Therapeutic laparoscopy for superior mesenteric artery syndrome in 5 children: Treatment experience and lite-rature review北大核心CSCD

Objective To investigate the clinical characteristics and therapeutic laparoscopy treatment for superior mesenteric artery syndrome(SMAS) in children. Methods A retrospective study on 5 children with SMAS who failed to respond to the conservative treatment in the Second Hospital of Shandong University from March 2017 to March 2022 was carried out.All the patients underwent the laparoscopic lysis of the ligament of Treitz and duodenal lowering procedure.The clinical data were analyzed and literature retrieved from Wanfang and PubMed databases were reviewed. Results There were 3 boys and 2 girls in the 5 cases with SMAS.The average age was (12.4±1.4) years, and the preoperative body mass index was (15.2±0.8) kg/m2.The patients were admitted to the hospital with vomiting, abdominal pain and emaciation symptoms, and the duration of symptoms was (8.4±7.8) months.Gastroscopy, laparoscopic lysis of the ligament of Treitz and duodenal lowering procedure were performed successfully in all the patients, without conversions to open surgery.The average operation duration was (66.0±10.7) min.Food intake was allowed 1 day after surgery.Chylous fistula occurred 4 days after operation in one patient, whose symptom relieved after 11 days of conservative treatment.One patient still had nausea and vomiting symptoms 10 days after operation and was healed by knee-chest posture treatment.No other short-term complications were observed.The median postoperative duration of stay in the hospital was 7 (6-22) days.The patients were followed up for 9-56 (median: 21) months.All of them recovered well and gained weight.One patient had mild recurrent symptoms, and was cured after conservative treatment.A total of 15 literature on the treatment of SMAS by laparoscopic lysis of the ligament of Treitz was retrieved, and the cure rate was 40%-100%.Only one group of these cases had a cure rate below 75%. Conclusions The laparoscopic lysis of the ligament of Treitz and duodenal lowering procedure is safe, effective, simple and minimally invasive treatment for children with SMAS.It can be used as the first choice for most pediatric patients. © 2022 Chinese Journal of Applied Clinical Pediatrics. All rights reserved.     

A review of the risk factors associated with juvenile-onset recurrent respiratory papillomatosis:genetic,immune and clinical aspects

Background Juvenile-onset recurrent respiratory papillomatosis(JoRRP)is one of the most common benign lesions of hyperplastic respiratory epithelial tissue in children and is predominantly caused by human papillomaviruses(HPVs)6 and 11.The clinical course of the disease is variable,and some patients even develop a malignancy.The purpose of this review was to summarize the related factors affecting the disease course in patients with JoRRP.Data sources We used databases,including PubMed and Google Scholar,to search for publications on factors associated with the genetic,immune,and clinical aspects of JoRRP.The most relevant articles to the scope of this review were chosen for analysis.Results Mother-to-child transmission is the most important mode of disease transmission;other factors,such as immune condition or genetic susceptibility,may be important determinants of JoRRP occurrence.Genetically,the presence of DRBI*0301 and HFV 6/11 E6/E7 is associated with a more severe disease.Immunewise,patients have an enhanced T helper 2-like response.In addition,regulatory T cells are enriched in tumors and may become one of the effective prognostic indicators.For clinical characteristics,patients infected with HPV-11 have more aggressive disease.However,compared with HPV type,age at first onset is a more important factor related to the aggressiveness of JoRRP.Furthermore,socioeconomic status may also affect the course.Conclusions Genetic,immune,and some clinical factors have been noted to play an important role in the course of JoRRP.Exploring definite influencing factors will be an important direction of research in the future.     

Clinical practice guidelines in multisystem inflammatory syndrome(MIS-C)related to COVID-19:a critical review and recommendations

