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Videofluoroscopy swallow studies (VFSS) are gold standard to diagnose aspiration in children but require resources and radiation compared with clinical feeding evaluation (CFE). We evaluated their added value for diagnosis, feeding management and clinical status.A retrospective single-centre cross-sectional study of children aged 0-18 years, with respiratory morbidity, referred for VFSS at a tertiary pediatric hospital.A total of 113 children, median age (range) 2.2 years (0.1-17.9), underwent VFSS. Diagnosis included chronic pulmonary aspiration (CPA), 87 (77%); neurological, 73 (64%); gastrointestinal, 73 (64%) and congenital heart disease, 42 (37%), not mutually exclusive. Forty-six (41%) aspirated, 9 (8%) only overtly and 37 (33%) including silent aspirations. Those with CPA or cerebral palsy were more likely to have VFSS aspiration, OR 3.2 and 9.8 respectively. Feeding recommendations after VFSS differed significantly from those based on prior CFE, p 0.001: The rate of exclusively orally fed children rose from 65% to 79%, p = 0.006; exclusively enterally fed children from 10% to 14%; p = 0.005. During the year after VFSS, there were significantly less antibiotic courses, total and respiratory admissions.In this population with high prevalence of clinically suspected CPA, VFSS altered feeding management compared with CFE and may have contributed to subsequent clinical improvement.

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

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.

Adherence to treatment by adolescents and adults with cystic fibrosis (CF) is often poor.To assess the impact of a focused clinical intervention on adherence in individual patients, including help in problem-solving key barriers to adherence. To implement a patient-centered problem-solving intervention using CF My Way tools. To identify and overcome a selected barrier to adherence.Medication possession ratios (MPRs), number of airway clearance sessions, forced expiratory volume (FEV1), body mass index (BMI), and health-related quality of life (HRQoL) were measured before and after the intervention.Sixteen patients with CF, aged 23.4 ± 6.7 years, participated. MPR increased for colistimethate sodium and tobramycin inhalations from a median of 21 (range 0-100) to 56 (range 0-100), P = 0.04 and 20 (range 0-100) to 33.3 (range 25-100), P = 0.03, respectively. BMI standard deviation score rose from -0.37 to -0.21, P = 0.05. No significant improvements were found in FEV1, airway clearance, or HRQoL scores.The CF My Way problem-solving intervention increased adherence to medical treatments by removing barriers directly related to the needs and goals of young adults with CF.

Summary Background Tezacaftor–ivacaftor is an approved cystic fibrosis transmembrane conductance regulator (CFTR) modulator shown to be efficacious and generally safe and well tolerated over 8–24 weeks in phase 3 clinical studies in participants aged 12 years or older with cystic fibrosis homozygous for the Phe508del CFTR mutation (F/F; study 661-106 [EVOLVE]) or heterozygous for the Phe508del CFTR mutation and a residual function mutation (F/RF; study 661-108 [EXPAND]). Longer-term (>24 weeks) safety and efficacy of tezacaftor–ivacaftor has not been assessed in clinical studies. Here, we present results of study 661-110 (EXTEND), a 96-week open-label extension study that assessed long-term safety, tolerability, and efficacy of tezacaftor–ivacaftor in participants aged 12 years or older with cystic fibrosis who were homozygous or heterozygous for the Phe508del CFTR mutation. Methods Study 661-110 was a 96-week, phase 3, multicentre, open-label study at 170 clinical research sites in Australia, Europe, Israel, and North America. Participants were aged 12 years or older, had cystic fibrosis, were homozygous or heterozygous for Phe508del CFTR, and completed one of six parent studies of tezacaftor–ivacaftor: studies 661-103, 661-106, 661-107, 661-108, 661-109, and 661-111. Participants received oral tezacaftor 100 mg once daily and oral ivacaftor 150 mg once every 12 h for up to 96 weeks. The primary endpoint was safety and tolerability. Secondary endpoints were changes in lung function, nutritional parameters, and respiratory symptom scores; pulmonary exacerbations; and pharmacokinetic parameters. A post-hoc analysis assessed the rate of lung function decline in F/F participants who received up to 120 weeks of tezacaftor–ivacaftor in studies 661-106 (F/F) and/or 661-110 compared with a matched cohort of CFTR modulator-untreated historical F/F controls from the Cystic Fibrosis Foundation Patient Registry. Primary safety analyses were done in all participants from all six parent studies who received at least one dose of study drug during this study. This study was registered at ClinicalTrials.gov ( NCT02565914 ). Findings Between Aug 31, 2015, to May 31, 2019, 1044 participants were enrolled in study 661-110 from the six parent studies of whom 1042 participants received at least one dose of study drug and were included in the safety set. 995 (95%) participants had at least one TEAE; 22 (2%) had TEAEs leading to discontinuation; and 351 (34%) had serious TEAEs. No deaths occurred during the treatment-emergent period; after the treatment-emergent period, two deaths occurred, which were both deemed unrelated to study drug. F/F (106/110; n=459) and F/RF (108/110; n=226) participants beginning tezacaftor–ivacaftor in study 661-110 had improvements in efficacy endpoints consistent with parent studies; improvements in lung function and nutritional parameters and reductions in pulmonary exacerbations observed in the tezacaftor–ivacaftor groups in the parent studies were generally maintained in study 661-110 for an additional 96 weeks. Pharmacokinetic parameters were also similar to those in the parent studies. The annualised rate of lung function decline was 61·5% (95% CI 35·8 to 86·1) lower in tezacaftor–ivacaftor-treated F/F participants versus untreated matched historical controls. Interpretation Tezacaftor–ivacaftor was generally safe, well tolerated, and efficacious for up to 120 weeks, and the safety profile of tezacaftor–ivacaftor in study 661-110 was consistent with cystic fibrosis manifestations and with the safety profiles of the parent studies. The rate of lung function decline was significantly reduced in F/F participants, consistent with cystic fibrosis disease modification. Our results support the clinical benefit of long-term tezacaftor–ivacaftor treatment for people aged 12 years or older with cystic fibrosis with F/F or F/RF genotypes. Funding Vertex Pharmaceuticals Incorporated.

