Publications by authors named "Meir Mei-Zahav"

47 Publications

Low flow intermittent bronchoscopic oxygen insufflation to identify occult tracheo-esophageal fistulas.

Respir Med 2021 Jul 24;186:106544. Epub 2021 Jul 24.

Pulmonary Institute, Schneider Children's Medical Center, Petach Tikva, Israel; Sackler Faculty of Medicine, Tel Aviv University, Ramat Aviv, Israel.

Background: Esophageal atresia and tracheo-esophageal fistula (TEF), a well described congenital anomaly of the aero-digestive tract, commonly presents with inability to swallow and feed immediately after birth. However, diagnosis of recurrent or isolated TEF can be challenging and requires a combination of endoscopic and contrast studies. We describe a hitherto unreported technique of low flow intermittent oxygen insufflation into the suspicious tract and examine its safety and diagnostic yield for identification of occult TEF.

Methods: A retrospective single center cohort study, analyzing case notes of patients with TEF who underwent bronchoscopic oxygen insufflation for suspected recurrent or isolated TEF between 2006 and 2019 at a tertiary pediatric hospital.

Results: One-hundred and seven patients with TEF underwent 142 bronchoscopies during the study period. Of these, 22 patients underwent 28 bronchoscopies with oxygen insufflation. Twelve (43%) open fistulas were identified; of these, 9 (75%) were found using oxygen insufflation, revealing the fistula in 4/9 (44%) cases that had not been apparent using simple bronchoscopic visualization alone. One fistula was missed with multiple investigations, including bronchography and found only using oxygen insufflation. No complications were encountered.

Conclusions: Recurrent or isolated TEF may be missed using ordinary flexible bronchoscopy and imaging studies. Low flow oxygen insufflation can be applied safely and may detect otherwise occult TEF.
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http://dx.doi.org/10.1016/j.rmed.2021.106544DOI Listing
July 2021

Effects of Wearing Facemasks During Brisk Walks: A COVID-19 Dilemma.

J Am Board Fam Med 2021 Jul-Aug;34(4):798-801

From the Pulmonary Institute, Schneider Children's Medical Center of Israel, Petach Tikva, Israel (OB-O, YG, PS, HL, GS, ES, DP, MM-Z); Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel (PS, HL, DP, MM-Z); The Department of Nursing, Ariel University, Ariel, Israel (YG).

Background: During the Coronavirus disease 2019 (COVID-19) pandemic, wearing facemasks became obligatory worldwide.

Objectives: The objective of this study was to evaluate the effects of facemasks on gas exchange.

Methods: Healthy adults were assessed at rest and during slow and brisk 5-minute walks, with and without masks. We monitored O saturation, end-tidal carbon dioxide (EtCO), and heart and respiratory rates. Participants graded their subjective difficulty and completed individual sensations questionnaires.

Results: Twenty-one participants with a median age of 38 years (range, 29-57 years) were recruited. At rest, all vital signs remained normal, without and with masks. However, during slow and brisk walks, EtCO increased; the rise was significantly higher while wearing masks: slow walk, mean EtCO (mmHg) change +4.5 ± 2.4 versus +2.9 ± 2.3, = .004; brisk walk EtCO change +8.4 ± 3.0 versus +6.2 ± 4.0, = .009, with and without masks, respectively. Wearing masks was also associated with higher proportions of participant hypercarbia (EtCO range, 46-49 mmHg) compared with walking without masks, though this was only partially significant. Mean O-saturation remained stable (98%) while walking without masks but decreased by 1.2 % ± 2.2 while walking briskly with a mask ( = .01). Mild desaturation (O range, 93% to 96%) was noted during brisk walks among 43% of participants with masks, compared with only 14% without masks ( = .08). Borg's scale significantly increased while walking with a mask, for both slow and brisk walks ( < .001). Sensations of difficulty breathing and shortness of breath were more common while walking with masks.

Conclusion: While important to prevent viral spread, wearing facemasks during brisk 5-minute walks might be associated with mild hypercarbia and desaturation. The clinical significance of these minor gas exchange abnormalities is unclear and should be further investigated.
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http://dx.doi.org/10.3122/jabfm.2021.04.200559DOI Listing
October 2020

Incidence of SARS-CoV-2 in people with cystic fibrosis in Europe between February and June 2020.

J Cyst Fibros 2021 Apr 18. Epub 2021 Apr 18.

Centre for Cystic Fibrosis, Hospital de Santa Maria, Lisbon, Portugal.

Background: Viral infections can cause significant morbidity in cystic fibrosis (CF). The current Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic could therefore have a serious impact on the health of people with CF (pwCF).

Methods: We used the 38-country European Cystic Fibrosis Society Patient Registry (ECFSPR) to collect case data about pwCF and SARS-CoV-2 infection.

Results: Up to 30 June 2020, 16 countries reported 130 SARS-CoV-2 cases in people with CF, yielding an incidence of 2.70/1000 pwCF. Incidence was higher in lung-transplanted patients (n=23) versus non-transplanted patients (n=107) (8.43 versus 2.36 cases/1000). Incidence was higher in pwCF versus the age-matched general population in the age groups <15, 15-24, and 25-49 years (p<0.001), with similar trends for pwCF with and without lung transplant. Compared to the general population, pwCF (regardless of transplantation status) had significantly higher rates of admission to hospital for all age groups with available data, and higher rates of intensive care, although not statistically significant. Most pwCF recovered (96.2%), however 5 died, of whom 3 were lung transplant recipients. The case fatality rate for pwCF (3.85%, 95% CI: 1.26-8.75) was non-significantly lower than that of the general population (7.46%; p=0.133).

Conclusions: SARS-CoV-2 infection can result in severe illness and death for pwCF, even for younger patients and especially for lung transplant recipients. PwCF should continue to shield from infection and should be prioritized for vaccination.
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http://dx.doi.org/10.1016/j.jcf.2021.03.017DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8053246PMC
April 2021

Risk factors for respiratory syncytial virus bronchiolitis hospitalizations in children with chronic diseases.

Pediatr Pulmonol 2021 Jul 7;56(7):2204-2211. Epub 2021 May 7.

Pediatric Pulmonology Institute, Schneider Children's Medical Center of Israel, Petach Tikva, Israel.

Background: Respiratory syncytial virus (RSV) bronchiolitis is the most common lower respiratory tract disorder causing hospitalization in infants. Due to decreased hospitalization rates of premature infants following Palivizumab immune prophylaxis, the proportion of infants with chronic diseases not eligible for Palivizumab has increased.

Aim: To characterize infants hospitalized during 2014-2018 with RSV bronchiolitis, to compare between those with and without chronic conditions, and to identify risk factors for severe disease.

