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Extra Renal Rhabdoid Tumor: A Rare Cause of Congenital Soft Tissue Tumor

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Open Journal of pediatrics 2024年第3期14卷 579-584页

作者： Tazi Charki Mohammed Akammar Amal Dardar Hajar Abdellaoui Hicham Atarraf Karima Boubou Meryem Afifi Moulay Abderrahmane Department of pediatric Orthopedic and Traumatology University Hospital Hassan II Fez Morocco The Faculty of Medicine and Pharmacy Sidi Mohamed Ben Abdellah University Fez Morocco Department of radiology University Hospital Hassan II Fez Morocco

Rhabdoid tumors (RTs) are a well-defined entity in the kidney or central nervous system of infants or children. However, soft-tissue involvement is uncommon. It’s an exceptional neonatal tumor of soft tissue. The imaging characteristics of this tumor are not specific. Biopsy allows diagnosis;the histomorphological characteristics of rhabdoid tumors, their immunoreactivity to epithelial markers and vimentin, and the INI-1 loss are important tools for diagnosis. RT tumors are aggressive and have a rapidly fatal clinical course in most cases. Despite multidisciplinary therapy, the survival rate is very low. We report a rare case occurring in a male neonate who presents at birth with a voluminous right axillary mass. A CT scan showed a well-limited tumor mass with lobulated contours. An ultrasound-guided biopsy was performed on day 8, showing the morphology and immunoprofile of RT. The mass showed rapid growth. The child was admitted for respiratory distress at 3 weeks. A thoraco-abdominal CT showed an increase in the size of the mass with the appearance of multiple lymph nodes and pleural, hepatic, and renal metastases. The child died two days later.

关键词： Congenital Rhabdoid Tumor Soft Tissue Diagnosis Immunohistochemistry INI-1

Diseases of bile duct in children

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World Journal of Gastroenterology 2024年第9期30卷 1043-1072页

作者： Sutha Eiamkulbutr Chomchanat Tubjareon Anapat Sanpavat Teerasak Phewplung Nimmita Srisan Palittiya Sintusek Department of pediatrics King Chulalongkorn Memorial HospitalBangkok 10330Thailand Department of pediatrics Faculty of MedicineChulalongkorn University and King Chulalongkorn Memorial HospitalBangkok 10330Thailand Department of Pathology Chulalongkorn UniversityBangkok 10330Thailand Department of radiology Chulalongkorn UniversityBangkok 10330Thailand Department of Surgery Faculty of MedicineChulalongkorn University and King Chulalongkorn Memorial HospitalBangkok 10330Thailand Center of Excellence in Thai pediatric Gastroenterology Hepatology and ImmunologyDivision of GastroenterologyDepartment of PediatricsKing Chulalongkorn Memorial HospitalChulalongkorn UniversityBangkok 10330Thailand

Several diseases originate from bile duct *** studies on these diseases,certain etiologies of some of them still cannot be *** most common disease of the bile duct in newborns is biliary atresia,whose prognosis varies according to the age of surgical *** diseases such as Alagille syndrome,inspissated bile duct syndrome,and choledochal cysts are also time-sensitive because they can cause severe liver damage due to *** majority of these diseases present with cholestatic jaundice in the newborn or infant period,which is quite difficult to differentiate regarding clinical acumen and initial *** cholangiography is potentially necessary to make an accurate diagnosis,and further treatment will be performed synchronously or planned as findings *** article provides a concise review of bile duct diseases,with interesting cases.

关键词： Bile duct Cholestasis Biliary atresia Biliary hypoplasia Biliary imaging Inspissated bile syndrome Choledochal cyst

Hernandezine promotes cancer cell apoptosis and disrupts the lysosomal acidic environment and cathepsin D maturation

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Chinese Journal of Natural Medicines 2024年第5期22卷 387-401页

