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中华医学杂志(英文版) 2024年第14期137卷 1753-1755页

作者： Tan Shanshan Cao Ling Department of pulmonology The Children’s Hospital Affiliated to the Capital Institute of Pediatrics Beijing China

To the Editor: cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. To date, over 2000 CFTR mutations have been identified. CF mostly occurs in the Caucasian population, and its incidence is approximately 1/25,000 to 1/1800. However, the number of CF cases in China is small. To discover the gene mutations and clinical characteristics of Chinese children with CF, as well as their correlation, the clinical data of 10 children with CF or CFTR-related disorders (CFTR-RD) were retrospectively analyzed in this study.

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Fecal calprotectin in pediatric gastrointestinal diseases:Pros and cons

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World Journal of Clinical pediatrics 2024年第2期13卷 198-220页

作者： Mohammed Al-Beltagi Nermin Kamal Saeed Adel Salah Bediwy Reem Elbeltagi Department of pediatric Faculty of MedicineTanta UniversityTanta 31511AlghrabiaEgypt Department of pediatrics University Medical CenterKing Abdulla Medical CityArabian Gulf UniversityManama 26671ManamaBahrain Department of pediatrics University Medical CenterDr.Sulaiman Al Habib Medical GroupBahrainManama 26671ManamaBahrain Medical Microbiology Section Department of PathologySalmaniya Medical ComplexMinistry of HealthKingdom of BahrainManama 12ManamaBahrain Medical Microbiology Section Department of PathologyIrish Royal College of SurgeonBahrainBusaiteen 15503MuharraqBahrain Department of pulmonology Faculty of MedicineTanta UniversityTanta 31527AlghrabiaEgypt Department of pulmonology University Medical CenterKing Abdulla Medical CityArabian Gulf UniversityManama 26671ManamaBahrain Department of pulmonology University Medical CenterKing Abdulla Medical CityDr.Sulaiman Al Habib Medical GroupManama 26671ManamaBahrain Department of Medicine The Royal College of Surgeons in Ireland-BahrainBusiateen 15503MuharraqBahrain

BACKGROUND Fecal calprotectin is a valuable biomarker for assessing intestinal inflammation in pediatric gastrointestinal ***,its role,pros,and cons in various conditions must be comprehensively *** To explore the role of fecal calprotectin in pediatric gastrointestinal diseases,including its advantages and *** A comprehensive search was conducted on PubMed,PubMed Central,Google Scholar,and other scientific research engines until February 24,*** review included 88 research articles,56 review articles,six metaanalyses,two systematic reviews,two consensus papers,and two letters to the *** Fecal calprotectin is a non-invasive marker for detecting intestinal inflammation and monitoring disease activity in pediatric conditions such as functional gastrointestinal disorders,inflammatory bowel disease,coeliac disease,coronavirus disease 2019-induced gastrointestinal disorders,gastroenteritis,and cystic fibrosis-associated intestinal ***,its lack of specificity and susceptibility to various confounding factors pose challenges in *** these limitations,fecal calprotectin offers significant advantages in diagnosing,monitoring,and managing pediatric gastrointestinal *** Fecal calprotectin holds promise as a valuable tool in pediatric gastroenterology,offering insights into disease activity,treatment response,and *** protocols and guidelines are needed to optimize its clinical utility and mitigate interpretation *** research is warranted to address the identified limitations and enhance our understanding of fecal calprotectin in pediatric gastrointestinal diseases.

关键词： Fecal calprotectin pediatric gastrointestinal diseases Functional gastrointestinal disorders Inflammatory bowel disease Coeliac disease COVID-19-induced gastrointestinal disorders Infectious gastroenteritis cystic fibrosis

Variability of anti-human transglutaminase testing in celiac disease across Mediterranean countries

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World Journal of Gastroenterology 2017年第24期23卷 4437-4443页

作者： Andrea Smarrazzo Giuseppe Magazzù Mongi Ben-Hariz Maria Legarda Tamara Virtut Velmishi Elefhteria Roma Aydan Kansu Dusanka Micetic-Turk Enzo Bravi Pio Stellato Carmela Arcidiaco Luigi Greco Department of Translational Medical Sciences School of MedicineSection of PediatricsUniversity of Naples“Federico II” European Laboratory for Food Induced Diseases Celiac Regional Centre Pediatric Gastroenterology and Cystic Fibrosis UnitUniversity of Messina pediatric unit Mongi SLIM’s Hospital of Tunis Paediatric Gastroenterology unit Cruces University Hospital Service of pediatric Gastroenterology"Mother Teresa"Hospital First Department of pediatrics University of Athens Department of pediatric Gastroenterology School of MedicineAnkara University University Medical Centre Paediatric Department Eurospital SpA