Background Multisystem inflammatory syndrome in children(MIS-C)is a serious health condition that develops from and is linked to coronavirus disease 2019.MIS-C is considered a multi-organ dysfunction involving cardiac,renal,respiratory,hematologic,gastrointestinal and neurological symptoms and groups of signs and symptoms such as rash or bilateral non-purulent conjunctivitis,hypotension or shock and acute gastrointestinal problems,which require immediate therapeutic intervention to prevent the aggravation of the patient’s health condition.MIS-C is relatively new in the field of evidence-based medicine;however,there are several clinical guidelines for good clinical practice.For every disorder,the guidelines have different suggestions.Hence,based on the current status of the evidence,recommendations have been combined to form a unified guideline for therapeutic management.Methods This paper compares and evaluates the current MIS-C-specific clinical practice guidelines(namely,American Academy of Pediatrics,American College of Rheumatology,Helen DeVos Children’s Hospital Foundation,Children’s Hospital of The King’s Daughters,and the Infectious Diseases Society of America).The compiled literature was then assessed by the authors separately,and an algorithm was proposed for each disorder,taking into consideration the various guidelines proposed for the management of the disorder.Results The features of MIS-C patients are unified;this is very helpful in managing its symptoms and decreasing mortality rates.In addition,recommendations for pharmacological treatment for MIS-C symptoms are formulated after cross-comparison across five different guidelines.Conclusions This study provides a general interpretation of the results in the context of other evidence and implications for future research.It proposes a unified guideline based on the current evidence,with the best potential to maintain suitable clinical standards in the Saudi Arabian Ministry of Health.     

孕早期胎儿颈项透明层在产前超声筛查胎儿畸形中价值的Meta分析

ObjectiveTo evaluate the value of nuchal translucency (NT) as a screening test for fetal abnormalities in the first trimester ultrasound examination.MethodsA search in Cochrane Library, PubMed, OVID, Springer, China National Knowledge Infrastructure (CNKI) and Chinese Bio-edicine Database（CBM）was performed to identify relevant English and Chinese articles between Jan 1990 and August 2008. Criteria for inclusion were established based on validity criteria for diagnostic research. Subsequently, the characteristics of the included articles were extracted. Statistical analysis was performed with Meta-iSc 1.4. Heterogeneity of the included articles was tested to select proper effect model to calculate pooled weighted sensitivity and specificity. Summary receiver operating characteristic curve(SROC) was made and the area under the curve (AUC) was calculated. Finally, sensitivity analysis was performed.ResultsTwelve articles were included, with a total of 112 099 fetuses. Nine articles meeting inclusion criteria were analyzed for the value of screening for cardiac abnormalities by nuchal translucency thickness above the 95th percentile for gestational age. The pooled sensitivity and specificity was 29.8%, 96.9%，respectively, AUC=0.804 7. Seven articles meeting inclusion criteria were analyzed for the value of screening for cardiac abnormalities by nuchal translucency thickness above the 99th percentile for gestational age. The pooled sensitivity and specificity was 18.9%, 99.3%，respectively, AUC=0.971 2. Five articles meeting inclusion criteria were analyzed for the value of screening for fetal abnormalities by nuchal translucency thickness above the 95th percentile for gestational age. The pooled sensitivity and specificity was 25.0%, 98.3% respectively, AUC=0.183 0. The sensitivity of screening for cardiac abnormalities by nuchal translucency thickness above the 95th percentile for gestational age was significantly higher than the nuchal translucency thickness above the 99th percentile for gestational age（χ2=6.58， P<0.05）,no difference was found in the specificity. The random?瞖ffect model was used in the analysis of screening for cardiac abnormalities by nuchal translucency thickness above the 95th or 99th percentile for gestational age because of the heterogeneity. The fixed-ffect model was used in the analysis of screening for fetal abnormalities by nuchal translucency thickness above the 95th percentile for gestational age because of the homogenicity. ConclusionsThe results suggest that screening for fetal abnormalities by NT thickness above the 95th percentile for gestational age has a low accuracy and is unsuitable for prenatal screen. At present it is incompletely clear on the sensitivity and specificity of screening for different kinds of fetal abnormalities by NT thickness. Screening for cardiac abnormalities by first trimester NT thickness has a high accuracy and specificity and can be reliably used in prenatal screen. We need the further systemic reviews to evaluate the value of NT as a screening test for different kinds of fetal abnormalities. Further multi?瞔entre and prospective studies are badly needed to be performed to optimize the standard of nuchal translucency thickness, evaluate the value of NT as a screening test for fetal abnormalities (especially cardiac abnormalities) in the first trimester ultrasound examination by a cost-effectiveness analysis, and develop an operation guideline on first trimester nuchal translucency thickness measurement for the prenatal screening of cardiac abnormalities in China.     

