Valentina Fainardi

Tracheobronchial obstruction: follow-up study of 100 children treated with airway stenting

OBJECTIVE We described a cohort of 100 children with a wide variety of airway obstruction who underwent stent positioning in the last 7 years. The study examined the outcomes of this treatment in the largest series of paediatric patients reported in the literature with special concern over safety and clinical effectiveness. METHODS We performed a retrospective analysis of 100 consecutive paediatric patients who underwent stent insertions between January 2005 and May 2012. Statistical analysis was performed and exact likelihood was used. RESULTS A total of 235 stents were placed for severe airway obstruction. One hundred and twelve silicone stents (cylinder, hourglass or Y-shaped), 120 metallic stents (covered Nitinol stents, expandable coronary and vascular stents) and 3 biodegradable polydioxanone (PDS) stents were used. Eighty patients presented clinical improvement after stent insertion, 17 were weaned off mechanical ventilation and 3 showed no significant clinical improvement [95% confidence interval (CI) 0.1-8.0%]. Complications were different according to stent type. In our cohort, no fatal stent-related complications have been observed. At follow-up (median 41.4 months, range 1.1-145.4) complete resolution was registered for 60 patients (66%; 95% CI 55-76%), 17 are still under treatment, 9 were lost to follow-up, 8 underwent surgery and 6 died of causes not stent related. CONCLUSION Airway stenting represents a conservative treatment before more invasive surgical procedures and can be very effective when performed in selected children and in specialized centres by physicians experienced in rigid and flexible bronchoscopy.

Prevalence of scoliosis in cystic fibrosis.

The prevalence of idiopathic scoliosis in the general pediatric population is reported to be between 0.5% and 3.2%. Previous studies have reported an increased prevalence of scoliosis in children with cystic fibrosis (CF).

Short-Term Effects of High-Frequency Chest Compression and Positive Expiratory Pressure in Patients With Cystic Fibrosis

Background Cystic fibrosis patients require daily airway clearance therapies. The primary objective of this study was to compare the short-term efficacy of high-frequency chest compression and positive expiratory pressure mask on expectorated sputum, pulmonary function, and oxygen saturation in patients with CF hospitalized for an acute pulmonary exacerbation. Methods A controlled randomized cross-over trial with 24 hours between treatments was used. Thirty-four CF patients (26 ± 6.5 years) were included in the study. Before and 30 minutes after each treatment were recorded: pulmonary function testing, oxygen saturation, and perceived dyspnea. Preference for the two devices was assessed. Results No statistically significant difference between high-frequency chest compression and positive expiratory pressure mask was found in sputum production and in lung function testing. A reduction in SpO2 was found after positive expiratory pressure mask (98 ± 1.0% versus 97 ± 1.2%; P < 0.001). Both treatments induced a statistically significant increase in Borg scale for dyspnea without differences between them. Patients reported greater satisfaction with positive expiratory pressure mask than with high-frequency chest compression (P < 0.001). Conclusion High-frequency chest compression and positive expiratory pressure mask have comparable short-term effects on expectorated sputum and lung function. Although positive expiratory pressure mask was associated with a lower SpO2, it was better tolerated than high-frequency chest compression. Keywords Airway clearance therapies; High-frequency chest compression; Sputum; Cystic fibrosis

Mepolizumab in the treatment of severe eosinophilic asthma

IL-5 is crucial in the pathogenesis and evolution of eosinophilic asthma. Mepolizumab is a high-affinity humanized monoclonal antibody of the IgG1/k subtype that inhibits the binding of IL-5 to its receptor expressed on eosinophils, thereby inducing significant reduction in eosinophil circulation, as well as asthma exacerbations and corticosteroid treatment. This review deals with the currently available studies of mepolizumab in the treatment of patients with severe eosinophilic asthma.

Residual tracheobronchial malacia after surgery for vascular compression in children: treatment with stenting†.

OBJECTIVES: Surgery for vascular anomalies can occasionally fail to relieve symptoms, especially when severe tracheobronchial malacia persists. We studied outcomes in children who underwent airway stenting for severe post-surgical airway malacia and tested known clinical and surgical prognostic factors. METHODS: Among 257 children evaluated for tracheobronchial vascular compression, we reviewed the clinical charts for the 59 patients (23%) who underwent surgery. After surgery, children in whom severe malacia and respiratory symptoms persisted underwent airway stenting. RESULTS: Among the 59 patients (boys: 58.1%, median age: 6.4 months, age range: 0.1–182.8 months), 79.7% had major comorbidities, 39% additional upper airway anomalies and 15.2% abnormal bronchial anatomy. Diagnostic imaging identified seven vascular anomalies: innominate artery compression 27.1%, left bronchial compression from the descending aorta and left pulmonary artery 20.3%, lesions associated with right aortic arch 22.0%, double aortic arch 13.6%, bovine arch type 10.2%, aberrant right subclavian artery 5.1% and pulmonary artery sling 1.7%. For severe residual malacia and severe persistent respiratory symptoms, 20 patients (33.9%) had silicone or metallic stents inserted. The most frequent indication for stenting was double aortic arch (P = 0.02 by chi-square test). A clinical prognostic factor for permanent stenting was left bronchial compression from the descending aorta and left pulmonary artery [odds ratio (OR): 14.667, 95% confidence interval (CI): 2.881–74.659], particularly if associated with congenital heart disease (OR: 30.00, 95% CI: 4.349– 206.927). All silicone stents but one were successfully removed; metallic uncovered stents were patent and completely re-epithelialized. CONCLUSIONS: When surgery leaves severe airway obstruction and respiratory symptoms unchanged, children with tracheobronchial malacia can safely undergo airway stenting.

