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Implementation of a High-Risk Outpatient clinic for Children with Complex Congenital Heart Disease in a Reference Service in Brazil

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Congenital Heart Disease 2023年第6期18卷 649-656页

作者： Gustavo Foronda Vanessa Ferreira Amorim de Melo Claudia Regina Pinheiro de Castro Grau Ingrid Magatti Piva Glaucia Maria Penha Tavares Ana Cristina Sayuri Tanaka Nana Miura Department of pediatric Cardiology and Adult Congenital Heart Diseases Heart Institute of the University of São PauloSão PauloBrazil Foronda clinic São PauloBrazil Institute of Orthopedics and Traumatology University of São PauloSão PauloBrazil Department of Echocardiography Heart Institute of the University of São PauloSão PauloBrazil

Background:Children with congenital heart disease(CHD),even after surgical approaches,and especially those who undergo staged procedures in theﬁrst months of life,remain vulnerable to readmissions and complications,requiring very close monitoring and differentiated intervention ***:Descriptive and exploratory study,of the experience report type,which presents the process of building the high-risk outpatient clinic for complex congenital heart diseases(AAR)at the Instituto do Coração(InCor).Results:Report of the path taken to structure the AAR,demonstrating the organization,interface with the multidisciplinary team,admission and discharge criteria,training,and patient proﬁ*** theseﬁve years of care,275 patients were treated,59.65%with biventricular interstage physiology,followed by univentricular interstage physiology(34.55%),residual defects after surgical procedures(3.63%),tumors with risk of mechanical obstruction(1.45%)and patients with an intrauterine approach(0.72%).The signiﬁcant number of critical patients who were successfully discharged from hospital(44.72%),the low mortality rate due to sudden deaths at home and the high adherence to follow-up cor-roborate the impact of this specialized ***:Despite the limitations of the study,this experi-ence report showed that with few resources,there is the possibility of organizing an AAR with differentiated care,with the objective of early detection and treatment of residual injuries,identiﬁcation of early interventions,edu-cation of parents for follow-up of their children,resulting in individualized treatment,promoting a better quality of life for this population.

关键词： Congenital heart defects ambulatory care heart defects

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Outcomes in children with Clostridium difficile infection:results from a nationwide survey

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Gastroenterology Report 2016年第4期4卷 293-298,I0002页

作者： Arjun Gupta Darrell S Pardi Larry M Baddour Sahil Khanna Divisions of Infectious Diseases Mayo ClinicRochesterMNUSA Divisions of Gastroenterology and Hepatology Mayo ClinicRochesterMNUSA Department of Internal Medicine University of Texas Southwestern Medical CenterDallasTXUSA

Objective:Hospital-and population-based studies demonstrate an increasing incidence of Clostridium difficile infection(CDI)in adults and children;although pediatric CDI outcomes are incompletely *** analysed United States National Hospital Discharge Survey(NHDS)data to study CDI in hospitalized ***:NHDS data for 2005–2009(demographics,diagnoses and discharge status)were obtained;cases and comorbidities were identified using ICD-9 *** univariate and multivariate analyses were performed to ascertain incidence of CDI;associations between CDI and outcomes[length of stay(LOS),colectomy,all-cause in-hospital mortality and discharge to a care facility(DTCF)].Results:Of an estimated 13.8 million pediatric inpatients;46176 had CDI;median age was 3 years;overall incidence was 33.5/10000 *** annual frequency of CDI did not vary from 2005 to 2009(0.24–0.43%;P=0.64).On univariate analyses,children with CDI had a longer median LOS(6 vs 2 days),higher rates of colectomy[odds ratio(OR)2.0;95%confidence interval(CI)1.7–2.4],mortality(OR 2.5;95% CI 2.3–2.7),and DTCF(OR 1.6;95% CI 1.6–1.7)(all P<0.0001).After adjusting for age,sex and comorbidities,CDI was an independent and the strongest predictor of increased LOS(adjusted mean difference,6.4 days;95% CI 5.4–7.4),higher rates of colectomy(OR 2.1;95% CI 1.8–2.5),mortality(OR 2.3;95% CI 2.2–2.5),and DTCF(OR 1.7;95% CI 1.6–1.8)(all P<0.0001).On excluding infants from the analysis,children with CDI had higher rates of mortality,DTCF and longer LOS than children without ***:Despite increased awareness and advancements in management,CDI remains a significant problem and is associated with increased LOS,colectomy,in-hospital mortality and DTCF in hospitalized children.

