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Role of practice standardization in outcome optimization for CDH

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World Journal of pediatric surgery 2024年第2期7卷 110-118页

作者： Alexandra Dimmer Robert Baird Pramod Puligandla Harvey E.Beardmore Division of pediatric surgery Department of Pediatric SurgeryMontreal Children's HospitalMontrealQuebecCanada Division of pediatric General and Thoracic surgery British Columbia Children's HospitalVancouverBritish ColumbiaCanada Harvey E.Beardmore Department of pediatric surgery McGill UniversityMontrealQuebecCanada

Standardization of care seeks to improve patient outcomes and healthcare delivery by reducing unwanted variations in care as well as promoting the efficient and effective use of healthcare *** are many types of standardization,with clinical practice guidelines(CPGs),based on a stringent assessment of evidence and expert consensus,being the hallmark of high-quality *** article outlines the history of CPGs,their benefits and shortcomings,with a specific focus on standardization efforts as it relates to congenital diaphragmatic hernia management.

关键词： congenital clinical shortcomings

Multiple thoracic and abdominal foregut duplication cysts:A case report

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World Journal of clinical Cases 2024年第8期12卷 1504-1509页

作者： Tuqa Adil Alsinan Tariq Ibrahim Altokhais Department of pediatric surgery Prince Sultan Military Medical CityRiyadh 12233Saudi Arabia Devision of pediatric surgery Department of SurgeryCollege of MedicineKing Saud UniversityRiyadh 4545Saudi Arabia

BACKGROUND Congenital enteric duplication cysts are tubular or cystic structures that normally lie alongside the gastrointestinal(GI)*** duplication cysts are typically solitary lesions that occur anywhere near the GI tract from the neck to the rectum,but having multiple duplication cysts is rare,and presentation within the pancreas is extremely *** SUMMARY We herein demonstrate a case of esophageal,gastric,and gastric-type duplication cyst of the pancreas in a seventeen-month-old girl who presented with failure to thrive,abdominal pain,vomiting,hematemesis,and melena since the age of three *** cysts were excised by thoracoscopy and laparoscopy in the same *** our knowledge,no such case has been *** Enteric duplications can occur throughout the entire alimentary *** they occur in the pancreas,they present a formidable challenge in both diagnosis and *** to the risk of complications and malignant transformation,surgical removal is the recommended treatment of all duplication cysts.

关键词： Congenital Duplication cyst Foregut duplication Gastric duplication Pancreas Case report

Primary repair of esophageal atresia Gross type C via thoracoscopic magnetic compression anastomosis:Is it the best option?

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World Journal of Gastrointestinal surgery 2024年第5期16卷 1474-1481页

作者： Sonia Pérez-Bertólez Jorge Godoy-Lenz Department of pediatric surgery Sant Joan de Deu HospitalUniversity of BarcelonaBarcelona 08950Spain Department of pediatric surgery Clínica Alemana of SantiagoSantiago 7650568Chile

Magnetic compression anastomosis is a promising treatment option for patients with complex esophageal atresia;but,at the present time,should not be the first therapeutic option in those cases where the surgeon can per... 详细信息

关键词： Esophageal atresia Tracheoesophageal fistula Thoracoscopy Magnamosis Magnetic anastomosis

Emerging role of regulated cell death in intestinal failure-associated liver disease

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Hepatobiliary & Pancreatic Diseases International 2024年第3期23卷 228-233页

作者： Si-Yang Cheng Lu Jiang Ying Wang Wei Cai Department of pediatric surgery Xinhua HospitalSchool of MedicineShanghai Jiao Tong UniversityShanghai 200092China Shanghai Key Laboratory of pediatric Gastroenterology and Nutrition Shanghai 200092China Shanghai Institute for pediatric Research Shanghai 200092China Division of pediatric Gastroenterology and Nutrition Xinhua HospitalSchool of MedicineShanghai Jiao Tong UniversityShanghai 200092China

Intestinal failure-associated liver disease(IFALD)is a common complication of long-term parenteral nutrition that is associated with significant morbidity and *** is mainly characterized by cholestasis in children and steatohepatitis in ***,there is no effective approach to prevent or reverse the *** cell death(RCD)represents a fundamental biological paradigm that determines the outcome of a variety of liver *** cell death is reclassified into several types,based on the mechanisms and morphological *** evidence has linked different modes of RCD,such as apoptosis,necroptosis,ferroptosis,and pyroptosis to the pathogenesis of liver *** studies have shown that different modes of RCD are present in animal models and patients with *** the pathogenic roles of cell death may help uncover the underlying mechanisms and develop novel therapeutic strategies in *** this review,we discuss the current knowledge on how RCD may link to the pathogenesis of *** highlight examples of cell death-targeted interventions aiming to attenuate the disease,and provide perspectives for future basic and translational research in the field.

