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Two congenital coronary abnormalities affecting heart function： anomalous origin of the left coronary artery from the pulmonary artery and congenital left main coronary artery atresia

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Chinese Medical Journal 2014年第21期127卷 3724-3731页

作者： Xiao Yanyan Jin Mei Han Ling Ding Wenhong Zheng Jianyong Sun Chufan Lyu Zhenyu Department of Pediatric Cardiology Beijing Anzhen Hospital Capital Medical University Beijing 100029 China Department of Cardiology Navy General Hospital of Pepole's Liberation Army Beijing 100000 China

Background The anomalous origin of the left coronary artery （LCA） from the pulmonary artery （ALCAPA） and congenital left main coronary artery atresia （CLMCA-A） are two kinds of very rare coronary heart diseases which affect heart function profoundly.This study aimed to retrospectively illustrate the clinical features and therapy experience of ALCAPA and CLMCA-A patients.Methods From April 1984 to July 2012,in Beijing Anzhen Hospital,23 patients were diagnosed with ALCAPA and 4 patients with CLMCA-A.We summarized the clinical data of the 27 cases and retrospectively analyzed the clinical manifestation,diagnosis,and treatments of these two kinds of congenital coronary abnormalities.Results The 23 patients （13 males and 10 females,aged ranging from 2.5 months to 65 years） identified with ALCAPA were classified into infantile type （age of onset younger than 12 months,16 cases） and adult type （age of onset older than 12 months,7 cases）.Four patients were diagnosed with CLMCA-A （three males and one female,aged ranging from 3 months to 2 years）.The main clinical manifestations of infantile-type ALCAPA and CLMCA-A include repeated respiratory tract infection,heart failure,dyspnea,feeding intolerance,diaphoresis,and failure to thrive.And these two congenital coronary abnormalities might be misdiagnosed as endocardial fibroelastosis,dilated cardiomyopathy,and acute myocardial infarction.As for the adult-type ALCAPA,cardiac murmurs and discomfort of the precordial area are the most common presentations and might be misdiagnosed as coronary heart disease,myocarditis,or patent ductus arteriosus.In ECG examination：Infantile-type ALCAPA and CLMCA-A showed abnormal Q waves with T wave inversion in leads I,avL,and V4-V6,especially in lead avL.However,ECG of adult-type ALCAPA lacked distinct features.In chest radiography：pulmonary congestion and cardiomegaly were the most common findings in infantile-type ALCAPA and CLMCA-A,while pulmonary artery segment dilation was more common in adult type.In echocardiography,the common feature

关键词： congenital coronary abnormalities ALCAPA coronary artery atresia

Liver fibrosis in biliary atresia

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World Journal of Pediatrics 2019年第2期15卷 117-123页

作者： Wen-Jun Shen Gong Chen Min Wang Shan Zheng Department of Pediatric Surgery Children's Hospital of Fudan University399 Wanyuan RoadMinhang DistrictShanghai 201102China

Background Biliary atresia (BA) is the most common cause of obstructive jaundice in infants.Although the Kasai procedure has greatly improved the progno sis,most patients still need liver transplantation (LT) for long-term survival.The pathogenesis of BA has not been fully clarified,and liver fibrosis in BA is far beyond biliary obstructive cirrhosis.Data sources Literature reviews were underwent through PubMed.Persistent inflammation,immune response,biliary epithelial-mesenchymal transition,matrix deposition,decompensated angiogenesis,and unique biliary structure development all contribute to the fibrosis process.Observed evidences in such fields have been collected and form the backbone of this review.Results Interactions of the multiple pathways accelerate this process.Conclusions Understanding the mechanisms of the liver fibrosis in BA may pave the way to improved survival after the Kasai procedure.

