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pediatric Wilson disease presenting as acute liver failure:Prognostic indices

World Journal of Clinical Cases 2021年第14期9卷 3273-3286页

作者： Wei-Yuan Fang Kuerbanjiang Abuduxikuer Peng Shi Yi-Ling Qiu Jing Zhao Yu-Chuan Li Xue-Yuan Zhang Neng-Li Wang Xin-Bao Xie Yi Lu A S Knisely Jian-She Wang Center for pediatric Liver Diseases Children’s Hospital of Fudan UniversityShanghai 201102China Medical Statistics Department Children’s Hospital of Fudan UniversityShanghai 201102China Institut für Pathologie Medizinische Universität GrazGraz 8010Austria

BACKGROUND Acute liver failure(ALF)can be a primary presentation of Wilson disease(WD).Mortality rates are high in WD with ALF(WDALF).Predictions of mortality in WDALF vary by model and are sometimes contradictory,perhaps because few patients are studied or WD diagnoses are *** To determine the outcomes among well-documented WDALF patients and assess mortality model performance in this *** We reviewed the medical records of our pediatric WDALF patients(n=41 over 6-years-old,single-center retrospective study)and compared seven prognostic models(King’s College Hospital Criteria,model for end-stage liver disease/pediatric end-stage liver disease scoring systems,Liver Injury Unit[LIU]using prothrombin time[PT]or international normalized ratio[INR],admission LIU using PT or INR,and Devarbhavi model)with one *** Among the 41 Han Chinese patients with ALF,WD was established by demonstrating ATP7B variants in *** 5 others,Kayser-Fleischer rings and Coombs-negative hemolytic anemia permitted *** died during hospitalization and three underwent liver transplantation(LT)within 1 mo of presentation and survived(7.3%each);35(85.4%)survived without LT when given enteral D-penicillamine and zinc-salt therapy with or without urgent *** significantly correlated with mortality included encephalopathy,coagulopathy,and gamma-glutamyl transpeptidase activity,bilirubin,ammonia,and serum sodium *** under the receiver operating curves varied among seven prognostic models from 0.981 to 0.748 with positive predictive values from 0.214 to *** WDALF children can survive and recover without LT when given D-penicillamine and Zn with or without plasmapheresis,even after enlisting for LT.

关键词： Acute liver failure ATP7B D-penicillamine Liver transplant Wilson disease Zinc

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Clinical cardiac regenerative studies in children

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World Journal of Cardiology 2017年第2期9卷 147-153页

作者： Imre J Pavo Ina Michel-Behnke Division of pediatric Car-diology Department of Pediatrics and Adolescent MedicineMedical University of ViennaA1090 ViennaAustria

Although the incidence of pediatric heart failure is low, the mortality is relatively high, with severe clinical symptoms requiring repeated hospitalization or intensive care treatment in the surviving patients. Cardiac biopsy specimens have revealed a higher number of resident human cardiac progenitor cells, with greater proliferation and differentiation capacity, in the neonatal period as compared with adults, demonstrating the regeneration potential of the young heart, with rising interest in cardiac regeneration therapy in critically ill pediatric patients. We review here the available literature data, searching the MEDLINE, Google Scholar and EMBASE database for completed, and *** homepage for ongoing studies involving pediatric cardiac regeneration reports. Because of difficulties conducting randomized blinded clinical trials in pediatric patients, mostly case reports or cohort studies with a limited number of individuals have been published in the field of pediatric regenerative cardiology. The majority of pediatric autologous cell transplantations into the cardiac tissue have been performed in critically ill children with severe or terminal heart failure. Congenital heart disease, myocarditis, and idiopathic hypertrophic or dilated cardiomyopathy leading to congestive heart failure are some possible areas of interest for pediatric cardiac regeneration therapy. Autologous bone marrow mononuclear cells, progenitor cells, or cardiospheres have been applied either intracoronary or percutaneously intramyocardially in severely ill children, leading to a reported clinical benefit of cell-based cardiac therapies. In conclusion, compassionate use of autologous stem cell administration has led to at least short-term improvement in heart function and clinical stability in the majority of the critically ill pediatric patients.

