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High-flow nasal cannula (HFNC) therapy may be better tolerated than traditional noninvasive ventilation (NIV) and is rapidly gaining acceptance in pediatric acute care. In Israel, HFNC is approved for domestic use. We aim to describe its indications, efficacy, parental satisfaction, and safety.Retrospective study of children treated with home HFNC therapy in three pediatric centers. Data included demographic parameters, indication of use, weight and days of hospitalization before and after initiation. Safety, tolerability, and parental satisfaction were assessed via standardized telephone questionnaire.Median (interquartile range [IQR]) age of initiating home HFNC in 75 children was 8.3 (2.2, 29.6) months. Indications were obstructive sleep apnea (33; 44%), airway malacia (19; 25%), chronic lung disease (15; 20%), neuromuscular disease (4; 5%), and postextubation support (4; 5%). Weight standard deviation score rose from -2.3 pre-HFNC to -1.7 at 6.7 months post-HFNC initiation, p 0.001. Hospital admission days during the 2 months pre- versus post-HFNC initiation were 22 (5.5, 60) and 5 (0, 14.7) respectively, p 0.008. Median (IQR) parental satisfaction score was 5/5 (4, 5). Fifty of 60 (83%) respondents would recommend home HFNC to other families in a similar situation. There were no serious adverse events.In our population, domestic HFNC appeared safe and well tolerated for a variety of indications. Its introduction was associated with improved weight gain, fewer hospitalization days and high parental satisfaction. Further work is required to characterize groups of children most likely to benefit from HFNC, as opposed to traditional modes of NIV.

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.

In Duchenne muscular dystrophy (DMD), lung disease contributes significantly to morbidity and mortality. This study aimed to assess the usefulness of various pulmonary function tests in evaluating DMD severity.This retrospective study analysed lung function tests of patients with DMD treated in the multidisciplinary respiratory neuromuscular clinic at Schneiders' Children Medical Center of Israel. Data were analysed according to age, ambulatory status and glucocorticoid treatment.Among 90 patients with DMD, 40/63 (63.5%) ambulatory patients and 22/27 (81.5%) non-ambulatory patients successfully performed spirometry. Significant annual declines were demonstrated among non-ambulatory patients, in percentile predicted forced vital capacity (3.8%) and in total lung capacity (5.5%) per year. The decline correlated with age and loss of ambulation but not with steroid treatment. Peak cough flow values were randomly distributed, and did not correlate with age, ambulation or treatment. In non-ambulatory patients, transcutaneous carbon dioxide measurement correlated significantly to age (r=0.55, p=0.02).Forced vital capacity, total lung capacity and transcutaneous carbon dioxide correlated with the clinical severity of disease in children with DMD. These measures may be useful in follow up and clinical trials. A comparable correlation was not found for peak cough flow.

Introduction: Several dry-powder inhalers (DPIs) contain lactose which may be contaminated with milk proteins. Confusion exists pertaining to DPI use in patients with cow’s milk protein allergy (CMPA). Methods: A computerized survey sent via e-mail to pediatric pulmonologists and allergologists. Results: A total of 77 out of 232 (33.2%) doctors replied, of whom 80.5% were pediatric pulmonologists. A total of 69 of 77 (89.6%) were specialists, 37.6% with more than 15 years of experience. The most commonly used DPIs were formoterol + budesonide and vilanterol + fluticasone. A total of 62 out of 77 (80.5%) responders knew these DPIs contained lactose. A total of 35 out of 77 (45.5%) doctors who replied did not know that DPI leaflets list CMPA as a contra-indication to DPI administration. Of these, 4 (11.4%) stated that they would instruct patients with CMPA to stop DPIs, and 7 (20%) would avoid recommending DPIs. A total of 42 out of 77 (54.5%) responders were aware of this warning, yet 13 of these 42 (30.9%) continued to recommend lactose-containing DPIs without hesitation and 18 of these 42 (42.8%) responders prescribed DPIs but considered allergy severity. Conclusions: Almost half of certified, experienced pediatric pulmonologists and allergologists were unaware of the warning to administer DPIs to patients with CMPA. Most doctors who do know of this warning still continue to prescribe these DPIs.

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

Systematic data are lacking on pediatric long COVID. This study prospectively assessed 90 children with persistent symptoms who presented to a designated multidisciplinary clinic for long COVID. In nearly 60%, symptoms were associated with functional impairment at 1-7 months after the onset of infection. A comprehensive structured evaluation revealed mild abnormal findings in approximately half the patients, mainly in the respiratory aspect.

