Lung Involvement In Children Research Articles

Congenital cytomegalovirus and pulmonary hypertension.

Lung involvement in children with congenital cytomegalovirus infection has been scarcely described. We describe three new cases of persistent pulmonary hypertension in children with congenital cytomegalovirus and review the other seven cases reported in the literature since 1988. All children had a symptomatic infection, including severe central nervous system or visceral findings. Morbidity and mortality were high. Persistent pulmonary hypertension may be a rare complication in severely symptomatic congenital cytomegalovirus infants. It is important to screen for congenital cytomegalovirus in cases of idiopathic refractory persistent pulmonary hypertension. Intensive treatment should be undertaken to treat this potentially rare lung involvement in combination with antiviral treatment.

Pulmonary Function in Paediatric Patients with Inflammatory Bowel Disease

Background: Among the extraintestinal manifestations of inflammatory bowel disease (IBD), those involving the lungs are relatively rare and often overlooked. There are only scarce data on the prevalence of IBD-associated lung involvement in children. Objectives: The aim of our study was to assess pulmonary function in IBD children by different methods and to evaluate the influence of immunosuppressive therapy on disease severity. Methods: Seventy-two children with IBD (mean age of 14.45 ± 2.27 years) and 40 age-matched healthy controls (mean age of 14.17 ± 2.82) were included in the study. Pulmonary function tests (PFTs) were carried out by means of spirometry, oscillometry (IOS) and fractional exhaled nitric oxide (FeNO) to assess the pulmonary involvement. Results: Certain differences were observed between the study group and the control group, regarding the spirometric and oscillometry parameters. The fractions of exhaled nitric oxide did not differ between the group with IBD patients and the control group with regards to disease activity, the duration of illness and the administered immunosuppressive treatment. Conclusions: The mean spirometry results were significantly different in the study group compared to the controls, although they were still within the normal limits. The pulmonary function abnormalities did not depend on either the disease activity or the immunosuppressive therapy. Oscillometry could be a supplementary method to assess pulmonary resistance. In turn, FeNO does not appear to be useful either in screening IBD children for pulmonary involvement or for the evaluation of disease activity. It appears then that only general screening of asymptomatic patients is a suitable method and a necessary recommendation in this population, prompting a revision of the current diagnostic approach.

Lung involvement in children with newly diagnosed rheumatic diseases: characteristics and associations

BackgroundPulmonary complications of rheumatic diseases may cause functional impairment and increase mortality. However, reports regarding detection of lung involvement in children with treatment-naive, newly diagnosed rheumatic diseases are scarce. Herein, we aimed to describe the characteristics of such patients and explore the association between lung involvement and rheumatic disease.MethodsFrom January 2019 to June 2021, 48 pediatric patients with treatment-naive, newly diagnosed rheumatic diseases at Department of Rheumatology and Immunology, Shanghai Children's Medical Center, School of Medicine, Shanghai Jiao Tong University were included with pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) findings, and 51 age-matched healthy controls were examined based on PFTs. Univariate and multivariable logistic regression analyses were used to investigate the clinical characters and laboratory parameters associated with lung involvement in these patients.ResultsAsymptomatic patients had a faster respiratory rate and a higher ratio of forced expiratory volume in 1 s/forced vital capacity than the controls (P < 0.05). More patients than controls were observed to have a decreased DLCO below the lower limit of normal (18 of 45 [40.0%] vs. 6 of 36, respectively; P = 0.041). Among the 48 patients, 8 (16.7%) had abnormal HRCT findings and 27 (56.3%) had abnormal PFT results. Thirty-one (64.6%) patients had lung involvement. Logistic regression revealed that increases in the erythrocyte sedimentation rate (ESR) and CD4/CD8 ratio were associated with increased odds ratio of lung involvement (1.037, 95% CI: 1.003–1.072; 9.875, 95% CI: 1.296–75.243, respectively).ConclusionsPediatric patients with treatment-naive, newly diagnosed rheumatic diseases are prone to pulmonary involvement. Increased ESR and CD4/CD8 are associated with elevated odds of lung involvement in patients. We recommend routine pulmonary evaluation in such patients, especially in high-risk patients, even in the absence of respiratory symptoms, once they are diagnosed with rheumatic disease.