Duchenne muscular dystrophy (DMD) is the most common childhood muscular dystrophy. As a result of progressive muscle weakness, pulmonary function decreases during the second decade of life and lung disease contributes significantly to morbidity and mortality in these patients. Corticosteroids are the current standard of care for patients with DMD, despite known adverse effects such as obesity and immunosuppression. Over the past year (2020), the novel coronavirus (COVID-19/SARS-CoV2) outbreak has caused a global pandemic. Restrictive lung disease due to low lung volumes, chronic immunosuppressive treatment with corticosteroids, and obesity are potential risk factors that may contribute to a more severe course of the disease. Out of 116 Duchenne/Becker muscular dystrophy patients treated in our tertiary neuromuscular center, six patients with DMD and one with advanced Becker muscular dystrophy were found to be positive for COVID-19 infection. Two of the DMD patients were admitted for hospitalization, of whom one was dependent on daily nocturnal non-invasive ventilation. All patients recovered without complications despite obesity, steroid treatment and severe restrictive lung disease.

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.

Background The Q359K/T360K mutation, described in Jewish CF patients of Georgian decent, is of questionable clinical significance. Methods Clinical records of patients with the Q359K/T360K mutation from three CF centers were studied for phenotypic expression and putative mechanism of dysfunction. Computer models of mutant CFTR were constructed. Results Nine patients (4 homozygous) of Georgian Jewish origin were included. Age at diagnosis was 9.4 (0.25–38.2) years, median (range). Sweat chloride was 106 ± 13 meq/L, mean ± SD. Nasal Potential Difference performed in three, was abnormal. All had pulmonary symptoms since early childhood and bronchiectasis. Median FEV1 was 88 (40–121)%. Five had chronic mucoid P. aeruginosa. Homozygous patients were pancreatic insufficient. Enzyme supplementation was initiated at 3.8 (1–14.7) years, median (range). Structural models hint at possible interference of this mutation with transmembrane chloride transport. Conclusion In our cohort, the Q359K/T360K mutation resulted in a severe CF phenotype, although with residual early CFTR function. The CFTR2 database should consider defining this mutation as CF-causing.

Background Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare, potentially life-threatening delayed drug-induced hypersensitivity reaction. The most frequently reported drugs causing DRESS are aromatic antiepileptic agents. Prompt withdrawal of the offending drug and administering systemic corticosteroids is the most widely accepted and used treatment. The treatment of severe DRESS not responsive to systemic corticosteroids is uncertain. Objective The objective of this study was to describe a case series of pediatric patients with DRESS who were treated successfully with intravenous immunoglobulins (IVIGs). Methods A retrospective review of all children hospitalized in a tertiary care children's hospital with severe DRESS syndrome who received IVIG in addition to offending drug withdrawal and systemic corticosteroids during 1999-2017 is performed. Results Seven severe DRESS patients (4 males, age: 9.5 ± 5.7 years) are described. The offending drugs were antiepileptics in all but one case. Clinical findings included fever, rash, lymphadenopathy, dyspnea, anasarca, and hepatic involvement. After IVIG treatment (total dosage: 1-2 g/kg), fever resolved within a median time of 1 (range, 0-5) day, rash disappeared after 6.3 ± 1.6 days, and liver enzymes substantially improved after 3.8 ± 1.6 days. Patients were discharged 6.1 ± 2.7 days after IVIG commencement. There was no mortality. Conclusion The addition of IVIG in DRESS syndrome resistant to regular drug withdrawal and systemic corticosteroid therapy may hasten disease recovery.

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 FEV1 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 FEV1 was 69.1 (±6.7) % at visit one and 65.3 (±9) % at visit two. Maximal FEV1 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

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.

Aim This study evaluated the effectiveness of three different treatments for bronchiolitis in a tertiary pediatric facility. Methods Patients with bronchiolitis who were younger than two years of age and were randomly allocated to three general wards at Schneider Children's Medical Center, Israel, after admission were included. Different treatment protocols in the wards were retrospectively compared. Results The study comprised 286 children. The clinical and laboratory parameters on admission were similar between the wards. In Ward C where nebulised hypertonic saline was infrequently administered (6.7%), the mean number of days with oxygen saturation under 92% and the meanlength of hospital stay (1.8 days and 3.8 days) were significantly lower than Ward A (2.8 days and 5.3 days) and Ward B, (2.9 days and 4.7 days) where nebulised hypertonic saline was given more frequently (38.7% to 74.7%). Multivariate analysis indicated that low saturation on admission, leukocytosis and use of nebulised hypertonic saline or adrenalin were independent predictors of a longer period of desaturation and hospital stay. Conclusion Different treatment protocols for bronchiolitis were used in three paediatric wards in this real life study. No treatment regimen proved superior. Inhalations of hypertonic saline or adrenaline were associated with a longer hospital stay. This article is protected by copyright. All rights reserved.

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.What is the long-term effect of palivizumab immunization and extreme prematurity (29 weeks) on respiratory symptoms and pulmonary function in adolescence?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.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, FEVPalivizumab 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.