关键词： Liver fibrosis Biliary atresia Children

Increased osteopontin and liver stiffness measurement by transient elastography in biliary atresia

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World Journal of Gastroenterology 2010年第43期16卷 5467-5473页

作者： Sittisak Honsawek Maneerat Chayanupatkul Voranush Chongsrisawat Paisarn Vejchapipat Yong Poovorawan Department of Biochemistry Faculty of Medicine Chulalongkorn University and King Chulalongkorn Memorial Hospital Department of Physiology Faculty of Medicine Chulalongkorn University Center of Excellence in Clinical Virology Department of Pediatrics Faculty of Medicine Chulalongkorn University Department of Surgery Faculty of Medicine Chulalongkorn University

AIM: To analyze plasma osteopontin levels and liver stiffness using transient elastography in postoperative biliary atresia (BA) children compared with healthy controls. METHODS: Thirty children with postoperative BA and 10 normal controls were enrolled. The patients were categorized into two groups according to their jaundicestatus. Plasma levels of osteopontin were determined using commercially available enzyme-linked immunosorbent assay. Liver stiffness was measured by using transient elastography (Fibroscan). Ten validated Fibroscan measurements were performed in each patient and control with the result expressed in kilopascals (kPa). RESULTS: Plasma osteopontin was significantly elevated in BA children compared with that of healthy controls (47.0 ± 56.4 ng/mL vs 15.1 ± 15.0 ng/mL, P = 0.01). The liver stiffness measurement was markedly elevated in the patients with BA compared with that of controls (26.9 ± 24.6 kPa vs 3.9 ± 0.7 kPa, P = 0.001). Subgroup analysis showed that the BA patients with jaundice had more pronounced plasma osteopontin levels than those without jaundice (87.1 ± 61.6 ng/mL vs 11.9 ± 6.1 ng/mL, P = 0.001). Furthermore, the mean liver stiffness was significantly greater in the jaundiced BA patients compared with non-jaundiced patients (47.7 ± 21.8 kPa vs 8.7 ± 3.0 kPa, P = 0.001). Additionally, plasma osteopontin was positively related to serum total bilirubin (r = 0.64, P < 0.001). There was also a correlation between plasma osteopontin and liver stiffness values (r = 0.60, P < 0.001). CONCLUSION: High plasma osteopontin positively correlated with degree of hepatic fibrosis and could be used as a biochemical parameter reflecting disease severity in postoperative BA children.

关键词： Biliary atresia Fibroscan Jaundice Liver stiffness Osteopontin

Oesophageal atresia: The growth gap

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World Journal of Gastroenterology 2020年第12期26卷 1262-1272页

作者： Isabelle Traini Jessica Menzies Jennifer Hughes Steven Thomas Leach Usha Krishnan School of Women’s and Children’s Health University of New South WalesSydneyNSW 2052Australia Department of Nutrition and Dietetics Sydney Children’s HospitalRandwickNSW 2031Australia Department of Speech Pathology Sydney Children’s HospitalRandwickNSW 2031Australia Department of Paediatric Gastroenterology Sydney Children’s HospitalRandwickNSW 2031Australia

Poor growth is an under-recognised yet significant long-term sequelae of oesophageal atresia(OA)repair.Few studies have specifically explored the reasons for growth impairment in this complex cohort.The association between poor growth with younger age and fundoplication appears to have the strongest supportive evidence,highlighting the need for early involvement of a dietitian and speech pathologist,and consideration of optimal medical reflux management prior to referring for anti-reflux surgery.However,it remains difficult to reach conclusions regarding other factors which may negatively influence growth,due to conflicting findings,inconsistent definitions and lack of validated tool utilisation.While swallowing and feeding difficulties are particularly frequent in younger children,their relationship with growth remains unclear.It is possible that these morbidities impact on the diet of children with OA,but detailed analysis of dietary composition and quality,and its relationship with these complications and growth,has not yet been conducted.Another potential area of research in OA is the role of the microbiota in growth and nutrition.While the microbiota has been linked to growth impairment in other paediatric conditions,it is yet to be investigated in OA.Further research is needed to identify the most important contributory factors to poor growth,the role of the intestinal microbiota,and effective interventions to maximise growth and nutritional outcomes in this cohort.

关键词： Oesophageal atresia Growth Malnutrition Feeding difficulty Dysphagia Microbiota

Adjuvant steroid treatment following Kasai portoenterostomy and clinical outcomes of biliary atresia patients: an updated meta-analysis

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World Journal of Pediatrics 2017年第1期13卷 20-26页

作者： Min-Zhong Zhang Peng-Cheng Xun Ka He Wei Cai Department of Pediatric Surgery Xinhua hospitalShanghai Jiao Tong University School of MedicineNo.1665Kongjiang RoadShanghaiChina Shanghai Key Laboratory of Pediatric Gastroenterology and Nutrition No.1665Kongjiang RoadShanghaiChina Department of Epidemiology and Biostatistics School of Public Health-BloomingtonIndiana University1025 E.Seventh StreetBloomingtonINUSA