关键词：先天的心疾病心失败心脏的新生基于房间的治疗住院孩子

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Outcome after Discharge from Hospital of Children with Complicated Severe Acute Malnutrition and Predictors Factors of Non-Response during Outpatient Treatment, in Senegal

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Open Journal of pediatrics 2023年第4期13卷 473-483页

作者： Abou Ba Aliou Thiongane Babacar Niang Younoussa Keïta Fatou Ly Aliou Abdoulaye Ndongo Ousmane Ndiaye pediatric Department of Dalal Jamm National Hospital Center Dakar Senegal Institute of Social pediatrics of Cheikh Anta DIOP University Dakar Senegal Albert Royer Children’s National Hospital Center Dakar Senegal Abass Ndao Hospital Center Dakar Senegal Pikine National Hospital Center Dakar Senegal

Introduction: Malnutrition is a public health problem. It is responsible for high morbidity and mortality in children aged 6 - 59 months. The aim of this study was to determine the outcome of children with complicated severe acute malnutrition after hospital discharge. Methods: This is a descriptive and analytical cross-sectional study conducted from March 1 to November 31, 2021 at the Albert Royer Children’s Hospital. Children discharged from hospital for complicated severe acute malnutrition were included. Results: A total of 103 children were included. The mean age of children at hospitalization was 16.41 ± 10.11 months and mean duration of follow-up after hospital discharge was 29.63 ± 8.59 months. Three children (2.91%) died after hospital discharge. The mean z-score of the weight-for-current height was -1.34 ± 1.08. Almost a quarter (24.27%) of the children remain acute malnourished with 3.88% of severe acute malnutrition (SAM). The predictors’ factors associated with non-response were weaning before 2 years of age (ORaj: 12.21;95% CI [6.82 - 18.44];p = 0.04) and tuberculosis (ORaj: 21.06;95% CI [12.54 - 41.09];p = 0.03). Conclusion: The rate of recovery of nutritional status in children with complicated severe acute malnutrition is satisfactory. Ablactation before the age of two and the existence of tuberculosis are significantly associated with non-recovery of nutritional status.

关键词： Children Severe Acute Malnutrition Recovery Non-Response

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Outcome in neonates with necrotizing enterocolitis and patent ductus arteriosus

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世界儿科杂志:英文版 2016年第1期12卷 55-59页

作者： Ulf Kessler Franzisca Schulte Dietmar Cholewa Mathias Nelle Stephan C.Schaefer Peter M.Klimek Steffen Berger Department of pediatric Surgery InselspitalBern University Hospital and University of BernSwitzerland Division of Neonatology Department of PediatricsInselspitalBern University Hospital and University of BernSwitzerland Institute of Pathology InselspitalUniversity of BernBernSwitzerland Department of Surgery HFR Fribourg-Cantonal HospitalSwitzerland

Background:There is no agreement of the influence of patent ductus arteriosus(PDA)on outcomes in patients with necrotizing enterocolitis(NEC).In this study,we assessed the infl uence of PDA on NEC ***:A retrospective study of 131 infants with established NEC was ***(death,disease severity,need for surgery,hospitalization duration),as well as multiple clinical parameters were compared between NEC patients with no congenital heart disease(n=102)and those with isolated PDA(n=29).Univariate,multivariate and stepwise logistic regression analyses were ***:Birth weight and gestational age were significantly lower in patients with PDA[median(95%CI):1120 g(1009-1562 g),28.4 wk(27.8-30.5 wk)]than in those without PDA[median(95%CI):1580 g(1593-1905 g),32.4 wk(31.8-33.5 wk);P<0.05].The risk of NEC-attributable fatality was higher in NEC patients with PDA(35%)than in NEC patients without PDA(14%)[univariate odds ratio(OR)=3.3,95%CI:1.8-8.6,P<0.05;multivariate OR=2.4,95%CI:0.82-2.39,P=0.111].Significant independent predictors for nonsurvival within the entire cohort were advanced disease severity stage III(OR=27.9,95%CI:7.4-105,P<0.001)and birth weight below 1100 g(OR=5.7,95%CI:1.7-19.4,P<0.01).Conclusions:In patients with NEC,the presence of PDA is associated with an increased risk of ***,when important differences between the two study groups are controlled,only birth weight and disease severity may independently predict mortality.

