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study to determine guidelines for pediatric colonoscopy

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World Journal of Gastroenterology 2017年第31期23卷 5773-5779页

作者： Shinichiro Yoshioka Hidetoshi Takedatsu Shuhei Fukunaga Kotaro Kuwaki Hiroshi Yamasaki RyosukeYamauchi Atsushi Mori Hiroshi Kawano Tadahiro Yanagi Tatsuki Mizuochi Kosuke Ushijima Keiichi Mitsuyama Osamu Tsuruta Takuji Torimura Division of Gastroenterology Department of Medicine Kurume University School of Medicine Department of Gastroenterology and Medicine Fukuoka University Faculty of Medicine Department of pediatrics and Child Health Kurume University school of Medicine

To investigated characteristics, diagnosis, bowel-cleansing preparation, sedation, and colonoscope length and diameter in Japanese pediatric patients receiving total colonoscopy. METHODSThe present study evaluated consecutive patients aged ≤ 15 years who had undergone their first colonoscopy in Kurume University between January 2007 and February 2015. Data were retrospectively analyzed. We identified 110 pediatric patients who had undergone colonoscopy that had reached the cecum, allowing the observation of the total colon. RESULTSHematochezia, abdominal pain, and diarrhea were the most common symptoms. For bowel-cleansing preparation, pediatric patients aged ≤ 12 years were treated with magnesium citrate, and patients aged 13-15 years were treated with polyethylene glycol 4000. For sedation, thiamylal with pentazocine, which has an analgesic effect, was used in patients aged ≤ 6 years, and midazolam with pentazocine was used in patients aged ≥ 7 years. Regarding the choice of endoscope, short and thin endoscopes were selected for younger patients, particularly patients aged ≤ 3 years. Positive diagnoses were made in 78 patients (70.9%). Inflammatory bowel disease (n = 49, 44.5%), including ulcerative colitis (n = 37, 33.6%) and Crohn’s disease (n = 12, 10.9%), was the most common diagnosis. CONCLUSIONColonoscopy offers a high diagnostic capability for pediatric patients with gastrointestinal symptoms. The selection of appropriate management the performance of colonoscopy is important in pediatric patients.

关键词： Pediatric endoscopy Sedation Bowel cleansing preparation Inflammatory bowel disease Complication

Double trisomy 48,XXX,+18 with multiple dysmorphic features

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World Journal of pediatrics 2015年第1期11卷 83-88页

作者： Zi-Yan Jiang Xiao-Hui Wu Chao-Chun Zou Department of pediatrics Children's Hospital Zhejiang University School of Medicine and the Key Laboratory of Reproductive Genetics(Zhejiang University) Ministry of Education

Background:Chromosomal abnormality is a common cause of congenital anomalies,psychiatric disorders,and mental ***,the double trisomy 48,XXX,+18 is a rare chromosome ***:Case report and literature ***:A 7-hour-old girl presented to our unit because of poor response after *** presented with multiple dysmorphic features,including small for gestational age infant,flat nasal bridge,widely-spaced eyes,the left thumb deformities,flat facial profile,raised sternum,ventricular septal defect,the third lateral brain ventricle enlargement,and small *** case expands the spectrum of malformations reported in association with the double trisomy 48,XXX,+*** literature on 16 fetuses or infants with the 48,XXX,+18 were also ***:These data suggested that in patients with clinical features similar to trisomy 18,especially with anomalies of the ears and/or reproductive malformations,double trisomy(48,XXX,+18)should be considered and karyotyping should be performed although it is a rare disease.