作者： FENG Qianwen SUN Lu Muhammad Jibran Sualeh ZHAO Qingli ZHAO Songji CUI Zhengguo INADERA Hidekuni Department of Public Health Graduate School of Medicine and Pharmaceutical SciencesUniversity of ToyamaToyama 930-8501Japan Department of pediatric Cardiology Heart CenterGuangzhou Women and Children’s Medical CenterGuangzhou Medical UniversityGuangzhou 510000China Department of Basic Medical Sciences College of MedicineUniversity of SharjahSharjah 57545United Arab Emirates Department of radiology Graduate School of Medicine and Pharmaceutical SciencesUniversity of ToyamaToyama 930-8501Japan Advanced Clinical Research Center Fukushima Global Medical Science CenterFukushima Medical UniversityFukushima CityFukushima 960-8074Japan Department of Environmental Health School of Medical SciencesUniversity of FukuiFukui 910-8580Japan

Hernandezine(Her),a bisbenzylisoquinoline alkaloid extracted from Thalictrum flavum,is recognized for its range of biological activities inherent to this herbal *** its notable properties,the anti-cancer effects of Her have remained largely *** this study,we elucidated that Her significantly induced cytotoxicity in cancer cells through the activation of apoptosis and necroptosis ***,Her triggered autophagosome formation by activating the AMPK and ATG5 conjugation systems,leading to LC3 *** findings revealed that Her caused damage to the mitochondrial membrane,with the damaged mitochondria undergoing mitophagy,as evidenced by the elevated expression of mitophagy ***,Her disrupted autophagic flux,demonstrated by the upregulation of p62 and accumulation of autolysosomes,as observed in the RFP-GFP-LC3 reporter ***,we determined that Her did not prevent the fusion of autophagosomes and ***,it inhibited the maturation of cathepsin D and increased lysosomal pH,indicating an impairment of lysosomal *** use of the early-stage autophagy inhibitor,3-methyladenine(3-MA),did not suppress LC3II,suggesting that Her also induces noncanonical autophagy in autophagosome *** application of Bafilomycin A1,an inhibitor of noncanonical autophagy,diminished the recruitment of ATG16L1 and the accumulation of LC3II by Her,thereby augmenting Her-induced cell *** observations imply that while autophagy initially plays a protective role,the disruption of the autophagic process by Her promotes programmed cell *** study provides the first evidence of Her’s dual role in inducing apoptosis and necroptosis while also initiating and subsequently impairing autophagy to promote apoptotic cell *** insights contribute to a deeper understanding of the mechanisms underlying programmed cell death,offering potential avenues for enhancing cancer prevention and therapeutic st

关键词： Hernandezine Apoptosis Mitophagy Autophagic flux Lysosome Noncanonical autophagy

Contribution and Relevance of Transfontanellar Ultrasound in Newborns in Ouagadougou: Multicentre Study on 1000 Cases

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Open Journal of radiology 2024年第2期14卷 64-73页

作者： Ouedraogo Pakisba Ali Ouedraogo Nina-Astrid Somé Milckisédek Judickael Dabire Stive Arnauld Tankoano Ida Aida Napon Aischa Madina Nikiema Zakari Diallo Ousseini Lougue Sorgho Leonie Claudine Cisse Rabiou Department of Medical Imaging Ouahigouya Regional University Hospital Centre Ouahigouya University Ouahigouya Burkina Faso Department of Diagnostic and Interventional radiology Bogodogo University Hospital Ouagadougou Burkina Faso Department of Medical Imaging Souro Sanon University Hospital Bobo Dioulasso Burkina Faso Medical Imaging Service Yalgado Oudraogo University Hospital Ouagadougou Burkina Faso Department of Medical Imaging Charles de Gaulle Pediatric University Hospital Ouagadougou Burkina Faso Radiodiagnostic and Medical Imaging Laboratory Health Sciences Training and Research Unit Joseph Ki-Zerbo University Ouagadougou Burkina Faso

