Long-Term Pulmonary Damage in Surviving Antitoxin-Treated Mice following a Lethal Ricin Intoxication.

Ricin, a highly potent plant-derived toxin, is considered a potential bioterrorism weapon due to its pronounced toxicity, high availability, and ease of preparation. Acute damage following pulmonary ricinosis is characterized by local cytokine storm, massive neutrophil infiltration, and edema formation, resulting in respiratory insufficiency and death. A designated equine polyclonal antibody-based (antitoxin) treatment was developed in our laboratory and proved efficacious in alleviating lung injury and increasing survival rates.

Pleural line slope in point of care ultrasound assessment of paediatric wheeze may reflect respiratory effort.

Aim: Asthma scoring systems rely on physical examination findings. Point of care ultrasound may provide an objective means to document improvement in the work of breathing in paediatric lower airway obstruction.

Methods: Thirty children with wheeze on physical examination (cases) and 15 children presenting with abdominal pain (controls) were studied.

The clinical yield of bronchoscopy in the management of cystic fibrosis: A retrospective multicenter study.

Background: Pulmonary disease is the leading cause of morbidity and mortality in people with cystic fibrosis (pwCF). Several studies have shown no benefit for bronchoscopy and bronchoalveolar lavage (BAL) over sputum to obtain microbiological cultures, hence the role of bronchoscopy in pwCF is unclear.

Aim: To analyze how bronchoscopy results affected clinical decision-making in pwCF and assess safety.

Respiratory physiotherapy in patients with cystic fibrosis and upper limb deep vein thrombosis.

We report physiotherapy management of two patients with severe cystic fibrosis (CF) lung disease and upper limb deep vein thrombosis (DVT). These patients were admitted due to a pulmonary exacerbation. Following peripherally inserted central catheters, they were diagnosed with an upper limb DVT.

Manual external chest compression reverses respiratory failure in children with severe air trapping.

We report manual external chest compression (MECC) as an effective treatment for acute respiratory failure due to severe air trapping. In this retrospective study, we describe our experience with MECC administered to five children suffering from severe air trapping as a consequence of severe asthma or bronchiolitis. These children were admitted to the Pediatric Intensive Care Unit (PICU) with clinical and blood gases parameters compatible with acute respiratory failure.

Ethical Dilemma: Elexacaftor-Tezacaftor-Ivacaftor or Lung Transplantation in Cystic Fibrosis and End-Stage Lung Disease?

Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR). Novel, highly effective, modulator therapies correcting and potentiating CFTR function are changing the course of this disease. We present an ethical dilemma involving an 11-year-old child with CF and end-stage lung disease.

The Use of Infant Pulmonary Function Tests in the Diagnosis of Neuroendocrine Cell Hyperplasia of Infancy.

Background: Infant pulmonary function tests (iPFTs) in subjects with neuroendocrine cell hyperplasia of infancy (NEHI) have demonstrated substantial expiratory airflow obstruction and air trapping.

Research Question: Can indices from iPFTs be used in the diagnosis of NEHI?

Study Design And Methods: This is an observational case-control study evaluating iPFT results from a registry of patients assessed at the Hadassah Hebrew University Medical Center between 2008 and 2018. iPFTs results in infants with NEHI were compared to two disease control infant groups (infants evaluated for recurrent wheezing and infants evaluated due to prematurity) and a spirometry control group of infants with normal expiratory airflow, using the Kruskal-Wallis test.

How abnormal is the normal? Clinical characteristics of CF patients with normal FEV.

Background: Normal values (>80%) of Forced Expiratory Volume in one second (FEV ) in patients with cystic fibrosis (CF) may lead to the interpretation that there is no lung disease. This study is a comprehensive analysis of lung involvement in CF patients having normal FEV .

Methods: Patients were recruited from two CF centers: Hadassah Medical Center, Jerusalem and Vall d' Hebron Hospital, Barcelona.

Betamethasone versus dexamethasone for inpatient preschool wheezing-A case-control study.

Background And Objectives: Wheezing is one of the most common reasons for the presentation of children to primary care or the emergency ward, before 7 years of age. Current guidelines recommend a short course of oral corticosteroids (OCS) for those children with a wheezing attack severe enough to require hospitalization. However, the optimal choice of therapy is controversial.

Whole-exome sequencing accuracy in the diagnosis of primary ciliary dyskinesia.

The diagnosis of primary ciliary dyskinesia (PCD) relies on clinical features and sophisticated studies. The detection of bi-allelic disease-causing variants confirms the diagnosis. However, a standardised genetic panel is not widely available and new disease-causing genes are continuously identified.

Ivacaftor in People with Cystic Fibrosis and a → or Residual Function Mutation.

Ivacaftor's clinical effects in the residual function mutations and warrant further characterization. To evaluate ivacaftor's effect in people with cystic fibrosis aged ≥6 years with or residual function mutations and to explore the correlation between ivacaftor-induced organoid-based cystic fibrosis transmembrane conductance regulator function measurements and clinical response to ivacaftor. Participants were randomized (1:1) in this placebo-controlled crossover study; each treatment sequence included two 8-week treatments with an 8-week washout period.

Giant lung cysts following necrotizing pneumonia: Resolution with conservative treatment.