关键词： congenital heart disease necrotizing enterocolitis neonatal mortality patent ductus arteriosus

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Endothelin-1 Antagonist in the Management of Refractory Systemic Hypertension in Children with End Stage Kidney Disease: A Case Series

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World Journal of Cardiovascular Diseases 2014年第5期4卷 217-224页

作者： Jayanthi Chandar Wacharee Seeherunvong Carolyn Abitbol Chryso Katsoufis Alcia Edwards-Richards Teresa Cano Gastón Zilleruelo Department of pediatrics Division of Pediatric Nephrology Holtz Children’s Hospital University of Miami Miller School of Medicine Miami USA

Uncontrolled hypertension is a significant problem in children with end stage kidney disease. We studied 10 children on chronic hemodialysis who received the endothelin-1 receptor antagonist, bosentan as adjunctive therapy in refractory systemic hypertension. Data were retrospectively analyzed on systolic (SBP) and diastolic blood pressure (DBP), antihypertensive medications and hospitalization days per patient per year, at baseline and 3 months after initiation of bosentan. The standard deviation (Z) score of BP for age, height and gender was calculated. Mean age and weight of these children were 11.4 ± 4.8 years (range 1 - 17.5 years) and 32.9 ± 14.4 kg (range 7.4 - 57 kg) respectively and 90% were African American. Seven were on hemodialysis ≥4 times weekly. The average inter-dialytic weight gain was 4.7% ± 1.8%. The pre-dialysis SBP and DBP Z scores were 4.9 ± 1.8 and 4.0 ± 1.0 at baseline and decreased to 1.3 ± 0.4 and 1.3 ± 0.8, 3 months after initiation of bosentan (p hospitalization days decreased from 35.6 ± 52.4 days to 1.5 ± 3.3 days (p < 0.0001). This preliminary report indicates bosentan may be beneficial in the management of children with severe refractory hypertension.

关键词： Children End Stage Kidney Disease Hypertension Bosentan

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Liver cirrhosis in a child associated with Castleman's disease: A case report

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World Journal of Clinical Cases 2020年第9期8卷 1656-1665页

作者： Soya Kobayashi Ayano Inui Tomoyuki Tsunoda Syuichiro Umetsu Tsuyoshi Sogo Masaaki Mori Masato Shinkai Tomoo Fujisawa Department of pediatric Hepatology and Gastroenterology Saiseikai Yokohama City Tobu HospitalYokohama 230-8765Japan Department of Lifetime Clinical Immunology Graduate School of Medical and Dental SciencesTokyo Medical and Dental UniversityTokyo 113-8519Japan Department of Surgery Kanagawa Children’s Medical CenterYokohama 232-8555Japan

BACKGROUND Castleman's disease(CD)is a lymphoproliferative *** syndrome is classified as a variant of CD based on its key clinical manifestations of thrombocytopenia,anasarca(generalized edema and pleural effusion),fever(pyrexia),reticulin fibrosis in the bone marrow and the proliferation of megakaryocytes,and organomegaly(such as hepatosplenomegaly and multiple lymphadenopathies);TAFRO syndrome is mainly reported in Japanese *** our knowledge,this is the first pediatric case report detailing a CD-associated disorder progressing to *** SUMMARY A 10-year old male patient presented with fever and *** months before hospitalization,he had remarkable abdominal ***,he visited a clinic for a fever that lasted 5 *** physical findings were marked hepatosplenomegaly and cervical lymphadenopathy.A blood test revealed leukocytosis,microcytic anemia,aspartate aminotransferase-dominant transaminase elevation,high levels of C-reactive protein,polyclonal hypergammaglobulinemia,and high levels of interleukin-6 and vascular endothelial growth *** contrast computed tomography and magnetic resonance imaging suggested cirrhosis,which was confirmed by liver *** findings in the enlarged hepatic lymph nodes revealed both hyperplasia and atrophy of lymphoid follicles with some vascular hyperplasia and moderate plasmacytosis between the lymphoid follicles,which is compatible with lymph node histology in TAFRO *** was not effective in reducing the patient’s symptoms;therefore,the patient was prescribed *** date,the patient remains free of fever and continues to receive *** We described the clinicopathological features of TAFRO syndrome to highlight the clinical presentation of this rare disease in a pediatric case.