Primary Ciliary Dyskinesia (PCD) is rare and its features in Israel have not been described.to assess prevalence utilizing state-of-the-art diagnostic techniques, and describe clinical features, diagnostic and management practices in Israel.A national multicenter study from 2012 to 2013 recruited patients diagnosed or suspected of having PCD. Diagnosis was verified using: nasal Nitric Oxide (nNO); High-speed Video Microscope Analysis (HVMA); Transmission Electron Microscopy (TEM) of cilia; Immuno-fluorescence staining (IF) for ciliary proteins, and genetic analysis.Of the 203 patients recruited from 14 pediatric centers, 150 had a PCD diagnosis verified. Median age was 15.05y, with range 0.15-60.5y. PCD prevalence was 1:54,000 for the general population and 1:25,000 in children (5-14 y). For the non-Jewish (mainly Druze and Arab Moslem) compared to Jewish populations, prevalence was 1:16,500 and 1:139,000 respectively (p 0.0001) and parental consanguinity was 85.4% and 21.9% respectively (p 0.0001). Clinical features included bronchiectasis (88%), rhinitis (81%), recurrent pneumonia (78%), recurrent otitis (62%), neonatal pneumonia (60%) and situs inversus (42%). Prior diagnostic practices varied widely between centers with TEM assessed in 55% and abnormal in 61% of these. Management included antibiotics and airway clearance. Diagnostic verification revealed for 150 PCD patients: 81% nNO233 ppb, 62% abnormal HVMA, 51% diagnostic TEM, 58% diagnostic IF and, 57% genetic diagnosis.PCD in Israel is rare, with comprehensive diagnostic tests showing prevalence in children similar to Europe. Prevalence was higher in non-Jews, associated with parental consanguinity. Diagnostic and management practices vary. Referral centers providing comprehensive diagnostic and care capabilities should be established.

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. Lately, bronchiectasis is gaining renewed attention among the medical community, with growing basic and clinical research-based data. In Israel, no registered treatments exist for bronchiectasis, which makes it difficult to treat these patients. This paper is a summary of the position of the Israeli Pulmonology Association and the Israeli Pediatric Pulmonology Association for diagnosis and treatment of bronchiectasis.

Objectives To assess the characteristics that correlate with better outcomes after lung transplantation for patients with cystic fibrosis (CF). Methods We retrospectively reviewed the charts of all patients with CF who underwent lung transplantation between 1996-2014 at Rabin Medical Center, Israel. Results Fifty patients with CF underwent 55 lung transplantations. Eighteen patients (36%) died during the study period. Actuarial survival was 83%, 68%, 62% and 39% at 1, 3, 5 and 10 years, respectively. Better survival correlated with: BMI at 6-months and 1-year after transplantation (p=0.002 and p=0.003, respectively), ischemic time of less than 300 minutes (p=0.023), absence of liver disease (p=0.012) and Jewish compared to Arab ethnicity (p=0.007). Freedom from bronchiolitis obliterans syndrome (BOS) was 87%, 75% and 72% at 1, 3 and 5 years, respectively. BOS was more common and appeared earlier in the Arab than in the Jewish population (p=0.012, p=0.007). Additionally, prolonged time free of BOS correlated with male gender (p=0.039), older age (p

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.

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.Infants with acute bronchiolitis were tested with sulfur hexafluoride (SFTwenty-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).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.

Aim Data on the causative pathogens and optimal empirical therapy of aspiration pneumonia in children are limited. This study sought to describe the bacteriology of aspiration pneumonia in hospitalised children with a high aspiration risk. Methods Respiratory tract specimens were prospectively collected using the induced sputum technique from children with a high aspiration risk who were hospitalised for aspiration pneumonia in a tertiary paediatric medical centre from 2009 to 2014. Clinical, microbiological and treatment data were recorded and analysed for each admission. Results The cohort comprised 50 children with 235 hospital admissions. Of the 183 respiratory tract cultures performed, 110 were positive for bacteria, with 169 isolates, mostly Gram-negative. The most common Gram-negative pathogen was Pseudomonas aeruginosa. If patients had Pseudomonas aeruginosa isolation, the risk of them having the pathogen again was 81%. The multivariate analysis showed that the use of antibiotic prophylaxis and number of hospitalisations were significantly associated with Pseudomonas aeruginosa isolation. Conclusion Gram-negative bacilli, especially Pseudomonas aeruginosa, were the major causative agents of paediatric aspiration pneumonia in our study. Empiric antipseudomonas treatment should be considered, particularly in patients who are receiving antibiotic prophylaxis, have experienced recurrent hospitalisations or with previous respiratory cultures that showed Pseudomonas aeruginosa isolation.

Catastrophic antiphospholipid syndrome (APS) in pediatric medicine is rare. We report 3 adolescents who presented with acute onset of severe abdominal pain as the first manifestation of probable catastrophic APS. The 3 patients, 2 male patients and 1 female patient were 14 to 18 years old. One had been diagnosed with systemic lupus erythematosus in the past, but the other 2 had no previous relevant medical history. All presented with excruciating abdominal pain without additional symptoms. Physical examination was noncontributory. Laboratory results were remarkable for high inflammatory markers. Abdominal ultrasonography was normal, and abdominal computed tomography scan showed nonspecific findings of liver infiltration. Only computed tomography angiography revealed evidence of extensive multiorgan thrombosis. All patients had elevated titers of antiphospholipid antibodies. The patients were treated with full heparinization, high-dose steroids, and intravenous immunoglobulin with a resolution of symptoms. One patient was resistant to the treatment and was treated with rituximab. In conclusion, severe acute abdominal pain can be the first manifestation of a thromboembolic event owing to catastrophic APS even in previously healthy adolescents. Diagnosis requires a high index of suspicion with prompt evaluation and treatment to prevent severe morbidity and mortality.