Can laboratory findings predict pulmonary involvement in children with COVID-19 infection?

BackgroundStudies investigating clinical and imaging findings of coronavirus disease 2019 (COVID‐19) pneumonia and predictors for lung injury mostly focus on adults. In this study, we aimed to evaluate the role of laboratory findings in predicting lung involvement in children with COVID‐19.MethodsChildren with COVID‐19 confirmed by reverse‐transcription polymerase chain reaction or COVID‐19 IgM and who underwent chest computed tomography (CT) scans were reviewed retrospectively. Admission absolute neutrophil count (ANC), absolute lymphocyte count (ALC), ANC/ALC ratio, platelet count, d‐dimer, fibrinogen, ferritin, procalcitonin, C‐reactive protein (CRP), and lactate dehydrogenase levels were compared in patients with normal and abnormal CT scans.ResultsA total of 101 children were included. Among the patients, 68 (67.3%) had normal CT scans, and 33 (32.7%) had pulmonary involvement. The median CRP, ferritin, and fibrinogen levels were significantly higher in children with abnormal CT findings. The model of binary logistic regression based on the presence of cough, shortness of breath, fibrinogen, ferritin, and CRP levels showed that the possibility of having abnormal CT was 1.021 times more likely for every one unit increase in fibrinogen levels.ConclusionFibrinogen might be useful to predict pulmonary involvement of COVID‐19 in children. Restricting radiological imaging to patients with significant symptoms and high fibrinogen levels might be helpful in children with COVID‐19 infections.

Lung ultrasound in outpatient approach to children with suspected COVID 19

BackgroundChildren with COVID 19 infection (CV19) generally have a mild disease whose main symptoms are fever and cough. Dyspnoea and hypoxemia are rarely reported and few data are available on the frequency and extent of lung involvement in children with CV19. In addition, due to the limited availability of diagnostic tests in Italy during the pandemic period and the relative reliability of the test results, the diagnostic suspicion of CV19 infection in most of the children was difficult to confirm. The aim of this study is to evaluate if lung ultrasound (LUS) was able to highlight typical interstitial lung lesions in children with persistent cough and suspected CV19, providing corroborating evidence of CV19 infection.MethodsWe retrospectively analysed the data of 32 children who came consecutively to our outpatient observation in the period between March 1st and April 30th, 2020 because of the presence of persistent cough for at least 3 days and with suspected CV19. All the children undergone clinical examination, oximetry measurement and LUS.ResultsTwenty over thirty-two children had US lesions compatible with the presence of CV19, many of them without clinical signs of respiratory distress. LUS is much more sensitive than clinical examination to detect lung injury in children with suspected CV19.ConclusionIn the absence of reliable, highly sensitive diagnostic tests or when nasal swab is unworkable or there are too many requests to be performed quickly due to the pandemic, LUS should be used in children with persistent cough for whom a CV19 is suspected because it can highlight undiagnosed interstitial lung lesions and reinforce the diagnostic suspicion of CV19 This approach can be very useful in outpatient settings and in areas with limited medical resources such as developing countries.

Clinical Features and Temporal Changes of RT-PCR and Chest CT in COVID-19 Pediatric Patients.

Objective: This work aims to investigate the clinical features and the temporal changes of RT-PCR and CT in COVID-19 pediatric patients.Methods: The clinical, RT-PCR, and CT features of 114 COVID-19 pediatric in-patients were retrospectively reviewed from January 21 to March 14, 2020. All patients had chest CT on admission and were identified as positive by pharyngeal swab nucleic acid test. The clinical features were analyzed, as well as the features and the temporal changes of RT-PCR and CT.Results: Fever (62, 54%) and cough (61, 54%) were the most common symptoms. There were 34 (30%) cases of concurrent infections. The most common imaging features on CT were ground-glass opacities (46, 40%) and consolidation (46, 40%). The bilateral lower lobes were the most common pattern of involvement, with 63 cases (55%) involving one to two lobes, and in 32 (28%) cases CT was normal. Throughout the whole duration of COVID-19 in children, the diagnostic positive rate of RT-PCR has been far higher than that of CT (all P < 0.05). For RT-PCR follow-up, reliable negative results were obtained only 7 days after the onset of symptoms. Though lung involvement on chest CT progressed rapidly in several cases, lung involvement in children with COVID-19 is mild, with a median value of 2 on CT score.Conclusions: RT-PCR is more reliable than CT in the initial diagnosis of pediatric patients with COVID-19. On follow-up, reliable negative RT-PCR results are available 7 days after the initial symptoms. The use of CT should be considered for follow-up purposes only if necessary.