In vitro studies have demonstrated rescue of CFTR function with Elexacaftor/Tezacaftor/Ivacaftor (ETI) in several mutations other than F508del. However, clinical efficacy was not tested in vivo in people with CF (pwCF) carrying mutations other than F508del. We report effects of treatment with ETI in pwCF with non-F508del mutations.We retrospectively analyzed pwCF with non-F508del mutations who received treatment with ETI. We evaluated sweat chloride, nutritional status, spirometry, antibiotic treatment, and pulmonary exacerbations (PEx), at baseline and 3-6 months after commencing treatment with ETI.We included 16 pwCF, including eight without previous use of CFTR modulators. Median time on treatment was 5.3 (range, 1.8-7.7) months. Compared to baseline, in the "naïve" group sweat chloride concentration was reduced from 113.0 (98-129) to 64.0 (32-97) mEq/L (n=7; median (IQR), p=0.018), and rate of pulmonary exacerbations declined from a median of 1.5 (IQR 1, 2.75) in the previous year to 0 (0,0) (p= 0.019) with a significant decline in annualized days with antibiotics (oral + parenteral) per year: 36 (17.5; 42) in the year before to 0 (0,0) (median (IQR), p= 0.027). Mean FEVWe demonstrate the clinical efficacy of ETI in pwCF carrying CFTR processing non-F508del mutations which are predicted to respond by in vitro studies. Our results support routine clinical use of ETI in this patient group.

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.

Background: Inhaled bronchodilators are frequently used among patients with primary ciliary dyskinesia (PCD), although neither the effectiveness nor the prevalence of their use is known, due to the paucity of relevant studies. Methods: This is a retrospective analysis of pre- and post-bronchodilator spirometry results, of patients with PCD from two centers. Correlations were examined of bronchodilator response, with asthma and atopy markers. Results: Of 115 patients, 46 (40%) completed spirometry pre- and post-bronchodilation. Of these, 26 (56.5%) demonstrated reversible airway obstruction (increase in %FEV1 predicted ≥ 10%). Obstruction reversibility was not found to be associated with a family history of asthma, blood eosinophil level, elevated IgE, or atopy symptoms. Of the 46 patients who completed bronchodilator spirometry, 29 (63%) were regularly using bronchodilators and inhaled corticosteroids. Conclusions: More than half of patients with PCD presented with reversible airway obstruction, without any correlation to markers of personal or familial atopy. Inhaled bronchodilators and corticosteroid therapies are commonly used for treating PCD. Evaluating bronchodilator response should be considered, and its effectiveness should be further studied.

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.

Background Influenza virus infection is an important cause of under-five mortality. Maternal vaccination protects children younger than 3 months of age from influenza infection. However, it is unknown to what extent paediatric influenza-related mortality may be prevented by a maternal vaccine since global age-stratified mortality data are lacking. Methods We invited clinicians and researchers to share clinical and demographic characteristics from children younger than 5 years who died with laboratory-confirmed influenza infection between January 1, 1995 and March 31, 2020. We evaluated the potential impact of maternal vaccination by estimating the number of children younger than 3 months with in-hospital influenza-related death using published global mortality estimates. Findings We included 314 children from 31 countries. Comorbidities were present in 166 (53%) children and 41 (13%) children were born prematurely. Median age at death was 8·6 (IQR 4·5–16·6), 11·5 (IQR 4·3–24·0), and 15·5 (IQR 7·4–27·0) months for children from low- and lower-middle-income countries (LMICs), upper-middle-income countries (UMICs), and high-income countries (HICs), respectively. The proportion of children younger than 3 months at time of death was 17% in LMICs, 12% in UMICs, and 7% in HICs. We estimated that 3339 annual influenza-related in-hospital deaths occur in the first 3 months of life globally. Interpretation In our study, less than 20% of children is younger than 3 months at time of influenza-related death. Although maternal influenza vaccination may impact maternal and infant influenza disease burden, additional immunisation strategies are needed to prevent global influenza-related childhood mortality. The missing data, global coverage, and data quality in this study should be taken into consideration for further interpretation of the results. Funding Bill & Melinda Gates Foundation.

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.

BackgroundVideo fluoroscopy swallow studies (VFSS) are considered gold standard for the diagnosis of aspiration in children but require resources and radiation compared to clinical feeding evaluation (CFE). We evaluated their added value for diagnosis, feeding management and clinical status.MethodsA retrospective single-center cohort study of children aged 0-18 years, referred for VFSS at a tertiary pediatric hospital. Results 113 children, median age (range) 2.2 years (0.1-17.9) successfully completed VFSS. Forty-six (41%) had oropharyngeal aspiration, 9 (8%) overt alone and 37 (33%) including silent aspirations. Underlying medical conditions included clinically suspected aspiration lung disease (ALD), 87 (77%); neurologic disease, 73 (64%); gastrointestinal disease, 73 (64%) and congenital heart disease, 42 (37%), not mutually exclusive. Those with ALD or cerebral palsy were more likely to have aspiration by VFSS, OR 3.2 and 9.8 respectively. Sensitivity and specificity of CFE for VFSS diagnosis of aspiration were 71% and 84% respectively. Feeding recommendations after VFSS differed significantly from those based on prior CFE, pConclusions In this population of children with a high prevalence of clinically suspected ALD, VFSS refined diagnosis and altered feeding management compared to CFE, with subsequent clinical improvement.