Rationale: Necrotizing pneumonia is characterized by destruction and liquefaction of the lung tissue and loss of the normal pulmonary parenchymal architecture. During the course of resolution areas of hyperlucency are formed, sometimes with the development of giant lung cysts that can be a field with fluid resembling lung abscess. There is no consensus on the management of these abnormalities.

Increasing Vitamin D Serum Levels Is Associated With Reduced Pulmonary Exacerbations in Patients With Cystic Fibrosis.

Context: In 2012, The North American Cystic Fibrosis Foundation (NACFF) published new guidelines for the treatment of vitamin D deficiency in individuals with cystic fibrosis (CF).

Objective: The objectives of our study were to assess the efficacy of these guidelines, and to test the effect of increasing vitamin D dosage on pulmonary function and exacerbations.

Design: Pulmonary function tests and serum concentrations of 25-hydroxyvitamin D [25(OH)D] were measured 1 year before increasing vitamin D dosage according to the guidelines and at least 1 year later.

[DIAGNOSIS AND TREATMENT OF BRONCHIECTASIS: POSITION PAPER OF THE ISRAELI PULMONOLOGY SOCIETY AND THE ISRAELI PEDIATRIC PULMONOLOGY SOCIETY].

Bronchiectasis is anatomically defined by irreversible distortion of the bronchi. Clinically, its manifestations are cough with sputum production and a predisposition to pulmonary infections. Unlike asthma and COPD, where ample clinical data are present regarding the course and effective treatment, knowledge of bronchiectasis has yet to evolve.

Attention deficit hyperactivity disorder symptoms in patients with cystic fibrosis.

Background: Cystic fibrosis (CF) is a chronic life-threatening disease. In patients who suffer from chronic disease, Attention Deficit Hyperactivity Disorder (ADHD) is associated with functional impairment that can affect adherence to treatment and consequently influence prognosis.

Methods: CF patients filled in the ADHD Rating Scale (ADHD-RS) adapted to the DSM5 and were assessed on a continuous performance task (MOXO-CPT), a standardized-computerized test designed to evaluate several domains of attention.

Clinical impact of Pseudomonas aeruginosa colonization in patients with Primary Ciliary Dyskinesia.

Background: Airway infections in Primary Ciliary Dyskinesia (PCD) are caused by different microorganisms, including pseudomonas aeruginosa (PA). The aim of this study was to investigate the association of PA colonization and the progression of lung disease in PCD.

Methods: Data from 11PCD centers were retrospectively collected from 2008 to 2013.

Predictors of Prolonged Hospitalizations in Pediatric Complicated Pneumonia.

Background: Pediatric community-acquired complicated pneumonia (PCACP) is characterized by a prolonged clinical course, but this may be highly variable.

Methods: A multicenter observational study was conducted to develop and validate a clinical prediction tool for prolonged hospitalizations in PCACP. The derivation and validation cohorts consisted of 144 and 169 patients with PCACP, respectively, hospitalized between the years 1997 and 2017 in three tertiary care hospitals.

Comparison of activity and fatigue of the respiratory muscles and pulmonary characteristics between post-polio patients and controls: A pilot study.

Objectives: To compare pulmonary function measures, maximal respiratory pressure and fatigue of respiratory muscles between patients with Post-Polio Syndrome (PPS) and controls.

Design: Cross-sectional study.

Patients: Patients with PPS (N = 12; age 62.

Autosomal dominant gain of function STAT1 mutation and severe bronchiectasis.

Background: In a substantial number of patients with non-cystic fibrosis (CF) bronchiectasis an etiology cannot be found. Various complex immunodeficiency syndromes account for a significant portion of these patients but the mechanism elucidating the predisposition for suppurative lung disease often remains unknown.

Objective: To investigate the cause and mechanism predisposing a patient to severe bronchiectasis.

Collecting clinical data in primary ciliary dyskinesia- challenges and opportunities.

Primary ciliary dyskinesia (PCD) is under diagnosed and underestimated. Most clinical research has used some form of questionnaires to capture data but none has been critically evaluated particularly with respect to its end-user feasibility and utility. To critically appraise a clinical data collection questionnaire for PCD used in a large national PCD consortium in order to apply conclusions in future PCD research.

Comparison of Nasal Potential Difference and Intestinal Current Measurements as Surrogate Markers for CFTR Function.

Objectives: Nasal potential difference (NPD) measurement is part of the diagnostic criteria for cystic fibrosis (CF) and now used routinely as an endpoint in clinical trials of correcting the basic defect in CF. Intestinal current measurement (ICM), measured ex vivo on a rectal biopsy, has been used to study cystic fibrosis transmembrane conductance regulator (CFTR) function but has not been compared to NPD in the same subject in adults and children. The aim of the study is to evaluate the potential usefulness of ICM as a marker of CFTR function for treatment studies compared NPD in patients with CF and in healthy control subjects.

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.

Complicated community acquired pneumonia in childhood: Different types, clinical course, and outcome.

Unlabelled: The incidence of pediatric community acquired complicated pneumonia (PCACP) is increasing. Questions addressed: Are different types of PCACP one disease? How do different treatment protocols affect the outcome?

Methods: Retrospective analysis of medical records of PCACP hospitalizations in the three major hospitals in Jerusalem in the years 2001-2010 for demographics, clinical presentation, management, and outcome.

Results: Of the 144 children (51% aged 1-4 years), 91% of Jewish origin; 40% had para-pneumonic effusion (PPE), 40% empyema (EMP), and 20% necrotizing pneumonia (NP).