality of life(QoL)are strongly associated with outcomes in pediatric heart transplant recipients.The data in pediatric transplantation,however,is limited.This study aims to in...Background:Psychosocial functioning and quality of life(QoL)are strongly associated with outcomes in pediatric heart transplant recipients.The data in pediatric transplantation,however,is limited.This study aims to investigate the associations of perioperative anxiety and depression with postoperative complications,sociodemographic and clinical characteristics.Methods:This observational,analytical,longitudinal study included 42 pediatric participants aged 8 to 16 years old.Preoperative psychological assessments were completed by 36 children,the remaining 6 were unable to participate due to invasive ventilation,extracorporeal membrane oxygenation(ECMO),and physical debilitation.Postoperatively,all 42 subjects completed the psychosocial evaluations.Data on recipients characteristics,family characteristics and clinical parameters were collected.Anxiety and depression were assessed using the Screen for Child Anxiety Related Emotional Disorders(SCARED)and the Depression Self-Rating Scale for Children(DSRSC).The Short Form-36 Health Survey(SF-36)was applied to assess the health-related QoL.Results:Before transplantation,91.7%(33/36)of the children exhibited symptoms of anxiety,and an identical proportion(91.7%,33/36)showed signs of depression.After transplantation,the rates of anxiety and depression decreased to 35.7%(15/42)and 11.9%(5/42),respectively.Longer disease course(p=0.042),preoperative hypoalbuminemia(p=0.032),older age(p=0.024),postoperative hypertension(p=0.011),and postoperative CRRT(p=0.015)result in depression symptoms.Preoperative hypoalbuminemia(p=0.032)was also more common in the anxiety group.Additionally,children with psychosocial risks had significantly lower QoL scores in general health(p=0.008)and mental health(p=0.015).Conclusions:Perioperative anxiety and depression are highly prevalent among pediatric heart transplant recipients.Although significant improvements in psychosocial risks were observed at posttransplantation,approximately 40%children continued to experience psychosocial challenges.展开更多
Objective:The aim of the present study was to investigate long-term efficacy and safety of percutaneous stent implantation for residual pulmonary artery stenosis(PAS)in pediatric patients after surgical repair of comp...Objective:The aim of the present study was to investigate long-term efficacy and safety of percutaneous stent implantation for residual pulmonary artery stenosis(PAS)in pediatric patients after surgical repair of complicated congenital heart diseases(CHDs).Methods:All pediatric patients diagnosed with residual PAS after surgical repair of complicated CHDs between 1996 and 2020 were retrospectively enrolled in the study.Results:A total of 41 patients(30 males,11 females;median age 5.0 years,median weight 17 kg)were followed-up for a median of 7.1 years.Follow-up echocardiography results demonstrated that the target vessel diameter increased from(3.4±1.1)mm preoperatively to(6.2±1.9)mm one year post-procedure and(6.0±1.5)mm at the final follow-up(p<0.05).The pressure gradient across the stenosis decreased from(52.6±15.8)mmHg preoperatively to(35.8±19.1)mmHg one year post-procedure and(33.1±19.7)mmHg at the final follow-up(p<0.05).Cardiac computed tomography scans indicated that target vessel/distal vessel diameter ratio increased from(0.4±0.2)pre-operatively to(0.8±0.2)one year post-procedure and(0.9±0.3)at the final follow-up(p<0.05).A total of six adverse events were documented,comprising two cases of in-stent restenosis requiring surgical reintervention,three cases of in-stent restenosis managed with regular clinical surveillance,and one case of percutaneous pulmonary valve replacement due to severe pulmonary regurgitation.Kaplan-Meier event-free survival analysis demonstrated that elevated preprocedural right ventricular systolic pressure(>72 mmHg)was significantly associated with long-term adverse events(p=0.024).Conclusion:Percutaneous stent implantation for residual PAS after surgical repair of complicated CHDs effectively relieves vessel stenosis,stabilizes cardiac function,and improves long-term prognosis in pediatric patients.In-stent restenosis remains an unresolved complication,necessitating further advancements in interventional strategies.展开更多
Objective: This study aimed to demonstrate the feasibility of laparoscopic-resection of the abdominal cystic lymphangiomas in Pediatric Surgery and describe the morbidity associated to this management in Queen fabiola...Objective: This study aimed to demonstrate the feasibility of laparoscopic-resection of the abdominal cystic lymphangiomas in Pediatric Surgery and describe the morbidity associated to this management in Queen fabiola children’s university hospital (HUDERF) in Brussels. Methods: We retrospectively conducted a study at the Pediatric Surgery Department of HUDERF, Brussels. The studied period was from January 1, 2014, to January 1, 2024;a span of 10 years. All patients with a confirmed diagnosis of cystic lymphangiomas and who underwent laparoscopic surgery were included in our study. Those who have been operated exclusively by open surgery have been excluded. The technique involved either total laparoscopic resection or laparoscopic-assisted with extra-abdominal resection of the tumor. Parameters that were studied included age, sex, weight, symptoms, preoperative diagnosis, imaging assessment, location, size of the tumor, type of mass, surgical procedure, duration of the surgery, conversion to open surgery, morbidity, and histopathology. Data were analyzed using Microsoft Office Excel 2010 and SPSS. Results: We retrieved 10 files of patients presenting with abdominal cystic lymphangiomas within two patients underwent exclusive open surgery and have excluded from our study. Then our sample was constituted with 8 patients. The mean age of the patients was 6.4 years (standard deviation: 3.6 years, range: 1 to 11 years). Male patients were predominant. The mean weight was 26.7 kg (standard deviation: 14.7 kg, range: 10 to 55 kg). The most common symptom was abdominal pain. Preoperative diagnosis of abdominal cystic lymphangioma was made in 8 cases. Abdominal ultrasound was performed in all patients. MRI was done in 5 patients, and CT scan in 2 patients. All patients presented a multicystic mass. Pure laparoscopic resection of the cyst was done in 2 cases. Laparoscopic-assisted resection in 4 cases (with extra-peritoneal with small bowel resection and mesenteric detorsion in 1 case), and conversion in 2 cases due to the complex location of the cyst. After a follow-up period of 5 years, morbidity was noted in one patient (Patient 3) who developed postoperative bowel obstruction 1 month post-surgery. This patient was re-operated on with a favorable clinical outcome following conventional small bowel resection and anastomosis. The other patients (1, 2, 4, 5, 6, 7 and 8) had a simple clinical course, and no recurrence was observed in our series. Conclusion: Laparoscopic-resection of the abdominal cystic lymphangiomas is feasible in Pediatric Surgery. As minimally invasive surgery it gives many advantages even for complex abdominal cystic lymphangiomas with less morbidity as shown in our series.展开更多