Objectives: To demonstrate the contribution and relevance of ETFs through the study of 1000 examination reports carried out in the medical imaging departments of the OUAGADOUGOU CHU. Material and method: Analytical descriptive study with retrospective collection, extended from 1st January 2020 to 1st January 2022. Results: Of the 1000 transfontanellar ultrasound reports, the mean age of patients was 7.61 +/ 7.5 days, with extremes of zero and 28 days. Sex was specified in 989 cases. Males accounted for 54.49% and females for 45.51%. 555 transfontanellar ultrasound were performed in 2020. 441 in 2021 and 4 in 2022. 61.9% of transfontanellar ultrasound were performed at the Bogodogo University Hospital, 205 at Charles de Gaulle and 176 at Tengandogo. Indications for transfontanellar ultrasound were dominated by neonatal distress (65.8%), followed by convulsions (10.2%) and prematurity (9.1%). Transfontanellar ultrasound was normal in 570 cases (57%) and abnormal in 430 cases (43%). Abnormalities were dominated by haemorrhage and ischaemic lesions in 66.28% (285) and 21.63% (93) of cases respectively. In the group of normal transfontanellar ultrasound, neonatal distress represented 59.65% of indications and prematurity 10.7% of indications. As for abnormal transfontanellar ultrasound, neonatal suffering accounted for 73.95% of indications and convulsions for 12.56%. The average age ofpatients with an abnormal transfontanellar ultrasound was 8.74 days +/ 7.89 days. The indication for investigations was relevant in 42.2% of cases and irrelevant in 57.8%;of the transfontanellar ultrasound with relevant indications, 0.71 were normal and 99.29 abnormal;of the transfontanellar ultrasound with irrelevant indications, the transfontanellar ultrasound was normal in 98.1% and abnormal in 1.9%. Conclusion: Transfontanellar ultrasound is an important part of ultrasound in current practice. Haemorrhage, anoxic-ischaemic lesions and hydrocephalus are the most frequent pathologies fou

关键词： Transfontanellar Ultrasound Neonatal Distress Relevance

Rhabdomyosarcoma in Children: About 10 Cases

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Open Journal of pediatrics 2024年第3期14卷 522-530页

作者： Ayad Ghanam Manal Azizi Hind Zahiri Houssain Benhaddou Imane Kamaoui Amal Bennani Aziza El Ouali Abdeladim Babakhouya Maria Rkain Noufissa Benajiba Department of pediatrics Mohammed VI University Hospital Faculty of Medicine and Pharmacy Mohammed First University Oujda Morocco Department of pediatric Surgery Mohammed VI University Hospital Faculty of Medicine and Pharmacy Mohammed First University Oujda Morocco Department of radiology Mohammed VI University Hospital Faculty of Medicine and Pharmacy Mohammed First University Oujda Morocco Department of Anatomopathology Mohammed VI University Hospital Faculty of Medicine and Pharmacy Mohammed First University Oujda Morocco

Introduction: Rhabdomyosarcoma (RMS) is a malignant soft-tissue tumor arising from striated muscle cells. It accounts for 60% - 70% of malignant mesenchymal tumors and 5% of pediatric cancers. Two-thirds of these cancers are diagnosed in children under 6 years of age, with a slight male predominance. Materials and Methods: This is a retrospective descriptive study of 10 cases of RMS collected in the pediatric hematology and oncology department of the Oujda university hospital, over a 5-year period, running from January 2018 to December 2022. Results: The median age at diagnosis was 3 years, with a sex ratio of 1. The mean time to diagnosis was 2 months. The most common site was the head and neck (50%), followed by the genitourinary tract (20%), the extremities (20%) and finally the abdomen (10%). The most frequent mode of discovery was a mass or swelling found in 90% of patients (all sites included), followed by exophthalmos in 30% of cases. At the diagnostic stage, CT scans were performed in 70% of cases and MRI in 5 patients (50%). Histological diagnosis was determined by immunohistochemical pathology in all our patients, with a predominance of embryonal (70%) versus alveolar (20%) and spindle cell types (10%). All patients underwent an extension workup, and a cervico-thoraco-abdominopelvic CT was performed in all patients (100%);MRI was performed in 2 patients (20%);lymph node involvement was present in 5 patients (50%). Metastases at the time of diagnosis were noted in only 1 patient (10%), who simultaneously presented with two metastatic sites;testicular and abdominal wall. Sixty percent of patients presented with advanced disease (high risk) and 40% with standard risk. Chemotherapy was used in all patients (100%), with upfront tumor resection performed in 40%. Fifty percent of patients received radiotherapy at a mean dose of 43 Gy, with the orbit the most frequently irradiated area (30%). All patients underwent CTscan and/or MRI and/or ultrasound surveillance.