关键词： Castleman disease Case report Multi-centric Castleman’s disease Liver cirrhosis Tocilizumab Child

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Seroprevalence of SARS-CoV-2 infections among children visiting a hospital

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pediatric Investigation 2020年第4期4卷 236-241页

作者： Ran Wang Fang Jin Shuhui Cao Hong Yuan Jingchen Qu Jiaqi Zhang Yuxuan Li Xiangpeng Chen Wenqi Song Zhengde Xie Beijing Key Laboratory of pediatric Respiratory Infectious Diseases Key Laboratory of Major Diseases in ChildrenMinistry of EducationNational Clinical Research Center for Respiratory DiseasesResearch Unit of Critical Infection in ChildrenChinese Academy of Medical Sciences2019RUO16Laboratory of Infectionand VirologyBeijing Pediatric Research InstituteBeijing Children's HospitalNational Center for Children's HealthCapital Medical UniversityBeijingChina Department of Laboratory Medicine Beijing Children's HospitalNational Center for Children's HealthCapital Medical UniversityBeijingChina

Importance:In this study,we retrospectively investigated the seroprevalence of severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)antibodies within serum samples from children in Beijing,*** findings provide preliminary guidance regarding population susceptibility to SARS-CoV-2,which will aid in establishing policy toward coronavirus disease 2019(COVID-19)prevention and ***:To understand the seropositivity of anti-SARS-CoV-2 IgM/IgG antibodies among children in Beijing,China,evaluate the susceptibility of children in Beijing to SARS-CoV-2,and provide prima facie evidence to guide SARS-CoV-2 prevention and ***:IgM/IgG antibody kits(colloidal gold)were used to conduct preliminary screening of SARS-CoV-2 IgM/IgG antibodies in serum samples of children who presented to Beijing Children’s Hospital,Capital Medical University,having fever or requiring hospitalization,from March 2020 to August *** analysis of anti-SARS-CoV-2 antibody seropositivity was performed according to the children’s general demographic characteristics,timing of admission to hospital,presence of pneumonia,and viral nucleic acid test ***:The study included 19797 children with both IgM and IgG antibody ***-four children had anti-SARS-CoV-2 IgM-positive results(positive rate of 1.2‰),twelve children had anti-SARS-CoV-2 IgG-positive results(positive rate of 0.6‰).Viral nucleic acid test results were negative for the above-mentioned children with positive antibody findings;during the study,two children exhibited positive viral nucleic acid test results,but their anti-SARS-CoV-2 IgM/IgG antibody results were ***-SARS-CoV-2 IgM antibody seropositivity was higher in the<1-year-old group than in the≥6-year-old *** rates of anti-SARS-CoV-2 IgM seropositivity was highest in August from March to August;IgG results did not significantly differ over *** rates of anti-SARS-CoV-2 IgM or IgG seropositivity among children

关键词： Severe acute respiratory syndrome coronavirus 2(SARS-CoV-2) Coronavirus disease 2019(COVID-19) Antibody Seroprevalence Children

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Bacterial Meningitis of Infants and Children under Five Years Old in Brazzaville-Congo

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Open Journal of pediatrics 2017年第4期7卷 300-308页

作者： E. Moyen S. S. J. Dinga Boudjoumba J. Kambourou A. P. G. Oko A. R. Okoko G. Moyen Faculty of Health Sciences Marien Ngouabi University Brazzaville Congo pediatric Intensive Cares Unit Brazzaville Teaching Hospital Brazzaville Congo

Objective: to determine the epidemiological characteristics and outcomes of bacterial meningitis of infants and the children in Brazzaville after the introduction of Haemophilus influenzae type b and Pneumococcal vaccines. Patients and Methods: files of children aged from 1 to 59 months, hospitalized for bacterial meningitis between January, 2004 and December, 2013 in the Paediatric Intensive Care Unit of the Teaching Hospital of Brazzaville were reviewed. Studied variables included: age, gender, place of residence, immunization status, motive of hospitalization, laboratory analysis of the cerebrospinal fluid, and the outcome. Results: Overall 219 cases were included out of 22,148 admitted children, thus a frequency of 0.99%. Of the 219 children, 144 (65.8%) did not receive any dose of the Haemophilus influenzae b vaccine, 15 (6.8%) were incompletely vaccinated, and 60 (2.4%) were up-to-date. Concerning the Pneumococcal vaccine, 213 (97.2%) were not vaccinated, three (1.4%) were incompletely vaccinated, and the other three (1.4%) were up-to-date. Meningitis was severe in 139 cases (63.3%). The main isolated germs were: the Streptococcus pneumoniae with 46 cases (41.0%), and Haemophilus influenzae b with 16 cases (14.3%). Ninety-five children (43.4%) died in the course of the disease. The mortality rate was 7.1% before the introduction of the Haemophilus influenzae b vaccine, and 9.9% after its introduction, 8.5% after the introduction of the Pneumococcal vaccine. The lethality was 37% before and 45.1% after the introduction of the Haemophilus influenzae b vaccine and 52.8% after the introduction of the Pneumococcal vaccine. Conclusions: The high frequency and mortality rate due to meningitis necessitates the strengthening of the Expanded Program on Immunization.