Background We evaluated the effect of adding dexamethasone to antibiotic therapy in the clinical course of septic arthritis in children. Methods A randomized double-blind placebo-controlled trial was performed. The study group included 49 children with septicarthritis. In addition to antibiotic therapy given, patients were randomly assigned to receive intravenous dexamethasone 0.15 mg/kg every 6 hours for 4 days or placebo. The groups were compared for clinical and laboratory parameters, length of hospital stay, and late sequelae. Results Mean age was 33±42 months (range: 6 to 161 mo). There was no significant difference between the dexamethasone and placebo groups in age, duration of symptoms, joint affected, or levels of acute phase reactants. Bacteria were isolated from joint fluid in 17 patients (35%) and from blood in 4 patients. Compared with the placebo group, patients treated with dexamethasone had a significantly shorter duration of fever (P=0.021; mean first day without fever 1.68 vs 2.83) and local inflammatory signs (P=0.021; mean first day without pain 7.18 vs 10.76), lower levels of acute phase reactants (P=0.003; mean last day of erythrocyte sedimentation rate>25 mm/h 3.76 vs 8.40), shorter duration of parenteral antibiotic treatment (P=0.007; mean of 9.91 d vs 12.60 d), and shorter hospital stay. No side effects of treatment were recorded in either group. Conclusions A 4-day course of dexamethasone given at the start of antibiotic treatment in children with septic arthritis, is safe, and leads to a significantly more rapid clinical improvement, shortening duration of hospitalization compared with those treated with antibiotics alone. Level of evidence I.

Environmental factors, such as nutritional status, physical activity, and drug therapy, can affect bone mineralization. Our objective was to evaluate the relationship between nutritional status, physical activity, and bone mineralization as assessed by multisite quantitative ultrasound technology in children. The study group comprised 67 children, aged 6-17 years (mean, 9.4), attending a primary care clinic. Data on calcium intake and physical activity were collected using a detailed questionnaire. Speed of sound measurements were performed at the distal 1/3 radius and the midshaft tibia using Sunlight Omnisense apparatus. The reported mean calcium intake was 1105 mg/day. There was a significant difference in Z-scores at the radius and tibia between the low-and high-calcium-intake groups (P = 0.004, P = 0.035, respectively). A similar difference was found between the low-and normal-physical-activity groups (P = 0.015, P = 0.036, respectively). In this pilot study, a positive association was found between calcium intake, physical activity, and bone status, as assessed by the quantitative ultrasound technique.

Purpose:The incidence of Haemophilus influenzae type B (HIB) infection, once the most common cause of periorbital cellulitis, declined dramatically after the introduction of HIB vaccine in 1990. The aim of the current study was to determine the predisposing factors and agents in the pathogenesis of periorbital cellulitis in hospitalized children in the post-HIB vaccination era.Methods:Children with clinical findings of periorbital inflammation who were hospitalized in a tertiary pediatric hospital in Israel in 2000–2001 were observed prospectively. Special attention was directed to the predisposing medical condition in each case.Results:One hundred sixty-three patients had a final discharge diagnosis of periorbital cellulitis. Mean age was 34 months (median = 24 months). The predisposing conditions were conjunctivitis (42.9%), infected wound or trauma (20.9%), insect bites (9.8%), sinusitis (8%), dacryostenosis (4.9%), and Streptococcus pneumoniae bacteremia (0.6%). Children with conjunctivitis and sinusitis had the most severe inflammatory signs. None of the cultures was positive for HIB, although only 71% of the children had complete immunization.Conclusion:The epidemiology of periorbital cellulitis in children has changed in the post-HIB vaccine era. The most common predisposing medical conditions are conjunctivitis or an infected wound in the vicinity of the eye. Bacteremia is rarely a source of the disease. These findings have important clinical implications in terms of choice of treatment.

Background Henoch-Schonlein purpura (HSP), also known as anaphylactoid purpura is a clinically recognizable systemic disorder occurring in children, mainly from ages 3 to 10 years. Objectives To describe the clinical, epidemiological, and laboratory findings in a group of patients with recurrent HSP, admitted to a tertiary pediatric center. Methods Retrospective analysis of medical records of patients hospitalized due to HSP between 1969 and 2004. Results Two hundred sixty children (56.7% males) were hospitalized due to HSP, 7 (2.7%) more than once. There were no statistically significant differences in demographic or clinical characteristics between the patients with 1 event of HSP and patients with recurrence. Mean age of the subgroup with recurrence was 3.67 years (10 months to 7.4 years) at the first episode, and 5.03 years (2.2-10 years) at the second one, with a mean lag period of 13.5 +/- 2.8 months (range 2-26). The duration of the recurrent clinical symptoms ranged from 9 to 30 days, and in 72% of those patients, resolution took more than 14 days. Conclusion In our inpatient population, no clinical or laboratory characteristics were found to be predictive of recurrence; the second episode was longer than the first and the lag period between the 2 episodes was substantially longer than previously reported. Hospital admissions for recurrent HSP are not common. Nevertheless, a good prognosis was the rule of our admitted patients.

An elevated expression of the alloantigen D8/17 on B lymphocytes has been previously proposed as a susceptibility marker in rheumatic fever. The aim of the study was to investigate the presence of the D8/17 marker on B lymphocytes in poststreptococcal reactive arthritis (PSRA). The study sample included 19 patients (15 boys, 4 girls; mean age 11.7 +/- 5.4 years) who were diagnosed with PSRA (mean age at diagnosis, 9.4 +/- 5.6 years) and 18 healthy controls (10 boys and 8 girls) matched for ethnic background. B-cell D8/17 expression was tested by flow cytometry assay using monoclonal antibodies. Laboratory results showed a higher expression of D8/17 in the patient group (23.1 +/- 10.4%, range 11.6-51.9%) than in the control group (17.1 +/- 8.2%, range 7.1-35.7) in controls; this difference was statistically significant after log transformation of the data (P = 0.035). The high rate of expression of the D8/17 marker in patients with PSRA suggests that RF and PSRA share the same genetic susceptibility.