Methods: This retrospective study analyzed demographic and clinical data of patients younger than 2 years admitted with bronchiolitis during four consecutive RSV seasons.

Results: Of 1124 hospitalizations due to RSV bronchiolitis, 244 (22%) were in infants with chronic diseases. Although 20/1124 qualified for RSV prophylaxis, only eight received immune prophylaxis. Compared to otherwise healthy infants, children with chronic diseases had longer hospitalizations, median 4.8 days (interquartile range [IQR]: 3.4-8.3) versus 3.7 days (IQR: 2.7-5.1), p < .001; and higher pediatric intensive care unit (PICU) and readmission rates (9% vs. 4.5%, p = .007% and 3% vs. 1%, p = .055, respectively). Children with Down's syndrome comprised 2% of all hospitalizations, but 8% of PICU admissions; their median length of hospitalization was 10.7 days (IQR: 6.6-17.6). Respiratory tract malformations were present in 2% of hospitalizations, and comprised 4% of PICU admissions.

Conclusion: Among infants admitted with RSV bronchiolitis, those with chronic diseases had longer hospitalizations and higher rates of transfer to the PICU. Children with multiple comorbidities, and especially those with Down's syndrome, are at particularly high risk for severe hospitalization and may benefit from RSV immune prophylaxis.
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http://dx.doi.org/10.1002/ppul.25435DOI Listing
July 2021

Diagnostic value of sputum cultures in children under 2 years of age with chronic suppurative lung diseases.

Pediatr Pulmonol 2020 12 12;55(12):3421-3428. Epub 2020 Oct 12.

Graub CF Center Pulmonary Institute, Schneider Children's Medical Center of Israel, Petach Tikva, Israel.

Background: Acquiring sputum cultures from infants is considered challenging. We describe their yield in infants with cystic fibrosis (CF) and other chronic suppurative lung diseases (CSLDs).

Methods: Retrospective medical record review over a 4-year period, for infants aged 0-2 years with ≥2 airway bacterial cultures acquired by deep suction or induced sputum ≥4 weeks apart. Data included demographics, culture results, and clinical status.

Results: A total of 98 infants (16 CF) were evaluated and 534 sputum cultures acquired, 201 in CF and 333 in CSLD. There were 12 (2-23), median (range) cultures/CF infant, and 3 (2-21)/CSLD infant. Age at first culture was 3.8 (1-19.5) months for CF and 10.4 (0.5-22) months for CSLD; p = .016. In total, 360 cultures (67%) were positive for any bacteria, with 170/234 (73%) positive during exacerbations, compared with 190/300 (63%) during routine visits; p = .05. More infants with CF than CSLD had cultures positive for Staphylococcus aureus (SA; 75% vs. 34%; p = .004) throughout the period. Pseudomonas aeruginosa (PA) was common in both CF and CSLD (56% and 44%, respectively; p = .42) and increased over time for CF but was high throughout for CSLD. The number of hospital days before PA acquisition was 6 (10.2) for CF and 28.8 (38.7) for CSLD (p = .003). No CF but 6/82 (7%) CSLD infants had chronic PA (p = .56).

Conclusions: Sputum cultures showed that infection, in particular PA, is common in CF and CSLD whereas SA is more common in CF. Prospective studies are warranted to elucidate the role of active surveillance in guiding antibiotic therapy.
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http://dx.doi.org/10.1002/ppul.25103DOI Listing
December 2020

Topical Propranolol Improves Epistaxis Control in Hereditary Hemorrhagic Telangiectasia (HHT): A Randomized Double-Blind Placebo-Controlled Trial.

J Clin Med 2020 Sep 28;9(10). Epub 2020 Sep 28.

Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 69978, Israel.

Epistaxis is a common debilitating manifestation in hereditary hemorrhagic telangiectasia (HHT), due to mucocutaneous telangiectases. The epistaxis can be difficult to control despite available treatments. Dysregulated angiogenesis has been shown to be associated with telangiectases formation. Topical propranolol has demonstrated antiangiogenic properties. We performed a two-phase study, i.e., a double-blind placebo-controlled phase, followed by an open-label phase. The aim of the study was assessment of safety and efficacy of nasal propranolol gel in HHT-related epistaxis. Twenty participants with moderate-severe HHT-related epistaxis were randomized to eight weeks of propranolol gel 1.5%, or placebo 0.5 cc, applied to each nostril twice daily; and continued propranolol for eight weeks in an open-label study. For the propranolol group, the epistaxis severity score (ESS) improved significantly (-2.03 ± 1.7 as compared with -0.35 ± 0.68 for the placebo group, = 0.009); hemoglobin levels improved significantly (10.5 ± 2.6 to 11.4 ± 2.02 g/dL, = 0.009); and intravenous iron and blood transfusion requirement decreased. The change in nasal endoscopy findings was not significant. During the open-label period, the ESS score improved significantly in the former placebo group (-1.99 ± 1.41, = 0.005). The most common adverse event was nasal mucosa burning sensation. No cardiovascular events were reported. Our results suggest that topical propranolol gel is safe and effective in HHT-related epistaxis.
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http://dx.doi.org/10.3390/jcm9103130DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7601781PMC
September 2020

Second International Guidelines for the Diagnosis and Management of Hereditary Hemorrhagic Telangiectasia.

Ann Intern Med 2020 12 8;173(12):989-1001. Epub 2020 Sep 8.

Mayo Clinic, Rochester, Minnesota (V.N.I.).

Description: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease with an estimated prevalence of 1 in 5000 that is characterized by the presence of vascular malformations (VMs). These result in chronic bleeding, acute hemorrhage, and complications from shunting through VMs. The goal of the Second International HHT Guidelines process was to develop evidence-based consensus guidelines for the management and prevention of HHT-related symptoms and complications.

Methods: The guidelines were developed using the AGREE II (Appraisal of Guidelines for Research and Evaluation II) framework and GRADE (Grading of Recommendations Assessment, Development and Evaluation) methodology. The guidelines expert panel included expert physicians (clinical and genetic) in HHT from 15 countries, guidelines methodologists, health care workers, health care administrators, patient advocacy representatives, and persons with HHT. During the preconference process, the expert panel generated clinically relevant questions in 6 priority topic areas. A systematic literature search was done in June 2019, and articles meeting a priori criteria were included to generate evidence tables, which were used as the basis for recommendation development. The expert panel subsequently convened during a guidelines conference to conduct a structured consensus process, during which recommendations reaching at least 80% consensus were discussed and approved.