关键词： 48,XXX,+18 chromosome abnormality double trisomy multiple dysmorphic features

Applications of lung clearance index in monitoring children with cystic fibrosis

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World Journal of Clinical pediatrics 2019年第2期8卷 15-22页

作者： Andrew Fretzayas Konstantinos Douros Maria Moustaki Ioanna Loukou school of Medicine University of Athens Department of pediatrics Athens Medical Center Athens University Medical School Respiratory Unit Third Department of Pediatrics Athens University Medical School "Attikon" University Hospital Department of Cystic Fibrosis "Agia Sofia" Children’s Hospital

A sensitive, reproducible and feasible measure of lung function for monitoring the respiratory health is a prerequisite for the optimization of management of the patients with cystic fibrosis(CF). Spirometry has been considered the method of choice, although it is applicable only in children older than 6 years of age, as good cooperation is necessary for its proper performance. However, over the last15 years, scientific interest in gas dilution techniques and particularly in multiple breath wash out(MBW) method has been revived. The most commonly reported index of MBW is lung clearance index(LCI). The aim of this review is to present the most recent developments in the application of LCI as a monitoring index of respiratory status of CF patients. LCI is a sensitive and reproducible marker of ventilation inhomogeneity. It is more sensitive than spirometry and, unlike spirometry; it can be performed across the whole pediatric age range. Since it is dependent on body size, until at least the age of 6 years, the relative and not the absolute changes are more appropriate for providing clinically meaningful conclusion on ventilation inhomogeneity. Until now, MBW has been mainly used as a research tool. Based on the currently available data LCI cannot safely predict high-resolution computed tomography findings in children with CF, especially in infants. It can be used as an end-point measure for the assessment of beneficial effect of interventions. However, its utility as an outcome measure for the efficacy of therapeutic interventions seems to be dependent on the pathophysiologic mechanisms that underlie each intervention. It seems that more studies,especially longitudinal ones, are required in order to fully clarify the clinical usefulness of LCI, not only in the research setting, but also in every day practice of CF clinic.

关键词： Cystic fibrosis Respiratory health Lung clearance index Ventilation inhomogeneity

The role of surfactant and non-invasive mechanical ventilation in early management of respiratory distress syndrome in premature infants

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World Journal of pediatrics 2014年第3期10卷 204-210页

作者： Narayan Prabhu Iyer Maroun Jean Mhanna Department of pediatrics Division of NeonatologyCase Western Reserve University School of MedicineMetroHealth Medical CenterClevelandOhio 44109USA

Background:Surfactant replacement therapy has been used for few decades for the treatment of respiratory distress syndrome(RDS)and has significantly improved morbidity and mortality in premature ***-invasive respiratory support has recently emerged as a strategy in the early management of *** this review,we discuss the different strategies of early management of *** sources:A literature search of PubMed database was conducted to review the *** quality of evidence of key clinical studies was graded according to a modified grading system of the international GRADE ***:Continuous positive airway pressure(CPAP)with selective surfactant is a safe alternative to routine intubation,surfactant and mechanical ventilation in preterm infants with spontaneous breathing,and such an approach has been associated with decreased risk of death and bronchopulmonary *** is a risk of pneumothorax when using a high pressure of CPAP(≥8 cm of H_(2)O),a high partial pressure of carbon dioxide(PCO_(2)>75 mm of Hg),and a high fraction of inspired oxygen(FiO_(2)>0.6)as a threshold for intubation while on ***:Not all preterm infants need surfactant treatment,and non-invasive respiratory support is a safe and effective approach.

关键词： non-invasive mechanical ventilation prematurity respiratory distress syndrome

AfterWards:A Narrative Medicine Program at Johns Hopkins Medicine and in China

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Chinese Medicine and Culture 2023年第2期6卷 213-217页

作者： Lauren Small Department of pediatrics Johns Hopkins University School of MedicineBaltimoreMD 21205USA