Background:It is controversial whether adjuvant steroid treatment should be given to biliary atresia rBAl patients following a Kasai portoenterostomy(KPE).This study aimed to quantitatively and systematically evaluate... 详细信息

关键词： biliary atresia Kasai portoenterostomy steroid treatment

Four cancer cases after esophageal atresia repair: Time to start screening the upper gastrointestinal tract

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World Journal of Gastroenterology 2018年第9期24卷 1056-1062页

作者： Floor WT Vergouwe Madeleine Gottrand Bas PL Wijnhoven Hanneke IJsselstijn Guillaume Piessen Marco J Bruno René MH Wijnen Manon CW Spaander Department of Gastroenterology and Hepatology Erasmus MC University Medical Center Department of Pediatric Surgery Erasmus MC University Medical Center - Sophia Children's Hospital Department of Pediatrics Jeanne de Flandre Children’s Hospital - Univ.Lille CHU Lille Department of Surgery Erasmus MC University Medical Center Department of Digestive and Oncological Surgery Claude Huriez Hospital - Univ.Lille CHU Lille

Esophageal atresia(EA) is one of the most common congenital digestive malformations and requires surgical correction early in life. Dedicated centers have reported survival rates up to 95%. The most frequent comorbidities after EA repair are dysphagia(72%) and gastroesophageal reflux(GER)(67%). Chronic GER after EA repair might lead to mucosal damage, esophageal stricturing, Barrett's esophagus and eventually esophageal adenocarcinoma. Several long-term follow-up studies found an increased risk of Barrett's esophagus and esophageal carcinoma in EA patients, both at a relatively young age. Given these findings, the recent ESPGHAN-NASPGHAN guideline recommends routine endoscopy in adults born with EA. We report a series of four EA patients who developed a carcinoma of the gastrointestinal tract: three esophageal carcinoma and one colorectal carcinoma in a colonic interposition. These cases emphasize the importance of lifelong screening of the upper gastrointestinal tract in EA patients.

关键词： Adenocarcinoma Esophageal atresia Esophageal cancer Screening Barrett’s esophagus Squamous cell carcinoma

Expression of programmed death-1 and its ligands in the liver of biliary atresia

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World Journal of Pediatrics 2017年第6期13卷 604-610页

作者： Pan-Liang Wang Jun Wang Ying Zhou Xiao-Song Chen Ke-Jun Zhou Jie Wen Jian-Jun Zhang Wei Cai Department of Pediatric Surgery Xinhua HospitalSchool of MedicineShanghai Jiaotong UniversityShanghaiChina Department of Transplantation and Hepatic Surgery Renji HospitalSchool of MedicineShanghai Jiaotong UniversityShanghaiChina Shanghai Key Laboratory of Pediatric Gastroenterology and Nutrition ShanghaiChina Department of Pediatric Surgery Xinhua HospitalSchool of MedicineShanghai Jiaotong UniversityShanghaiChina Shanghai Key Laboratory of Pediatric Gastroenterology and Nutrition ShanghaiChina Shanghai Institute of Pediatric Research ShanghaiChina

Background:An aberrant immune response is the predominant pathogenetic factor in biliary atresia (BA).Programmed death-1 (PD-1) and its two ligands,programmed death ligand-1 and programmed death ligand-2 (PD-L1 and PD-L2,respectively) play an important inhibitory role in immune reactions.We aimed to illustrate the expression of these molecules in BA.Methods:Liver specimens were obtained from infants with BA during the Kasai procedure (early BA) and fiver transplantation (late BA).Intrahepatic expression of PD-1,PD-L1,and PD-L2 were examined by immunostaining and compared with that in patients with neonatal hepatitis syndrome and normal controls.The correlation between the expression levels of these molecules in the liver and clinicopathological parameters was analyzed for each group.Results:Enhanced expression of PD-1 and its ligands occurred in the livers with early BA.In the BA-affected livers,PD-1 was correlated with the degree of peri-bifiary inflammation,while PD-L2 was linked more directly with portal fibrosis.None of the three molecules was correlated with the prognosis of the Kasai procedure in patients with early BA.Conclusion:Only PD-1 and PD-L1 are involved in the immune reactions of early BA.Elucidation of the detailed role of PD-L2 in BA requires further research.