关键词： Clostridium difficile infection children pediatric outcomes epidemiology

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Presentation of the Diverticulum of the Caecum, Cause of the Acute Abdomen

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Surgical Science 2016年第2期7卷 54-57页

作者： Afrim Avdaj Nexhmi Hyseni Curr Gjocaj Agron Bytyqi Artur Avdaj Hatim Baxhaku Division of Surgery Regional Hospital “Primarius Daut Mustafa” Prizren Kosova Surgical pediatric clinic Department of Pediatric Surgery University Clinic Centre of Pristina Prishtina Kosovo Hospital and University clinical Service of Kosovo Prishtina Kosovo Division of Education Regional Hospital “Primarius Daut Mustafa” Prizren Kosova Medical Faculty in Pristina Pristina Kosovo

Solitary caecal diverticulum is an uncommon entity and therefore it is difficult to diagnose except during surgery exploration. It is extremely difficult to differentiate it preoperatively from acute appendicitis. We report a case of an enlarged colon segment, presenting macroscopically as tumor diverticulum in a 27-year-old female patient, presenting with a 2 day history of a severe abdominal right lower quadrant pain with accompanying anorexia, nausea, vomiting and high body temperature. After clinical assessment, laboratory examination, X-ray, and CT are performed, the indication for surgical treatment is set.

关键词： Caecum Diverticulum Acute Abdomen Abdominal Pain

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Fibrolamellar hepatocellular carcinoma:Exploring molecular mechanisms and differentiation pathways to better understand disease outcomes and prognosis

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Liver Research 2017年第4期1卷 187-192页

作者： Cassandra A.Kersten Elise N.Sloey Eric Zhou Ying Peng Michael S.Torbenson Yi Guo Department of Biochemistry and Molecular Biology Mayo ClinicRochesterMNUSA Division of Gastroenterology and Liver Pathology Department of Laboratory Medicine and PathologyMayo ClinicRochesterMNUSA Division of Gastroenterology and Hepatology Mayo ClinicRochesterMNUSA

Fibrolamellar hepatocellular carcinoma(FLC)is a rare but aggressive liver cancer of children that occurs predominantly in teenagers without a history of liver *** resection remains the only therapeutic option,and the recurrence rate is extremely high(>50%within 3 years).A newly discovered chromosomal deletion that occurs in the majority of FLCs generates a novel kinase fusion between DnaJ heat shock protein family member B1(DNAJB1)and protein kinase cAMP-activated catalytic subunit alpha(PRKACA)(DNAJB1-PRKACA).Despite its high penetrance and apparent specificity for FLC,the oncogenic role of this fusion event remains *** this review article,we discuss the histology,presentation and diagnosis,current treatment,and roles of the DNAJB1-PRKACA as well as research models contributing to our understanding of this disease.

关键词： Fibrolamellar hepatocellular carcinoma(FLC) DnaJ heat shock protein family member B1 (DNAJB1) Protein kinase cAMP-activated catalytic subunit alpha(PRKACA)pediatric cancer Liver cancer

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Semen analysis in chronic bacterial prostatitis： diagnostic and therapeutic implications

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Asian Journal of Andrology 2009年第4期11卷 461-477页