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.

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.

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.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.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 FEV11 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.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.

Anticonvulsant hypersensitivity syndrome is a specific severe idiosyncratic reaction to the aromatic antiepileptic drugs. The most frequent presenting symptoms are fever and rash, lymphadenopathy owing to lymphoid hyperplasia, hepatitis, and interstitial nephritis. Severe skin reactions (Stevens-Johnson syndrome or toxic epidermal necrolysis) have also been reported. Early detection is crucial owing to the high mortality rate. Although withdrawal of the offending drug is critical, the optimal treatment approach remains controversial. Previous studies report a severe course and prolonged hospital stay for cutaneous drug-related reactions, including referral to a burn center, skin débridement, and allograft skin coverage. The aim of the present report is to describe four adolescents with antiepileptic drug hypersensitivity syndrome who were treated with intravenous immunoglobulin and systemic corticosteroids. All recovered completely following an uncomplicated and relatively short course and hospitalization. Findings indicate that this regimen might be a promising treatment option in this patient population. Larger, controlled trials are needed to reach a definitive conclusion. ( J Child Neurol 2006;21:380—384; DOI 10.2310/7010.2006.00122).

Acute cerebellar ataxia is a relatively common neurologic disorder among children. Our aim was to characterize the clinical picture, etiology, and prognosis of acute cerebellar ataxia. The medical records of all children with a diagnosis of acute cerebellar ataxia hospitalized in our center and Hasharon Medical Center from 1990 to 2001 were reviewed. The diagnosis of acute cerebellar ataxia was based on the following criteria: acute onset of ataxia with or without nystagmus; absence of known genetic predisposing factors, such as familial degenerative disorders; and absence of drug intoxication, bacterial meningitis, and metabolic disorders. Thirty-nine children were identified; 54% were male; mean age at presentation was 4.8±3.8 years. All patients were observed for at least 1 year. A prodromal febrile illness was noted in 74.4%: varicella, 31%; mumps, 20%; nonspecific viral infection, 15.4%; mycoplasma, 5%; Epstein Barr virus, 3%. Latency from the prodromal illness to the onset of ataxia was 8.8±7.4 days. The most common associated neurologic findings were nystagmus and dysmetria. Full gait recovery took less than 2 weeks on average, and the longest duration of neurologic signs was 24 days (mumps-related). Acute cerebellar ataxia in childhood is a self-limited disease. The recovery was faster than that reported in previous publications and was complete in all children without any neurologic sequelae. Imaging studies are needed only in atypical presentation or if there is no spontaneous improvement after 1 to 2 weeks.

Objective. Preterm infants, especially those with chronic lung disease (CLD), are considered more susceptible to severe respiratory illness from respiratory syncytial virus (RSV) infection than healthy term infants, and are therefore targeted for prophylactic administration of immune globulins. The impact of this practice on the more severe cases of bronchiolitis (ie, pediatric intensive care unit [PICU] admission, mechanical ventilation, mortality) has not been reported to date. The aim of this study was to evaluate PICU admissions, need for mechanical ventilation, and mortality attributable to RSV bronchiolitis in Israel before the introduction of RSV prophylaxis to the country. Design and Setting. Prospective survey of 11 PICUs in Israel during the RSV season (November 2000-March 2001). Patients. All patients admitted to the PICU because of bronchiolitis, and the subgroups who needed mechanical ventilation or who died, were analyzed for known risk factors, namely, prematurity, CLD, and chronic oxygen dependence. Results. A total of 105 patients with RSV bronchiolitis met the inclusion criteria, of whom 33 were mechanically ventilated. Most of the patients (84% total admission, 88% ventilated) were born after 32 weeks’ gestation, and 89% and 88%, respectively, did not have CLD. Only 16% and 9%, respectively, met any of the American Academy of Pediatrics criteria for RSV prophylaxis, such that 84% of the whole sample and 91% of the ventilated patients were not candidates for RSV prophylaxis. Five patients died, 2 of them with cyanotic heart disease. Conclusions. Most of the infants with severe RSV bronchiolitis were born at term and did not have CLD. The great majority of patients admitted to the PICU for bronchiolitis were not candidates for RSV prophylaxis. Administration of RSV prophylaxis to the predefined high-risk population could be expected to yield no significant change in PICU admissions or number of infants needing mechanical ventilation. New risk-stratified guidelines for RSV prophylaxis are needed.

Levels of lactic dehydrogenase (LDH) in the cerebrospinal fluid (CSF) have been used to evaluate a variety of neurologic disorders. However, few studies have been performed on neonates. CSF samples were collected from 39 neonates, 19 aged 1-4 weeks and 20 aged 5-8 weeks with neonatal fever. Findings were compared to those in 15 infants mean age 10 +/- 6.4 months with suspected nervous system disease and normal CSF findings. Mean total LDH activity in the CSF was significantly higher in the first month of life than in the second month of life and compared with the control group (p

BackgroundNontuberculous mycobacteria (NTM) are emerging infections in the CF population.AimsTo assess NTM infection prevalence and associated features in our CF clinic population.MethodsPatient 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.ResultsIncidence rate of NTM infection increased from 0 in 2002 to 8.7% in 2011 (p

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.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.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 at2 years despite therapeutic intervention.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.

