scholarly journals Multiple Breath Wash-out for early assessment of pulmonary complications in patients with Primary Antibody Deficiencies: an observational study in pediatric age

2021 ◽  
Author(s):  
Teresa Secchi ◽  
Lucia Augusta Baselli ◽  
Maria Chiara Russo ◽  
Irene Borzani ◽  
Federica Carta ◽  
...  
Keyword(s):  
Pediatric Patients ◽  
Pulmonary Function Tests ◽  
Pulmonary Complications ◽  
Lung Damage ◽  
Primary Antibody ◽  
Early Assessment ◽  
High Resolution Computed Tomography ◽  
Specific Test ◽  
Ventilation Inhomogeneity ◽  
Primary Antibody Deficiencies

Abstract Purpose. In Primary Antibody Deficiencies (PADs), pulmonary complications are the main cause of morbidity, despite immunoglobulin substitutive therapy, antibiotic treatment of exacerbations and respiratory physiotherapy. Current recommendations for surveillance of PADs respiratory complications include the annual assessment of spirometry and the execution of chest High Resolution Computed Tomography (HRCT) every four years.This study aims to evaluate the effectiveness of Lung Clearance Index (LCI) as early marker of lung damage in patients with PADs. LCI is measured by Multiple Breath Wash-out, a non-invasive and highly specific test widely used in Cystic Fibrosis patients.Methods. Pediatric patients with PADs (n=17, 10, M, 7, F, age range 5-15 years) underwent baseline assessment of lung involvement with chest HRCT, Spirometry and Multiple Breath nitrogen Wash-out. Among them, 13 patients were followed up to repeat HRCT after four years, while performing Pulmonary Function Tests annually. Their baseline and control LCI and FEV1 values were compared, taking HRCT as gold standard, using logistic regression analysis.Results. LCI (OR 2.43 at baseline, OR 14.95 at control) has a stronger discriminating power between altered and normal HRCT rather than FEV1 (OR 0.82 at baseline, OR 1.91 at control).Conclusion. Within the context of a limited sample size, LCI seems to be more predictive of HRCT alterations than FEV1 and more sensitive than HRCT in detecting ventilation inhomogeneity, in absence of bronchiectasis. A study of a larger cohort of pediatric patients followed longitudinally in adulthood is needed to challenge these findings.

scholarly journals Clinical and follow-up study of bronchiolitis obliterans in pediatric patients in China

2017 ◽  
Vol 15 (3) ◽  
pp. 152-159 ◽  
Author(s):  
Li Huang ◽  
Yaping Xie ◽  
Huifeng Fan ◽  
Gen Lu ◽  
Jialu Yu ◽  
...  
Keyword(s):  
Risk Factors ◽  
Bronchiolitis Obliterans ◽  
Pediatric Patients ◽  
Medical Center ◽  
Pulmonary Function Tests ◽  
Age At Onset ◽  
Airflow Obstruction ◽  
High Resolution Computed Tomography ◽  
Significant Difference ◽  
Post Infectious

Bronchiolitis obliterans (BO) is in general a rare and life-threatening form of non-reversible obstructive lung disease in which the bronchioles are compressed and narrowed by fibrosis and/or inflammation. The purpose of this study was to evaluate the clinical features of BO in pediatric patients and explore its risk factors. The medical records of 35 pediatric patients with BO at Guangzhou Women and Children’s Medical Center were evaluated. The age at onset of symptoms was 2–42 months (mean 13.3 ± 8.9 months), with age at diagnosis of 5 months–4 years (mean 17.8 ± 9.0 months). High-resolution computed tomography findings included mosaic pattern (100%), atelectasis (37.1%), air trapping (31.4%), and bronchiectasis (20.0%). Three patients received lung biopsies and mainly exhibited an inflammatory process surrounding the lumen of bronchioles. BO predominantly resulted from post-infectious causes (91.4%) which were primarily caused by adenovirus (50%), followed by Mycoplasma pneumoniae (46.7%) and influenza (20%). Pulmonary function tests (PFTs) showed severe and fixed airflow obstruction, decreased compliance, and increased resistance. No significant difference was found between before and after steroid treatment ( P > 0.05). Two patients died owing to severe pulmonary complications, one of whom had inherent immunodeficiency. Our study suggests that the occurrence of BO, especially post-infectious BO, in China is relatively high and might result from primary immunodeficiency diseases in severe cases. Recurrent aspiration pneumonia caused by congenital dysplasia of the larynx and vaccination not on schedule might be potential risk factors for persistent and recurrent BO.

scholarly journals The Relationship between Pulmonary Damage and Peripheral Vascular Manifestations in Systemic Sclerosis Patients