To verify the precision and accuracy of transglutaminase antibodies (TGA) assays across Mediterranean countries. METHODSThis study involved 8 referral centres for celiac disease (CD) in 7 Mediterranean countries. A central laboratory prepared 8 kits of 7 blinded and randomized serum samples, with a titrated amount of Human TGA IgA. Each sample was analysed three times on three different days, with each centre running a total of 21 tests. The results were included in a blindly coded report form, which was sent to the coordinator centre. The coordinator estimated the mean coefficient of Variation (CoVar = σ/μ), the mean accuracy (Accur = Vobserved - Vreal) and the mean percent variation (Var% = [(Vobserved - Vreal)/Vreal] × 100). RESULTSThe analysis showed that 79.17% of the mean variation fell between -25% and +25% of the expected value, with the accuracy and precision progressively increasing with higher titres of TGA. From values 1.25 times greater than the normal cut-off, the measurements were highly reliable. CONCLUSIONTGA estimation is a crucial step for the diagnosis of CD; given its accuracy and precision, clinicians could be confident in establishing a diagnosis.

关键词： Coeliac disease Gluten Transglutaminase Non-invasive testing Accuracy Precision

Long-term follow-up of distal intestinal obstruction syndrome in cystic fibrosis

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World Journal of Gastroenterology 2015年第1期21卷 318-325页

作者： Moran Lavie Tzipora Manovitz Daphna Vilozni Sarina Levy-Mendelovich Ifat Sarouk Ilana Weintraubv David Shoseyov Malena Cohen-Cymberknoh Joseph Rivlin Ori Efrati pediatric Pulmonary unit and the National Center for cystic fibrosis Edmond and Lily Safra Children’s HospitalSheba Medical CenterTel Hashomer 52621Israel cystic fibrosis Center and pediatric Pulmonary unit Hadassah Hebrew University Medical CenterJerusalem 91120Israel cystic fibrosis Center Carmel Medical CenterHaifa 34362Israel

AIM: To investigate the long-term follow-up of distal intestinal obstruction syndrome(DIOS) in Israeli cystic fibrosis(CF) ***: This is a multi-center,comparative,retrospective study in which we reviewed the medical records of all CF patients from three major CF centers in Israel who were treated in the period from 1980 to *** diagnosed with DIOS were defined as the study *** patients were diagnosed with DIOS based on their clinical presentation and typical findings on either abdominal X-ray or computerized tomography *** the control group,CF patients with no DIOS were matched to the patients in the study group for age,sex,and cystic fibrosis transmembrane conductance regulator(CFTR) *** both groups,the collected data included age,sex,CFTR genotype,weight,height,and body mass *** data included respiratory function tests in the last five years prior to the study,respiratory function test immediately before and after the DIOS event,number of hospitalizations,sputum culture results,and CFrelated conditions diagnosed according to the CF clinical practice *** the study group,data on the DIOS treatment and tendency for DIOS recurrence were also ***: The medical charts for a total of 350 CF patients were *** the 350 CF patients,26(7.4%) were diagnosed with *** control group included 31 CF patients with no DIOS *** mean follow-up period was 21.6 ± 8.2 *** total of DIOS episodes in the follow-up period was *** distribution of DIOS episodes was as follows: 6/26(23.1%) study patients had one episode of DIOS intheir lifetime,7/26(26.9%) had two episodes,7/26(26.9%) had three episodes,and 6/26(23.1%) had four or more *** to the control group,DIOS patients had a significantly higher incidence of meconium ileus in the past(65.4% vs 0%,respectively,P < 0.02),more Aspergillus ***(34.6% vs 3.2%,respectively,P < 0.02),and a higher number of hospitali

关键词： Distal intestinal obstruction syndrome cystic fibr

Comparative Variability of Nasal Potential Difference Measurements in Human and Mice

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Open Journal of Respiratory Diseases 2012年第2期2卷 43-56页

作者： Anissa Leonard Bob Lubamba Barbara Dhooghe Sabrina Noel Pierre Wallemacq Patrick Lebecque Teresinha Leal Louvain Centre for Toxicology and Applied Pharmacology Institut de Recherche Clinique et Expérimentale Université Catholique de Louvain Brussels Belgium pediatric pulmonology & cystic fibrosis unit St Luc University Hospital Brussels Belgium