Lung Involvement in Children with Hereditary Autoinflammatory Disorders.

Short-lived systemic inflammatory reactions arising from disrupted rules in the innate immune system are the operating platforms of hereditary autoinflammatory disorders (HAIDs). Multiple organs may be involved and aseptic inflammation leading to disease-specific phenotypes defines most HAIDs. Lungs are infrequently involved in children with HAIDs: the most common pulmonary manifestation is pleuritis in familial Mediterranean fever (FMF) and tumor necrosis factor receptor-associated periodic syndrome (TRAPS), respectively caused by mutations in the MEFV and TNFRSF1A genes, while interstitial lung disease can be observed in STING-associated vasculopathy with onset in infancy (SAVI), caused by mutations in the TMEM173 gene. The specific pleuropulmonary diseases may range from sub-clinical abnormalities during inflammatory flares of FMF and TRAPS to a severe life-threatening disorder in children with SAVI.

Lung involvement in children with lysinuric protein intolerance.

Lysinuric protein intolerance (LPI) is a rare multisystemic metabolic disease. The objective of the study was to describe presentation and course of lung involvement in a cohort of ten children. Retrospective review of patients followed at Necker-Enfants Malades University Hospital between 1980 and 2012 for a LPI. In patients with lung involvement, clinical data, chest radiographs, pulmonary function tests, bronchoalveolar lavages, and lung biopsies were analyzed. The first and last high-resolution computed tomography (HRCT) were also reviewed. Lung involvement was observed in ten of 14 patients (71 %). Five patients had an acute onset of respiratory symptoms, three had a progressive onset and two were free of symptoms. During the period studied, six patients (60 %) died, all in a context of respiratory failure. Clinical presentation and course were highly variable, even in the same family. HRCT were performed in seven cases, showing in all cases an interstitial pattern and fibrosis in four. All ten patients had pulmonary alveolar proteinosis (PAP) confirmed by histopathological analysis. Five patients had pulmonary fibrosis (at biopsy and/or HRCT scan). Two patients underwent whole lung lavages, without efficiency. PAP is a constant feature in children with LPI and lung involvement. Pulmonary fibrosis is frequent and these two pathologies may develop independently. This study shows the heterogeneity of presentation and outcome. Lung injury could be secondary to impaired phagocytic function and abnormal inflammatory and immune responses intrinsic to the SLC7A7 mutant phenotype. HRCT is recommended to detect lung involvement.

The lung is involved in juvenile dermatomyositis

Juvenile dermatomyositis (JDM) is the main cause of chronic idiopathic inflammatory myopathy of autoimmune origin in children. The aim of this multicenter prospective study was to describe respiratory status and treatment of children followed for JDM. Clinical manifestations, pulmonary function tests (PFT), chest high-resolution computed tomography (HRCT) scan results, and treatments and their adverse effects were analyzed in children followed for JDM. Twenty-one patients (median age: 9.9 years; range: 20 months-18 years) were included. The median of disease duration at the time of the analysis was 3 years (range: 6 months-9 years 4 months). Overall 16 (76%) of 21 children presented with a respiratory involvement related to JDM including interstitial lung disease (n = 3) and/or respiratory muscle involvement (n = 7). Seven patients presented with other nonspecific manifestations. Three children had aspiration pneumonia. A chest HRCT was performed in 15 children, and abnormalities were observed in 12. PFT were performed in 20 of 21 patients. Seven showed functional abnormalities: restrictive ventilatory defect (n = 3) or obstructive ventilatory defect (n = 4). Six patients had abnormal respiratory muscle tests, including three with a restrictive ventilatory defect and one with an obstructive ventilatory defect. One other child with an acute aspiration pneumonia had a clearly muscle respiratory involvement but was too young to perform respiratory muscle tests and confirm this diagnosis. Treatment comprised systemic corticosteroid for all patients and adjuvant immunosuppressive therapy for 11. Adverse effects linked to treatment were reported in eight patients. The frequency of lung involvement in children with JDM justifies systematic respiratory assessment with PFT including measures of respiratory muscle strength. We suggest that a chest HRCT scan is indicated in cases of respiratory symptoms and/or PFT abnormalities. Longitudinal studies are needed to assess pediatric characteristics, long-term outcomes, and responses to treatment taking into account the risk-benefit ratio.