2021 ◽  
Vol 14 (5) ◽  
pp. 403
Author(s):  
Barbara Ruaro ◽  
Marco Confalonieri ◽  
Francesco Salton ◽  
Barbara Wade ◽  
Elisa Baratella ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Function Tests ◽  
Organ Damage ◽  
Right Heart Catheterization ◽  
Lung Damage ◽  
Organ Involvement ◽  
Heart Catheterization ◽  
High Resolution Computed Tomography ◽  
Microvascular Damage ◽  
Pulmonary Damage

Systemic sclerosis (SSc) is an autoimmune disease, characterized by the presence of generalized vasculopathy and tissue fibrosis. Collagen vascular disorder in SSc is due to fibroblast and endothelial cell dysfunctions. This leads to collagen overproduction, vascular impairment and immune system abnormalities and, in the last stage, multi-organ damage. Thus, to avoid organ damage, which has a poor prognosis, all patients should be carefully evaluated and followed. This is particularly important in the initial disease phase, so as to facilitate early identification of any organ involvement and to allow for appropriate therapy. Pulmonary disease in SSc mainly involves interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). High-resolution computed tomography (HRCT) and pulmonary function tests (PFT) have been proposed to monitor parenchymal damage. Although transthoracic echocardiography is the most commonly used screening tool for PAH in SSc patients, definitive diagnosis necessitates confirmation by right heart catheterization (RHC). Moreover, some studies have demonstrated that nailfold videocapillaroscopy (NVC) provides an accurate evaluation of the microvascular damage in SSc and is able to predict internal organ involvement, such as lung impairment. This review provides an overview of the correlation between lung damage and microvascular involvement in SSc patients.

scholarly journals Metabolic and hematologic changes occurring after rapid intravenous infusion of gammaglobulin in patients with antibody deficiency syndromes

1998 ◽  
Vol 116 (5) ◽  
pp. 1815-1820 ◽  
Author(s):  
Beatriz Tavares Costa-Carvalho ◽  
Marisa Lin ◽  
Dirceu Solé ◽  
Magda Maria Sales Carneiro-Sampaio ◽  
Ricardo Uhr Sorensen ◽  
...  
Keyword(s):  
Side Effects ◽  
Intravenous Infusion ◽  
Infusion Rate ◽  
Pediatric Patients ◽  
Antibody Deficiency ◽  
Primary Antibody ◽  
Rapid Infusion ◽  
Replacement Treatment ◽  
Primary Antibody Deficiencies ◽  
Ivig Preparation

OBJECTIVE: We wished to investigate whether increased IgG infusion rates are associated with metabolic and hematologic changes in pediatric patients with antibody deficiency syndromes. METHODS: We studied 7 patients (2-16 years old) with primary antibody deficiencies who had been on regular IgG replacement treatment, 350-600 mg/kg/dose every 3 weeks with a 3% IVIG preparation, for periods ranging from 6 months to 4 years. Initially, the IgG concentration of IVIG preparations was increased to 6, 9 and 12% in consecutive infusions at a constant IgG infusion rate of 4 mg/kg/min. Subsequently, the infusion rates were increased to 8, 12, and 16 mg/kg/min using the IVIG 12% preparation. RESULTS: Clinically, all patients tolerated increases in IVIG concentrations while the infusion rate was 4 mg/kg/min. However, 3 patients presented side effects when the infusion rate was increased to 8 and 16 mg/kg/min. CONCLUSION: We conclude that metabolic and hematologic sides effects occur with rapid infusion of IVIG even in patients who tolerate the increased infusion rate clinically. The advantages of using high infusion rates have to be re-evaluated.

Pulmonary function after COVID-19 in early convalescence phase

2020 ◽  
pp. 7-12 ◽  
Author(s):  
O. I. Savushkina ◽  
A. V. Cherniak ◽  
E. V. Kryukov ◽  
I. Ts. Kulagina ◽  
M. V. Samsonova ◽  
...  
Keyword(s):  
Pulmonary Function ◽  
Pulmonary Function Tests ◽  
Lung Damage ◽  
Diffusing Capacity ◽  
Diffusion Test ◽  
High Resolution Computed Tomography ◽  
Oxygen Saturation Level ◽  
Group 2 ◽  
Convalescence Phase ◽  
Group 1

Pulmonary function after COVID-19 in early convalescence phase. The aim of the study is to investigate the influence of Coronavirus disease 2019 (COVID-19) on pulmonary function in early convalescence phase.Materials and methods. The study included 44 patients (35 male) after COVID-19 without concomitant bronchopulmonary pathology, with a median age of 47.5 years. All patients underwent standard pulmonary function tests (PFTs): spirometry, body plethysmography, diffusion test. Besides, dyspnea on the mMRC scale was assessed, oxygen saturation level (SpO2 ) was measured. Depending on degree of lung damage determined using high-resolution computed tomography (CT), the patients were divided into 2 groups: group 1 (22 patients) — CT 1 and CT 2, group 2 (22 patients) — CT 3 and CT 4.Results. The medians of standard PFTs parameters were in normal values. However, there were statistically significant differences between groups: VC, FVC, FEV1 and TLC were lower in second group. Diffusing capacity was reduced in 52% of patients. Statistical significant correlations were established between lung damage by CT and the parameters of VC, FVC, FEV1 , TLC, IC and DLCO.Conclusion. The degree of functional disorders of lungs depended on the extent of abnormal CT. Impaired diffusing capacity were detected in more than half of the COVID-19 patients in early convalescence phase.