Many pathogens have been reported to ause bronchiolitis during the winter season including he respiratory syncytial virus (RSV), influenza, oetapneumovirus, etc. The last H1N1 A influenza pandemic ccurred during the respiratory syncytial virus (RSV) eason. The role of the pandemic influenza strain as a ause of bronchiolitis was not documented.Assessment of the prevalence of co-infections of RSV and H1N1 influenza virus among children aged 12 months and younger, hospitalized with bronchiolitis.A retrospective study design was used. Clinical data on 93 infants, 12 months old and younger, hospitalized for bronchiolitis, were retrospectively collected, including test results for RSV and H1N1 A influenza infection.Sixty-six out of the 90 (73.3 %) patients tested were positive for RSV; 2 out of the 81 patients tested (2.5%) were positive for H1N1 influenza. No patient was positive for both.The results of the present study clearly show that in infants younger than one year of age, who suffer from upper and lower respiratory tract infection (bronchiolitis), the main pathogen is RSV (also appeared in the middle of he H1N1 A influenza pandemic last winter). We conclude that the H1N1 A influenza infection is uncommon in infants hospitalized with bronchiolitis during the winter season.

We report a case of Guillain-Barré syndrome in a previously healthy child who received a hepatitis A vaccination (HAVRIX) 5 days before onset of symptoms. No other precipitating factor could be identified. The relevant literature is reviewed and discussed.

Summary Objective: Otitis externa (OE), also known as ‘‘swimmer’s ear’’, is an inflammation or infection of the external auditory canal. Many risk factors have been identified, mainly excessive moisture in the canal from swimming. Study design/ methods: To study the leading risk factors of otitis externa. Eighty-seven children aged 3.5–12 years (mean 68 ± 6.5 months) who were diagnosed with otitis externa from December 1999 to March 2001 were studied for age, sex, cerumen cleaning habits, signs and symptoms. Findings were compared to an age-matched control group of 90 children without otitis externa. Results: Sixty-one children (70.1%) in the study group had their ears cleaned with a cotton-tip applicator (Q-tip) during the 10 days preceding the diagnosis of otitis externa. In the control group, only 31(34%) used applicators routinely during the 10 days prior to diagnosis (P < 0.001). Other risk factors for otitis externa were swimming in a pool (34%), wax removal (5.8%) and ventilation tubes (1.1%). Conclusions: Use of a cotton-tip applicator to clean the ear seems to be the leading cause of otitis externa in children and should be avoided. © 2003 Elsevier Ireland Ltd. All rights reserved.

The aim of this study was to determine the long-term pulmonary outcome of extreme prematurity at a single tertiary-care center from 1997 to 2001 in the postsurfactant era.We assessed symptoms, exhaled nitric oxide, spirometry, methacholine challenge (provocative concentration of methacholine required to decrease FEV₁ by 20% [PC(20)]), lung volumes, diffusion, and cardiopulmonary exercise tolerance.Of 279 infants born, 192 survived to discharge, and 79 of these developed bronchopulmonary dysplasia (BPD) (65 mild, 12 moderate, two severe). We studied a subgroup of 53 neurologically intact preterm subjects aged 10 ± 1.5 years (28 with BPD [born, 26.2 ± 1.4 weeks; birth weight, 821 ± 164 g] and 25 without BPD [born, 27.2 ± 1 weeks; birth weight, 1,050 ± 181 g]) and compared them with 23 term control subjects. Of the BPD cases, 21 were mild, seven were moderate, and none was severe; 77.4% of subjects received antenatal steroids, and 83% received postnatal surfactant. Sixty percent of the preterm subjects wheezed at age2 years compared with 13% of the control subjects (P.001), but only 13% wheezed in the past year compared with 0% of control subjects (not significant). For preterm and control subjects, respectively (mean ± SD), FEV₁ % predicted was 85% ± 10% and 94% ± 10% (P.001), with limited reversibility; residual volume/total lung capacity was 29.3% ± 5.5% and 25% ± 8% (P.05); diffusing capacity/alveolar volume was 89.6% ± 9.2% and 97% ± 10% (P.005); and PC(20) was 6.5 ± 5.8 mg/mL and 11.7 ± 5.5 mg/mL (P.001). PC(20) was4 mg/mL in 49% of preterm subjects despite normal exhaled nitric oxide. Most measurements were similar in premature subjects with and without BPD. Peak oxygen consumption and breathing reserve were normal, but % predicted maximal load (measured in Watts) was 69% ± 15% for subjects with BPD compared with 88% ± 23% for subjects without and 86% ± 20% for control subjects (P.01).Pulmonary outcome was encouraging at mid-childhood for neurologically intact survivors in the postsurfactant era. Despite mechanical ventilation and oxygen therapy, most had no or mild BPD. Changes found probably reflect the hypoplastic lungs of prematurity.

Neonates with midline lumbar, thoracic, or occipital cutaneous lesions should be suspected of having spinal dysraphism and should undergo an imaging study. The aim of the present study was to evaluate whether sacral nevus flammeus simplex (SNFS) in neonates is associated with spinal dysraphism. During a period of 6 months, we prospectively examined all neonates in our center for the presence of SNFS. Those affected were evaluated for associated anomalies by physical and neurologic examination and ultrasound imaging of the lumbosacral area. Twenty-eight of 3623 neonates (0.77%) were diagnosed as having SNFS. In 64% of the SNFS patients, nevus flammeus simplex (NFS) was noted as well. Physical and neurologic examinations were unremarkable. Ultrasound imaging of the lumbosacral area was performed in 25 patients (89%) and revealed diastematomyelia in one. The results show that SNFS may constitute the only cutaneous sign of spinal dysraphism. In conclusion, we recommend that neonates with SNFS be investigated for occult spinal dysraphism.