Narrative medicine is a multidisciplinary field of inquiry and practice based on the premise that medical care takes place in the context of *** on narrative medicine training suggests that it conveys benefits such as improved communication skills and personal and professional growth to physicians,medical students,and other health care *** medicine can promote empathy and trust between patients and physicians and foster *** 2014,the author and a colleague started an ongoing inter-disciplinary narrative medicine program in the Children’s Center of the Johns Hopkins Hospital called *** program,which meets monthly,is open to all on a volunteer *** literature,art,and writing,AfterWards nurtures empathy,encourages reflective practice,and builds community among a diverse group of health care *** a series of lectures and workshops at Johns Hopkins Medicine,Peking Union Medical College,and Fudan Hospital in Shanghai,the author has introduced AfterWards to Chinese medical educators and *** with *** Hanson,she created an AfterWards Facilitator’s Guide for the use of Chinese practitioners.A recent White Paper on Chinese health care indicates that an infusion of humanities-based education,of which narrative medicine forms a part,can help rebuild patient-physician ***,there has been an increase in interest in narrative medicine in the United States and ***,more research is needed to demonstrate the impact of programs like *** to the implementation of narrative medicine programs remain,most significantly in terms of expertise,resources,and time.

关键词： AfterWards Narrative medicine

Circulating immunocompetent cell profiles during oral cyclosporin therapy for immunoglobulin-resistant Kawasaki disease

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World Journal of pediatrics 2021年第6期17卷 671-673页

作者： Seigo Okada Yuji OhnishiTakashi Furuta Yasuo Suzuki Akiko Kawakami-Miyake Chie Matsuguma Takako Waniishi Hiroki Yasudo Shunji Hasegawa Department of pediatrics Yamaguchi University Graduate School of Medicine1-1-1 MinamikogushiUbeYamaguchi 755-8505Japan

Although the standard treatment for Kawasaki disease(KD)is intravenous immunoglobulin(IVIG)combined with oral aspirin,18%of 1st IVIG is refractory[1].Recently,the efficacy of immunomodulatory drugs for IVIG-resistant KD,such as prednisolone,infliximab(IFX),and cyclosporine(CsA),has been reported[2-5].However,optimal immunomodulatory therapy for IVIG-resistant KD has not yet been established.

关键词： immuno drugs immunoglobulin

Application of isoxanthopterin as a new pterin marker in the differential diagnosis of hyperphenylalaninemia

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World Journal of pediatrics 2019年第1期15卷 66-71页

作者： Pei-Zhong Bao Jun Ye Lian-Shu Han Wen-Juan Qiu Hui-Wen Zhang Yong-Guo Yu Jian-Guo Wang Xue-Fan Gu Department of Endocrinology and Genetics Shanghai Institute of PediatricsXinhua HospitalShanghai Jiaotong University School of Medicine1665 Kong Jiang RoadShanghai 200092China

Background This study aimed to explore the value of applying a new pterin marker (isoxanthopterin) to the traditional urine pterin analysis to reduce the rate of mis-diagnosis of 6-pyruvoyltetrahydropterin synthase deficiency (PTPSD) and improve the accuracy of *** We compared the urine neopterin (N),biopterin (B),isoxanthopterin (Iso),B% and Iso% levels between patients with phenylalanine hydroxylase deficiency and those with PTPSD,and found the most specific pterin biomarkers by ROC analysis.A positive cut-off value of urine pterins was *** effect of combined Iso% + B + B% in reducing PTPSD mis-diagnosis was evaluated,and the different urine pterin levels in PTPSD and false PTPSD (FPTPSD) were *** concordance of PTPSD diagnosis by the new pterin scheme and gene mutation analysis was *** (1) Urinary B,B%,Iso and Iso% were significantly lower in PTPSD than those in phenylalanine hydroxylase-deficiency group (P < 0.01);(2) Iso%,B%,and B were the most specific markers;(3) The positive cut-off values of B,B%,Iso% for PTPSD were < 0.17 mmoL/moLCr,< 5.0%,and < 9.5%,respectively;(4) urinary B +B% + Iso% scheme significantly reduced the false-positive rate of PTPSD compared to traditional *** Iso% levels in FPTPSD group were higher than the ones in PTPSD group;(5) an accuracy of diagnosis for PTPSD was increased by 9-19% when Iso% was introduced to urinary pterin *** Iso% is helpful to reduce the rate of misdiagnosis of PTPSD in the diagnosis by urinary pterin analysis for hyperphenylalaninemias and improve the accuracy of *** approach is worthy of further development and increased utilization.