Acute alithiasic cholecystitis (AAC) is the inflammatory disease of the gallbladder in the absence of gallstones. It is a rare pathology in children. The aim was to describe the clinical, diagnostic and therapeutic ch...Acute alithiasic cholecystitis (AAC) is the inflammatory disease of the gallbladder in the absence of gallstones. It is a rare pathology in children. The aim was to describe the clinical, diagnostic and therapeutic characteristics of this disease. Materials and Method: This was a 3-year retrospective study (January 2008 to December 2010) including 66 patient records collected for CAA. Results: The frequency of AAC was 3.57%, the mean age of the patients was 8 ± 3.52 years, and there were 36 boys and 30 girls. The main clinical features were right hypochondrial pain (66 cases), positive Murphy’s (66 cases), fever (53 cases) and jaundice or sub-jaundice (51 cases). The main ultrasound signs were thickened vesicular wall 50 cas (75.76%), vesicular distension 50cas (75.76%) and positive ultrasound Murphy 47 cas (71.21%). Medical treatment was exclusive in 64 patients (96.97%) and surgical treatment in 2 patients (3.03%). Conclusion: Acute alithiasic cholecystitis is a rare pathology in children, the clinical picture is not very specific and abdominal ultrasound is the key to early diagnosis. Conservative treatment is the first choice in the absence of any complications.展开更多
I<span><span style="font-family:Verdana;">ntroduction: Acute thoracic syndrome is the appearance of a new pulmonary </span><span style="font-family:Verdana;">infiltrate</...I<span><span style="font-family:Verdana;">ntroduction: Acute thoracic syndrome is the appearance of a new pulmonary </span><span style="font-family:Verdana;">infiltrate</span><span style="font-family:Verdana;"> on radiology associated with fever, desaturation </span><span style="font-family:Verdana;">or</span><span style="font-family:Verdana;"> respiratory signs. It is the second leading cause of hospitalization and</span></span><span style="font-family:Verdana;"> the first cause of death in sickle cell patients. It is an acute pulmonary complication whose pathophysiological mechanisms are still poorly understood. This study aims to</span><span><span style="font-family:Verdana;"> study the epidemiological, clinical, therapeutic </span><span style="font-family:Verdana;">and</span><span style="font-family:Verdana;"> evolutionary aspe</span></span><span style="font-family:Verdana;">c</span><span><span style="font-family:Verdana;">ts of Acute Chest Syndrome in children at the Pediatrics Department of Donka National Hospital. Method: This is a prospective study of descriptive type for a period of 6 months from February 19 to August 19, </span><span style="font-family:Verdana;">2019</span><span style="font-family:Verdana;"> on patients with sickle cell disease who developed an ATS in the pediatrics department of Donka National Hospital. Epidemiological, clinical, therapeutic </span><span style="font-family:Verdana;">and</span><span style="font-family:Verdana;"> evolutionary data were studied and proportionate data were calculated. Results: The frequency of ATS was 39%. The mean age of our patients was 9.83 years with the extremes of</span></span><span style="font-family:Verdana;"> 4 and 16 years. The age group from 6 to 10 years with a frequ</span><span style="font-family:Verdana;">ency of 66.7% was the most affected. Fever was the main clinical manifestation, followed by hepatome</span><span><span style="font-family:Verdana;">galy. All our patients were homozygous SS and undergoing folic acid prophylaxis. 96% of our patients did not receive any specific vaccine. Antibiotic therapy, hyperhydration </span><span style="font-family:Verdana;">and</span><span style="font-family:Verdana;"> analgesics were administered to all our patients. 96% of our patients were transfused with red blood cell concentrate. 96% of our patients were transfused with packed red blood cells. 96% of our patients were transfused with packed red blood cells and 96% of them had a favorable outcome. Conclusion: ATS is an acute co</span></span><span style="font-family:Verdana;">mplication of sickle cell disease responsible for significant mortality and morbidity in the pediatric population. Its treatment is symptomatic and must be started early. Emphasis must be placed on prevention to prevent or limit its occurrence.</span>展开更多
Hodgkin lymphoma(HL)is a heterogenous lymphoproliferative disorder of B-cell origin and represents one of the most common malignancies in children and young adults.In addition to well-known underlying factors-such as ...Hodgkin lymphoma(HL)is a heterogenous lymphoproliferative disorder of B-cell origin and represents one of the most common malignancies in children and young adults.In addition to well-known underlying factors-such as Epstein-Barr virus infection-the familial aggregation demonstrated in large population studies suggested a genetic predisposition.First-degree relatives of patients with HL have an approximately threefold increased risk of developing the disease compared to the general population.These observations have recently prompted several whole-genome studies in affected families,identifying variants possibly implicated in lymphomagenesis,including alterations in DICER1(a member of the ribonuclease III family),POT1(protection of telomeres 1),KDR(kinase insert domain receptor),KLHDC8B(kelch domain-containing protein 8B),PAX5(paired box protein 5),GATA3(GATA binding protein 3),IRF7(interferon regulatory factor 7),EEF2KMT(eukaryotic elongation factor 2 lysine methyltransferase),and POLR1E(RNA polymerase I subunit E).In this article,we review current insights into the etiopathogenesis and risks of familial HL,and present case reports involving two sisters diagnosed with HL nearly 17 years apart.Recognizing the risk for first-degree relatives may potentially increase awareness of early symptoms among family members of HL patients,leading to earlier diagnosis and better outcomes.Conversely,understanding that the hereditary risk,though higher than in the general population,remains relatively low may provide reassurance for affected families.展开更多