Pulmonary and hepatic nodular lesions precede the diagnosis of Crohn’s disease in an 8‐year‐old girl: a case study and review of the literature

To describe a novel clinical observation and to provide a review the literature about lung involvement in children with Crohn disease (CD). An 8-year-old girl presented with pulmonary and hepatic granulomatous lesions, followed 6 months later by abdominal and general symptoms, resulting in the diagnosis of CD. Differential diagnosis between CD and sarcoidosis and a review of the literature about lung lesions in children with CD are discussed. Crohn disease can be associated with pulmonary lesions, developing before, at the same time or after occurrence of digestive symptoms. This report highlights not only the importance of looking for lung lesions in CD but also underscores the necessity of considering the diagnosis of CD in incomplete presentation evoking sarcoidosis.

Pulmonary manifestations in Proteus syndrome: Pulmonary varicosities and bullous lung disease

We report on two patients with Proteus syndrome (PS), with emphasis on its pulmonary manifestations. The first patient was a 6-year-old girl diagnosed with PS at 5 years of age. The pulmonary abnormalities first observed at age 3 years and included streaky densities with accentuated vascular markings detected by chest radiography. The patient had persistent abnormalities on follow-up chest radiographs. Chest computed tomography (CT) scans showed diffuse pulmonary venous dilatations. The second patient was a 10-year-old boy diagnosed with PS at age 4 years. Chest radiography and CT scans showed patchy and streaky densities intermixed with small bullae, which were interpreted as pneumonia with post-inflammatory pneumatoceles. The patient developed diffuse enlargement of air spaces of the lungs at age 10 years with severe respiratory compromise. Although pulmonary manifestations in PS are uncommon, recognition of pulmonary vein malformation and the presentation of enlarged air spaces in the lungs at an earlier age are important for accurate diagnosis. The plain radiograph findings of accentuated vascular markings seen in patients with PS may appear similar to interstitial or chronic pneumonia. This report emphasizes the features of lung involvement in children with PS and suggests that specific attention be paid to pulmonary manifestations using chest CT scans. © 2011 Wiley-Liss, Inc.

Pediatric Uveitis Secondary to Probable, Presumed, and Biopsy-Proven Sarcoidosis

To describe pediatric patients with uveitis diagnosed as having sarcoidosis. Medical records of pediatric patients evaluated between 1987 and 2008 were reviewed to identify those with ocular inflammation in whom a diagnosis of sarcoidosis was considered. A classification system including ocular findings and results of laboratory testing was devised and used to classify likelihood of sarcoidosis. Four hundred sixty children younger than 17 years were evaluated. Based on the classification system designed, 13 patients (2.8%) had probable, presumed, or definite sarcoidosis. The mean age was 11.6 years (range: 5 to 16 years). Elevated angiotensin-converting enzyme was measured in 6 patients and lysozyme in 5 patients. Five of 12 patients in whom chest imaging was performed had signs of sarcoidosis. Anterior segment involvement was non-granulomatous more often than granulomatous. Seven patients had multifocal choroiditis and 4 patients had retinal periphlebitis. Ocular sarcoidosis is uncommon in children, even at a tertiary referral center. Pulmonary involvement was detected in slightly less than half of the patients who had imaging, in contrast to previous reports of almost universal lung involvement in children 8 to 15 years old. The classification system of presumed, probable, and definite sarcoidosis presented may be useful in clinical practice.