关键词： biliary atresia immunostaining liver neonatal hepatitis syndrome programmed death-1

Post-inflammatory acquired atresia of the external auditory canal

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Journal of Otology 2019年第4期14卷 149-154页

作者： Michel Kmeid Jade Nehme School of Medicine Lebanese UniversityBeirutLebanon Lebanese University Department of OtolaryngologyHead and Neck SurgeryBeirutLebanon

Acquired atresia of the external auditory canal(EAC)is a rare cause of conductive hearing loss.It has been traditionally classified into 4 categories:traumatic,post-operative,neoplastic and inflammatory.Post-inflammatory acquired auditory canal atresia is thought to be the result of chronic and repetitive infectious bouts affecting the auditory canal.Nevertheless,the underlying pathophysiology of this disorder is yet to be fully elucidated.Current data fail to clearly state the impact that certain underlying systemic disorders may have on the EAC.The possible association to metabolic disturbances such as iron deficiency is also emphasized.In the light of these findings,this analysis can be used to improve the classification of this entity thereby standardizing the assessment of therapeutic approaches.

关键词： Stenosing external otitis Meatal atresia External auditory canal Conductive hearing loss Canaloplasty

Autoimmune liver disease-related autoantibodies in patients with biliary atresia

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World Journal of Gastroenterology 2018年第3期24卷 387-396页

作者： Shu-Yin Pang Yu-Mei Dai Rui-Zhong Zhang Yi-Hao Chen Xiao-Fang Peng Jie Fu Zheng-Rong Chen Yun-Feng Liu Li-Yuan Yang Zhe Wen Jia-Kang Yu Hai-Ying Liu Clinical Laboratory Guangzhou Women and Children’s Medical CenterGuangzhou Medical University Guangzhou Institute of Pediatrics Guangzhou Women and Children’s Medical CenterGuangzhou Medical University Department of Pathology Guangzhou Women and Children’s Medical CenterGuangzhou Medical University Department of Neonatal Surgery Guangzhou Women and Children’s Medical CenterGuangzhou Medical University

AIM To investigate the prevalence and clinical significance of autoimmune liver disease(ALD)-related autoantibodies in patients with biliary atresia(BA).METHODS Sera of 124 BA patients and 140 age-matched non-BA controls were assayed for detection of the following autoantibodies: ALD profile and specific anti-nuclear antibodies(ANAs), by line-blot assay; ANA and antineutrophil cytoplasmic antibody(ANCA), by indirect immunofluorescence assay; specific ANCAs and antiM2-3 E, by enzyme linked immunosorbent assay. Associations of these autoantibodies with the clinical features of BA(i.e., cytomegalovirus infection, degree of liver fibrosis, and short-term prognosis of Kasai procedure) were evaluated by Spearman's correlation coefficient.RESULTS The overall positive rate of serum autoantibodies in preoperative BA patients was 56.5%. ALD profile assay showed that the positive reaction to primary biliary cholangitis-related autoantibodies in BA patients was higher than that to autoimmune hepatitis-related autoantibodies. Among these autoantibodies, anti-BPO was detected more frequently in the BA patients than in the controls(14.8% vs 2.2%, P < 0.05). Accordingly, 32(25.8%) of the 124 BA patients also showed a high positive reaction for anti-M2-3 E. By comparison, the controls had a remarkably lower frequency of anti-M2-3 E(P < 0.05), with 6/92(8.6%) of patients with other liver diseases and 2/48(4.2%) of healthy controls. The prevalence of ANA in BA patients was 11.3%, which was higher than that in disease controls(3.3%, P < 0.05), but the reactivity to specific ANAs was only 8.2%. The prevalence of ANCAs(ANCA or specific ANCAs) in BA patients was also remarkably higher than that in the healthy controls(37.9% vs 6.3%, P < 0.05), but showed no difference from that in patients with other cholestasis. ANCA positivity was closely associated with the occurrence of postoperative cholangitis(r = 0.61, P < 0.05), whereas none of the autoantibodies showed a correlation to cytomegalovirus infection or the stages of liver fibrosis.

关键词： Biliary atresia Anti-nuclear antibody Antineutrophilic cytoplasmic antibody Autoimmune liver diseases Autoantibodies