Pediatric negative pressure pulmonary edema: Case series and review of the literature.

INTRODUCTION: Negative pressure pulmonary edema (NPPE) is a potentially life-threatening complication that develops rapidly following acute upper airway obstruction. The condition is rare, dramatic but resolves quickly. Prompt recognition and appropriate supportive treatment may prevent unnecessary investigations and iatrogenic complications. METHODS: We describe a spectrum of etiologies and clinical manifestation of pediatric NPPE in our center and review of previous publications. CONCLUSION: The etiology for the development of NPPE in children has shifted over the years. Although dramatic in presentation, this type of pulmonary edema often resolves quickly with minimal support.

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

BACKGROUND: Primary Ciliary Dyskinesia (PCD) is rare and its features in Israel have not been described. AIMS: to assess prevalence utilizing state-of-the-art diagnostic techniques, and describe clinical features, diagnostic and management practices in Israel. METHODS: A national multicenter study from 2012 to 2013 recruited patients diagnosed or suspected of having PCD. 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. RESULTS: 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% nNO<233 ppb, 62% abnormal HVMA, 51% diagnostic TEM, 58% diagnostic IF and, 57% genetic diagnosis. CONCLUSIONS: 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.

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

Rationale: Primary ciliary dyskinesia (PCD) is under diagnosed and underestimated. Most clinical research has used some form of questionnaires to capture data but none has been critically evaluated particularly with respect to its end-user feasibility and utility. Objective: To critically appraise a clinical data collection questionnaire for PCD used in a large national PCD consortium in order to apply conclusions in future PCD research. Methods: We describe the development, validation and revision process of a clinical questionnaire for PCD and its evaluation during a national clinical PCD study with respect to data collection and analysis, initial completion rates and user feedback. Results: 14 centers participating in the consortium successfully completed the revised version of the questionnaire for 173 patients with various completion rates for various items. While content and internal consistency analysis demonstrated validity, there were methodological deficiencies impacting completion rates and end-user utility. These deficiencies were addressed resulting in a more valid questionnaire. Conclusions: Our experience may be useful for future clinical research in PCD. Based on the feedback collected on the questionnaire through analysis of completion rates, judgmental analysis of the content, and feedback from experts and end users, we suggest a practicable framework for development of similar tools for various future PCD research.

Bronchial Reactivity, Inflammatory and Allergic Parameters, and Vitamin D Levels in Children With Asthma.

BACKGROUND: A low serum vitamin D level may represent a marker of other perplexing factors that may lead to increased asthma prevalence and severity. Our aim was to assess the correlation between vitamin D levels and asthma and allergy markers in a subgroup of children with fewer confounding factors. METHODS: Non-obese children (6-18 y old) with asthma who were not receiving anti-inflammatory treatment were recruited. Subjects underwent spirometry with a methacholine challenge test, and fractional exhaled nitric oxide (FENO), serum vitamin D levels, total immunoglobulin E (IgE) levels, blood eosinophil counts, and high-sensitivity C-reactive protein levels were determined. The primary end point was the correlation between vitamin D level and airway hyper-responsiveness as assessed by a methacholine challenge test. The secondary end point was the correlation between vitamin D level and FENO, systemic inflammatory markers, and allergy. RESULTS: Seventy-one children with asthma (25 females, 35%; 12.5 ± 3.6 y of age) were included. The median vitamin D level was 23 ng/mL (range of 6-48.5, mean of 23.02 ± 7.74), the median IgE level was 305 IU/mL (range of 4.3-4,240), the median provocational concentration of methacholine that produced a 20% decrease in FEV1 was 1.1 mg/mL (range of 0-13.9), and the median FENO was 26.5 ppb (range of 3.6-285). No correlation was found between vitamin D level and response to the methacholine challenge test, FENO, high-sensitivity C-reactive protein levels, IgE levels, eosinophil counts, and frequency of allergic rhinitis or atopic dermatitis. CONCLUSIONS: In our group of children with asthma, no correlation was found between the level of vitamin D and the degree of airway reactivity, airway inflammation, and allergy. The cause-and-effect relationship between vitamin D, asthma, and allergy should be further clarified. (ClinicalTrials.gov registration NCT01287455).