Juvenile dermatomyositis:advances in clinical presentation,myositis-specific antibodies and treatment

Background Juvenile dermatomyositis(JDM)is a chronic autoimmune disease characteristic by inflammation of small vessels within the skin,muscle and vital organs.But the clinical features and treatment of JDM have not been fully clarified.Data sources Databases underwent through PubMed for articles about the clinical features,myositis-specific antibodiesof JDM and its treatment,and we selected publications written in English which were relevant to the topic of this review.Results Clinical features and myositis-specific antibodies may predict the severity and prognosis of disease.Although the mortality rate has been lower with traditional treatments,such as corticosteroid,intravenous immunoglobulin,and diseasemodifying anti-rheumatic drugs such as methotrexate,their usages are variable.Novel biological therapies seem to be effective for refractory JDM patients,but more clinical trials are necessary.Conclusions JDM is a sever disease of childhood.We need to better understand recent advances of JDM in the context of clinical features including skin manifestations,muscle weakness and organ damage,myositis-specific antibodies and their associated outcomes and the treatment of disease.     

Group B <Emphasis Type="Italic">Streptococcus</Emphasis> causes severe sepsis in term neonates: 8 years experience of a major Chinese neonatal unit

Background:In contrast to industrialized countries,the clinical characteristics of neonatal sepsis caused by Group B Streptococcus (GBS) are largely unexplored in China.Methods:A retrospective case series study was performed at a high-capacity neonatal unit in Shanghai,China from January 2008 to December 2015.Clinical characteristics of neonates with culture-proven GBS sepsis and antibiotic susceptibility of isolated strains were analyzed.Results:Forty-three term neonates were included during the study period.The majority (74.4％) had early-onset sepsis with symptoms of respiratory distress.Meningitis was significantly more common in lateonset sepsis than in early-onset sepsis (81.5％ vs.18.8％,P＜0.0001).Approximately one third of all patients (n=16)developed severe sepsis,defined as sepsis with organ dysfunctions,and respiratory dysfunction/failure was the most common (32.6％).The in-hospital mortality rate of GBS sepsis was 4.7％.Neonates who progressed to severe sepsis had significantly lower pH level at the onset of symptoms than those who did not (7.26±0.12 vs.7.39±0.05,P=-0.006).Treatment of severe GBS sepsis required lots of medical resources including extracorporeal membrane oxygenation.All tested GBS strains were susceptible to penicillin,but the rate of resistance to clindamycin (84.0％) and erythromycin (88.0％) was high.Conclusions:GBS as a pathogen for neonatal sepsis has been receiving little attention in China.Our data demonstrated that GBS sepsis was likely to be fulminant.Early recognition followed by antibiotics and adequate supportive therapies was critical for successful treatment.Chinese clinicians should be aware of GBS infection when treating neonatal sepsis,especially in the absence of universal maternal GBS screening.     

Etiology and clinical features of viral bronchiolitis in infancy

Background:Bronchiolitis is a common lower respiratory tract infection in infancy.The aim of this review is to present the clinical profile of viral bronchiolitis,the different culprit viruses and the disease severity in relation to the viral etiology.Data sources:Databases including PubMed and Google Scholar were searched for articles about the clinical features of bronchiolitis and its viral etiology.The most relevant articles to the scope of this review were analyzed.Results:Currently there are two main definitions for bronchiolitis which are not identical,the European definition and the American one.The most common viral pathogen that causes bronchiolitis is respiratory syncytiai virus which was identified in 1955;now many other viruses have been implicated in the etiology of bronchiolitis such as rhinovirus,adenovirus,metapneumovirus,and bocavirus.Several studies have attempted to investigate the correlation of bronchiolitis severity with the type of detected virus or viruses.However,the results were not consitent.Conclusions:For the time being,the diagnosis of bronchiolitis remains clinical.The isolation of the responsible respiratory pathogens does not seem to confer to the prognosis of the disease severity.     