The forced oscillation technique is a noninvasive and effort-independent technique that is well suited for lung function measurement in young children. FOT employs small-amplitude pressure oscillations superimposed on normal breathing. Therefore, it has the advantage over conventional lung function techniques in that it does not require patient cooperation for conducting respiratory maneuvers.To test the feasibility of the FOT test in preschool children and to compare the results to the commonly used spirometry before and after the administration of bronchodilator therapy.Forty-six children (median age 4.9 years, range 1.8-18.3) attending the pulmonary clinic at Schneider Children's Medical Center tried to perform FOT and routine spirometry. Results were retrospectively analyzed.Of the 46 children 40 succeeded in performing FOT and only 29 succeeded in performing simple spirometry. All but one of the 32 children aged 4 years and above (97%) could perform both tests. Nine of 14 children (64%) aged 4 and less could perform the FOT but only 3 (21%) could perform spirometry. Baseline values of respiratory resistance measured at 6 Hz (R6) negatively correlated with body length (r2 = 0.68, P0.005). Twenty-four children performed both tests before and after bronchodilator therapy. A significant concordance was found between the measured responses to bronchodilators by FOT and spirometry (P0.01). Only one child had a negative response by FOT but a positive response by spirometry.The FOT is a simple, non-invasive technique that does not require subject cooperation and thus can be utilized for measuring lung function in children as young as 2 years old. Furthermore, the FOT was shown to reliably measure response to bronchodilator therapy.

Objective: Although circumcision is commonly believed to protect against urinary tract infection (UTI), it is not unusual in neonates in Israel, where almost all male infants are circumcised. The aim of the study was to evaluate the burden of neonatal UTI in Israel and its relationship to circumcision. Design: Medical records of neonates (≤T2 months old) hospitalized with UTI were reviewed and demographic and clinical data were collected. The second part of the study consisting of a telephone survey to assess timing and details concerning the circumcision, included two groups: the study group consisting of parents of male infants, aged 8-30 days, hospitalized with UTI and a control group consisting of healthy neonates. Results: 162 neonates (108 males, 54 females) were hospitalized with UTI. Mean age at admission was significantly lower in males (27.5 vs 37.7 days, p=0.0002). The incidence of UTI in males peaked at 2-4 weeks of age, i.e. the period immediately following circumcision. In females, the incidence tended to rise with age. Accordingly, male predominance disappeared at 7 weeks and the male-to-female ratio reversed. In the second part of the study, 111 males (≤T1 month old) were included: 48 post-UTI and 63 as a control group. While evaluating the impact of circumcision technique, we found that UTI occurred in 6 of the 24 infants circumcised by a physician (25%), and in 42 of the 87 infants (48%) circumcised by a religious authority; the calculated odds ratio for contracting UTI was 2.8 (95% CI: 1-9.4). Conclusions: There was a higher preponderance of UTI among male neonates. Its incidence peaked during the early post-circumcision period, as opposed to the age-related rise in females. UTI seems to occur more frequently after traditional circumcision than after physician performed circumcision. We speculate that changes in the hemostasis technique or shortening the duration of the shaft wrapping might decrease the rate of infection after Jewish ritual circumcision.

OBJECTIVE. Hypoalbuminemia is a common finding in children with massive parapneumonic pleural effusion; however, its incidence and pathogenesis are unclear. The objective of this study was to assess the presence and severity of hypoalbuminemia in children with parapneumonic pleural effusion and to propose a possible pathophysiologic mechanism. METHODS. The clinical charts of patients who were hospitalized in a tertiary pediatric center with bacterial pneumonia complicated by pleural effusion were reviewed. The volume of pleural fluid was assessed semiquantitatively and categorized as small, moderate, or large. The lowest serum albumin level was recorded, and caloric intake and protein loss were evaluated. Findings were compared with age- and gender-matched children who had bacterial pneumonia without pleural effusion and with children who had acute illnesses other than pneumonia. RESULTS. Of the 50 patients in the study group, 15 (30%) had small effusions, 16 (32%) had moderate effusions, and 19 (38%) had large effusions. Moderate-to-severe hypoalbuminemia was found in 52% of the study group, 6% of the patients with pneumonia without pleural effusion, and none of the patients with other illnesses. Mean serum albumin level was lower in patients with large pleural effusions than in patients with small effusions (2.66 ± 0.37 vs 3.66 ± 0.47 g/dL). There was no evidence of albumin loss or significant malnutrition. Estimation of the amount of albumin in the drained pleural fluid suggested an albumin shift from blood to pleural fluid. CONCLUSIONS. Significant hypoalbuminemia is common in children with parapneumonic pleural effusion. Large effusions are associated with low serum albumin levels, which might be explained in part by a shift from blood to pleural fluid.

Chronic inflammation and infection in patients with cystic fibrosis (CF) and other lung diseases begin early, making noninvasive diagnostic techniques vital. As induced sputum (IS) testing is useful in older patients, we investigated its adaptation to young nonexpectorating children.Following the inhalation of a 4.5% saline solution, sputum was collected by nasopharyngeal or oropharyngeal suction for culture and testing for inflammatory markers, with paired preceding oropharyngeal cough swabs (OCSs) in a subgroup. Specimens from 48 IS procedures (46 successful) in 20 CF children (median age, 3 years) were compared with 8 specimens from 8 non-CF pulmonary patients (median age, 4.5 years).The procedure was safe, with arterial oxygen saturation remaining ator = 96%. Cultures from 14 of 46 CF patients (30%) grew Pseudomonas aeruginosa, whereas cultures from 19 of 46 CF patients (41%) had no growth. Cultures from seven of eight non-CF subjects grew bacteria, but none were P aeruginosa. Comparing 29 paired IS and OCS samples, 11 and 5 samples, respectively, cultured P aeruginosa (not significant), whereas 12 and 21 samples, respectively, had no growth (p = 0.02). A correlation was found between the independent inflammatory markers NE and both interleukin (IL)-8 (r = 0.85; p0.001) and the percentage of neutrophils (r = 0.35; p0.05), confirming the validity of IS samples in evaluating early airway disease. IL-8 levels also increased with age (r = 0.41; p0.05). Inflammation was similar in CF and non-CF subjects.IS testing in the young is feasible, safe, and clinically useful, and could serve as an outcome measure for new therapies.

