Adapted first-line treatment of infection in Algerian children.

Background: () infection is acquired in early life and continues to have a high prevalence, especially in developing countries. Growing antibiotic-resistant strains necessitate adapted treatments. This study aimed to compare the efficacy, side effects, and influence of resistance of strains between two different treatments.

Long term results of the revisited Meary closing wedge tarsectomy for the treatment of the fixed cavo-varus foot in adolescent with Charcot-Marie-Tooth disease.

Background: Various techniques have been proposed for the treatment of cavovarus feet (CVF). The aim of this study was to report outcomes of the revisited Meary's dorsal closing wedge tarsectomy for fixed CVF secondary to Charcot-Marie-Tooth (CMT) disease.

Methods: All CVF operated on between 1977 and 2011 were included.

The development of new clinical instruments in laryngopharyngeal reflux disease: The international project of young otolaryngologists of the International Federation of Oto-rhino-laryngological Societies.

Introduction: To analyze the epidemiological characteristics of placebo controlled randomized trials (RCTs) that evaluated the effectiveness of medical treatments over placebo in laryngopharyngeal reflux (LPR).

Material And Methods: PubMed, Cochrane database, and Scopus were assessed for subject headings using the PRISMA recommendations. Placebo RCTs published between 1990 and 2018 describing clinical evolution throughout LPR treatment were extracted and analyzed for evidence-based level, number of patients, inclusion and exclusion criteria, gender, age, symptoms and signs used as therapeutic outcomes, and treatment schemes.

Two year outcome in treatment-naive patients with neovascular age-related macular degeneration (nAMD) using an individualized regimen of Aflibercept.

Purpose: To evaluate the 2 year visual and anatomical results of intravitreal aflibercept injection (IAI) in nAMD in treatment-naive eyes in real life using a flexible regimen combining a PRN and modified treat-and-extend (TAE) regimen.

Patients And Methods: This is a retrospective study including 48 eyes of 38 patients with nAMD treated with aflibercept as first line therapy. The modified T&E protocol consisted of a loading phase with 3 monthly IAI followed by an adaptation phase during which patients were monitored and treated as needed at the same visit from week 12 to week 32, then a T&E phase per se, for which the treatment interval was determined based on history of disease recurrence.

Helicobacter pylori Infection in Pediatric Patients: Update on Diagnosis and Eradication Strategies.

Helicobacter pylori infection is acquired mainly in childhood and remains an essential cause of peptic ulcer disease and gastric cancer. This article provides commentary on the last ESPGHAN/NASPGHAN guidelines and on publications made after the consensus conference of 2015. The majority of infected children are asymptomatic and pediatric studies do not support a role for H.

CoDE-seq, an augmented whole-exome sequencing, enables the accurate detection of CNVs and mutations in Mendelian obesity and intellectual disability.

Objective: The molecular diagnosis of extreme forms of obesity, in which accurate detection of both copy number variations (CNVs) and point mutations, is crucial for an optimal care of the patients and genetic counseling for their families. Whole-exome sequencing (WES) has benefited considerably this molecular diagnosis, but its poor ability to detect CNVs remains a major limitation. We aimed to develop a method (CoDE-seq) enabling the accurate detection of both CNVs and point mutations in one step.

Predictors of poor outcome in patients with pulmonary arterial hypertension: A single center study.

Objectives: Pulmonary arterial hypertension (PAH) is a rare disorder with unfavorable prognosis despite implementation of specific PAH-oriented therapy. The aim of the study was to define predictors of poor prognosis in patients from one center treated according to the Polish National Health Fund program.

Patients And Methods: Forty-seven consecutive patients (30 women; aged 39±17 years) with PAH diagnosis were enrolled to the study.

Metastasis-free interval in breast cancer patients: Thirty-year trends and time dependency of prognostic factors. A retrospective analysis based on a single institution experience.

Introduction: Breast cancer remains the leading cause of cancer death in French women in spite of continuously improving management. The objectives of this study were to analyse trends in the metastasis-free interval over the past 30 years and to identify the prognostic factors of survival, while accounting for time dependency.

Methods: A total of 1613 patients diagnosed with invasive non-metastatic breast cancer at Saint Vincent de Paul Hospital, Lille, France between 1977 and 2013, were followed for outcome (metastasis-free interval).

Thirty-Year Trends of Survival and Time-Varying Effects of Prognostic Factors in Patients With Metastatic Breast Cancer-A Single Institution Experience.

Background: Metastatic breast cancer is generally considered an incurable disease. In our study we aimed to detect a time trend of survival over the past 30 years and account for time-varying effects of the prognostic factors.

Patients And Methods: A total of 446 patients diagnosed with breast cancer at Saint Vincent de Paul Hospital, Lille, France between 1977 and 2013 who developed metastatic disease after a disease-free interval longer than 3 months and were followed-up for outcome.

Helicobacter pylori infection in children.

Helicobacter pylori infection in children differs from that in adults, from the point of view of epidemiology, host response, clinical features, related diseases, and diagnosis, as well as treatment strategies. The prevalence of H. pylori infection, in both children and adults, is decreasing in the Western World as well as in some developing countries, which contrasts with the increase in childhood asthma and allergic diseases.

Prognostic factors in pulmonary arterial hypertension: Literature review.

Pulmonary arterial hypertension is a disease that has a bad influence on the patient's prognosis. Recently, the possibility of therapy has dramatically changed. Nowadays, the treatment of this disease is concerned mainly with the pathophysiological target.

Rehospitalization Through Childhood and Adolescence: Association with Neonatal Morbidities in Infants of Very Low Birth Weight.

Objective: To evaluate the impact of major neonatal morbidities on the risks for rehospitalization in children and adolescents born of very low birth weight.