Recommendations: The expert panel generated and approved 6 new recommendations for each of the following 6 priority topic areas: epistaxis, gastrointestinal bleeding, anemia and iron deficiency, liver VMs, pediatric care, and pregnancy and delivery (36 total). The recommendations highlight new evidence in existing topics from the first International HHT Guidelines and provide guidance in 3 new areas: anemia, pediatrics, and pregnancy and delivery. These recommendations should facilitate implementation of key components of HHT care into clinical practice.
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http://dx.doi.org/10.7326/M20-1443DOI Listing
December 2020

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome - Is It Related to Ethnicity? An Israeli Multicenter Cohort Study.

J Pediatr 2020 12 14;227:268-273. Epub 2020 Aug 14.

Department of Pediatrics B, Ruth Rappaport Children's Hospital, Rambam Medical Center, Haifa, Israel; The Ruth and Bruce Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel; Pediatric Rheumatology Service, Ruth Rappaport Children's Hospital, Rambam Medical Center, Haifa, Israel.

Objectives: To evaluate the ethnic distribution of Israeli patients with the syndrome of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA).

Study Design: The medical records of patients with PFAPA attending 2 pediatric tertiary medical centers in Israel from March 2014 to March 2019 were retrospectively reviewed. Patients with concomitant familial Mediterranean fever were excluded. Ethnicity was categorized as Mediterranean, non-Mediterranean, and multiethnic. Findings were compared with patients with asthma under treatment at the same medical centers during the same period.

Results: The cohort included 303 patients with PFAPA and 475 with asthma. Among the patients with PFAPA, 178 (58.7%) were of Mediterranean descent (Sephardic Jews or Israeli Arabs), 96 (33.0%) were multiethnic, and 17 (5.8%) were of non-Mediterranean descent (all Ashkenazi Jews). Patients with PFAPA had a significantly higher likelihood of being of Mediterranean descent than the patients with asthma (58.7% vs 35.8%; P < .0001). The Mediterranean PFAPA subgroup had a significantly earlier disease onset than the non-Mediterranean subgroup (2.75 ± 1.7 vs 3.78 ± 1.9 years, P < .04) and were younger at disease diagnosis (4.77 ± 2.3 vs 6.27 ± 2.9 years, P < .04).

Conclusions: PFAPA was significantly more common in patients of Mediterranean than non-Mediterranean descent. Further studies are needed to determine the genetic background of these findings.
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http://dx.doi.org/10.1016/j.jpeds.2020.08.033DOI Listing
December 2020

An international, multicenter study of intravenous bevacizumab for bleeding in hereditary hemorrhagic telangiectasia: the InHIBIT-Bleed study.

Haematologica 2021 Aug 1;106(8):2161-2169. Epub 2021 Aug 1.

Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.

Hereditary hemorrhagic telangiectasia (HHT, Osler-Weber-Rendu disease) is a rare multisystem vascular disorder causing chronic gastrointestinal bleeding, epistaxis, and severe anemia. Bevacizumab, an anti-vascular endothelial growth factor antibody, may be effective to treat bleeding in HHT. This international, multicenter, retrospective study evaluated the use of systemic bevacizumab to treat HHT-associated bleeding and anemia at 12 HHT treatment centers. Hemoglobin, epistaxis severity score, red cell units transfused, and intravenous iron infusions before and after treatment were evaluated using paired means testing and mixed-effects linear models. 238 HHT patients received bevacizumab for a median of 12 (range, 1-96) months. Compared with pretreatment, bevacizumab increased mean hemoglobin by 3.2 g/dL (95% CI, 2.9-3.5 g/dL) [mean hemoglobin 8.6 (8.5, 8.8) g/dL versus 11.8 (11.5, 12.1) g/dL, p.
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http://dx.doi.org/10.3324/haematol.2020.261859DOI Listing
August 2021

Aerosol treatments for childhood asthma in the era of COVID-19.

Pediatr Pulmonol 2020 08 8;55(8):1871-1872. Epub 2020 Jun 8.

Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.

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http://dx.doi.org/10.1002/ppul.24849DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7301021PMC
August 2020

Palivizumab Following Extremely Premature Birth Does Not Affect Pulmonary Outcomes in Adolescence.

Chest 2020 08 13;158(2):660-669. Epub 2020 Apr 13.

Pulmonary Institute, Schneider Children's Medical Center of Israel, Petah Tikva; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv. Electronic address:

Background: Prematurity is a risk factor for impaired lung function. We sought to assess the long-term effect of palivizumab immunization and extreme prematurity (<29 weeks gestation) on respiratory symptoms and pulmonary function in adolescence.

Research Question: What is the long-term effect of palivizumab immunization and extreme prematurity (<29 weeks) on respiratory symptoms and pulmonary function in adolescence?

Study Design And Methods: We examined survivors of extreme prematurity (<29 weeks gestation) at 13 to 18 years of age (study group). Study group babies who were born immediately before palivizumab immunization (nonpalivizumab group [NPG]) were compared with those babies who were born just after implementation (PG) and with a control group. For study group patients, lung function in adolescence was further compared longitudinally with that at primary school age.

Results: Sixty-four adolescents aged 15.76 ± 1.52 years were included: 46 in the study group (17 PG and 29 NPG) and 18 in the control group. For the study group, wheezing episodes, inhaler use, and hospitalizations were uncommon. For the study group compared with the control group, FEV percent predicted was 82.60% ± 13.54% vs 105.83% ± 13.12% (P < .001), and the lung clearance index was 7.67 ± 1.02 vs 7.46 ± 0.70 (P = .48), respectively. Study group adolescents with bronchopulmonary dysplasia had a higher lung clearance index than did adolescents with no bronchopulmonary dysplasia (7.94 ± 1.11 vs 7.20 ± 0.60; P = .002). PG and NPG adolescents were not significantly different. Comparing the study group in adolescence with primary school age, we found improvement in mean FEV percent predicted bronchodilator response (0.37% ± 9.98% vs 5.67% ± 9.87%; P = .036) and mean provocative concentration causing 20% decline in FEV (12.16 ± 4.71 mg/mL vs 4.14 ± 4.51 mg/mL, respectively; P < .001).

Interpretation: Palivizumab did not provide any discernable long-term protective effect. Nevertheless, adolescent survivors of extreme prematurity showed good clinical and physiologic outcomes, except for mildly raised lung clearance index in patients with bronchopulmonary dysplasia. Airway hyperreactivity detected at primary school age, decreased by adolescence.
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http://dx.doi.org/10.1016/j.chest.2020.02.075DOI Listing
August 2020

Validation of a computerized scoring system for foreign body aspiration: An observational study.

Pediatr Pulmonol 2020 03 7;55(3):690-696. Epub 2020 Jan 7.

Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Objective: The diagnosis of foreign body aspiration (FBA) is challenging. In a previous study, we developed a computerized scoring system (CSS) to support decision-making. In the present study, we aimed to validate it on a further cohort.