scholarly journals Diagnosing ventilation inhomogeneity after COVID-19 by multiple-breath nitrogen washout test

2021 ◽  
Vol 31 (1) ◽  
pp. 30-36
Author(s):  
E. V. Kryukov ◽  
O. I. Savushkina ◽  
A. V. Chernyak ◽  
I. C. Kulagina
Keyword(s):  
Frequency Dependence ◽  
Lung Damage ◽  
Study Cohort ◽  
Impulse Oscillometry ◽  
Body Plethysmography ◽  
High Resolution Computed Tomography ◽  
Cross Sectional ◽  
Nitrogen Washout ◽  
Ventilation Inhomogeneity ◽  
Group 2

The aim was to evaluate the ventilation inhomogeneity (VIH) by the multiple-breath nitrogen washout test (MBNW) after COVID-19 and to identify the relationship of the lung clearance index (LCI) with other functional parameters of the respiratory system. Methods. The cross-sectional study included 35 patients (97% men); the median age was 44 years. Spirometry, body plethysmography, diffusing capacity of the lung for carbon monoxide (DLco), MBNW test, and impulse oscillometry were performed. Dyspnea was evaluated by mMRC scale. MBNW test was performed using the Easy-one Pro, MBW Module (ndd Medizintechnik AG, Switzerland). Results. The patients were divided into 2 groups. Group 1 included 21 (60%) patients who were not diagnosed with VIH. Group 2 included 14 (40%) patients with VIH. The median testing period was 72 (47 - 109) days from the onset of COVID-19. The median of the maximum volume of lung damage determined by high-resolution computed tomography (CTmax) was 50% in the acute period of the disease and 12% during the study. The medians of all analyzed parameters remained normal in the study cohort as well as in groups 1 and 2, except the resonance frequency (fres) in group 2. Statistically significant differences were found between groups 1 and 2 in the absolute frequency dependence of resistance (R5 - R20), reactance area (AX), fres. Significant differences were also found in pathological changes of vital capacity, forced expiratory volume in the first second (FEV1), (R5 - R20). The abnormalities were more common in group 2. A significant correlation was shown between LCI with the ratio of residual lung volume to total lung capacity, (R5 - R20), AX, fres, relative frequency dependence of resistance, CTmax, FEV1 and trasfer-factor (DLco). Conclusion. Seventy-two days after the onset of CoVID-19, the ventilation inhomogeneity was detected in 40% of the patients, decreased DLco - in 23%, airway obstruction - in 11.4%, and restrictive ventilatory defect - in 8.6%. Correlations were found between LCI and DLco, spirometry parameters, body plethysmography, impulse oscillometry, and CTmax.

scholarly journals The broad spectrum of lung diseases in primary antibody deficiencies

2018 ◽  
Vol 27 (149) ◽  
pp. 180019 ◽  
Author(s):  
Francesco Cinetto ◽  
Riccardo Scarpa ◽  
Marcello Rattazzi ◽  
Carlo Agostini
Keyword(s):  
Broad Spectrum ◽  
Lung Diseases ◽  
Adaptive Immune Response ◽  
Interstitial Lung Diseases ◽  
Pulmonary Complications ◽  
Primary Antibody ◽  
Primary Antibody Deficiencies ◽  
Pulmonary Manifestations ◽  
Relevant Role ◽  
Immune Defects

Human primary immunodeficiency diseases (PIDs) represent a heterogeneous group of more than 350 disorders. They are rare diseases, but their global incidence is more relevant than generally thought. The underlying defect may involve different branches of the innate and/or adaptive immune response. Thus, the clinical picture may range from severe phenotypes characterised by a broad spectrum of infections to milder infectious phenotypes due to more selective (and frequent) immune defects. Moreover, infections may not be the main clinical features in some PIDs that might present with autoimmunity, auto-inflammation and/or cancer. Primary antibody deficiencies (PADs) represent a small percentage of the known PIDs but they are the most frequently diagnosed, particularly in adulthood. Common variable immunodeficiency (CVID) is the most prevalent symptomatic PAD.PAD patients share a significant susceptibility to respiratory diseases that represent a relevant cause of morbidity and mortality. Pulmonary complications include acute and chronic infection-related diseases, such as pneumonia and bronchiectasis. They also include immune-mediated interstitial lung diseases, such as granulomatous-lymphocytic interstitial lung disease (GLILD) and cancer. Herein we will discuss the main pulmonary manifestations of PADs, the associated functional and imaging findings, and the relevant role of pulmonologists and chest radiologists in diagnosis and surveillance.

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