Background: Nasal potential difference (NPD) test has long been used to assist in the diagnosis of cystic fibrosis (CF) and more recently as an outcome measure in clinical trials of new CF therapies. This test has also been adapted to the mouse nose. Objectives: We aimed at evaluating variability of the NPD measurements in CF patients displaying two severe CFTR mutations and in sex-matched healthy controls. NPD recorded from F508del-CF and normal wild-type mice were also compared. Methods and results: In each setting, tests were performed by a single qualified operator. In the clinical setting, the latest standardized operation protocol of the CF foundation was followed. A total of 80 tracings were obtained from 10 patients (23.2 y;range 14 to 32) and 10 healthy subjects (34 y;range 24 to 53), each tested twice, in both nostrils. Two CF and two controls were excluded from the statistical data analysis due to the presence of a single non interpretable NPD tracing (4/80, 5%). To achieve equal sample size, tests were obtained from 8 CF mice and normal wild-type. Comprehensive multivariate analysis of paired data showed a good reproducibility of NPD parameters in the clinical and the preclinical setting;lower variability was observed in mice. However, 95% repeatability limits of NPD parameters were large indicating a large measurement error, poor precision and low within-subject repeatability. In both settings, chloride secretion was shown to be the most reproducible and repeatable parameter. Conclusion: In human as in mice, NPD showed good reproducibility but limited within-subject repeatability.

关键词： Animal Models CFTR cystic fibrosis Nasal Potential Difference

Role of vitamin D in cystic fibrosis and non-cystic fibrosis bronchiectasis

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World Journal of Clinical pediatrics 2017年第3期6卷 132-142页

作者： Maria Moustaki Ioanna Loukou Kostas N Priftis Konstantinos Douros cystic fibrosis unit "Aghia Sophia" Children’s Hospital pediatric Allergy and Respiratory unit 3rd Department of Pediatrics "Attikon" Hospital University of Athens School of Medicine

Bronchiectasis is usually classified as cystic fibrosis(CF) related or CF unrelated(non-CF); the latter is not considered an orphan disease any more, even in developed countries. Irrespective of the underlying etiology, bronchiectasis is the result of interaction between host, pathogens, and environment. Vitamin D is known to be involved in a wide spectrum of significant immunomodulatory effects such as down-regulation of pro-inflammatory cytokines and chemokines. Respiratory epithelial cells constitutively express 1α-hydroxylase leading to the local transformation of the inactive 25(OH)-vitamin D to the active 1,25(OH)_2-vitamin D. The latter through its autocrine and paracrine functions up-regulates vitamin D dependent genes with important consequences in the local immunity of lungs. Despite the scarcity of direct evidence on the involvement of vitamin D deficiency states in the development of bronchiectasis in either CF or non-CF patients, it is reasonable to postulate that vitamin D may play some role in the pathogenesis of lung diseases and especially bronchiectasis. The potential contribution of vitamin D deficiency in the process of bronchiectasis is of particular clinical importance, taking into consideration the increasing prevalence of vitamin D deficiency worldwide and the significant morbidity of bronchiectasis. Given the well-established association of vitamin D deficiency with increased inflammation, and the indicative evidence for harmful consequences in lungs, it is intriguing to speculate that the administration of vitamin D supplementation could be a reasonable and cost effective supplementary therapeutic approach for children with non-CF bronchiectasis. Regarding CF patients, maybe in the future as more data become available, we have to re-evaluate our policy on the most appropriate dosage scheme for vitamin D.

关键词： Vitamin D Bronchiectasis cystic fibrosis Vitamin D supplementation

The airway epithelium dysfunction in the pathogenesis of asthma: The evidence

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Health 2013年第2期5卷 331-338页

作者： Salvatore Leonardi Alfina Coco Michele Miraglia Del Giudice Gianluigi L. Marseglia Mario La Rosa Clinical pediatric 2nd University of Naples Naples Italy IRCCS University Hospital San Martino University of Pavia Pavia Italy pediatric Bronchopneumology and cystic fibrosis unit Department of Medical and Paediatric Sciences University of Catania Catania Italy

Asthma is traditionally defined as a chronic, multisystem, multicellular disease characterized by bronchial hyper-responsiveness (BHR) and lung inflammation. In this illness is particularly involving the airway epithelium as the place where the inflammation begins, develops and often returns through airway remodelling. In specific way there is also an evolving awareness of the active participation of structural elements, such as the airway epithelium, airway smooth muscle, and endothelium, in this process. The airway inflammation and remodelling together likely explain the clinical manifestations of asthma that is demonstrated by complicated respiratory manifestations in which wheeze occurring secondary to bronchoconstriction in the setting of airway hyper-responsiveness and mucous hypersecretion. This is confirmed by also the histopathological analyses of bronchial biopsies from asthmatic subjects. For this reason is very important to understand the physiological role of the airway epithelium and their changes during the exacerbation of disease.

