Experience in the surgical treatment of chronic suppurative lung disease in children

Operations were performed on 750 children with cystic hypoplasia and dysontogenetic and acquired bronchiectases. The operation volume: pulmonectomy, lobectomy, combined resections, resection of pulmonary tissue with extirpation of bronchi, extirpation of bronchi. Complications: atelectasis, pneumonia, intrapleural hemorrhage were encountered in 87 (11.6%) children. Six (0.8%) children died. Recurrent or residual bronchiectases were found in 27 children, 18 underwent a second operation. Recurrences were encountered mainly in children with dysontogenetic bronchiectases.     

Pulmonary resection in the prone position for suppurative lung disease in children

Lung resection for suppurative inflammatory disease is hazardous in children whose small airway diameter precludes the use of standard methods of bronchial separation. A prospective evaluation of the prone position for thoracotomy in 17 children referred for operation with severe inflammatory disease was done. Bronchography showed whole lung bronchiectasis eight, destroyed lung in three, and lobar bronchiectasis five. Pulmonary resections performed with the child prone included left pneumonectomy (nine), right pneumonectomy (four), lingulectomy with lower lobectomy (two), and other lobectomy (two). No endobronchial or intrapleural spillage occurred. One child required reexploration for bleeding and one child developed a postoperative empyema that ultimately caused death. The remaining 16 children were discharged within 8 days of operation, and follow-up of 1 to 18 months records favorable progress.     

The fungal airway microbiome in cystic fibrosis and non-cystic fibrosis bronchiectasis

BackgroundThe prevalence of fungal disease in cystic fibrosis (CF) and non-CF bronchiectasis is increasing and the clinical spectrum is widening. Poor sensitivity and a lack of standard diagnostic criteria renders interpretation of culture results challenging. In order to develop effective management strategies, a more accurate and comprehensive understanding of the airways fungal microbiome is required. The study aimed to use DNA sequences from sputum to assess the load and diversity of fungi in adults with CF and non-CF bronchiectasis.MethodsNext generation sequencing of the ITS2 region was used to examine fungal community composition (n = 176) by disease and underlying clinical subgroups including allergic bronchopulmonary aspergillosis, chronic necrotizing pulmonary aspergillosis, non-tuberculous mycobacteria, and fungal bronchitis. Patients with no known active fungal disease were included as disease controls.ResultsITS2 sequencing greatly increased the detection of fungi from sputum. In patients with CF fungal diversity was lower, while burden was higher than those with non-CF bronchiectasis. The most common operational taxonomic unit (OTU) in patients with CF was Candida parapsilosis (20.4%), whereas in non-CF bronchiectasis sputum Candida albicans (21.8%) was most common. CF patients with overt fungal bronchitis were dominated by Aspergillus spp., Exophiala spp., Candida parapsilosis or Scedosporium spp.ConclusionThis study provides a framework to more accurately characterize the extended spectrum of fungal airways diseases in adult suppurative lung diseases.     

Comparison of ultrasound and biopsy findings in children with cystic fibrosis related liver disease.

BACKGROUND: The objective of our study was to determine if hepatic ultrasound findings in paediatric patients with cystic fibrosis and suspected liver disease are related to histopathological results derived from liver biopsies. METHODS: A retrospective analysis of ultrasound and liver biopsy findings using published criteria was performed in 30 children with cystic fibrosis suspected as having liver disease on clinical, biochemical and ultrasonographic criteria. The results were correlated and assessed for intra- and interobserver agreement. RESULTS: A significant association was found for the prediction of fibrosis or cirrhosis on the basis of ultrasound (p=0.03). There was no significant association between normal or indeterminate ultrasound and histology results. A high intra- and interobserver variability was found in sonographic assessment of the hepatic echostructure. CONCLUSIONS: The diagnosis of early liver disease in cystic fibrosis cannot reliably be made on the basis of ultrasound alone. A normal ultrasound does not preclude significant liver fibrosis in cystic fibrosis. An abnormal US that suggests cirrhosis predicts the presence of moderate to severe liver disease.     

Nailfold capillaroscopic findings in systemic sclerosis related lung fibrosis and in idiopathic lung fibrosis

ObjectiveInterstitial lung diseases (ILDs) are heterogeneous pathological conditions, which can be idiopathic or associated to other diseases, such as connective tissue diseases (CTD), especially systemic sclersosis (SSc). Both in primary and secondary forms of ILDs, fibroblastic and vascular anomalies are involved in the progressive structural destruction of lung parenchyma. Nailfold video-capillaroscopy (NFC) is a non-invasive diagnostic tool that permits detection of the main local microvascular alterations in SSc, which are an expression of the systemic vascular changes characteristic of this disease. The aim of this study is to record the range of capillaroscopic anomalies in patients with idiopathic pulmonary fibrosis (IPF) and to detect the main differences compared to patients with pulmonary fibrosis associated to SSc (SSc-PF).MethodsWe performed NFC on 23 patients with PF secondary to systemic sclerosis (SSc-PF), 20 patients with IPF, and 22 patients with chronic obstructive pulmonary disease (COPD) and we analysed the differences in morphological capillaroscopic parameters between the study groups.ResultsThe main finding of this study was the detection of minor capillaroscopic alterations in patients affected by IPF compared to SSc-PF patients, the latter having typical capillary loop changes. Particularly, we found that in IPF patients capillary density was significantly reduced and neoangiogenic aspects was significantly greater compared to patients with COPD.ConclusionsAn altered nailfold capillaroscopic pattern in IPF, as observed in this study, seems to support the hypothesis that a systemic vascular dysfunction in these patients plays a role in the pathogenesis of the disease.     