Study Design: In this observational study, 100 children referred to the emergency department of a tertiary pediatric hospital for suspected FBA and treated according to standard protocol, were assigned a probability score using the CSS, between 0 and 1 (0, very low probability; 1, very high). The diagnosis of FBA was based on bronchoscopy, and if discharged without bronchoscopy, determined via telephone questionnaire, 4 to 6 months after discharge, supplemented by clinical re-evaluation and bronchoscopy, if respiratory symptoms persisted.

Results: Thirty-five out of 100 children (35%) underwent bronchoscopy with 12 of 35 (34%) positive for FBA. Sixty-five patients were discharged without bronchoscopy and completed a telephone questionnaire. Seven patients required clinical re-evaluation for persistent respiratory symptoms, in two out of them, additional bronchoscopies were performed and were negative. The CSS median probability score was 0.94 in patients with FBA, as compared to 0.73 in patients without FBA (P = .007). The CSS area under the receiver operating curve was 0.74. At a probability score threshold of 0.6, the sensitivity and specificity were 100% and 41%, respectively.

Conclusion: The present validation study suggests a high sensitivity of the CSS for the identification of FBA in children. We suggest that it might aid decision-making with regard to the need for bronchoscopy in children presenting to the emergency room.
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http://dx.doi.org/10.1002/ppul.24632DOI Listing
March 2020

Cystic fibrosis related diabetes in Europe: Prevalence, risk factors and outcome; Olesen et al.

J Cyst Fibros 2020 03 31;19(2):321-327. Epub 2019 Oct 31.

Belgian Cystic Fibrosis Registry, Sciensano, Belgium. Electronic address:

Background: Cystic fibrosis related diabetes (CFRD) has implications for morbidity and mortality with several risk factors identified. We studied the epidemiology of CFRD in the large dataset of the European Cystic Fibrosis Society Patient registry.

Methods: Data on CF patients were investigated for the prevalence of CFRD as well as for any association with suggested risk factors and effects.

Results: CFRD increased by approximately ten percentage points every decade from ten years of age. Prevalence was higher in females in the younger age groups. CFRD was associated with severe CF genotypes (OR = 3.11, 95%CI: 2.77-3.48), pancreatic insufficiency (OR = 1.46, 95%CI: 1.39-1.53) and female gender (OR = 1.28, 95%CI: 1.21-1.34). Patients with CFRD had higher odds of being chronically infected with Pseudomonas aeruginosa, Burkholderia cepacia complex and Stenotrophomonas maltophilia than patients without CFRD, higher odds of having FEV1% of predicted <40% (OR = 1.82, 95%CI: 1.70-1.94) and higher odds of having BMI SDS ≤-2 than patients without CFRD (OR = 1.24, 95%CI: 1.15-1.34).

Conclusions: Severe genotype, pancreatic insufficiency and female gender remain considerable intrinsic risk factors for early acquisition of CFRD. CFRD is associated with infections, lower lung function and poor nutritional status. Early diagnosis and aggressive treatment of CFRD are more important than ever with increasing life span.
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http://dx.doi.org/10.1016/j.jcf.2019.10.009DOI Listing
March 2020

Changing epidemiology of the respiratory bacteriology of patients with cystic fibrosis-data from the European cystic fibrosis society patient registry.

J Cyst Fibros 2020 05 3;19(3):376-383. Epub 2019 Sep 3.

Pediatric Pulmonology, Department of Pediatrics, University of Leuven, Leuven, Belgium. Electronic address:

Background: Monitoring changes in the epidemiology of cystic fibrosis (CF) pathogens is essential for clinical research, quality improvement, and clinical management.

Methods: We analyzed data reported to the European Cystic Fibrosis Society Patient Registry (ECFSPR) from 2011 to 2016 to determine the overall and the age-specific annual prevalence and incidence of selected CF pathogens and their trends during these years. The ECFSPR collects data on three chronic infections: Pseudomonas aeruginosa (PsA), Burkholderia cepacia complex Species (BCC) and Staphylococcus aureus (SA), as well as on the occurrence of non-tuberculous mycobacteria (NTM) and Stenotrophomonas maltophilia (SM). The same analyses were performed for different country groups, according to their gross national income (GNI).

Results: The pathogens with the highest prevalence were SA and PsA, with prevalence, in 2016, equal to 38.3% and 29.8% respectively, followed by SM (8.1%). The pathogens with the lowest prevalence were NTM (3.3%) and BCC (3.1%). The overall prevalence and incidence significantly decreased for PsA; they also decreased for BCC, while they increased significantly for SA. The overall prevalence of NTM and SM increased significantly. The most considerable prevalence changes were observed for PsA, which decreased across all income country groups and all age strata (with the exception of 0-1 years) The prevalence and incidence of pathogens differed significantly according to GNI.

Conclusions: The epidemiology of CF pathogens in Europe has changed; epidemiologic data differ significantly among countries with different socio-economic status. The causes of these observations are multifactorial and include improvements in clinical care and infection control.
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http://dx.doi.org/10.1016/j.jcf.2019.08.006DOI Listing
May 2020

Healthcare utilization in infants and toddlers with asthma-like symptoms.

Pediatr Pulmonol 2019 10 12;54(10):1567-1577. Epub 2019 Jul 12.

Schneider Children's Medical Center of Israel, Pulmonary Institute, Petah Tikva, Israel.

Background: Recurrent asthma-like symptoms are common in infants, but few population studies describe diagnostic and treatment practice.

Methods: Using the electronic data repository of Clalit Health Services, the largest integrated health care provider in Israel, we evaluated children born 2005-2012, who before 3 years of age had >3 episodes of asthma-like symptoms and/or >2 bronchodilator purchases within a year. We described health care utilization and the odds ratio for subsequent utilization after 3 and 12 months' controller therapy. The primary outcome measure was respiratory-related doctor visits. Linear and categorical regression analysis measured overall effectiveness of therapy.

Results: Among 689 171 infants, 262 900 (38.1%) had > 3 asthma-like episodes/year during at least 1 year. Of those, 26 108 (10%) purchased controller therapy: 20 316 (77.8%) inhaled corticosteroids (ICS) with or without leukotriene receptor antagonists (LTRA), and 5792 (22.2%) LTRA alone. For these 26 108 over 3 months there were 93 845 respiratory-related doctor visits, 3110 hospital admissions, 5568 diagnoses of pneumonia, 9960 chest X-rays, 37 127 purchases for oral steroids, and 45 142 for antibiotics courses. Healthcare utilization decreased following ICS ± LTRA and LTRA alone, respectively, as follows: doctor visits 7% and 3%, chest X-rays 16% and 17%, bronchodilators 20% and 11%, systemic steroids 17% and 12%, and antibiotics by 35% and 22%, (P < .001 for all). Twelve months' therapy remained effective.