腹腔镜辅助肛门直肠成形术致后尿道憩室的预防措施探讨CSCD

Objective To explore the preventive measures of posterior urethral diverticulum (PUD) after laparoscopic assisted anorectoplasty (LAARP). Methods From August 2013 to December 2018, perioperative and postoperative clinical data were reviewed for 6 children with anorectal malformation (ARM) developing PUD after LAARP. PubMed database was searched for all published English literatures of PUD after ARM surgery from 2000 to 2021. Since international naming of PUD is not standardized, there are two major names, i. e. remnant of original fistula and PUD. Thus retrieval keywords were anorectal malformation, remnant of original fistula and PUD. Results The diagnostic age was 17 months to 5 years after LAARP. The clinical manifestations included mucuria, recurrent urinary tract infection and dysuria, etc. Computed tomography (CT) and retrograde urethrography revealed prerectal cyst. A total of 13 articles were retrieved, reporting 102 cases of PUD after ARM surgery. The age was 1 — 44 years and the diameter of PUD 3 —70 mm. The major clinical symptoms were frequent urination, dysuria, recurrent urinary tract infection, urinary calculi, hematuria and urinary incontinence, etc. Some non-symptomatic cases were only discovered accidentally during follow-ups. ARM procedures included LAARP (n = 36), posterior sagittal anorectopasty (PSARP,n = 15), abdominoperineal pull-through (n=15) and APP (n=50). Conclusion PUD is a rare complication after ARM surgery. And potential risks of PUD may be minimized by strengthening perioperative managements and improving surgical techniques. © 2022, Journal of Clinical Pediatric Surgery. All rights reserved.     

Prevention and treatment strategies for esophageal stenosis after thoracoscopy for type Ⅲ esophageal atresia北大核心CSCD

Objective To explore the roles of esophageal stenosis control strategies in children with esophageal atresia (EA). Methods Retrospective analysis was conducted for clinical data of 56 children with type Ⅲ EA undergoing thoracoscopy from January 2017 to December 2022.There were 30 boys and 26 girls.The clinical types were ⅢA (n=12) and ⅢB (n=11) in group A. Birth weight was (2573.26±112.77) gram and operative age (31.70±5.86) hours.Eight cases had concurrent congenital heart disease (CHD). And 33 patients received traditional postoperative observation (group B). The clinical types were ⅢA (n=18) and ⅢB (n=15) in group B. Birth weight was (2 598.79±94.88) gram and operative age (37.00±7.15) hour.Six cases had concurrent CHD.Preoperative profiles and postoperative recovery of two groups were compared along with differential efficacy between the prevention and treatment strategy after comparing EA and traditional management mode. Results No statistically significant inter-group differences existed in operative age (P=0.593), body weight (P=0.863) or proportion of CHD (P=0.438). One-stage surgery was performed for repairing tracheoesophageal fistula and esophageal reconstruction.Later they were cured and discharged with regular follow-ups.The incidence of postoperative anastomotic stenosis was 26.09%(6/23) in group A versus 60.61%(20/33) in group B. The inter-group difference was insignificant (P=0.125). There were 0 cases (0%) of anastomotic leakage in group A versus 3 cases in group B (9.09%)(P=0.001). Average dilation frequency was[(0.61±0.27) vs (2.00±0.41), P=0.014]and incidence of postoperative refractory stenosis[0(0%) vs 4(12.12%), P=0.001]. The differences were statistically significant. Conclusions Perioperative comprehensive management plays some roles in the prevention and treatment of esophageal stricture after EA thoracoscopy.The clinical outcomes are satisfactory. © 2023 Chinese Medical Journals Publishing House Co.Ltd.. All rights reserved.     

Drug-induced Stevens-Johnson syndrome and toxic epidermal necrolysis in children: 20 years study in a tertiary care hospital