Lung disease in niemann–pick disease

Lung involvement in children with Niemann-Pick disease has rarely been studied systematically. To assess the involvement of the lung and the value of bronchoalveolar lavage in children with Niemann-Pick diseases. Retrospective analysis of patient records. Thirteen patients, with type A (n = 1), type B (n = 10), and type C (n = 2) Niemann-Pick disease, aged 2 months to 9 years at diagnosis, were included in the study. Lung involvement was assessed by clinical evaluation, chest radiograph, lung computed tomography (CT) scan, pulmonary function tests, and bronchoalveolar lavage fluid analysis. Respiratory symptoms were present at diagnosis in 10 patients and developed during follow up in the three other patients. All patients showed signs of interstitial lung disease on chest X-ray and lung CT scan. Bronchoalveolar lavage fluid analysis (n = 7) revealed a marked accumulation of foamy macrophages (Niemann-Pick cells) in all patients. At follow up, one patient died of respiratory failure, five patients required long term oxygen therapy and seven other patients presented a chronic obstructive pulmonary disease (n = 6) or chronic cough (n = 1). Lung disease was observed in all the patients included in the present study. Bronchoalveolar lavage may be useful in Niemann-Pick diseases by showing the presence of characteristic Niemann-Pick cells.

Isolated parenchymal lung involvement in children with stage IV Hodgkin's disease: results of the UKCCSG HD8201 and HD9201 studies.

We retrospectively reviewed the case notes of 27 patients who were diagnosed with stage IV Hodgkin's disease (HD) because of isolated parenchymal pulmonary involvement on chest radiograph and computerized tomography scan (excluding subcategory E). Ten were boys and 15 had B symptoms. Median age at diagnosis was 13.6 years (range 6.1-16.2). All received 6-8 cycles of ChlVPP (chlorambucil, vinblastine, procarbazine and prednisolone) and two had additional whole lung irradiation (12 Gy). Ten patients (37%) relapsed or progressed. Seven survive following second-line therapy while three died, two of HD and one of secondary acute myeloid leukaemia 4 years from diagnosis. At the time of analysis, the median follow-up of patients was 56 months (range 9-127). The event-free survival (EFS) was 58.4% (95% CI 38.5-75.8%) at both 5 and 10 years from diagnosis, and the overall survival (OS) was 84.2% (95% CI 61.8-94.6%) at both 5 and 10 years from diagnosis. We conclude that the outcome for HD patients defined as stage IV, because of isolated parenchymal lung involvement, is encouraging and compares favourably with other extra lymphatic organ involvement. Combination chemotherapy is effective in achieving long-term remission and whole lung irradiation is unnecessary.

High Resolution CT in Children with Cystic Fibrosis

High resolution CT (HRCT) was performed in 24 children (median age 57.9 months) suffering from cystic fibrosis (CF). In 23 patients (one examination unacceptable because of motion artifacts) the most frequent finding was bronchial wall thickening, shown in 21 patients (91%), followed by bronchiectasis in 15 patients (65%). Less frequent findings were mucus plugging and patchy consolidations, which could be demonstrated in 11 patients each (48%). Findings were classified using a CT scoring system and including only irreversible pulmonary changes; a statistically significant correlation with lung function tests (FEV1/FVC; MEF50) could be established. HRCT to date seems to be the most valuable method to determine extent and severity of lung involvement in children with CF and should therefore be routinely used for the staging of this disease.

Clinical utility of non invasive methods in the evaluation of scleroderma lung in pediatric age.

Lung involvement in children affected with Systemic Sclerosis (SSc) has been studied. We used ventilation and perfusion scans and compared the results to those obtained with traditional pulmonary function tests. The vascular pulmonary area was examined with a gama-camera after infusion of 99mTc microspheres, while the alveolo-capillary membrane and the airways were investigated after administration of a radioaerosol, (99mTc DTPA). Pulmonary function tests revealed either alteration of the airways or a considerable involvement of the alveolo-capillary membrane in two patients. On the other hand ventilation scan showed airways alterations and a severe decrease of the alveolocapillary permeability in all patients. Our study indicates that ventilation scans may be useful in the diagnosis of early modifications of the lung function also in paediatric age and may complement the data obtained from traditional function tests.

Hydatid disease in childhood

Five children with hydatid disease are reported. The varied symptomatology and inconsistent laboratory data in the cases illustrates the difficulties in establishing the diagnosis. The frequency of lung involvement in children is stressed and outlines of management given.