关键词： Parenteral nutrition Apoptosis Necroptosis Cholestasis Steatosis

Perforation of Meckel's diverticulum in two neonates with single umbilical artery

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World Journal of pediatric surgery 2024年第2期7卷 158-160页

作者： Lifeng Zhang Shannan Wu Xuefeng Miao Yonglin Li Xiaojian Yuan Zhigang Gao General pediatric surgery Children's HospitalZhejiang University School of MedicineHangzhouChina Department of pediatric surgery Yiwu Branch of Children's HospitalZhejiang University School of MedicineYiwuChina

Meckel's diverticulum(MD)is the most common congenital anomaly of the gastro-intestinal(GI)*** main clinical mani-festation of MD in childhood is GI ***,symptomatic manifestation in the neonatal period is rare.1 Single umbil-ical artery(SUA)was first reported by Hyrtl in 1870.2 In recent years,SUA has been reported to be a marker for fetal congenital malformations,chromosomal abnormali-ties,premature birth,and low birth weight,which can lead to severe adverse *** hereby report two cases of MD perfora-tion in neonates with SUA in the Yiwu Branch of Children's Hospital,Zhejiang university School of Medicine.

关键词： Meckel artery fetal

A novel structural variant of RET causes familial Hirschsprung's disease via nonsense-mediated mRNA decay

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Genes & Diseases 2024年第1期11卷 7-10页

作者： Qi Wang Siyu Pu Bo Xiang Jing Chen Department of pediatric surgery and Laboratory of pediatric surgery Rare Diseases CenterInstitute of Immunology and InflammationFrontiers Science Center for Disease-Related Molecular NetworkWest China HospitalSichuan UniversityChengduSichuan 610041China

Hirschsprung's disease(HsCR)is the most common type of developmental gastrointestinal malformation causing intestinal obstruction in children,with an incidence of 1/5000 in live births.1 it is characterized by the absence of intrinsic ganglion cells in the myenteric and submucosal plexuses of the distal gastrointestinal tract,leading to the dysregulation of smooth muscle contraction/relaxation and resulting in intestinal *** on the length of the aganglionic intestine,HSCR can be categorized into short-segmental HSCR(S-HSCR),which affects up to the sigmoid colon from the anus,and long-segmental HSCR(LHSCR),which affects beyond the sigmoid colon.1 In some rare conditions,aganglionic lesions can affect the full length of the colon or even the whole intestine.

关键词： intestine colon relaxation

Nationwide questionnaire survey on pediatric pancreatic tumors in Japan

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World Journal of Gastrointestinal Oncology 2024年第10期16卷 4166-4176页

作者： Satoshi Makita Hiroo Uchida Motohiro Kano Naonori Kawakubo Hiromu Miyake Akihiro Yoneda Tatsuro Tajiri Koji Fukumoto Department of pediatric surgery Nagoya University Graduate School of MedicineNagoya 466-8550Japan Department of pediatric surgery National Center for Child Health and DevelopmentTokyo 157-8535Japan Department of pediatric surgery Reproductive and Developmental MedicineFaculty of Medical SciencesKyushu UniversityFukuoka 819-0395Japan Department of pediatric surgery Shizuoka Children’s HospitalShizuoka 420-8660Japan

BACKGROUND pediatric pancreatic tumors are rare and account for<0.1%of all childhood *** primary treatment for pancreatic tumors is surgical ***,because of the lack of knowledge regarding pediatric pancreatic tumors,n... 详细信息