作者： Vittorio Magri Florian M. E. Wagenlehner Emanuele Montanari Emanuela Marras Viviana Orlandi Antonella Restelli Erminio Torresani Kurt G. Naber Gianpaolo Perletti Urology and Sonography Primary Care clinic Hospital：＂Istituti Clinici di Perfezionamento＂ Milano 20100 Italy Department of Urology and pediatric Urology Justus-Liebig-University Giessen 35390 Germany Third Urological clinic San Paolo Hospital University of Milan Milan 20142 Italy Laboratory of Toxicology and Pharmacology Department of Structural and Functional Biology University of Insubria Busto A. 21052 Italy Laboratory of Molecular and Environmental Microbiology Department of Structural and Functional Biology University of lnsubria Varese 21100 Italy Microbiology Unit Hospital：＂Osp. Maggiore Policlinico Mangiagalli Regina Elena＂ Milano 20122 Italy Technical University Munich Munich 80333 Germany

The significance and diagnostic value of semen analysis in chronic bacterial prostatitis has been extensively debated and remains controversial. To investigate the diagnostic relevance of semen culture in the bacteriological workup of prostatitis patients, we retrospectively analyzed a clinical database of 696 symptomatic patients. All patients were routinely subjected to a four-glass test, followed by semen culture and analysis. This allowed to dissect from the database three different diagnostic scenarios, and to compare the ＇two-glass＇ pre-/post- massage test and the standard ＇four-glass＇ test with a ＇five-glass＇ test （four-glass plus post-VB3 semen culture）. The ＇five-glass＇ test showed 3.6- or 6.5-fold increases in relative sensitivity and lesser reductions （-13.2% or -14.7%） in relative specificity for traditional uropathogens （TUs） compared with the four-glass or two-glass test, respectively. The area under the ROC curve and Jouden＇s index were increased, whereas positive and negative likelihood ratios were lower than comparators, indicating that the ＇five-glass＇ assay may be superior in confirming the negative outcome of both standard tests. The five-, four-, and two-glass tests detected TUs （Enterobacteriaceae, Enterococci, etc.） in 120, 33, and 20 patients and unusual pathogens （Streptococci, other Gram-positive species, Mycoplasmata, and others） in 130, 56, and 45 patients, respectively. When patients were subjected to pharmacological treatment, including a combination of a fluoroquinolone and a macrolide, no differences in eradication rates were observed between groups diagnosed with different tests, irrespective of pathogen category. Eradication was associated with long-term sign/symptom remission; no significant intergroup differences in sign/symptom scores were observed throughout a 24-month off-therapy follow-up period. In conclusion, our data support the usefulness of semen analysis in the diagnostic workup ofprostatitis patients when this test is u

关键词： alfuzosin azithromycin chronic bacterial prostatitis chronic pelvic pain syndrome ciprofloxacin Meares and Stamey test National Institutes of Health Chronic Prostatitis Symptom Index prostatitis semen analysis seminal fluid Serenoa repens

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Impact of Seven Valent Pneumococcal Conjugate Vaccine on Nasopharyngeal Carriage in Young Children in Okinawa, Japan

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World Journal of Vaccines 2015年第2期5卷 88-95页

作者： Hideki Akeda Bin Chang Yoshihisa Nakamura Hirotsune Hamabata Kenji Ameku Takaya Toma Eiichi Tamanaha Makoto Ohnishi Okinawa Prefectural Nanbu Medical Center & Children’s Medical Center Okinawa Japan Department of Bacteriology I National Institute of Infectious Diseases Tokyo Japan Nakamura clinic pediatric Dermatology and Internal Medicine Okinawa Japan Awase Daiichi clinic Okinawa Japan Syuri Child clinic Okinawa Japan Wanpaku clinic Okinawa Japan The Okinawa Society of Child Health Okinawa Japan