BACKGROUND Tuberculous osteitis is a chronic,granulomatous bone infection that frequently results in impaired bone healing following surgery.Despite surgical intervention and prolonged anti-tuberculous therapy,complet...BACKGROUND Tuberculous osteitis is a chronic,granulomatous bone infection that frequently results in impaired bone healing following surgery.Despite surgical intervention and prolonged anti-tuberculous therapy,complete bone regeneration often remains unachieved,contributing to subsequent orthopedic complications.AIM To investigate the efficacy and safety of pamidronate in promoting bone regeneration following surgical treatment of experimental animal tuberculous osteitis.METHODS A controlled randomized basic study of rabbit femoral tuberculosis induced by Mycobacterium tuberculosis strain H37Rv included surgical removal of infected tissue and implantation of osteoinductive bone grafts with the following animal allocation to one of three groups:(1)Bisphosphonates alone;(2)Bisphosphonates combined with anti-tuberculous therapy;and(3)Anti-tuberculous therapy alone.The control group consisted of animals that received no surgical or medical treatment.Clinical evaluations,biochemical markers,micro-computed tomography imaging,and histomorphometry analyses were conducted at 3 months and 6 months postoperatively.RESULTS Pamidronate treatment significantly reduced early implant resorption,increased osteoblastic activity,improved trabecular bone regeneration,and maintained graft integrity compared to the anti-tuberculous therapy-only group.Histologically,pamidronate led to enhanced vascular remodeling and increased bone matrix formation.Crucially,bisphosphonate therapy demonstrated safety,compatibility with anti-tuberculous medications,and did not exacerbate tuberculous inflammation.Furthermore,micro-computed tomography analysis revealed a significant increase in trabecular thickness and density in pamidronate-treated groups,underscoring the anabolic effects of bisphosphonates.Morphometric evaluation confirmed a marked reduction in osteoclast number and activity at graft interfaces.These combined radiological,histological,and biochemical data collectively demonstrate the efficacy of pamidronate as an adjunctive agent in enhancing bone repair outcomes following surgical intervention for tuberculous osteitis.CONCLUSION A single intravenous dose of pamidronate significantly enhances bone regeneration and prevents implant resorption following surgical treatment of tuberculous osteitis.The following prospective studies are needed.展开更多
文摘A recently published prospective study marks a breakthrough for congenital olfactory disorders in children.The study provides the first long-term,three-year follow-up data,robustly demonstrating the durable efficacy and safety of autologous nasal epithelial stem cell transplantation.This work reveals immense therapeutic potential for a condition traditionally considered untreatable.However,this milestone achievement also presents new challenges.To translate this pioneering therapy from a single-center success to a global standard,multicenter,controlled clinical trials must be initiated immediately.Only through rigorous validation can we ensure its widespread adoption and ultimately bring hope to millions of children worldwide.
文摘BACKGROUND Functional gastrointestinal disorders(FGIDs)in children present with chronic symptoms like abdominal pain,diarrhea,and constipation without identifiable structural abnormalities.These disorders are closely linked to gut-brain axis dysfunction,altered gut microbiota,and psychosocial stress,leading to psychia-tric comorbidities such as anxiety,depression,and behavioral issues.Under-standing this bidirectional relationship is crucial for developing effective,holistic management strategies that address physical and mental health.AIM To examine the psychiatric impacts of FGIDs in children,focusing on anxiety and depression and their association with other neurodevelopmental disorders of childhood,such as attention-deficit/hyperactivity disorder,emphasizing the role of the gut-brain axis,emotional dysregulation,and psychosocial stress.Key mechanisms explored include neurotransmitter dysregulation,microbiota imbalance,central sensitization,heightening stress reactivity,emotional dysregulation,and symptom perception.The review also evaluates the role of family dynamics and coping strategies in exacerbating FGID symptoms and contributing to psychiatric conditions.METHODS A narrative review was conducted using 328 studies sourced from PubMed,Scopus,and Google Scholar,covering research published over the past 20 years.Inclusion criteria focused on studies examining FGID diagnosis,gut-brain mechanisms,psychiatric comorbidities,and psychosocial factors in pediatric populations.FGIDs commonly affecting children,including functional constipation,abdominal pain,irritable bowel syndrome,gastroesophageal reflux,and cyclic vomiting syndrome,were analyzed concerning their psychological impacts.RESULTS The review highlights a strong connection between FGIDs and psychiatric symptoms,mediated by gut-brain axis dysfunction,dysregulated microbiota,and central sensitization.These physiological disruptions increase children’s vulnerability to anxiety and depression,while psychosocial factors-such as chronic stress,early-life trauma,maladaptive family dynamics,and ineffective coping strategies-intensify the cycle of gastrointestinal and emotional distress.CONCLUSION Effective management of FGIDs requires a biopsychosocial approach integrating medical,psychological,and dietary interventions.Parental education,early intervention,and multidisciplinary care coordination are critical in mitigating long-term psychological impacts and improving both gastrointestinal and mental health outcomes in children with FGIDs.