Study Design: An observational study was performed on data of the Israel Neonatal Network linked together with the Maccabi Healthcare Services medical records. After discharge from the neonatal intensive care unit, 6385 infants of very low birth weight born from 1995 to 2012 were registered with Maccabi Healthcare Services and formed the study cohort.

Joint ESPGHAN/NASPGHAN Guidelines for the Management of Helicobacter pylori in Children and Adolescents (Update 2016).

Background: Because of the changing epidemiology of Helicobacter pylori infection and low efficacy of currently recommended therapies, an update of the European Society for Paediatric Gastroenterology Hepatology and Nutrition/North American Society for Pediatric Gastroenterology, Hepatology and Nutrition recommendations for the diagnosis and management of H pylori infection in children and adolescents is required.

Methods: A systematic review of the literature (time period: 2009-2014) was performed. Representatives of both societies evaluated the quality of evidence using GRADE (Grading of Recommendation Assessment, Development, and Evaluation) to formulate recommendations, which were voted upon and finalized using a Delphi process and face-to-face meeting.

A Review of Gaucher Disease Pathophysiology, Clinical Presentation and Treatments.

Gaucher disease (GD, ORPHA355) is a rare, autosomal recessive genetic disorder. It is caused by a deficiency of the lysosomal enzyme, glucocerebrosidase, which leads to an accumulation of its substrate, glucosylceramide, in macrophages. In the general population, its incidence is approximately 1/40,000 to 1/60,000 births, rising to 1/800 in Ashkenazi Jews.

Primary ciliary dyskinesia in Israel: Prevalence, clinical features, current diagnosis and management practices.

Background: Primary Ciliary Dyskinesia (PCD) is rare and its features in Israel have not been described.

Aims: to assess prevalence utilizing state-of-the-art diagnostic techniques, and describe clinical features, diagnostic and management practices in Israel.

Methods: A national multicenter study from 2012 to 2013 recruited patients diagnosed or suspected of having PCD.

Ulcerative proctitis is a frequent location of paediatric-onset UC and not a minor disease: a population-based study.

Objective: Natural history of paediatric-onset ulcerative proctitis (UP) is poorly described. Our aim was to describe the phenotype and disease course of incident UP in a population-based study of paediatric-onset UC.

Patients And Methods: All patients with UC diagnosed <17 years from 1988 to 2004, and followed during >2 years have been extracted from a population-based registry.

Rare Genetic Forms of Obesity: Clinical Approach and Current Treatments in 2016.

Obesity results from a synergistic relationship between genes and the environment. The phenotypic expression of genetic factors involved in obesity is variable, allowing to distinguish several clinical pictures of obesity. Monogenic obesity is described as rare and severe early-onset obesity with abnormal feeding behavior and endocrine disorders.

Efficacy and Safety in Retinal Vein Occlusion Treated with at Least Three Consecutive Intravitreal Dexamethasone Implants.

Purpose. To evaluate the effects of repeated intravitreal dexamethasone implant (DI) (Ozurdex®) in eyes with macular edema (ME) due to retinal vein occlusion (RVO). Methods.

Systematic Analysis of CCNO Variants in a Defined Population: Implications for Clinical Phenotype and Differential Diagnosis.

Reduced generation of multiple motile cilia (RGMC) is a novel chronic destructive airway disease within the group of mucociliary clearance disorders with only few cases reported. Mutations in two genes, CCNO and MCIDAS, have been identified as a cause of this disease, both leading to a greatly reduced number of cilia and causing impaired mucociliary clearance. This study was designed to identify the prevalence of CCNO mutations in Israel and further delineate the clinical characteristics of RGMC.

Sequential Therapy for Helicobacter pylori Infection in Treatment-naïve Children.

Unlabelled: The goal of first-line Helicobacter pylori therapy is to reach an eradication rate of 90% to avoid further investigations, antibiotic use, and spreading of resistant strains.

Aim: To evaluate the eradication rate of high-dose sequential therapy in treatment-naïve children and to assess factors associated with failure.

Methods: Prospective data assessed in a registry from nine European centers between October 2009 and December 2011.

Impact of imiglucerase supply constraint on the therapeutic management and course of disease in French patients with Gaucher disease type 1.

Background: In 2009, a worldwide supply constraint of imiglucerase led to treatment modifications or interruptions for patients with Gaucher disease (GD) type 1. In France, joint treatment recommendations were issued to protect the most vulnerable patients. This observational study evaluated the impact of imiglucerase treatment modifications on the clinical and biological course of GD.

Safety of a New Amino Acid Formula in Infants Allergic to Cow's Milk and Intolerant to Hydrolysates.

Objectives: Amino acid-based formulas (AAFs) are recommended for children with cow's-milk allergy (CMA) failing to respond to extensively hydrolysed formulas (eHFs). We evaluated the effects of a new thickened AAF (TAAF, Novalac), containing a pectin-based thickener, and a reference AAF (RAAF, Neocate) on allergy symptoms and safety, through blood biochemistry analysis and growth.

Methods: Infants (ages < 18 months) with CMA symptoms failing to respond to eHFs were randomised in a double-blind manner to receive TAAF or RAAF for 3 months.

Immunofluorescence Analysis and Diagnosis of Primary Ciliary Dyskinesia with Radial Spoke Defects.

Primary ciliary dyskinesia (PCD) is a genetically heterogeneous recessive disorder caused by several distinct defects in genes responsible for ciliary beating, leading to defective mucociliary clearance often associated with randomization of left/right body asymmetry. Individuals with PCD caused by defective radial spoke (RS) heads are difficult to diagnose owing to lack of gross ultrastructural defects and absence of situs inversus. Thus far, most mutations identified in human radial spoke genes (RSPH) are loss-of-function mutations, and missense variants have been rarely described.