In Japan, the heptavalent pneumococcal conjugate vaccine (PCV7) became available in February 2010 and was subsidized by the national funding system from May 2011 in Okinawa, after which it was incorporated into the national immunization practice (NIP) in April 2013 using a 3 + 1 schedule for all infants. We conducted an annual survey in 2012 to determine the effect of PCV7 on nasopharyngeal colonization by pneumococcal serotypes and to analyze the risk factors for colonization in infants. Nasopharyngeal swabs for pneumococcal isolation and serotyping were obtained from infant 2 to 22 months of age before and after PCV7 immunization among 4 clinics in Okinawa, Japan. Between January 2012 and December 2012, nasopharyngeal swabs for bacterial cultures were obtained among 782 infants aged 2 to 22 months old and demographic data was obtained among 725 participant infants. Among the 725 evaluable infants, 193 pneumococcal strains were detected in 180 infants for an overall nasopharyngeal carriage of 24.8%. The main capsular serotypes isolated were 6C (16.1%), 19A (12.4%) and 15B (9.8%). Carriage of PCV7 serotypes accounted for 21.8% (42/193). The result of multivariate data analysis showed the pneumococcal carriage rate of non-PCV7 serotypes was significantly (P < 0.001) high in infant with siblings and daycare attendance. On the other hand, the result of multivariate data analysis showed that carriage rate of PCV7 serotype had only significantly high risk in infant with siblings and did not have a significant risk dependent on age and daycare attendance. Carriage PCV7 serotypes increased in the presence of other siblings, while PCV7 vaccination was shown to eliminate daycare attendance as a risk. The results of this study demonstrates that PCV7 vaccination decrease the overall nasopharyngeal carriage of PCV7 serotypes in vaccinated children including children at risk such as children attending day-care centers.

关键词： Nasopharynx Carriage Pneumococcal Conjugate Vaccine Japan Young Children

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Effectiveness of Huai Qi Huang Granules on Juvenile Collagen-induced Arthritis and Its Influence on Pyroptosis Pathway in Synovial Tissue

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Current Medical Science 2019年第5期39卷 784-793页

作者： Ting HE Xie XU Xin-yan ZHANG Pan SHEN Jia-yun LING Yan-xin-li HAN Yu WEN Xiu-fen HU Hui-ling LU Division of pediatric Rheumatology and Immunology Tongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430030China

Summary:Huai Qi Huang(HQH)exerts great effects in clinic,such as anti-inflammation,immune-regulation,anti-cancer,and so ***,the mechanism by which HQH protects juvenile idiopathic arthritis(JIA)is ***,we explored deeply the protective mechanisms in juvenile collagen-induced arthritis(CIA)rat *** is Gasdermin D(GSDMD)-dependent programmed cell death,involved in many diseases,such as *** investigated whether GSDMD-induced pyroptosis take part in mechanisms of juvenile CIA *** Wistar rats(3-4 weeks)were injected intradermally with fully emulsified bovine typeⅡcollagen and complete Freund's adjuvant to establish CIA rat ***,the CIA rats received oral administration of HQH(4.16 g/kg)once a day from the day 21 of modeling,with the treatment lasting for 28 *** of indicators were measured for evaluation of anti-inflammation effect of HQH,including hind paw swelling,arthritis scores,micro CT,and histopathological changes and the level of pro-inflammatory cytokines in the serum,including tumor necrosis factor alpha(TNF-α)and interleukin-18(IL-18).The expression of GSDMD and caspasein the joint synovial tissues was *** results demonstrated that the expression of the pyroptotic protein GSDMD and its upstream caspase-1 was significantly increased in the synovial tissues of CIA *** treatment of HQH ameliorated the symptoms in CIA rats,reduced levels of pro-inflammatory cytokines and hind paw swelling,down-regulated the expression of GDSMD and *** pyroptosis participated in the pathogenesis of CIA *** study supported that HQH can effectively improve joints inflammation of juvenile collagen-induced arthritis rats by inhibiting pyroptosis pathway in the joint synovial tissues.