BACKGROUND pediatric pancreatic tumors are rare and account forpediatric pancreatic tumors,no comprehensive treatment plans for pediatric pancreatic tumors have been *** To compared the clinical features,treatment methods,and prognosis of pediatric pancreatic tumors in Japan with those in other *** Questionnaires were sent to 213 pediatric surgical units in *** tumors that were not surgically treated were excluded from the *** primary survey investigated the number of patients aged 0-18 years who underwent pancreatic tumor surgery and the type of tumors managed during the 22-year study period(from January 1,2000 to December 31,2021)by post *** secondary survey assessed the clinical images,treatment methods,and tumor outcomes via *** The primary survey enrolled 228 *** the secondary survey,213 patients were eventually *** most common type of pancreatic tumor was solid pseudopapillary neoplasm(SPN)[n=164(77.0%)],followed by pancreatoblastoma[n=16(7.5%)],pancreatic endocrine tumor[n=14(6.6%)],non-epithelial tumor[n=9(4.2%)],pancreatic tumor[n=7(3.3%)],and metastatic pancreatic tumor[n=3(1.4%)].Overall,123(57.7%)patients underwent distal pancreatectomy,of whom 49 underwent laparoscopic ***-four(20.7%)patients underwent enucleation,of whom eight underwent laparoscopic ***-two(15.0%)patients underwent pancreaticoduodenectomy,of whom one underwent laparoscopic *** patients with SPN,including those with distant metastases and recurrent disease,*** SPN was more common in Japan than in other *** of the histological type,resection is the most effective treatment for pediatric pancreatic tumors.

关键词： Pancreatic tumors pediatric Nationwide survey Solid pseudopapillary tumors Pancreatoblastoma

Jaffe-Campanacci syndrome resulted in amputation: A case report

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World Journal of clinical Cases 2024年第10期12卷 1785-1792页

作者： Jun Jiang Min Liu Department of pediatric surgery West China Hospital of Sichuan UniversityChengdu 610041Sichuan ProvinceChina

BACKGROUND Jaffe-Campanacci syndrome(JCS)is a very rare *** treatment of JCS is more conservative,and most authors recommend that no surgery should be done in asymptomatic *** conventional concept holds that the natural course of non-ossifying fibromas(NOFs)grows with the development of bones,and the osteolytic region gradually stops expanding and self-healing through bone ossifying around the lesion and ossification within the *** in this case,the bone lesions were potentially biologically aggressive,which led to severe limb deformities and *** SUMMARY We present the case of a 5-year-old girl with JCS presenting with not only NOF sand café-au-lait macules,but also showed features not mentioned before,severe limb pain,and at last resulted in *** was admitted to our hospital after presenting with claudication and mild pain over her right thigh,which worsened when stretching or being *** examination revealed multiple café-au-lait macules on the neck,arm,axilla,and torso,including the nipples and *** revealed multiple lytic lesions in the proximal part of the right humerus,distal part of the right clavicle,proximal and distal parts of the right femur,and proximal parts of the right tibia and *** and biopsy were performed on the distal part of the right *** the age of 7,the girl was re-admitted to our hospital for a pathological fracture in the middle in the right femur and underwent Intralesional excision,internal fixation,bone grafting,and spica *** the age of 10,the girl came to our hospital again for severe pain of the right *** from the middle level of the right femur was *** present the case of a 5-year-old girl with JCS presenting with not only NOFs and café-au-lait macules,but also showed features not mentioned before,severe limb pain,and at last resulted in *** was admitted to our hospital after presenting with claudication and mild pain o

关键词： Jaffe-Campanacci syndrome Multiple non-ossifying fibromas Café-au-lait macule Amputation Case report

Stubborn Hypoxia in Neonates with D-Transposition of the Great Arteries after Arterial Switch Operation: Central Sleep Apnea as the Cause and Potential Indicator of Brain Immaturity

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Congenital Heart Disease 2024年第2期19卷 185-195页

作者： Camden L.Hebson Kyle Bliton Amr Y.Hammouda Kaitlyn Barr W.Hampton Gray Mohini Gunnett Waldemar F.Carlo Department of pediatrics Division of Pediatric CardiologyUniversity of Alabama BirminghamBirmingham35233USA Department of pediatrics Division of Pediatric PulmonologyUniversity of Alabama BirminghamBirmingham35233USA Department of pediatrics University of Alabama BirminghamBirmingham35233USA Department of surgery Division of Cardiothoracic SurgeryUniversity of Alabama BirminghamBirmingham35233USA

D-transposition of the great arteries(d-TGA)is surgically repaired with the arterial switch operation(ASO)with excellent results,however short and long-term morbidities still develop including neurocognitive *** significant central sleep apnea is uncommon in non-premature infants,but when present indicates immature autonomic control of respiration likely due to a neurologic *** report the unanticipatedfinding of central sleep apnea in four-term neonates with d-TGA after uncomplicated ASO,with the short-term complication of delayed hospital discharge and long-term concerns regarding this early marker of brain immaturity and its hindrance to normal *** this report,we will review each patient’s clinical course and then examine the literature on pediatric central sleep apnea,neurodevelopmental outcomes after ASO,and the important overlap of these entities in the care of patients going forward.

关键词： Transposition congenital heart disease sleep apnea polysomnography