Conclusions: Asthma-like symptoms are common in infants. Health care utilization is very high and physician practices should be reassessed. Following controller therapy, health care utilization decreased. Yet controllers were prescribed in only a minority of eligible children.
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http://dx.doi.org/10.1002/ppul.24429DOI Listing
October 2019

Inspiromatic-safety and efficacy study of a new generation dry powder inhaler in asthmatic children.

Pediatr Pulmonol 2018 10 15;53(10):1348-1355. Epub 2018 Jun 15.

Pulmonary Institute, Schneider Children's Medical Center of Israel, Petah Tikva, Israel.

Background: Dry powder inhalers (DPI) are effective but forceful inhalation required to fluidize the powder may be difficult for children and patients with airway disease. Inspiromatic is a new generation active DPI that actively suspends drugs in synchrony with inhalation. We evaluated safety and efficacy of Formoterol delivery via Inspiromatic, compared to Aerolizer, a conventional DPI, in pediatric asthmatic subjects.

Methods: A phase I/II, randomized, single-center, double-blind, double-dummy, placebo-controlled, cross-over study. Subjects aged 8-18 years with FEV 40-80% predicted were included. Patients were randomized to inhale Formoterol via the Inspiromatic, immediately followed by the placebo via the Aerolizer or vice versa, in a double-blind fashion. Spirometry, blood pressure, and heart rate were measured at baseline and 15, 30, and 60 min after drug administration. Capsule emptying, comfort of use, confidence in efficacy, and patient satisfaction were assessed. At a subsequent visit, three months later, patients inhaled the active drug via the other DPI.

Results: Twenty-nine patients, aged 12.6 (±2.3) years, mean (SD), completed the study. Baseline FEV was 69.1 (±6.7) % at visit one and 65.3 (±9) % at visit two. Maximal FEV increase was 16.6 (±7.1) % with Inspiromatic and 15.5 (±7.5) % with Aerolizer (P = 0.47). No differences in heart rate or blood pressure were observed; 24/28 capsules were emptied using the Inspiromatic and 19/28 with the Aerolizer (P = 0.5); 21/28 preferred the Inspiromatic and 7/28 the Aerolizer (P < 0.001). There were no adverse events.

Conclusions: Formoterol inhalation via the Inspiromatic is safe and as efficacious as with the Aerolizer. The device is well accepted by asthmatic subjects.
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http://dx.doi.org/10.1002/ppul.24077DOI Listing
October 2018

CFTR Genotype and Maximal Exercise Capacity in Cystic Fibrosis: A Cross-sectional Study.

Ann Am Thorac Soc 2018 02 15;15(2):209-216. Epub 2017 Nov 15.

University of Zurich, Zurich, Switzerland ;

Rationale: Cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in human skeletal muscle cells. Variations of CFTR dysfunction among patients with cystic fibrosis may be an important determinant of maximal exercise capacity in cystic fibrosis. Previous studies on the relationship between CFTR genotype and maximal exercise capacity are scarce and contradictory.

Objectives: This study was designed to explore factors influencing maximal exercise capacity, expressed as peak oxygen uptake (V.O2peak), with a specific focus on CFTR genotype in children and adults with cystic fibrosis.

Methods: In an international, multicenter, cross-sectional study, we collected data on CFTR genotype and cardiopulmonary exercise tests in patients with cystic fibrosis who were ages 8 years and older. CFTR mutations were classified into functional classes I–V.

Results: The final analysis included 726 patients (45% females; age range, 8–61 yr; forced expiratory volume in 1 s, 16 to 123% predicted) from 17 cystic fibrosis centers in North America, Europe, Australia, and Asia, all of whom had both valid maximal cardiopulmonary exercise tests and complete CFTR genotype data. Overall, patients exhibited exercise intolerance (V.O2peak, 77.3 ± 19.1% predicted), but values were comparable among different CFTR classes. We did not detect an association between CFTR genotype functional classes I–III and either V.O2peak (percent predicted) (adjusted β = −0.95; 95% CI, −4.18 to 2.29; P = 0.57) or maximum work rate (Wattmax) (adjusted β = −1.38; 95% CI, −5.04 to 2.27; P = 0.46) compared with classes IV–V. Those with at least one copy of a F508del-CFTR mutation and one copy of a class V mutation had a significantly lower V.O2peak (β = −8.24%; 95% CI, −14.53 to −2.99; P = 0.003) and lower Wattmax (adjusted β = −7.59%; 95% CI, −14.21 to −0.95; P = 0.025) than those with two copies of a class II mutation. On the basis of linear regression analysis adjusted for relevant confounders, lung function and body mass index were associated with V.O2peak.

Conclusions: CFTR functional genotype class was not associated with maximal exercise capacity in patients with cystic fibrosis overall, but those with at least one copy of a F508del-CFTR mutation and a single class V mutation had lower maximal exercise capacity.
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http://dx.doi.org/10.1513/AnnalsATS.201707-570OCDOI Listing
February 2018

Topical propranolol improves epistaxis in patients with hereditary hemorrhagic telangiectasia - a preliminary report.

J Otolaryngol Head Neck Surg 2017 Oct 4;46(1):58. Epub 2017 Oct 4.

Department of Hematology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.

Background: Severe epistaxis is often difficult to control in patients with hereditary hemorrhagic telangiectasia (HHT). Propranolol has been shown to have antiangiogenic properties in vitro and in vivo and is commonly used to treat hemangiomas. We present our experience with topical nasal propranolol for the treatment of moderate to severe epistaxis in patients with HHT.

Methods: Retrospective case series. Six patients with HHT were treated with 0.5 cm of 1.5% propranolol gel, applied to each nostril twice daily for at least 12 weeks. Outcome measures were epistaxis severity score (ESS), hemoglobin level, and number of blood transfusions prior to and while on treatment. Local and systemic side effects were recorded.

Results: The mean duration of treatment was 30 ± 5.6 weeks. A significant improvement in the ESS was found in all patients, with a mean decrease from 6.4 ± 2.1 at treatment onset to 3.5 ± 1.7 at 12 weeks (p = 0.028). Hemoglobin level increased significantly from 8.4 ± 3.1 to 11.0 ± 1.8 g/dL at 12 weeks (p = 0.043). The mean number of blood transfusions decreased from 4.5 ± 4.9 before treatment to 2.5 ± 2.9 at 12 weeks and 0.3 ± 0.8 at 24 weeks, but the difference did not reach statistical significance (p = 0.109 for both). No significant side effects of treatment were recorded.

Conclusions: These preliminary results suggest that topical propranolol may be effective for the treatment of epistaxis in patients with HHT. A prospective controlled trial is required to confirm our findings.
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http://dx.doi.org/10.1186/s40463-017-0235-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5628419PMC
October 2017

Nationwide genetic analysis for molecularly unresolved cystic fibrosis patients in a multiethnic society: implications for preconception carrier screening.