文摘Pediatric inflammatory bowel disease(PIBD)is a chronic inflammatory disorder of the gastrointestinal tract,with rising global incidence and prevalence.Over the past two decades,biologics have added to the therapeutic armamentarium and revolutionized the approach to treatment of inflammatory bowel disease.The available biologics include monoclonal antibodies which target inflammatory cytokines(anti-tumor necrosis factor alpha,anti-interleukin 12/23)or recruitment of leucocytes to the gastrointestinal tract(anti-alpha4beta7 integrin)and small molecules(Janus kinase inhibitors,sphingosine 1-phosphate-inhibitors)which modify the proinflammatory signaling.Considering their potential disease-modifying ability,recent pediatric guidelines from the West have advocated upfront use of biologics in appropriate clinical scenarios as a top-down approach rather than the conventional step-up approach.Although real-world studies are available regarding the clinical efficacy of biologics in PIBD,there is paucity of long-term outcome and safety data in children.Also,little information is available about the best approach in the newly industrialized-developing countries where PIBD is rising but at the same time,infections are prevalent and resources are limited.In this review,we summarize the efficacy and safety profile of biologics and small molecule drugs and discuss the challenges in the management of PIBD,especially in the developing world,and future directions.
文摘BACKGROUND Pediatric short bowel syndrome(SBS)poses management challenges,and teduglutide is a potential therapy.However,comprehensive data on its pediatric safety are lacking.AIM To evaluate the impact of teduglutide on infection and gastrointestinal adverse events in pediatric SBS patients via systematic review and meta-analysis.METHODS Following PRISMA 2009 guidelines and PROSPERO registration,we searched PubMed,Web of Science,and EMBASE for randomized controlled trials(RCTs)(pediatric SBS patients≤18 years;teduglutide vs placebo/standard care).Two reviewers screened studies,extracted data,and assessed bias(ROB2).Metaanalyses used RevMan 5.4(Mantel-Haenszel method,random-effects if I^(2)≠0).Trial sequential analysis and GRADE were applied.RESULTS Three RCTs involving 115 pediatric patients were included.Pooled analysis revealed no statistically significant differences between the teduglutide and control groups for the primary outcome of infection events[RR=0.83;(95%CI:0.44-1.56);P=0.57;I^(2)=0%;2 studies,n=55].Similarly,no significant differences were found for secondary outcomes:Upper respiratory tract infection[RR=0.68;(95%CI:0.32-1.47);P=0.33;I^(2)=0%],catheter site infection[RR=1.86;(95%CI:0.23-14.78);P=0.56;I^(2)=0%],vomiting[RR=1.35;(95%CI:0.10-18.23);P=0.82;I^(2)=72%],abdominal pain[RR=2.47;(95%CI:0.50-12.16);P=0.27;I^(2)=0%],nausea[RR=1.31;(95%CI:0.24-7.22);P=0.75;I^(2)=0%],diarrhea[RR=1.02;(95%CI:0.23-4.43);P=0.98;I^(2)=0%],and abdominal distension[RR=1.49;(95%CI:0.18-12.35);P=0.71;I^(2)=0%].The overall certainty of evidence assessed by GRADE was moderate.CONCLUSION Teduglutide does not increase infection or gastrointestinal adverse event risk in pediatric SBS,but small sample sizes limit conclusions.Larger studies are needed.
文摘Hepatitis A virus(HAV)infection remains a significant public health concern in many developing countries.The annual incidence of HAV infection is 1.5 million,though this figure may be underestimated owing to the infection’s asymptomatic nature and the presence of milder disease variants.The clinical spectrum of HAV infection now ranges from asymptomatic infection to fulminant hepatitis.Despite the availability of safe and highly effective vaccines,HAV infections remain a major contributor to acute viral hepatitis worldwide.
文摘This article examines the growing prevalence of pediatric obesity and its con-nection to metabolic dysfunction-associated steatotic liver disease(MASLD)in children and adolescents,focusing on the role of glucagon-like peptide-1 receptor agonists in treatment.Pediatric obesity and MASLD present significant long-term health risks,making early intervention crucial.The article reviews the patho-physiology of both pediatric obesity and MASLD,explores current therapeutic strategies,and discusses the emerging role of glucagon-like peptide-1 receptor agonists,such as liraglutide,semaglutide,exenatide,and dulaglutide,in managing obesity,as well as explores current limited pediatric literature on the use of these medications in MASLD.
文摘BACKGROUND Gastrointestinal diseases in young children are often anatomic or inflammatory in nature and can present with symptoms similar to those of Cow’s milk protein allergy(CMPA),complicating diagnosis.This case series highlights 3 pediatric patients initially misdiagnosed with CMPA,emphasizing the need for a thorough evaluation.CASE SUMMARY Case 1:A 3-year-old child with chronic abdominal distension and constipation was initially treated for CMPA and was later diagnosed with Hirschsprung disease through rectal biopsy.Surgical intervention involved a laparoscopic colostomy followed by a pull-through procedure,leading to a successful recovery.Case 2:A 2-month-old infant presented with greenish-yellow vomiting and abdominal distension.Initially misdiagnosed with CMPA,further investigation using barium studies revealed partial intestinal malrotation.The patient underwent a laparoscopic Ladd’s procedure and recovered well.Case 3:A 6-month-old infant with persistent vomiting and failure to thrive had been treated for CMPA.Detailed imaging studies indicated achalasia.The child underwent a Heller myotomy,which resulted in significant symptom improvement and weight gain CONCLUSION Thorough evaluation of gastrointestinal symptoms is necessary in children.A high suspicion for alternative diagnoses will prevent delays in accurate diagnosis and proper treatment,leading to improved outcomes.