Background:Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe life-threatening skin conditions.The most common cause of these manifestations is medications.Beside discontinued of the culprit drug,systemic corticosteroids were used as a primary treatment option among pediatric population.This study aimed to explore causative drugs (drug group/ latent period),treaments,complications,and treatment outcome (morbidity,mortality,length of hospital stay) of SJS and TEN in children.Methods:A retrospective chart was reviewed during the period of 1992 to 2012 at Srinagarind Hospital,Faculty of Medicine,Khon Kaen University,Thailand.SJS and TEN were clinically diagnosed and confirmed by pediatric dermatologists.Other possible causes other than drug-induced SJS and TEN were excluded.Results:A total of 30 patients was recorded,including 24 (80％) SJS patients and 6 (20％) TEN patients.The mean age was 6.9 years (SD 4.4).Male to female ratio was 1.5:1.Antiepileptic drug group was the most common causative drug (n=18,60％),followed by antibiotic drug group (n=8,26.6％),and others (n=4,13.3％) which included nonsteroidal antiinflammtory drugs (NSAIDs) and chemotherapy drugs.Systemic corticosteroids were used in 29 patients (96.6％).Intravenous immunoglobulin was used in one TEN patient (3.3％).There was a medium correlation between time to treatment (systemic corticosteroids) and the length of hospital stay (Spearman correlation coefficient=0.63,P=0.005).Two TEN patients (6.6％) died.Conclusions:Carbamazepine was the most common causative drug of SJS and TEN in our study.The severity of skin detachment is not correlated to severity of ocular findings.However,the persistent of ocular complications up to one year is suggested for promptly appropriate ocular treatment in all SJS and TEN patients.Our data suggested that early administration of systemic corticosteroid may reduce the length of hospital stay and should be considered for the treatment of pediatric drug-induced SJS and TEN.     

早产儿视网膜病的危险因素分析及随访调查

研究早产儿视网膜病(retinopathy of prematurity,ROP)的发生率、高危因素、治疗与随访情况。方法对2005年7月-2007年12月温州医学院附属第一医院NICU收治的符合ROP筛查标准的早产儿,于生后2周开始由资深眼科医师开始行间接眼底镜检查眼底,并进行随访。结果434例早产儿中ROP的发生率为5.5%(24/434例),24例ROP中Ⅰ期19例,Ⅱ期3例,Ⅲ期2例。Ⅲ期阈值病变者行激光光凝治疗,全部患儿均恢复正常。对434例早产儿行单因素分析得出,胎龄、出生体重、住院时间、吸氧、吸氧浓度、吸氧时间、呼吸暂停、新生儿肺透明膜病(RDS)、肺表面活性剂(PS)的应用、机械通气、输血、光疗时间、感染与ROP的发生有相关性(P<0.05)。Logistic回归分析显示胎龄、出生体重、胎数、吸氧时间、光疗时间、代谢性酸中毒、母亲妊高症、颅内出血是影响ROP发生的主要因素。结论早产是ROP的根本原因,防治各种并发症、合理的氧疗是预防ROP的关键。建立完善有效的ROP筛查制度,早期发现、早期治疗ROP,可改善ROP的预后。     

术中判断肠活力三种方法的比较

术中准确地评价肠活力是外科医师面临的一个普遍问题。应用兔肠缺血模型，比较静脉荧光素、表面血氧测定和激光多普勒三种技术判断肠活力的精确性。结果：诊断效率静脉荧光素法为７８％，表面血氧测定为６８％，激光多普勒为９５％。后者精确度显著优于前二者，且操作简便、迅速，具有临床应用前景。     

Spectrum of clinical presentations of vein of Galen aneurysm

Aneurysm of the vein of Galen is a rare intracranial vascular malformation. It is known to have diverse manifestations and varying severity. Four cases with different modes of presentation and outcome are reported. A mortality of 50 per cent was encountered. Among the survivors, one had neurologic sequelae whereas the other had attained age-appropriate developmental milestones. The former was a rare case of spontaneous thrombosis of the aneurysm while the latter was a boy who underwent therapeutic embolization.     

Outcome of the Treatment of Inborn Errors of Metabolism

The use of specific dietary restrictions, cofactor administration, mobilisation of insoluble substances, environmental modifications, product replacement and selective enzyme inhibition are now established for the treatment of some inborn errors of metabolism. There is no generally accepted application for enzyme administration, cytopharmacology (manipulation of the cytoskeleton) or for cell transplantation except for bone marrow transplantation in disorders where the bone marrow is primarily at fault. The other uses of bone marrow transplantation which have been proposed require further evaluation. Results of recent research suggest that the scope of this approach is gradually being widened. There is also scope for development in the field of organ transplantation taking advantage of recent technical¹ and immunological progress. The treatment of inborn errors of metabolism by genetic modification is not yet on a practical clinical level; more laboratory and animal studies are needed before this can be attempted in man. Adenosine deaminase deficiency appears to be the disease in which this will be first attempted using a retroviral vector to insert the gene into the genome of pluripotential bone marrow cells.     