Background Home nebulizers are in widespread use in cystic fibrosis (CF) and other chronic pulmonary diseases. Bacterial contamination may be a source of respiratory tract colonization. Objectives To investigate microbial contamination of home nebulizers in CF patients, compare with sputum cultures and relate to cleaning practices. Methods A total of 29 home nebulizers of CF patients were cultured. Families were interviewed regarding cleaning routines and patients had sputum cultures for bacteria and fungi. Results In total, 19/29 (65%) nebulizers were contaminated: 18 reservoir cups, 14 mouthpieces and five filters. Pseudomonas spp. were isolated from 10 nebulizers (35%) and all 10 had Pseudomonas aeruginosa airway infection although without genetic typing we could not be sure this was the same bacteria as that from their nebulizer unit. An additional 7/29 had Pseudomonas aeruginosa airway infection without a contaminated nebulizer (P = 0.001). No nebulizers were contaminated with Aspergillus. Only 4/19 contaminated nebulizers (22%) had been cleaned after every use, compared with seven of the 10 (70%) uncontaminated nebulizers (P = 0.017). Only 7/19 patients with contaminated nebulizers (37%) and 5/10 with clean nebulizers (50%) recalled receiving cleaning instructions (not significant). Conclusions Home nebulizers are frequently contaminated, particularly when cleaning instructions are inadequate, and may be a source of airway infection or reinfection especially following contamination from a patient chronically colonized with P. aeruginosa. Simple oral and written cleaning instructions should be offered.

The impact of paediatric asthma is far-reaching. Asthma quality of life (QOL) questionnaires are used in research but not readily incorporated in clinical care. We therefore computerised the Paediatric Asthma Quality of Life Questionnaire (standardised) (PAQLQ(S)) and Caregivers9 PACQLQ, with a colour-coded graphic printed report. Objectives: To 1) assess feasibility and impact of the electronic questionnaires in clinical care; 2) compare the child’s PAQLQ scores with parent9s score, physician’s clinical score and spirometry. Patients and Study Design: Asthmatic children were given a clinical severity score of 1-4 (increasing severity) using International Guidelines. Children then completed the PAQLQ(S) electronically (scores 1- 7 for increasing quality of life in emotional, symptoms and activity limitation domains) followed by spirometry and physician review. Parents completed the PACQLQ. Inclusion criteria required fluent Hebrew, and reliable performance of spirometry. Children with additional chronic diseases were excluded. Results: 147 asthmatic children aged 7-17y completed PAQLQs and 115 accompanying parents completed PACQLQs, taking 8.3 (4.3-15) and 4.4 (1.5-12.7) minutes respectively (mean (range)). Graphic reports enabled physicians to address QOL during even brief visits. Children9s (PAQLQ) and parents9 (PACQLQ) total scores correlated (r=0.61, p

Lung transplantation is a well-established therapeutic option for end-stage lung disease in cystic fibrosis. Although it confers a clear survival advantage, outcome differs among centers according to local experience, patient selection, transplantation procedure, and postoperative care.To evaluate the national Israeli experience with lung transplantation in patients with CF.We reviewed the medical charts of all CF patients who underwent lung transplantation between January 1996 and June 2005 at the two Israeli centers that perform this procedure.Eighteen transplantations were performed in 17 patients. Mean patient age at transplantation was 25.3 +/- 9.1 years, and mean duration of follow-up in survivors (n=14) was 37.2 months (range 1-113 months). The actuarial survival rate was 88% at 1 year and 74% at 5 years. Pulmonary function, expressed as percent of predicted normal forced expiratory volume in 1 sec, improved from 22.4 +/- 8.1% to 76 +/- 16.8% at one year after transplantation. Bronchiolitis obliterans syndrome was diagnosed in 5 patients (29%), of whom 2 died and 2 are currently candidates for retransplantation. Median time to onset of BOS was 34.2 months (range 17-64 months).In Israel, the early and intermediate-term results of lung transplantation for cystic fibrosis are encouraging. BOS remains a major complication that threatens long-term outcome.

D1152H is a type IV cystic fibrosis transmembrane regulator (CFTR) mutation associated with abnormal chloride gating. Although comprising 5-6% of mutations on genetic screening, clinical reports of cystic fibrosis (CF) are rare, suggesting that the disease is mild, atypical, or even absent. We describe our experience, which contrasts with this assumption, in a retrospective case series encompassing 91 CF patients (74 Jewish) aged 8 months to 56 years, from 2000-2005. Nine patients of varied Jewish ethnic origins were homozygous (2 patients) or compound heterozygous for D1152H with 11 of 182 potential alleles (6%). Five were diagnosed at age 33-49 years. Of 4 infants, 1 was diagnosed by prenatal screening, 1 had a prenatal dilated bowel, and 1 had pulmonary symptoms. Sweat chloride was 28-120 meq/l. Three adults had chronic mucoid Pseudomonas aeruginosa in sputum, and a forced expired volume in 1 sec (FEV1) of 20-55%. One was on bilevel positive airway pressure (BIPAP) ventilation. The infants had pulmonary symptoms that responded well to therapy. All 9 patients had good nutrition, 6 were pancreatic-sufficient, and 3 adults had subclinical pancreatic insufficiency. Three adults had recurrent pancreatitis. None had a bowel obstruction. Two of 3 adult males were fertile. Although asymptomatic at times, the D1152H mutation is associated with a broad clinical spectrum. This information is crucial for genetic counseling. Lung disease may be evident from infancy, and is severe in some adults, although all have outlived the median life expectancy of CF. Hopefully, with early diagnosis and therapy, prognosis can be good. A multicenter study of this mutation is warranted.