文摘The surgical treatment of severe scoliosis and kyphoscoliosis in the pediatric population is complicated and has high morbidity and mortality risks.Severe scoliosis has traditionally been defined by a coronal Cobb angle of greater than 90°or 100°.The usual corrective methods for these patients have been anterior or posterior release and osteotomies using a combined anterior-posterior or posterior-only approach.Many of these patients have pre-existing pulmonary compromise;therefore,an anterior approach is often not reasonable or possible.Acute correction of a deformity may also cause neurologic injury.Halo gravity traction(HGT)allows for progressive,gradual,and sustained correction of the spinal deformity in the coronal,sagittal,and axial planes,leading to a decrease in the amount of correction needed at definitive posterior fusion.This relates to decreased postoperative neurologic deficit and improved pulmonary function.Preoperative HGT has evolved to be the surgical adjunct in the treatment of severe spinal deformity.Indications for HGT,best protocols of application,and optimal duration of traction still lack uniformity;thus,a review of the literature remains relevant and necessary.This review summarizes the existing literature on HGT,including its indications,applications,duration of traction,and associated complications.
基金Supported by Takeda Argentina SA,No.TAK-633-4003.
文摘BACKGROUND Approval of teduglutide is an important addition to the limited treatment options for short bowel syndrome(SBS).However,real-world evidence on teduglutide therapy for SBS in Latin America is scarce.AIM To investigate the effectiveness and safety of teduglutide in clinical practice in Argentina with a 24-week follow-up.METHODS This non-interventional multicentre cohort study included consecutive patients(aged≥1 years)with SBS who were dependent on parenteral support(PS)and received≥1 dose of teduglutide according to currently approved indications.RESULTS The study population(n=45)included 21 adult and 24 pediatric patients.The proportion of adult and pediatric patients who showed clinical response(defined as a≥20%reduction in weekly PS volume)after 24 weeks of treatment was 90.4%[95%confidence interval(CI):69.6%-98.8%]and 83.3%(95%CI:62.6%-95.2%),respectively.Overall,12 patients(26.6%;95%CI:14.6-41.9)were weaned from PS support at the 24-week assessment,6(28.5%;95%CI:11.5-52.1)in the adult cohort,and 6(25%;95%CI:9.7-46.7)in the pediatric cohort.Only baseline PS requirement was inversely associated with weaning from PS(P=0.025).The most frequently reported treatmentemergent adverse events(TEAEs)were mild to moderate abdominal pain and abdominal distension(16.6%;and 9.5%,respectively).None of the reported TEAEs led to treatment discontinuation.CONCLUSION This prospective real-world study demonstrated the effectiveness and safety of teduglutide in adult and pediatric patients with SBS in Argentina.The clinical response observed in both adults and pediatric patients was greater than that reported in phase 3 trials and was consistent with the results of other real-world studies.
基金Hubei Provincial Department of Education Humanities and Social Sciences Youth Project(Project No.:19Q137)Hubei Provincial Department of Education Humanities and Social Sciences Youth Project(Project No.:23Q183)。
文摘Objective:To investigate the efficacy of a tripartite collaborative nursing intervention in enhancing resilience among families of pediatric leukemia patients.Methods:Based on Walsh’s family resilience theory and collaborative nursing principles,and after reviewing a large amount of literature,a tripartite intervention was constructed,which was led by the nursing team,coordinated by medical social workers,and supported by volunteers.Caregivers of pediatric hematology inpatients at a tertiary-level Class A hospital were selected as research subjects.which were divided into a control group(n=30)and an experimental group(n=30)according to a randomized block design.The control group are treated with standard nursing care,which included health education,counseling,and psychological support from nurses.The experimental group,in addition,was provided with a tripartite collaborative nursing intervention for 3 months.Results:The family resilience level,social support,and family function scores of the experimental group were higher than those of the control group,and the differences were statistically significant(P≤0.05).Conclusion:The implementation of tripartite collaborative nursing intervention improves the resilience of families of children suffered from leukemia.
基金Guangzhou Municipal Science and Technology Bureau,Municipal School and College Joint Funding Project,No.2024A03J1240.
文摘BACKGROUND Despite advances in treatment,the prognosis for patients with high-risk pediatric solid tumors remains dismal.Tandem autologous stem cell transplantation(ASCT)offers promise for improving outcomes in these patients.This study aimed to examine the efficacy and prognostic factors of tandem ASCT in pediatric patients with high-risk solid tumors.AIM To determine the survival outcomes and prognostic factors in pediatric patients with high-risk solid tumors undergoing tandem ASCT.METHODS A total of 40 pediatric patients with high-risk solid tumors treated from March 2015 to August 2022 were included in this retrospective study.The diagnoses of the patients included neuroblastoma,germ cell tumors,atypical teratoid/rhabdoid tumor,medulloblastoma,and pineoblastoma.After induction chemotherapy,all patients received tandem ASCT and were allocated into two groups(group A and group B)based on high-dose chemotherapy regimens.Prognostic relevance was evaluated by examining patient characteristics,such as sex,age,lactate dehydrogenase levels,primary site,the number of metastatic sites,and bone marrow involvement.RESULTS The median follow-up duration since the first ASCT was 24 months(range:1-91 months),with 5-year overall survival(OS)and event-free survival(EFS)rates of 73%and 70%,respectively,for the entire cohort.The 3-year OS rates were 67%for group A and 87%for group B(P=0.29),with corresponding 3-year EFS rates of 67%and 79%(P=0.57).Among neuroblastoma patients,the 5-year OS and EFS were 69%and 63%(P=0.23).Univariable analysis revealed a notable association of age≥36 months and elevated lactate dehydrogenase level at diagnosis with poorer OS.Despite acute adverse effects,all patients demonstrated good tolerance to the treatment,with no occurrences of transplant-related mortality.CONCLUSION Tandem ASCT demonstrates promising survival outcomes for patients with high-risk solid tumors,particularly neuroblastoma,with manageable toxicity and no transplant-related mortality.