关键词： Rhabdomyosarcoma Child Chemotherapy Surgery Radiotherapy

Salivary C-reactive protein and mean platelet volume as possible diagnostic markers for late-onset neonatal pneumonia

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World Journal of Clinical pediatrics 2024年第1期13卷 41-51页

作者： Wafaa Ahmed Metwali Abdelrahman Mohamed Elmashad Sahar Mohey Eldin Hazzaa Mohammed Al-Beltagi Mohamed Basiony Hamza Department of pediatric Faculty of MedicineTanta UniversityTanta 31511AlgahrbiaEgypt Department of Clinical Pathology Faculty of MedicineTanta UniversityTanta 31511AlgahrbiaEgypt Department of pediatric University Medical CenterDr.Suliaman Al Habib Medical GroupManama 26671ManamaBahrain Department of pediatric University Medical CenterKing Abdulla Medical CityArabian Gulf UniversityManama 26671ManamaBahrain

BACKGROUND Neonatal sepsis,a formidable threat to newborns,is a leading cause of neonatal mortality,with late-onset sepsis manifesting after 72 hours post-birth being particularly ***,a prevalent sepsis presentation,poses a significant risk,especially during the neonatal phase when lung defenses are *** diagnosis of pneumonia is imperative for timely and effective ***,a minimally invasive diagnostic medium,holds great promise for evaluating infections,especially in *** To investigate the potential of serum C-reactive protein(CRP),salivary CRP(sCRP),and mean platelet volume(MPV)as diagnostic markers for late-onset neonatal pneumonia(LONP).METHODS Eighty full-term neonates were systematically examined,considering anthropometric measurements,clinical manifestations,radiology findings,and essential biomarkers,including serum CRP,sCRP,and *** The study reveals noteworthy distinctions in serum CRP levels,MPV,and the serum CRP/MPV ratio between neonates with LONP and healthy *** exhibited a robust discriminatory ability[area under the curve(AUC)=0.87]with high sensitivity and specificity at a cutoff value of>*** between serum CRP,sCRP,and MPV were also ***,sCRP demonstrated excellent predictive value for serum CRP levels(AUC=0.89),underscoring its potential as a diagnostic *** This study underscores the diagnostic promise of salivary and serum biomarkers,specifically MPV and CRP,in identifying and predicting LONP among *** findings advocate for further research to validate their clinical utility in larger neonatal cohorts.

关键词： Neonatal sepsis Late-onset pneumonia Salivary C-reactive protein Mean platelet volume Diagnostic markers Newborn infections

Transjugular intrahepatic portosystemic shunt vs conservative treatment for recurrent ascites:A propensity score matched comparison

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World Journal of Gastroenterology 2022年第41期28卷 5944-5956页

作者： Martin Philipp Theresia Blattmann Jörn Bienert Kristian Fischer Luisa Hausberg Jens-Christian Kröger Thomas Heller Marc-AndréWeber Georg Lamprecht Department of Medicine Division of Gastroenterology and EndocrinologyRostock University Medical CenterRostock 18057Germany Institute of Diagnostic and Interventional radiology Pediatric Radiology and NeuroradiologyRostock University Medical CentreRostock 18057Germany

BACKGROUND Transjugular intrahepatic portosystemic shunt(TIPS) placement is an effective intervention for recurrent tense ascites. Some studies show an increased risk of acute on chronic liver failure(ACLF) associated with TIPS placement. It is not clear whether ACLF in this context is a consequence of TIPS or of the pre-existing liver *** To better understand the risks of TIPS in this challenging setting and to compare them with those of conservative *** Two hundred and fourteen patients undergoing their first TIPS placement for recurrent tense ascites at our tertiary-care center between 2007 and 2017 were identified(TIPS group). Three hundred and ninety-eight patients of the same time interval with liver cirrhosis and recurrent tense ascites not undergoing TIPS placement(No TIPS group) were analyzed as a control group. TIPS indication,diagnosis of recurrent ascites, further diagnoses and clinical findings were obtained from a database search and patient records. The in-hospital mortality and ACLF incidence of both groups were compared using 1:1 propensity score matching and multivariate logistic *** After propensity score matching, the TIPS and No TIPS groups were comparable in terms of laboratory values and ACLF incidence at hospital admission. There was no detectable difference in mortality(TIPS: 11/214, No TIPS 13/214). During the hospital stay, ACLF occurred more frequently in the TIPS group than in the No TIPS group(TIPS: 70/214, No TIPS: 57/214, P = 0.04). This effect was confined to patients with severely impaired liver function at hospital admission as indicated by a significant interaction term of Child score and TIPS placement in multivariate logistic regression. The TIPS group had a lower ACLF incidence at Child scores < 8 points and a higher ACLF incidence at ≥ 11 points. No significant difference was found between groups in patients with Child scores of 8 to 10 *** TIPS placement for recurrent tense as