The effect of vitamin D on airway reactivity and inflammation in asthmatic children: A double-blind placebo-controlled trial.

BACKGROUND: Cross-sectional studies have reported an association of vitamin D deficiency with increased asthma prevalence and severity, and with allergies. The effect of vitamin D as sole therapy on airway hyper-reactivity (AHR) and airway inflammation has not been reported. AIM: To evaluate the effect of vitamin D therapy on AHR as assessed by methacholine concentration, causing a 20% reduction in FEV1 (PC20 -FEV1 ) and fractional exhaled nitric oxide (FeNO), systemic markers of allergy and inflammation, and exhaled breath condensate cytokines. PATIENTS AND METHODS: Children aged 6-18 years with a clinical diagnosis of mild asthma currently not receiving anti-inflammatory therapy and with low vitamin D levels were included in this randomized, double-blind, placebo-controlled study assessing the effect of 6 weeks of treatment with oral vitamin D 14,000 units once weekly or placebo. RESULTS: Of the 39 patients included, 20 received vitamin D treatment and 19 received a placebo. Vitamin D replacement resulted in a significant increase in vitamin levels, which remained unchanged in the placebo group (P < 0.0001). There was no change in IgE, eosinophil count, high sensitivity C-reactive protein, FeNO levels or PC20 -FEV1 following treatment. Similar values of exhaled breath condensate cytokines (IL4, IL5, IL10, IL17, and γ interferon) were observed in both groups. CONCLUSIONS: In our small group of children with mild asthma, no difference could be demonstrated between the effect of vitamin D and placebo, despite significant increases in vitamin D blood levels. Larger interventional studies are needed to fully explore the possible effect of vitamin D in asthma.

Severe pertussis and hyperleukocytosis: is it time to change for exchange?

BACKGROUND: Pertussis is an important cause of infant death worldwide and continues to be a public health concern even in countries with high vaccination coverage. Severe (critical) pertussis with hyperleukocytosis is a severe form of the disease with up to 80% mortality rate. Attempts have been reported to reduce the white blood cell burden by exchange transfusion (ET) with conflicting conclusions. CASE REPORT: We report a case of critical pertussis in a neonate who was treated with ET, in view of a comprehensive literature review. RESULTS: The patient was discharged without any sequelae. CONCLUSION: ET should be considered in all infants suffering from critical pertussis and hyperleukocytosis, even in secondary care community hospitals.

Legionella pneumophila and Pneumocystis jirovecii coinfection in an infant treated with adrenocorticotropic hormone for infantile spasm: case report and literature review.

We describe an 8-month-old infant with infantile spasms treated with adrenocorticotropic hormone (ACTH) who presented with fatal Legionella pneumophila and Pneumocystis jirovecii infection. Emphasis is placed on the ensuing immunosuppression and infectious sequelae of ACTH therapy. Given that ACTH therapy may increase the risk of fatal infection, patients undergoing such treatment should be closely monitored, with particular attention paid to the functioning of the immune system.

Urinary tract infections caused by community-acquired extended-spectrum ß-lactamase-producing and nonproducing bacteria: a comparative study.