婴幼儿法乐四联症手术治疗近期疗效的影响因素

目的 探讨影响婴幼儿法乐四联症手术治疗近期疗效的各相关因素.方法 将2003年8月至2011年2月经作者一期手术纠治的117例年龄≤3岁的法乐四联症患儿分为疗效良好、疗效较差两组.分析手术时患儿年龄、体重、术前HCT值、McGoon指数、EDVI、主动脉阻断时间、转流时间、室间隔缺损大小、升主动脉与肺动脉干直径比、...     

主动脉缩窄的治疗研究进展

主动脉缩窄(coarctation of aorta,CoA)是主动脉的局限性狭窄,狭窄最常见于峡部,是一种常见的先天性心血管畸形,占所有先天性心脏病的5％ ～8％.缩窄导致上肢血压升高,下肢血压降低,并可引起心功能下降,未经治疗的CoA预后不佳.治疗方式包括外科治疗及经皮介入治疗,不同治疗方式的并发症发生率不同,术后长期监测其并发症、心功能是评估预后的重要指标.该文对主动脉缩窄的治疗方式及术后并发症、心功能情况的研究进展作一综述.     

早产儿脑病研究进展

早产儿脑病是复杂的原发性脑损伤和继发性脑发育异常疾病.过去几年对该病有了较为深刻的认识,该文就最新的有关早产儿脑病概念演变,脑损伤后的细胞分子机制和损伤后发育障碍,尤其是活化小胶质细胞介导的少突胶质细胞损伤机制、神经/髓鞘受损和丘脑、板层下神经元及大脑皮层的受损机制或发育成熟障碍进行综述,以便对该病有更深刻的认识.     

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??Inhaled corticosteroids??ICS?? are the most effective medicine for chronic airway inflammation nowdays. Atomization inhalation has been widely applied in clinics because of its efficacy??fewer side-effects and convenience. Here??we focused on some points which should be paid attention to??including how to choose appropriate patients??how to ensure the effectiveness of inhaled steroids and how to reduce possible side-effects.     

Treatment of retinopathy of prematurity

Retinopathy of prematurity is a potentially blinding disorder of premature infants. Retinal ablation of the avascular retina originally described using cryotherapy but now most commonly undertaken with laser photocoagulation, reduces the unfavourable structural outcomes and improves the functional visual acuity outcome. The CRYO-ROP study showed the long-term benefit of treatment of threshold disease compared with no treatment, however even with cryoablation 44.4% of treated eyes had a visual acuity of 6/60 or worse at 10 year follow-up. The ETROP study of earlier treatment for high-risk pre-threshold disease, rather than treatment at threshold, has shown that pre-threshold treatment of type 1 disease produces a significantly improved outcome. Despite treatment some infants develop retinal detachment for which various surgical treatments have been described, although not always with a good functional outcome. Future treatment modalities may include the use of anti-VEGF therapies.     

Epidemiology of respiratory distress of newborns

The present prospective study was conducted to find out the incidence, etiology and outcome of respiratory distress (RD) in newborns. All newborns (n=4505), delivered at this hospital over a period of 13 months, were observed for respiratory problems. Relevant antenatal, intranatal and neonatal information was noted. Cases were investigated for the cause of respiratory distress and followed up for the outcome. The overall incidence of RD was 6.7% Preterm babies had the highest incidence (30.0%) followed by post-term (20.9%) and term babies (4.2%). Transient tachypnea of newborn (TTN) was found to be the commonest (42.7%) cause of RD followed by infection (17.0%), meconium aspiration syndrome (10.7%), hyaline membrane disease (9.3%) and birth asphyxia (3.3%). TTN was found to be common among both term and preterm babies. While Hyaline membrane disease (HMD) was seen mostly among preterms, and Meconium aspiration syndrome (MAS) among term and post-term babies. Overall case fatality ration for RD was found to be 19%, being highest for HMD (57.1%), followed by MAS (21.8%) and infection (15.6%). Our results indicate that RD is a common neonatal problem. TTN accounts for a large proportion of thhese cases. MAS and infection also contribute significantly and are largely preventable. Without adequate ventilatory support HMD and MAS carry high mortality.