Background: Respiratory syncytial virus (RSV) bronchiolitis is the most common lower respiratory tract disorder causing hospitalization in infants. Palivizumab has shifted the profile of the hospitalized population away from premature infants and towards those with chronic morbidities who are not eligible for prophylaxis. Aim: To characterize RSV bronchiolitis hospitalizations in infants with chronic diseases, compared to otherwise healthy infants. Methods: A four consecutive RSV season retrospective analysis of patients younger than two years admitted with bronchiolitis. Background demographic and clinical data, including vital sign measurements, laboratory tests, and pediatric intensive care unit (PICU) admissions during hospitalization, were analyzed. Results: Of 1124 hospitalizations due to RSV bronchiolitis, 244 (22%) were in infants with chronic diseases. Although 20/1124 qualified for RSV prophylaxis, only 8 had been vaccinated. Compared to otherwise healthy infants, children with chronic diseases had longer hospitalizations, median 4 days (IQR 4-7) vs 3 days (2-5), p

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 O2 saturation, end-tidal carbon dioxide (EtCO2), 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, EtCO2 increased; the rise was significantly higher while wearing masks: slow walk, mean EtCO2 (mmHg) change +4.5 ± 2.4 versus +2.9 ± 2.3, P = .004; brisk walk EtCO2 change +8.4 ± 3.0 versus +6.2 ± 4.0, P = .009, with and without masks, respectively. Wearing masks was also associated with higher proportions of participant hypercarbia (EtCO2 range, 46-49 mmHg) compared with walking without masks, though this was only partially significant. Mean O2-saturation remained stable (98%) while walking without masks but decreased by 1.2 % ± 2.2 while walking briskly with a mask (P = .01). Mild desaturation (O2 range, 93% to 96%) was noted during brisk walks among 43% of participants with masks, compared with only 14% without masks (P = .08). Borg's scale significantly increased while walking with a mask, for both slow and brisk walks (P < .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.

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 (&minus, 2.03 &plusmn, 1.7 as compared with &minus, 0.35 &plusmn, 0.68 for the placebo group, p = 0.009), hemoglobin levels improved significantly (10.5 &plusmn, 2.6 to 11.4 &plusmn, 2.02 g/dL, p = 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 (&minus, 1.99 &plusmn, 1.41, p = 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.

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.

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 (SF6 ) 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

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 CFTR gene mutations suggesting that expanded preconception carrier screening might achieve higher preconception detection rates.

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.

Background: In children with suspected aspiration, clinical swallow assessment (CSA) may be supplemented with video-fluoroscopic swallow studies (VFSS) to inform feeding management. Aim: To assess the diagnostic accuracy of CSA, compared to VFSS and evaluate the impact of VFSS on feeding management and clinical status. Method: Retrospective case review of children who underwent CSA, followed by VFSS in a tertiary centre. Results: 113 children underwent VFSS at age 2.2 (0.1-17.9) years, median (range). Diagnoses were syndromic entities (38, 34%), CNS dysfunction (28, 25%), anatomic abnormalities (18, 16%), CNS malformation (7, 6%), neuromuscular disease (6, 5%) and other (16, 14%). 62% had laryngeal penetration or aspiration per CSA, compared to 38% per VFSS, p VFSS based feeding route recommendations differed significantly from those based on prior CSA, p=0.04. Following VFSS, the rate of orally fed children rose from 66% to 78%, p=0.006. However, exclusive gastrostomy feeding also increased, from 7% to 12%, p=0.005. In the year after VFSS, compared to the year prior, total hospital admissions fell from 364 to 252, p Conclusion: CSA overestimated the presence of overall swallowing abnormalities, but underestimated silent aspirations. VFSS led to change in feeding route and advice, which was associated with clinical improvement.

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.

Summary 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 FEV1 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 FEV1 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 FEV1 (P = 0.025) as did those conditioned with radiation, regarding both FVC (P = 0.003) and FEV1 (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. Pediatr Pulmonol. 2014; 49:1124–1132. © 2013 Wiley Periodicals, Inc.

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 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. Methods 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). Results The procedure was safe, with arterial oxygen saturation remaining at ≥ 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; p r = 0.35; p r = 0.41; p Conclusions IS testing in the young is feasible, safe, and clinically useful, and could serve as an outcome measure for new therapies.

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.