文摘The significance of gut microbiota(GM)in human health is being increasingly researched.An imbalance in GM composition,known as dysbiosis,is linked to various and other health issues.In addition,antibiotics are the primary and most significant factors leading to major changes in the composition and function of the GM,which may result in colonization by antimicrobial-resistant(AMR)pathogens.Therefore,alternative antibiotic strategies for combating AMR pathogens are urgently needed.This narrative review highlights current knowledge regarding various pertinent strategies for decolonizing bacterial pathogens from GM and emphasizes decolonization therapies’critical role in pediatric surgical disorders.Strategies such as decontamination of the digestive tract utilizing antibiotics,the use of probiotics,and particularly fecal microbiota transplantation have introduced new options for clinical treatment.These treatments show the potential to restore GM balance and have demonstrated advantages for intestinal disorders related to pediatric surgery,including inflammatory bowel disease,neonatal necrotizing enterocolitis,Hirschsprung-associated enterocolitis,and short bowel syndrome.Despite GM therapeutics,recent strategies are still in their developmental phase and exhibit challenges that need further research.Thus,potential future directions for GMtargeted decolonization therapies are under consideration.Innovative alternative strategies to combat AMR though GM modulation in disorders related to pediatric surgery appear to be promising and should continue to be prioritized for further research and development.
文摘Background:Precision medicine is an emerging approach for treating pediatric cancer due to its ability to target tumor-specific genetic drivers rather than provide broad and aggressive treatments.The study aimed to outline the establishment and impact of a Precision Medicine Clinic(PMC)in the setting of pediatric oncology,with the objective of offering targeted treatment options within the institution and creating a scalable model for adoption by other healthcare systems to achieve a wider impact.Methods:Recognizing this need for an individualized approach to treating patients,Cook Children’s Medical Center(CCMC)established a multidisciplinary molecular tumor board in 2019,followed by the launch of an official PMC in 2021.Before this,there was no dedicated place to discuss and evaluate genetic sequencing results.Results:In 2022 and 2023,the PMC discussed 69 patients with a wide variety of oncologic diagnoses.Through the clinic’s efforts,133 genetic variants across 101 genes have been identified,spanning oncogenic pathways related to cell cycling,DNA processing,and cell signaling.Of the sequenced patients,four have received targeted therapy according to recommendations from the PMC.Conclusion:While the PMC continues to evaluate patients and their long-term outcomes,the continually growing PMC at CCMC represents the beginning of the advancement of treating pediatric oncology patients through the interpretation of genetic sequencing results,making actionable targeted treatment recommendations,and continuing to follow the patient’s course of care over time.This additionally provides a framework for starting a PMC that can be adapted for specific clinical needs and implemented broadly.
文摘Background:Psychosocial functioning and quality of life(QoL)are strongly associated with outcomes in pediatric heart transplant recipients.The data in pediatric transplantation,however,is limited.This study aims to investigate the associations of perioperative anxiety and depression with postoperative complications,sociodemographic and clinical characteristics.Methods:This observational,analytical,longitudinal study included 42 pediatric participants aged 8 to 16 years old.Preoperative psychological assessments were completed by 36 children,the remaining 6 were unable to participate due to invasive ventilation,extracorporeal membrane oxygenation(ECMO),and physical debilitation.Postoperatively,all 42 subjects completed the psychosocial evaluations.Data on recipients characteristics,family characteristics and clinical parameters were collected.Anxiety and depression were assessed using the Screen for Child Anxiety Related Emotional Disorders(SCARED)and the Depression Self-Rating Scale for Children(DSRSC).The Short Form-36 Health Survey(SF-36)was applied to assess the health-related QoL.Results:Before transplantation,91.7%(33/36)of the children exhibited symptoms of anxiety,and an identical proportion(91.7%,33/36)showed signs of depression.After transplantation,the rates of anxiety and depression decreased to 35.7%(15/42)and 11.9%(5/42),respectively.Longer disease course(p=0.042),preoperative hypoalbuminemia(p=0.032),older age(p=0.024),postoperative hypertension(p=0.011),and postoperative CRRT(p=0.015)result in depression symptoms.Preoperative hypoalbuminemia(p=0.032)was also more common in the anxiety group.Additionally,children with psychosocial risks had significantly lower QoL scores in general health(p=0.008)and mental health(p=0.015).Conclusions:Perioperative anxiety and depression are highly prevalent among pediatric heart transplant recipients.Although significant improvements in psychosocial risks were observed at posttransplantation,approximately 40%children continued to experience psychosocial challenges.
基金approved by the Institutional Review Board of Guangdong Provincial People's Hospital(KY-Q-2022-336-03).