Mol Genet Genomic Med 2017 May 19;5(3):223-236. Epub 2017 Feb 19.

Medical GeneticsBarzilai Medical CenterAshkelonIsrael.

Background: Preconception carrier screening for cystic fibrosis (CF) is usually performed using ethnically targeted panels of selected mutations. This has been recently challenged by the use of expanded, ethnically indifferent, pan-population panels. Israel is characterized by genetically heterogeneous populations carrying a wide range of CFTR mutations. To assess the potential of expanding the current Israeli preconception screening program, we sought the subset of molecularly unresolved CF patients listed in the Israeli CF data registry comprising ~650 patients.

Methods: An Israeli nationwide genotyping of 152 CF cases, representing 176 patients lacking molecular diagnosis, was conducted. Molecular analysis included Sanger sequencing for all exons and splice sites, multiplex ligation probe amplification (MLPA), and next-generation sequencing of the poly-T/TG tracts.

Results: We identified 54 different mutations, of which only 16 overlapped the 22 mutations included in the Israeli preconception screening program. A total of 29/54 (53.7%) mutations were already listed as CF causing by the CFTR2 database, and only 4/54 (7.4%) were novel. Molecular diagnosis was reached in 78/152 (51.3%) cases. Prenatal diagnosis of 24/78 (30.8%) cases could have been achieved by including all CFTR2-causing mutations in the Israeli panel.

Conclusions: Our data reveal an overwhelming hidden abundance of gene mutations suggesting that expanded preconception carrier screening might achieve higher preconception detection rates.
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http://dx.doi.org/10.1002/mgg3.278DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5441412PMC
May 2017

Effect of allergic bronchopulmonary aspergillosis on FEV in children and adolescents with cystic fibrosis: a European Cystic Fibrosis Society Patient Registry analysis.

Arch Dis Child 2017 08 21;102(8):742-747. Epub 2017 Mar 21.

Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy.

Objective: To evaluate the effect of allergic bronchopulmonary aspergillosis (ABPA) on FEV percent predicted in children and adolescents with cystic fibrosis.

Design: Longitudinal data analysis (2008-2010).

Setting: Patients participating in the European Cystic Fibrosis Society Patient Registry.

Participants: 3350 patients aged 6-17 years.

Main Outcome Measure: FEV percent predicted was the main outcome measure (one measurement per year per child). To describe the effect of ABPA (main explanatory variable) on FEV while controlling for other prognostic factors, a linear mixed effects regression model was applied.

Results: In 2008, the mean (±SD) FEV percent predicted was 78.6 (±20.6) in patients with ABPA (n=346) and 88 (±19.8) in those without ABPA (n=2806). After considering other variables, FEV in subjects with ABPA on entry to the study was 1.47 percentage points lower than FEV in patients of similar age without ABPA (p=0.003). There was no FEV decline associated with ABPA over the subsequent study years as the interaction of ABPA with age was not significant (p>0.05). For patients aged 11.82 years (population mean age), poor body mass index had the greatest impact on FEV in 2008, followed by high-risk genotype (two severe mutations), female gender, diabetes mellitus, chronic infection and ABPA in descending order of effect size.

Conclusions: In contrast to the common clinical belief of ABPA having a serious impact on lung function, the difference in FEV between young patients with and without the complication was found to be modest when the effect of other prognostic factors was considered.
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http://dx.doi.org/10.1136/archdischild-2016-311132DOI Listing
August 2017

Propranolol treatment for infantile hemangioma does not increase risk of childhood wheezing.

Pediatr Pulmonol 2017 08 7;52(8):1071-1075. Epub 2017 Mar 7.

Sackler School of Medicine, Tel Aviv University, Israel.

Objective: Propranolol is the treatment of choice for infantile hemangiomas requiring medical intervention. Although contraindicated in asthma, its bronchoconstrictive effect in infants and children has not been extensively studied. We aimed to assess the incidence of wheezing episodes in infants and children treated with propranolol for infantile hemangiomas.

Study Design: A retrospective case-control study.

Setting: a tertiary pediatric hospital.

Patients: All Children followed for infantile hemangioma between 2009 and 2014. Children followed conservatively served as control group and were matched 1:1 for gender and month of birth by random matching to children treated with propranolol.

Interventions: All respiratory episodes (asthma, wheezing, stridor, and pneumonia) and respiratory associated hospitalizations were recorded from hospital records, from the primary care physician visits records and pharmacy prescriptions. The main outcome measure was the incidence of respiratory episodes in the treatment and the control groups.

Results: A total of 1828 clinic visits were reviewed for 683 children. In addition, primary care physician visits records were available in 80% of them. Two hundred and sixteen children were treated with propranolol. Incidence of respiratory episodes and recurrent respiratory episodes was similar in the propranolol and control groups (8.3% vs 12%, P = 0.265; 3.7% vs 6.5%, P = 0.274, respectively). Time to first episode was similar in the treatment and control groups (5.03 ± 3.32 vs 4.45 ± 3.21 months, respectively, P = 0.09). Respiratory hospital admission rate was similar in both groups.

Conclusions: Propranolol treatment does not exacerbate wheezing episodes in infants and children.
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http://dx.doi.org/10.1002/ppul.23683DOI Listing
August 2017

Feasibility of multiple breath washout measurements in infants with bronchiolitis: A pilot study.

Pediatr Pulmonol 2017 06 21;52(6):763-770. Epub 2017 Feb 21.

Pulmonary Institute, Schneider Children's Medical Center of Israel, Petah Tikva, Israel.

Background: Lung clearance index (LCI) reflects ventilation inhomogeneity and is raised in obstructive airway disease. Feasibility of multiple breath washout (MBW) measurement during acute lung disease in infants is unknown. As a further measure of disease, exhaled nitric oxide (eNO) may paradoxically decrease in acute bronchiolitis. We hypothesized that MBW measurements were attainable in infants with bronchiolitis and that LCI was raised and eNO reduced, compared to normal controls.

Methods: Infants with acute bronchiolitis were tested with sulfur hexafluoride (SF ) MBW during hospitalization and compared to controls. Tidal breathing and eNO parameters were obtained. Measurements were performed during natural sleep.

Results: Twenty-nine infants with bronchiolitis aged 3.7 ± 2.3 months (mean ± SD) and 23 controls aged 4.2 ± 2.5 months (P = 0.07) were evaluated. Fifteen of 29 (52%) infants with bronchiolitis and 19/23 (83%) controls achieved ≥2 valid MBW measurements. Reasons for test failure included waking up during facemask application and an irregular respiratory pattern. LCI was 8.4 ± 0.8 in the study group and 7.3 ± 0.7 in controls (P < 0.001). ENO was 2.3 ± 2.7 ppb in the study group and 7.9 ± 6.9 ppb in controls (P = 0.004).