Spontaneous Dissemination in Neighboring Provinces of DKA Prevention Campaign Successfully Launched in Nineties in Parma’s Province

Aim: to investigate how much effectiveness of the historical campaign of DKA prevention at T1D diagnosis has survived in Parma’s province where this was launched in Nineties, and how much it has spontaneously spread in the neighboring provinces. Method: children aged 6-14 years with newly diagnosed TID coming from province of Parma (Group 1) and from two other nearby provinces (Group 2) were investigated. Clinical and laboratory data were retrospectively collected from medical files of each patient and included age, gender, capillary pH, serum bicarbonate, 3-beta-hydroxybutyrate (3HB), glycated hemoglobin (HbAlc) at the time of admittance from 1st January 2012 and 31 December 2016. Results: no DKA condition was globally found in 25/36 patients (69.4%): 16/17 and 9/19 patients belonged to Group 1 and 2 respectively (p=0.002). Mild or moderate DKA was reported in 5.9% patients of Group 1 and in 47.31% (p=0.005) patients from Group 2. Severe DKA was observed in only 1 child from Group 2. Normal 3-beta-hydroxybutyrate (3HB) serum levels was reported in the 25 patients without DKA at diabetes diagnosis. Duration of hyperglycemia-related symptoms before overt T1D diagnosis was shorter (4.6±2.5 days) in patients with 3HB levels <1 mmol/dl than in those with 3HB levels exceeding 1 mmol/dl (9.6±4.2 days, p<0.0001). HbA1c values were on overage lower in patients without DKA (9.9±1.2%) than in patients with DKA at diabetes diagnosis (13.60±1.3%; p<0,001). Conclusion: 1) the campaign for DKA prevention, launched in Nineties and renewed at beginning of Twenties in Parma’s province, continues to be effective in the same province after several years; 2) in the two control provinces despite no information campaign being officially promoted in loco, an unexpected decrease in severe DKA incidence as well a shorter latency before overt T1D diagnosis were observed in the same period. (www.actabiomedica.it)

Right Aortic Arch Detected Prenatally: A Rare Case With Bilateral Arterial Duct and Nonconfluent Pulmonary Arteries

We describe a rare case of right aortic arch (RAA) and nonconfluent pulmonary arteries. RAA and a right-sided arterial duct (AD) were identified on the prenatal scan, but a second left-sided AD and disconnection of the left pulmonary artery were missed. The missed diagnosis in fetal life adversely affected postnatal management. We suggest that fetuses with a prenatal diagnosis of RAA and right-sided AD be delivered in tertiary care centres to rule out an association with bilateral AD and nonconfluent pulmonary arteries after birth. Prompt postnatal diagnosis will enable preservation of flow in the disconnected pulmonary artery through prostaglandin E1 infusion until surgical reconstruction.

The authors reply: Response to Letter

We thank Dr. Hathorn and colleagues for their interest in our manuscript and for reporting their own results. The ages of our patients were evenly spread throughout childhood (Fig. 1). We re-analyzed our data excluding patients below 10 years of age, and found a prevalence of scoliosis of 7/173 or 4%, still much less that Hathorn and others have reported. We did not have data on puberty in our population. Thus acquisition bias is not a likely explanation for our findings. The discrepancies between the various studies remain unexplained. —VALENTINA FAINARDI, MD* Department of Paediatrics Parma Children Hospital Parma, Italy

Lung function in a cohort of 5-year-old children born very preterm

We assessed lung function and respiratory health in an area‐based prospective cohort of preschool children born very preterm.

Lung function tests to monitor respiratory disease in preschool children

Pulmonary function tests are routinely used in the diagnosis and follow-up of respiratory diseases. In preschool children assessment and evaluation of lung function has always been challenging but improved techniques that require only minimal collaboration allowed obtaining reliable and useful results even in this group of patients. In this review we will describe the different techniques used in clinical practice to measure lung function in preschool children.(www.actabiomedica.it)