文摘Objective:The aim of the present study was to investigate long-term efficacy and safety of percutaneous stent implantation for residual pulmonary artery stenosis(PAS)in pediatric patients after surgical repair of complicated congenital heart diseases(CHDs).Methods:All pediatric patients diagnosed with residual PAS after surgical repair of complicated CHDs between 1996 and 2020 were retrospectively enrolled in the study.Results:A total of 41 patients(30 males,11 females;median age 5.0 years,median weight 17 kg)were followed-up for a median of 7.1 years.Follow-up echocardiography results demonstrated that the target vessel diameter increased from(3.4±1.1)mm preoperatively to(6.2±1.9)mm one year post-procedure and(6.0±1.5)mm at the final follow-up(p<0.05).The pressure gradient across the stenosis decreased from(52.6±15.8)mmHg preoperatively to(35.8±19.1)mmHg one year post-procedure and(33.1±19.7)mmHg at the final follow-up(p<0.05).Cardiac computed tomography scans indicated that target vessel/distal vessel diameter ratio increased from(0.4±0.2)pre-operatively to(0.8±0.2)one year post-procedure and(0.9±0.3)at the final follow-up(p<0.05).A total of six adverse events were documented,comprising two cases of in-stent restenosis requiring surgical reintervention,three cases of in-stent restenosis managed with regular clinical surveillance,and one case of percutaneous pulmonary valve replacement due to severe pulmonary regurgitation.Kaplan-Meier event-free survival analysis demonstrated that elevated preprocedural right ventricular systolic pressure(>72 mmHg)was significantly associated with long-term adverse events(p=0.024).Conclusion:Percutaneous stent implantation for residual PAS after surgical repair of complicated CHDs effectively relieves vessel stenosis,stabilizes cardiac function,and improves long-term prognosis in pediatric patients.In-stent restenosis remains an unresolved complication,necessitating further advancements in interventional strategies.
文摘Objective: This study aimed to demonstrate the feasibility of laparoscopic-resection of the abdominal cystic lymphangiomas in Pediatric Surgery and describe the morbidity associated to this management in Queen fabiola children’s university hospital (HUDERF) in Brussels. Methods: We retrospectively conducted a study at the Pediatric Surgery Department of HUDERF, Brussels. The studied period was from January 1, 2014, to January 1, 2024;a span of 10 years. All patients with a confirmed diagnosis of cystic lymphangiomas and who underwent laparoscopic surgery were included in our study. Those who have been operated exclusively by open surgery have been excluded. The technique involved either total laparoscopic resection or laparoscopic-assisted with extra-abdominal resection of the tumor. Parameters that were studied included age, sex, weight, symptoms, preoperative diagnosis, imaging assessment, location, size of the tumor, type of mass, surgical procedure, duration of the surgery, conversion to open surgery, morbidity, and histopathology. Data were analyzed using Microsoft Office Excel 2010 and SPSS. Results: We retrieved 10 files of patients presenting with abdominal cystic lymphangiomas within two patients underwent exclusive open surgery and have excluded from our study. Then our sample was constituted with 8 patients. The mean age of the patients was 6.4 years (standard deviation: 3.6 years, range: 1 to 11 years). Male patients were predominant. The mean weight was 26.7 kg (standard deviation: 14.7 kg, range: 10 to 55 kg). The most common symptom was abdominal pain. Preoperative diagnosis of abdominal cystic lymphangioma was made in 8 cases. Abdominal ultrasound was performed in all patients. MRI was done in 5 patients, and CT scan in 2 patients. All patients presented a multicystic mass. Pure laparoscopic resection of the cyst was done in 2 cases. Laparoscopic-assisted resection in 4 cases (with extra-peritoneal with small bowel resection and mesenteric detorsion in 1 case), and conversion in 2 cases due to the complex location of the cyst. After a follow-up period of 5 years, morbidity was noted in one patient (Patient 3) who developed postoperative bowel obstruction 1 month post-surgery. This patient was re-operated on with a favorable clinical outcome following conventional small bowel resection and anastomosis. The other patients (1, 2, 4, 5, 6, 7 and 8) had a simple clinical course, and no recurrence was observed in our series. Conclusion: Laparoscopic-resection of the abdominal cystic lymphangiomas is feasible in Pediatric Surgery. As minimally invasive surgery it gives many advantages even for complex abdominal cystic lymphangiomas with less morbidity as shown in our series.
文摘Acute alithiasic cholecystitis (AAC) is the inflammatory disease of the gallbladder in the absence of gallstones. It is a rare pathology in children. The aim was to describe the clinical, diagnostic and therapeutic characteristics of this disease. Materials and Method: This was a 3-year retrospective study (January 2008 to December 2010) including 66 patient records collected for CAA. Results: The frequency of AAC was 3.57%, the mean age of the patients was 8 ± 3.52 years, and there were 36 boys and 30 girls. The main clinical features were right hypochondrial pain (66 cases), positive Murphy’s (66 cases), fever (53 cases) and jaundice or sub-jaundice (51 cases). The main ultrasound signs were thickened vesicular wall 50 cas (75.76%), vesicular distension 50cas (75.76%) and positive ultrasound Murphy 47 cas (71.21%). Medical treatment was exclusive in 64 patients (96.97%) and surgical treatment in 2 patients (3.03%). Conclusion: Acute alithiasic cholecystitis is a rare pathology in children, the clinical picture is not very specific and abdominal ultrasound is the key to early diagnosis. Conservative treatment is the first choice in the absence of any complications.