Conclusion: MBW measurements during natural sleep are feasible but technically challenging in infants with acute bronchiolitis. LCI is raised compared to healthy controls. Larger trials, possibly using sedation protocols and shortened washout periods, are required to corroborate these findings. LCI can potentially serve as an objective indicator of severity and could be considered as a biomarker for future interventional trials.
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http://dx.doi.org/10.1002/ppul.23674DOI Listing
June 2017

Reply to Letter Regarding "Amplatzer Vascular Plugs Versus Coils for Embolization of Pulmonary Arteriovenous Malformations in Patients with Hereditary Hemorrhagic Telangiectasia".

Cardiovasc Intervent Radiol 2016 10 9;39(10):1520. Epub 2016 Aug 9.

Sackler Faculty of Medicine, Institute of Pediatric Cardiology, Tel Aviv University, Tel Aviv, Israel.

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http://dx.doi.org/10.1007/s00270-016-1448-5DOI Listing
October 2016

Amplatzer Vascular Plugs Versus Coils for Embolization of Pulmonary Arteriovenous Malformations in Patients with Hereditary Hemorrhagic Telangiectasia.

Cardiovasc Intervent Radiol 2016 Aug 5;39(8):1110-4. Epub 2016 May 5.

Institute of Pediatric Cardiology, Schneider Children's Medical Center of Israel, 14 Kaplan Street, Petach Tikva, Israel.

Purpose: Coil embolization of pulmonary arteriovenous malformations (PAVMs) has a high re-canalization/re-perfusion rate. Embolization with Amplatzer plugs has been previously described, but the long-term efficacy is not established. This study reports the experience of a referral medical center with the use of coils and Amplatzer plugs for treating PAVMs in patients with hereditary hemorrhagic telangiectasia.

Methods: The study was approved by the Institutional Review Board with waiver of informed consent. The cohort included all patients who underwent PAVM embolization in 2004-2014 for whom follow-up imaging scans were available. The medical files were retrospectively reviewed for background data, embolization method (coils, Amplatzer plugs, both), and complications. Re-canalization of treated PAVMs was assessed from intrapulmonary angiograms (following percutaneous procedures) or computed tomography angiograms. Fisher's exact test and Pearson Chi-squared test or t test were used for statistical analysis, with significance at p < 0.05.

Results: 16 patients met the study criteria. Imaging scans were available for 63 of the total 110 PAVMs treated in 41 procedures. Coils were used for embolization in 37 PAVMs, Amplatzer plugs in 21, and both in five. Median follow-up time was 7.7 years (range 1.4-18.9). Re-canalization was detected in seven vessels, all treated with coils; there were no cases of re-canalization in plug-occluded vessels (p = 0.0413).

Conclusion: The use of Amplatzer plugs for the embolization of PAVMs in patients with hemorrhagic telangiectasia is associated with a significantly lower rate of re-canalization of feeding vessels than coils. Long-term prospective studies are required to confirm these findings.
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http://dx.doi.org/10.1007/s00270-016-1357-7DOI Listing
August 2016

Clinical Impact of β-Lactamase-producing Enterobacteriaceae in Sputum of Cystic Fibrosis Patients.

Pediatr Infect Dis J 2016 Feb;35(2):216-9

From the *Graub CF Center, Pulmonary Institute, Schneider Children's Medical Center of Israel, Petah Tikva, Israel; †Sackler Faculty of Medicine, Tel Aviv University, Ramat Aviv, Tel Aviv, Israel; ‡Clalit Health Services, Sharon Shomron District, Israel; §Microbiology Laboratory, Rabin Medical Center, Petach Tikva, Israel; ¶Pediatrics A Department, and ‖Infectious Diseases Unit, Schneider Children's Medical Center of Israel, Petah Tikva, Israel.

This case series describes 18 cystic fibrosis (CF) patients of a 135-patient CF center cohort with extended spectrum β-lactamase-producing Enterobacteriaceae, from 2003 to 2012. Four had chronic infection. Prevalence increased annually from 0 to 6.35%. Risk factors compared with the 2010 CF center cohort included continuous inhaled antibiotics (P = 0.014) and courses of intravenous antibiotics during the year before first isolation (P = 0.009). Hospitalization rates were 1.05/year and 0.47/year preinfection and postinfection, respectively (P = 0.02). Slope of forced expiratory volume at 1 second% predicted remained unchanged during 12 months.
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http://dx.doi.org/10.1097/INF.0000000000000972DOI Listing
February 2016

The impact of a national population carrier screening program on cystic fibrosis birth rate and age at diagnosis: Implications for newborn screening.

J Cyst Fibros 2016 07 16;15(4):460-6. Epub 2015 Sep 16.

Kathy and Lee Graub Cystic Fibrosis Center, Schneider Children's Medical Center of Israel, Petach Tikva, Israel.

Background: Population carrier screening (PCS) has been available in Israel since 1999 and universally subsidized since 2008. We sought to evaluate its impact.

Methods: A retrospective review of governmental databanks, the national CF registry and CF centers.

Results: CF rate per 100,000 live births has decreased from 14.5 in 1990 to 6 in 2011. From 2004-2011 there were 95 CF births: 22 utilized PCS; 68 (72%) had 2 known CFTR mutations; 37% were pancreatic sufficient. At diagnosis, age was 6 (0-98) months; 53/95 had respiratory symptoms, 41/95 failure to thrive and 19/95 pseudomonas. Thirty-four (36%) were Arabs and 19 (20%) orthodox Jews, compared to 20% and 8% respectively, in the general population.

Conclusions: PCS markedly reduced CF birth rates with a shift towards milder mutations, but was often avoided for cultural reasons. As children regularly have significant disease at diagnosis, we suggest a balanced approach, utilizing both PCS and newborn screening.
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http://dx.doi.org/10.1016/j.jcf.2015.08.007DOI Listing
July 2016

Short- and Long-term Pulmonary Outcome of Palivizumab in Children Born Extremely Prematurely.

Chest 2016 Mar 5;149(3):801-8. Epub 2016 Jan 5.

Pulmonary Institute, Schneider Children's Medical Center of Israel, Petach Tikva, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Background: Palivizumab reduces the severity of respiratory syncytial virus infection in premature infants, but whether there is a protective effect beyond the preschool age is unknown. This study sought to assess the short- and long-term effects of palivizumab immunization on respiratory morbidity and pulmonary function at school age in children born extremely prematurely.

Methods: Infants born before 29 weeks' gestation in 2000 to 2003 were assessed at school age by parental questionnaire, hospital chart review, and lung function tests. Children born immediately before the introduction of routine palivizumab prophylaxis were compared with age-matched children who received palivizumab prophylaxis during the first respiratory syncytial virus season.