关键词： Huai Qi Huang juvenile collagen-induced arthritis Gasdermin D pyroptosis

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A Rare Case of Chronic Recurrent Multifocal Osteomyelitis with Undifferentiated Juvenile Idiopathic Arthritis, Uveitis, and Psoriasis

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Case Reports in clinical Medicine 2016年第7期5卷 225-232页

作者： Yongdong Zhao Shannon K. Foster Todd J. Murdock Margret Schlesinger Carol A. Wallace pediatric Rheumatology Seattle Children’s Hospital Seattle WA USA Western Montana clinic Missoula MT USA Rocky Mountain Eye Center Missoula MT USA

We report here a 17-year-old boy with a complicated presentation of undifferentiated juvenile idiopathic arthritis, vision-threatening uveitis and chronic recurrent multifocal osteomyelitis (CRMO) in the pelvis. His severe iritis needed subtenon injections and only responded to infliximab after failing multiple biologics. Unfortunately he later developed infliximab-associated psoriasis. A combination of infliximab and ustekinumab induced remission of his arthritis, osteomyelitis, uveitis and psoriasis without experiencing severe infections.

关键词： Juvenile Idiopathic Arthritis Uveitis Psoriasis Chronic Recurrent Multifocal Osteomyelitis Infliximab Ustekinumab

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Dealing with pediatric glaucoma: from medical to surgical management-a narrative review

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Annals of Eye Science 2021年第3期6卷 30-36页

作者： Matteo Sacchi Rosario Alfio Umberto Lizzio Gianluca Monsellato University Eye clinic San Giuseppe HospitalMilanoItaly

pediatric glaucoma is a potentially sight-threatening disease and is considered the second leading cause of treatable childhood *** glaucoma is a clinical entity including a wide range of conditions:primary congenital glaucoma,glaucoma secondary to ocular(e.g.,aniridia,Peter’s anomaly),or systemic disease(e.g.,Sturge Weber)and glaucoma secondary to acquired condition(pseudophakic,traumatic,uveitic glaucoma).The treatment algorithm of childhood glaucoma is a step-by-step approach,often starting with surgery,as in primary congenital glaucoma *** therapy is also crucial in the management of pediatric *** we reported the results of the randomized,controlled,clinical trials carried out in children treated with topical anti-glaucoma *** is worth knowing that prostaglandin analogues showed an excellent systemic safety profile,while serious systemic events have been reported in children taking topical *** surgery is the first surgical option in patients diagnosed with primary congenital glaucoma,with ab interno and ab externo approaches showing similar *** in children can be troublesome,as mitomycin C(MMC)can lead to bleb complications and a higher endophthalmitis rate than in *** drainage devices(GDD)are no longer a last resort and can be considered a suitable option for the management of uncontrolled pediatric glaucoma after angle surgery failure.

关键词： pediatric glaucoma anti-glaucoma medication angle surgery trabeculectomy glaucoma drainage device(GDD)

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Anesthetic Technique for Transesophageal Electrophysiology Studies in pediatric Patients with Wolff-Parkinson-White Syndrome

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Open Journal of pediatrics 2015年第1期5卷 17-22页

作者： George M. Gilly Walter J. Hoyt Donald E. Harmon Eric H. Busch Bobby D. Nossaman David M. Broussard Christopher S. Snyder Department of Anesthesiology Ochsner Clinic Foundation New Orleans LA USA Division of pediatric Cardiology University of Virginia Charlottesville VA USA Department of pediatrics Division of Pediatric Cardiology Rainbow Babies and Children’s Hospital Case Western Reserve School of Medicine Cleveland OH USA

Objective: Patients with Wolff-Parkinson-White (WPW) Syndrome require risk assessment to determine their potential for sudden cardiac death. Transesophageal electrophysiology studies (TEEPS) are an effective risk stratification tool. The purpose of this study is to describe a minimially invasive, effective anesthetic technique to employ during transesophageal electrophysiology studies. Methods: A retrospective review of anesthetic technique utilized during TEEPS. Inclusion criteria;WPW on ECG;age pediatric patients with Wolff-Parkinson-White syndrome underwent numerous attempts to make the procedure easy, reliable and reproducible for anesthesia and electrophysiologist. The eventual technique that proved to meet these criteria during a transesopheagel electrophysiology procedure was utilization of mask induction with continuous IV sedation.

关键词： Wolff Parkinson White Transesophagel Electrophysiology Study

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