文摘I<span><span style="font-family:Verdana;">ntroduction: Acute thoracic syndrome is the appearance of a new pulmonary </span><span style="font-family:Verdana;">infiltrate</span><span style="font-family:Verdana;"> on radiology associated with fever, desaturation </span><span style="font-family:Verdana;">or</span><span style="font-family:Verdana;"> respiratory signs. It is the second leading cause of hospitalization and</span></span><span style="font-family:Verdana;"> the first cause of death in sickle cell patients. It is an acute pulmonary complication whose pathophysiological mechanisms are still poorly understood. This study aims to</span><span><span style="font-family:Verdana;"> study the epidemiological, clinical, therapeutic </span><span style="font-family:Verdana;">and</span><span style="font-family:Verdana;"> evolutionary aspe</span></span><span style="font-family:Verdana;">c</span><span><span style="font-family:Verdana;">ts of Acute Chest Syndrome in children at the Pediatrics Department of Donka National Hospital. Method: This is a prospective study of descriptive type for a period of 6 months from February 19 to August 19, </span><span style="font-family:Verdana;">2019</span><span style="font-family:Verdana;"> on patients with sickle cell disease who developed an ATS in the pediatrics department of Donka National Hospital. Epidemiological, clinical, therapeutic </span><span style="font-family:Verdana;">and</span><span style="font-family:Verdana;"> evolutionary data were studied and proportionate data were calculated. Results: The frequency of ATS was 39%. The mean age of our patients was 9.83 years with the extremes of</span></span><span style="font-family:Verdana;"> 4 and 16 years. The age group from 6 to 10 years with a frequ</span><span style="font-family:Verdana;">ency of 66.7% was the most affected. Fever was the main clinical manifestation, followed by hepatome</span><span><span style="font-family:Verdana;">galy. All our patients were homozygous SS and undergoing folic acid prophylaxis. 96% of our patients did not receive any specific vaccine. Antibiotic therapy, hyperhydration </span><span style="font-family:Verdana;">and</span><span style="font-family:Verdana;"> analgesics were administered to all our patients. 96% of our patients were transfused with red blood cell concentrate. 96% of our patients were transfused with packed red blood cells. 96% of our patients were transfused with packed red blood cells and 96% of them had a favorable outcome. Conclusion: ATS is an acute co</span></span><span style="font-family:Verdana;">mplication of sickle cell disease responsible for significant mortality and morbidity in the pediatric population. Its treatment is symptomatic and must be started early. Emphasis must be placed on prevention to prevent or limit its occurrence.</span>
文摘Hodgkin lymphoma(HL)is a heterogenous lymphoproliferative disorder of B-cell origin and represents one of the most common malignancies in children and young adults.In addition to well-known underlying factors-such as Epstein-Barr virus infection-the familial aggregation demonstrated in large population studies suggested a genetic predisposition.First-degree relatives of patients with HL have an approximately threefold increased risk of developing the disease compared to the general population.These observations have recently prompted several whole-genome studies in affected families,identifying variants possibly implicated in lymphomagenesis,including alterations in DICER1(a member of the ribonuclease III family),POT1(protection of telomeres 1),KDR(kinase insert domain receptor),KLHDC8B(kelch domain-containing protein 8B),PAX5(paired box protein 5),GATA3(GATA binding protein 3),IRF7(interferon regulatory factor 7),EEF2KMT(eukaryotic elongation factor 2 lysine methyltransferase),and POLR1E(RNA polymerase I subunit E).In this article,we review current insights into the etiopathogenesis and risks of familial HL,and present case reports involving two sisters diagnosed with HL nearly 17 years apart.Recognizing the risk for first-degree relatives may potentially increase awareness of early symptoms among family members of HL patients,leading to earlier diagnosis and better outcomes.Conversely,understanding that the hereditary risk,though higher than in the general population,remains relatively low may provide reassurance for affected families.
基金Supported by Russian Science Foundation Grant,No.24-15-00185.
文摘BACKGROUND Tuberculous osteitis is a chronic,granulomatous bone infection that frequently results in impaired bone healing following surgery.Despite surgical intervention and prolonged anti-tuberculous therapy,complete bone regeneration often remains unachieved,contributing to subsequent orthopedic complications.AIM To investigate the efficacy and safety of pamidronate in promoting bone regeneration following surgical treatment of experimental animal tuberculous osteitis.METHODS A controlled randomized basic study of rabbit femoral tuberculosis induced by Mycobacterium tuberculosis strain H37Rv included surgical removal of infected tissue and implantation of osteoinductive bone grafts with the following animal allocation to one of three groups:(1)Bisphosphonates alone;(2)Bisphosphonates combined with anti-tuberculous therapy;and(3)Anti-tuberculous therapy alone.The control group consisted of animals that received no surgical or medical treatment.Clinical evaluations,biochemical markers,micro-computed tomography imaging,and histomorphometry analyses were conducted at 3 months and 6 months postoperatively.RESULTS Pamidronate treatment significantly reduced early implant resorption,increased osteoblastic activity,improved trabecular bone regeneration,and maintained graft integrity compared to the anti-tuberculous therapy-only group.Histologically,pamidronate led to enhanced vascular remodeling and increased bone matrix formation.Crucially,bisphosphonate therapy demonstrated safety,compatibility with anti-tuberculous medications,and did not exacerbate tuberculous inflammation.Furthermore,micro-computed tomography analysis revealed a significant increase in trabecular thickness and density in pamidronate-treated groups,underscoring the anabolic effects of bisphosphonates.Morphometric evaluation confirmed a marked reduction in osteoclast number and activity at graft interfaces.These combined radiological,histological,and biochemical data collectively demonstrate the efficacy of pamidronate as an adjunctive agent in enhancing bone repair outcomes following surgical intervention for tuberculous osteitis.CONCLUSION A single intravenous dose of pamidronate significantly enhances bone regeneration and prevents implant resorption following surgical treatment of tuberculous osteitis.The following prospective studies are needed.