Results: Sixty-three children with a mean age 8.9 years were included: 30 had received palivizumab and 33 had not (control subjects). The groups were similar in terms of gestational age, birth weight, need for mechanical ventilation, and oxygen supplementation. Fifty-three percent of the palivizumab group, compared with 39% of the control group, had bronchopulmonary dysplasia (P = .14). Wheezing occurred in the first 2 years of life in 27% of the palivizumab group and in 70% of control subjects (P = .008); respective hospitalization rates were 33% and 70% (P = .001). At school age, rates of hyperresponsiveness (provocative concentration leading to a 20% fall in FEV1 < 1 mg/mL) were 33% and 48%, respectively (P = .38). Spirometry, lung volumes, diffusion, and exhaled nitric oxide were within normal limits, with no significant differences between groups.

Conclusion: Palivizumab prophylaxis was associated with reduced wheezing episodes and hospitalizations during the first 2 years of life in children born extremely prematurely. However, it did not affect pulmonary outcome at school age.
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http://dx.doi.org/10.1378/chest.15-0328DOI Listing
March 2016

The Spectrum of Nocardia Lung Disease in Cystic Fibrosis.

Pediatr Infect Dis J 2015 Aug;34(8):909-11

From the *The Kathy and Lee Graub Cystic Fibrosis Center, Pulmonary Institute, Schneider Children's Medical Center of Israel, Petah Tikva, Israel; †Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; and ‡Pediatric Pulmonology Unit, Meyer Children's Hospital, Rambam Medical Center, The Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.

We reviewed all cases of Nocardia infection in cystic fibrosis patients at 2 centers. Eight of 200 patients had Nocardia in sputum. Four developed severe lung disease, including 3 with associated allergic bronchopulmonary aspergillosis; 4 remained clinically stable. Nocardia is often associated with significant lung disease in cystic fibrosis, possibly associated with allergic bronchopulmonary aspergillosis or steroids.
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http://dx.doi.org/10.1097/INF.0000000000000759DOI Listing
August 2015

Increasing nontuberculous mycobacteria infection in cystic fibrosis.

J Cyst Fibros 2015 Jan 7;14(1):53-62. Epub 2014 Jun 7.

Graub CF Center, Pulmonary Institute, Schneider Children's Medical Center of Israel, Israel; Sackler Faculty of Medicine, Tel-Aviv University, Ramat Aviv, Tel Aviv, Israel. Electronic address:

Background: Nontuberculous mycobacteria (NTM) are emerging infections in the CF population.

Aims: To assess NTM infection prevalence and associated features in our CF clinic population.

Methods: Patient records, 2002-2011, were reviewed for NTM infection. FEV1, pancreatic function, sputum microbiology, and serum cytokines were compared in patients with and without NTM infection.

Results: Incidence rate of NTM infection increased from 0 in 2002 to 8.7% in 2011 (p<0.001). NTM infection prevalence increased 3-fold from 5% (4/79) in 2003 to 14.5% (16/110) in 2011 (p=0.05). Prevalence of chronic NTM lung disease has decreased somewhat since a peak in 2009, with institution of aggressive triple therapy. Of NTM-infected compared to uninfected patients, 88.2% vs. 60.3% had a known 'severe' CFTR genotype (p=0.04), 88.2% vs. 58.9% were pancreatic insufficient (p=0.02); 70.6% vs. 43.8% had chronic Pseudomonas aeruginosa (p=0.06); 75% vs. 32% had Aspergillus infection (p=0.007) and 23.5% vs 2.7% had allergic bronchopulmonary aspergillosis (p=0.01). Patients infected with Mycobacterium abscessus had increased TGF-β, TNF-α, IL-1β, IL-2, IL-4 and IL-5 levels (p<0.05). There was no difference in cytokine levels for all NTM infected compared to uninfected patients. M. abscessus comprised 46% of all NTM infections. Comparing M. abscessus versus other NTM, duration was 10.5 (1-118) months versus 1 (1-70) month, median (range) (p=0.004); lung disease occurred in 69% versus 17% (p=0.0004), with sputum conversion in 4/11 versus 5/6, respectively (NS).

Conclusions: NTM incidence and prevalence have increased dramatically in our CF clinic, associated with a severe CF genotype and phenotype. M. abscessus, the most prevalent NTM, caused prolonged infection despite therapy. There has been some decrease in the prevalence of NTM lung disease since 2009.
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http://dx.doi.org/10.1016/j.jcf.2014.05.008DOI Listing
January 2015

Effectiveness of long-term routine pulmonary function surveillance following pediatric hematopoietic stem cell transplantation.

Pediatr Pulmonol 2014 Nov 4;49(11):1124-32. Epub 2013 Nov 4.

Pulmonary Institute, Schneider Children's Medical Center of Israel, Petach Tikva, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Introduction: Pulmonary complications following hematopoietic stem cell transplantation (HSCT) are common and often subclinical. Thus, periodic pulmonary function testing (PFT) is mandatory. This study sought to evaluate the effectiveness of long-term PFT surveillance for children undergoing HSCT and identify potential risk factors.

Methods: We reviewed long-term PFT for HSCT patients at a tertiary pediatric center. Inclusion criteria were PFT prior to and at least once following HSCT.

Results: Fifty-seven patients performed 202 spirometry and 193 plethysmographic maneuvers; 41 were tested during the first year after HSCT, but only 29 were evaluated consistently long term (2-12 years). FVC and FEV(1) decreased gradually suggesting a restrictive ventilatory defect: FVC % predicted [mean ± SD] dropped from 91 ± 14% to 85 ± 17% after 0-24 months and 80 ± 19% beyond 2 years (P = 0.01) whereas FEV(1) dropped from 95 ± 16% to 88 ± 19% and 82 ± 20%, respectively (P = 0.002). A slight reduction in TLC was observed. Those undergoing allogeneic HSCT had a greater decline in FVC (P = 0.025) and FEV(1) (P = 0.025) as did those conditioned with radiation, regarding both FVC (P = 0.003) and FEV(1) (P = 0.002). Decline occurred earlier (≤2 years) after chemotherapy compared with radiation. Seven children had severe irreversible obstruction at >2 years despite therapeutic intervention.

Conclusions: Most survivors of childhood HSCT maintain almost normal pulmonary function although mild restrictive lung disease may develop, particularly following allogeneic HSCT and conditioning with radiation. Severe airways obstruction developed in a small minority. The surveillance protocol for PFT needs to be followed more stringently to enable intervention possibly before early subclinical changes progress and become irreversible.
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http://dx.doi.org/10.1002/ppul.22944DOI Listing
November 2014
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