Risk factors for progression of structural lung disease in school-age children with cystic fibrosis

BackgroundComputed tomography (CT) is used to monitor progression of structural lung disease (SLD) in children with cystic fibrosis (CF). Our goals were to identify the risk factors for the annual progression of SLD and the impacts of airway pathogens on SLD.MethodSeventy-five school-aged children diagnosed with CF underwent 200 CT scans at Gothenburg CF Centre in the period 2003–2015. SLD was evaluated with a quantitative scoring system. Mixed models were used to calculate the yearly progression rates of SLD and FEV₁ and to analyse the effects of common airway pathogens in CF.ResultsThe yearly mean progression (95% CI) rates for total disease (%Dis), bronchiectasis (%Be), and FEV₁ were 0.62 (0.38–0.86), 0.43 (0.28–0.58) and −0.16 (−0.18–0.13), respectively. Adjusting for airway pathogens, the yearly mean progression rates for %Dis, %Be and FEV₁ were 0.23 (−0.04–0.51), 0.12 (0.00–0.25), and −0.12 (−0.16–0.08), respectively. A single infection with P aeruginosa was associated with significant increase in lung damage, assessed as %Dis (p = 0.044) and%Be (p = 0.0047), but not in FEV₁ (p = 0.96). At age of 7 years, there was a good correlation between the extent of SLD and subsequent progression of %Dis (r = 0.63, p = 0.0042) and %Be (r = 0.74, p = 0.0057) while there was no significant correlation between the FEV₁ and the rate of decline of FEV₁ (r = −0.22, p = 0.12).ConclusionIntermittent respiratory infections with P aeruginosa were associated with significant SLD but no change in FEV₁. More SLD at the age of 7 years signals a higher progression rate of SLD subsequently.     

Bronchoscopic findings in post-obstructive pulmonary oedema

Purpose

To present the first photographed bronchoscopic findings associated with negative pressure pulmonary oedema (NPPE).

Clinical features

A previously healthy patient underwent anterior C₃–C₄ disc removal and arthrodesis. Following tracheal extubation he developed acute respiratory distress manifested as stridor, tachypnoea, restlessness, and desaturation. Once the trachea was reintubated, he displayed the classic findings of pulmonary oedema. Bronchoscopy was performed to confirm tracheal tube position and to rule out tracheal injury secondary to surgical manipulation. Diffuse punctate haemorrhages were noted throughout the visualised tracheobronchial tree.

Conclusion

We believe that these haemorrhages represent disruption of the bronchial vasculature and may contribute to the clinical presentation of NPPE.     

Techniques and complications of one-lung ventilation in children with suppurative lung disease: experience in 15 cases.

OBJECTIVE: To evaluate lung isolation with Fogarty catheters and to analyze respiratory consequences of one-lung ventilation (OLV) in children with suppurative lung disease. DESIGN: Prospective. SETTING: University hospital. PARTICIPANTS: Fifteen children undergoing thoracotomy. INTERVENTIONS: Bronchial blockade with a 7F Fogarty catheter was attempted. In case of incomplete blockade or failure in directing the catheter into the desired mainstem bronchus, endobronchial intubation was done. Volume-controlled ventilation was performed with fraction of inspired oxygen (F(I)O2), 0.5; inspiratory-to-expiratory (I: E) ratio, 1:2; and 10 mL/kg tidal volume during two-lung ventilation (TLV). F(I)O2 was increased to 1.0 by the initiation of OLV. If peak airway pressure exceeded basal values during TLV by 35%, tidal volume was reduced to 8 mL/kg, inspiratory pause was zeroed, and I:E ratio was increased to 1:1. Hemodynamic and respiratory parameters were recorded during TLV and 30 minutes after initiation of OLV. Peripheral oxygen saturation and end-tidal carbon dioxide tension were recorded every 5 minutes. MEASUREMENTS AND MAIN RESULTS: Right lung isolation was successfully obtained by Fogarty catheters in 10 children undergoing right thoracotomy. Endobronchial intubation was performed in 2 children (40%) undergoing left thoracotomy. Three children (20%) developed episodes of severe hypercapnia and hypoxia requiring treatment during OLV. All of the parameters recorded at 30 minutes of OLV revealed statistically significant differences from TLV. OLV was transiently discontinued in 1 child. CONCLUSION: The use of Fogarty embolectomy catheters for lung isolation in children undergoing thoracotomy is recommended. Respiratory problems are not rare during OLV in children with suppurative lung disease and require immediate management.     

Interleukin-1 is associated with inflammation and structural lung disease in young children with cystic fibrosis

Background

Little is known about the role of interleukin (IL)-1 in the pathogenesis of cystic fibrosis (CF) lung disease. This study investigated the relationship between IL-1 signalling, neutrophilic inflammation and structural lung changes in children with CF.