关键词： Bacterial Meningitis Children Brazzaville-Congo

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Fever of unknown origin:a retrospective review of pediatric patients from an urban,tertiary care center in Washington,DC

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World Journal of pediatrics 2020年第2期16卷 177-184页

作者： Ann Marie Szymanski Hugo Clifford Tova Ronis Division of pediatric Rheumatology Children's National Health SystemWashingtonDCUSA National Institute of Arthritis and Musculoskeletal and Skin Diseases National Institutes of HealthBuilding 10Rm 12S251 MSC 156010 Center DriveBethesdaMD 20814USA The School of Medicine and Health Sciences The George Washington UniversityWashingtonDCUSA

Background Fever of unknown origin(FUO)continues to challenge clinicians to determine an etiology and the need for *** study explored the most common etiologies,characteristics,and average cost of hospitalization for FUO in a pediatric population at an urban,tertiary care hospital in Washington,*** Records from patients admitted to Children's National Health System between September 2008 and April 2014 with an admission ICD-9 code for fever(780.6)were *** charts of patients 2-18 years of age with no underlying diagnosis and a temperature greater than 38.3℃for 7 days or more at time of hospitalization were *** diagnoses,features of admission,and total hospital charges were *** 110 patients qualified for this *** majority of patients(n=42,38.2%)were discharged without a *** was followed closely by infection,accounting for 37.2%(n=41)of *** disease was next(n=16,14.5%),followed by miscellaneous(n=6,5.4%)and oncologic diagnoses(n=5,4.5%).The average cost of hospitalization was 40,295 US *** This study aligns with some of the most recent publications which report undiagnosed cases as the most common outcome in patients hospitalized with *** that,often no diagnosis is found may reassure patients,families,and *** cost associated with hospitalization for FUO may cause clinicians to reconsider inpatient admission for diagnostic work-up of fever,particularly given the evidence demonstrating that many patients are discharged without a diagnosis.

关键词： Fever Fever of unknown origin Infection Healthcare costs

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pediatric endoscopy across multiple clinical settings:Efficiency and adverse events

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World Journal of Gastrointestinal Endoscopy 2022年第6期14卷 367-375页

作者： Erin Crawford Ramy Sabe Thomas J Sferra Carolyn Apperson-Hansen Ali S Khalili Department of pediatrics University Hospitals Rainbow Babies and Children's HospitalClevelandOH 44113United States Department of pediatric Gastroenterology Hepatology and NutritionUniversity Hospitals Rainbow Babies and Children's HospitalClevelandOH 44106United States Department of Population and Quantitative Health Sciences Case Western Reserve UniversityClevelandOH 44106United States

BACKGROUND Endoscopic procedures are becoming increasingly important for the diagnosis and treatment of gastrointestinal disorders during childhood,and have evolved from a more infrequent inpatient procedure in the operating room to a routine outpatient procedure conducted in multiple care *** for these procedures is rapidly increasing and thus there is a need to perform them in an efficient ***,there are little data comparing the efficiency of pediatric endoscopic procedures in diverse clinical *** hypothesized that there are significant differences in efficiency between *** To compare the efficiency and examine adverse effects of pediatric endoscopic procedures across three clinical *** A retrospective chart review was conducted on 1623 cases of esophagogastroduodenoscopy(EGD)or combined EGD and colonoscopy performed between January 1,2014 and May 31,2018 by 6 experienced pediatric gastroenterologists in three different clinical settings,including a tertiary care hospital operating room,community hospital operating room,and free-standing pediatric ambulatory endoscopy center at a community *** following strict guidelines were used to schedule patients at all three locations:age greater than 6 mo;American Society of Anesthesiologists class 1 or 2;normal craniofacial anatomy;no anticipated therapeutic intervention(e.g.,foreign body retrieval,stricture dilation);and,no planned or anticipated hospitalization *** on demographics,times,admission rates,and adverse events were *** time(elapsed time from the endoscopist entering the operating room or endoscopy suite to the next patient entering)and patient time(elapsed time from patient registration to that patient exiting the operating room or endoscopy suite)were calculated to assess *** In total,58%of the cases were performed in the tertiary care operating *** median age of patients was 12 years and th

关键词： pediatric endoscopy Efficiency Adverse events Tertiary care operating room Community operating room Endoscopy center

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