关键词： 6-Pyruvoyltetrahydropterin synthase Hyperphenylalaninemia Isoxanthopterin Phenylalanine hydroxylase Pterin

Persistent hypertransaminasemia in asymptomatic children: A stepwise approach

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World Journal of Gastroenterology 2013年第18期19卷 2740-2751页

作者： Pietro Vajro Sergio Maddaluno Claudio Veropalumbo Department of Medicine and Surgery Pediatrics University of Salerno 84081 Baronissi Italy European Laboratory for Food Induced Disease 84081 Baronissi Italy Department of Translational Medical Sciences Pediatrics Medical School of the University of Naples "Federico Ⅱ" 80131 Naples Italy

We aimed to examine the major causes of isolated chronic hypertransaminasemia in asymptomatic children and develop a comprehensive diagnostic flow diagram. A MEDLINE search inclusive of publications throughout August 2012 was performed. We found only a small number of publications that had comprehensively investigated this topic. Consequently, it was difficult to construct a diagnostic flowchart similar to those already available for adults. In children, a "retesting panel" prescription, including gamma-glutamyl transpeptidase and creatine kinase in addition to aminotransferases, is considered a reasonable approach for proficiently confirming the persistence of the abnormality, ruling out cholestatic hepatopathies and myopathies, and guiding the subsequent diagnostic steps. If re-evaluation of physical and historical findings suggests specific etiologies, then these should be evaluated in the initial enzyme retesting panel. A simple multistep diagnostic algorithm incorporating a large number of possible pediatric scenarios, in addition to the few common to adults, is available. Accurately classifying a child with asymptomatic persistent hypertransaminas-emia may be a difficult task, but the results are critical for preventing the progression of an underlying, possibly occult, condition later in childhood or during transition. Given the high benefit/cost ratio of preventing hepatic deterioration, no effort should be spared in diagnosing and properly treating each case of persistent hypertransaminasemia in pediatric patients.

关键词： Transaminase Aminotransferase Hyper-transaminasemia Liver disease Children

Diagnosis,treatment and prevention of type 2 diabetes mellitus in children and adolescents

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World Journal of Diabetes 2021年第4期12卷 344-365页

作者： Anastasios Serbis Vasileios Giapros Eleni P Kotanidou Assimina Galli-Tsinopoulou Ekaterini Siomou Department of pediatrics University Hospital of IoanninaIoannina 45500Greece Department of Child Health University of IoanninaIoannina 45500Greece Department of pediatrics Medical SchoolAristotle University ThessalonikiThessaloniki 54636Greece

During the last two decades,there have been several reports of an increasing incidence of type 2 diabetes mellitus(T2 DM) in children and adolescents,especially among those belonging to minority ethnic *** trend,which parallels the increases in prevalence and degree of pediatric obesity,has caused great concern,even though T2 DM remains a relatively rare disease in *** T2 DM differs not only from type 1 diabetes in children,from which it is sometimes difficult to differentiate,but also from T2 DM in adults,since it appears to be an aggressive disease with rapidly progressive β-cell decline,high treatment failure rate,and accelerated development of *** the recent research,many aspects of youth T2 DM still remain unknown,regarding both its pathophysiology and risk factor contribution,and its optimal management and *** management approaches include lifestyle changes,such as improved diet and increased physical activity,together with pharmacological interventions,including metformin,insulin,and the recently approved glucagonlike peptide-1 analog *** is more important for everyone to realize though,from patients,families and physicians to schools,health services and policy-makers alike,is that T2 DM is a largely preventable disease that will be addressed effectively only if its major contributor(i.e.,pediatric obesity) is confronted and prevented at every possible stage of life,from conception until ***,relevant comprehensive,coordinated,and innovative strategies are urgently needed.

关键词： Type 2 diabetes Children Adolescents Diagnosis Treatment Prevention