OBJECTIVE: To study the clinical characteristics and associated risk factors of urinary tract infections (UTIs) caused by community-acquired extended-spectrum ß-lactamase (CA-ESBL)-producing Enterobacteriaceae. STUDY DESIGN: A case-control study at a large community hospital in northern Israel, comparing children who had UTI due to CA-ESBL (n = 25) and CA non-ESBL (n = 125) in 2008-2011. Data were collected from medical charts, telephonic questionnaires administered to all participants, and groups were compared. RESULTS: During the study period, the yearly incidence of CA-ESBL UTI increased significantly. There were no significant differences between the CA-ESBL and CA non-ESBL groups in demographics and clinical outcome. Compared with CA non-ESBL UTI, children with CA-ESBL UTI had a longer hospital stay (5.9 ± 3.3 vs 3.9 ± 2.3 days; P = .003) and higher rates of recent hospitalization (28% vs 4%; P = .001), previous UTI (40% vs 13%; P = .003), urinary tract anomalies (32% vs 5%; P < .001), UTI prophylaxis with cephalexin (32% vs 2%; P < .005), and aminoglycoside resistance. In a multivariate analysis, UTI prophylaxis (OR 12.5 [CI 2.7-58]), recent hospitalization (OR 4.8 [CI 1.1-21]), and Klebsiella spp. UTI (OR 4.7 [CI 1.3-17]), were risk factors for CA-ESBL UTI. CONCLUSIONS: Children prescribed UTI prophylaxis (due to urinary tract anomalies or recurrent UTI) with cephalexin and those with previous hospitalizations are at increased risk for CA-ESBL UTI. Although not associated with higher rates of complications, the multidrug resistant phenotype of CA-ESBL isolates poses a challenge in choosing appropriate empiric and definitive therapy and prolongs hospital stay.

Pericardial effusion in an infant with severe respiratory syncytial virus bronchiolitis.

Respiratory syncytial virus is the most common pathogen causing lower respiratory tract infection in infants. Respiratory syncytial virus infection is also associated with a number of extrapulmonary manifestations, including the cardiac system. Pericardial effusion, however, is a very rare occurrence with respiratory syncytial virus infection. We report a very young infant with respiratory syncytial virus bronchiolitis whose clinical course was associated with pericardial effusion, treated conservatively.

Intravenous fluids versus gastric-tube feeding in hospitalized infants with viral bronchiolitis: a randomized, prospective pilot study.

The American Academy of Pediatrics recommends intravenous fluids for infants with bronchiolitis who are unable to sustain oral feedings. Our randomized, prospective pilot study shows that gastric tube feeding (in 31 infants) is feasible and demonstrated comparable clinical outcomes with intravenous fluids (in 20 infants) among hospitalized infants ≤6 months of age with moderate bronchiolitis.

The potential use of spirometry during methacholine challenge test in young children with respiratory symptoms.

BACKGROUND: The concentration of methacholine that causes a fall of 20% from baseline forced expiratory volume in the first second (PC20-FEV1) in the methacholine challenge test (MCT) is not usually considered a diagnostic tool in preschool children since PC20-FEV1 may not be achievable <6 years of age. AIM: To assess the usefulness of various spirometry indices obtained during MCT in a large group of 3- to 6-year-old children with respect to their clinical diagnosis. METHODS: Standardized MCT (inhaled triple-concentration increments [0.057-13.925 mg] of methacholine solution) was performed by 84 children previously diagnosed with asthma (asthmatics) and 48 with prolonged cough (coughers). Spirometry was determined at baseline and between inhalations; PC20-FEV1 and PC25-FEV0.5 were calculated. RESULTS: PC20-FEV1 values were significantly less in the asthmatics than in the coughers (mean +/- SD was 3.21 +/- 4.32 vs. 22.35 +/- 3.66 ml/mg). Similarly, PC25-FEV0.5 was 1.48 +/- 3.08 in the asthmatics and 9.45 +/- 12.59 mg/ml/Mch in the coughers, P < 0.0001. A cut-off at 4.0 mg/ml for PC20-FEV1 had 77.4% sensitivity and 75.0% specificity, a cut-off at 2.2 mg/ml for PC25-FEV0.5 had 73.8% sensitivity and 72.9% specificity, for clinical diagnosis of asthma. PC25-FEV0.5 also showed a correlation with age. CONCLUSIONS: Our findings suggest that MCT can be performed in preschool children with various respiratory symptoms. PC25-FEV0.5 may be a better end-point parameter. Children with a clinical diagnosis of asthma respond to a lower MCT concentration than children with cough. Further studies are needed to determine airway responsiveness in healthy young children and to further assess the contribution of MCT to the clinical diagnosis in this age group.