关键词： Liver cirrhosis Ascites Transjugular intrahepatic portosystemic shunt Acute on chronic liver failure Mortality Propensity score

Mediastinal lesions in children

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World Journal of Clinical Cases 2023年第12期11卷 2637-2656页

作者： Hasibe GökçeÇinar Ali Osman Gulmez ÇiğdemÜner Sonay Aydin Department of pediatric radiology Ankara Etlik City HospitalAnkara 06000Turkey Department of radiology Erzincan Binali Yıldırım University Faculty of MedicineErzincan 24100Turkey

The mediastinum is where thoracic lesions most frequently occur in young *** histological spectrum of diseases caused by the presence of several organs in the mediastinum is *** lesions,infections,benign and malignant lesions,and vascular diseases are examples of *** should be taken to make the proper diagnosis at the time of diagnosis in order to initiate therapy *** task is currently made simpler by radiological imaging techniques.

关键词： Mediastinum Thoracic lesions Vascular pathologies Trachea and main bronchus pathologies Esophageal pressure Imaging in mediastinal lesions

Classifying Cardiac Anomalies in Right and Left Isomerism:Concordant and Discordant Patterns

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Congenital Heart Disease 2023年第1期18卷 97-111页

作者： Lilia Oreto Giuseppe Mandraffino Paolo Ciliberti Teresa P.Santangelo Placido Romeo Antonio Celona Placido Gitto Lorenzo Galletti Fiore S.Iorio Alfredo Di Pino Aurelio Secinaro Paolo Guccione Robert H.Anderson Salvatore Agati Mediterranean pediatric Cardiology Center Bambino GesùChildren’s HospitalVia SirinaTaorminaItaly Internal Medicine unit Department of Clinical and Experimental MedicineUniversity of MessinaMessinaItaly Department of pediatric Cardiology and Cardiac Surgery Bambino GesùChildren’s HospitalRomaItaly Advanced Cardiovascular Imaging unit Department of ImagingBambino GesùChildren’s HospitalRomaItaly radiology Department S.Vincenzo HospitalTaorminaItaly Biosciences Institute Newcastle UniversityNewcastle upon TyneUK

Aims:Evidence is emerging that,in the setting of isomerism,the atrial and bronchial arrangement are not always concordant,nor are these patterns always harmonious with the arrangement of the abdominal *** aimed to evaluate the concordance between these features in a cohort of patients with cardiac malformations in the setting of known isomerism,seeking to determine whether it was feasible to assess complexity on this basis,in this regard taking note of the potential value of bronchial as opposed to appendage *** and Results:We studied 78 patients known to have isomerism of the bronchuses,43 with right and 35 with left *** anatomy could be determined in 49 cases(63%),all but one of these being concordant with bronchial *** assessing abdominal features,in only 59 cases(76%)was splenic morphology in keeping with the thoracic *** expected,right isomerism was associated with greater complexity of cardiac malformations,with an odds ratio of 6.53,with confidence intervals from 2.2–19.3(p<0.001).The odds were slightly decreased with thoraco-abdominal disharmony,when lesions shown to carry higher risk were then found in the setting of left ***:Harmony is excellent between bronchial and appendage isomerism,but less so with the arrangement of the abdominal *** isomerism in our cohort,was indicative of a sixfold increase in intracardiac *** discordance was found between the systems,however,the cardiac anomalies were less typical of the anticipated findings for right *** isomerism of the appendages.

关键词： Isomerism asplenia polysplenia congenital heart disease bronchial isomerism atrial appendages