关键词： Airway Epithelium Dysfunction Asthma Evidence

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

Association of vitamin A status with lung function in children and adolescents with cystic fibrosis

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pediatric Investigation 2021年第2期5卷 125-129页

作者： Ioanna Loukou Maria Moustaki Olympia Sardeli Marina Plyta Christina N.Katsagoni Konstantinos Douros cystic fibrosis Department “Agia Sofia”Children’s HospitalAthensGreece pediatric Allergy and Respiratory unit Third Department of Pediatrics“Attikon”University HospitalNational and Kapodistrian University of AthensSchool of MedicineAthensGreece

Importance Vitamin A(or retinol)has considerable antioxidative and anti-inflammatory attributes and it may have protective effects on the respiratory health of patients with cystic fibrosis(CF).This issue,however,remains *** The purpose of the present study was to investigate the relationship between serum retinol levels(SRL)and force expiratory volume in 1 second(FEV1)in patients with *** All patients with pancreatic insufficiency attending the CF Department of“Agia Sofia”Children’s Hospital in Athens,Greece,aged 6 to 19 years during the 2012–2016 period,who could perform spirometry effectively,were included in the *** impact of SRL on FEV1 was examined longitudinally and analyzed with generalized estimating *** The study included 231 patients and 851 SRL measurements were *** 25(3.2%)cases the SRL were below the 5th percentile of reference distribution;none was above the 95th *** analysis showed that SRL was positively correlated with the FEV1(P<0.001).Interpretation In this sample of children and adolescents with CF,vitamin A deficiency was *** results suggest a positive relationship between SRL and FEV1.

关键词： cystic fibrosis Vitamin A Retinol Lung function

Inhaler Technique Questionnaire(InTeQ)in pediatric patients with asthma

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世界儿科杂志:英文版 2023年第8期19卷 798-804页

作者： Catalina Lizano‑Barrantes Olatz Garin Alexandra Lelia Dima Karina Mayoral Angels Pont Eva María Ortiz María Araceli Caballero‑Rabasco Manuel Praena‑Crespo Laura Valdesoiro‑Navarrete María Teresa Guerra Alberto Bercedo‑Sanz Gimena Hernández Camila Maroni Inés de Mir MaríaÁngeles Carrasco Marta Ortega Alberto Servan JoséAntonio Castillo Eva Tato Montse Ferrer Health Services Research Group IMIM(Hospital del Mar Medical Research Institute)Barcelona Biomedical Research ParkDoctor Aiguader8808003 BarcelonaSpain Department of Medicine and Life Sciences Universitat Pompeu FabraBarcelonaSpain Department of Pharmaceutical Care and Clinical Pharmacy Faculty of PharmacyUniversidad de Costa RicaSan JoseCosta Rica Centro de Investigación Biomédica en Red de Epidemiología y Salud Pública CIBERESP MadridSpain Research and Development unit Institut de Recerca Sant Joan de DéuBarcelonaSpain Department of pediatrics Obstetrics and Gynaecology and Preventive MedicineUniversitat Autònoma de BarcelonaBarcelonaSpain pediatric Allergy and pulmonology unit Pediatric ServiceHospital del MarBarcelonaSpain pediatric Allergy and pulmonology unit Pediatric ServiceHospital Universitari Parc TaulíSabadellSpain Centro de Salud La Candelaria Servicio Andaluz de SaludSevilleSpain Grupo de Vías Respiratorias de La Asociación Española de Pediatras de Atención Primaria(AEPAP) MadridSpain Centro de Salud Jerez Sur Servicio Andaluz de SaludSevilleSpain Centro de Salud La Sagrera BarcelonaSpain Centro de Salud Los Castros CantabriaSpain Facultat de Medicina Universitat Autònoma de BarcelonaBarcelonaSpain pediatric Allergy and pulmonology and Cystic Fibrosis UnitHospital Universitari Vall d’HebronBarcelonaSpain Consultorio Sevilla la Nueva MadridSpain Centro de Salud Dos de Mayo MadridSpain Hospital Miguel Servet ZaragozaSpain Hospital Universitario Araba Vitoria-GasteizSpain Departamento de Farmacologia Pediatría y RadiologíaUniversidad de SevillaSevilleSpain Institut d’Investigaciói InnovacióParc Taulí SabadellSpain

Asthma in school-aged children is a major public health problem worldwide[1,2].Inhaled medications are the mainstay of its pharmacological management[2],but only 8%-22%of children with asthma use their inhalers correc... 详细信息

关键词： asthma pediatric patients