scholarly journals Children’s Interstitial and Diffuse Lung Diseases (ChILD) in 2020

Children ◽  
2020 ◽  
Vol 7 (12) ◽  
pp. 280
Author(s):  
Valentina Agnese Ferraro ◽  
Stefania Zanconato ◽  
Andrea Zamunaro ◽  
Silvia Carraro
Keyword(s):  
Lung Diseases ◽  
Developmental Disorders ◽  
Respiratory Infections ◽  
Clinical Manifestations ◽  
Signs And Symptoms ◽  
Surfactant Protein ◽  
Exercise Intolerance ◽  
Patient Treatment ◽  
High Resolution Computed Tomography ◽  
Cell Hyperplasia

The term children interstitial lung diseases (chILD) refers to a heterogeneous group of rare diseases that diffusely affect the lung. ChILD specific to children younger than 2 years of age include diffuse developmental disorders, growth abnormalities, specific conditions of undefined etiology (neuroendocrine cell hyperplasia of infancy and pulmonary interstitial glycogenosis) and surfactant protein disorders. Clinical manifestations are highly variable, ranging from the absence of relevant symptoms to a severe onset. Most commonly, chILD presents with nonspecific respiratory signs and symptoms, such as dyspnea, polypnea, dry cough, wheezing, recurrent respiratory infections and exercise intolerance. In the diagnostic approach to a child with suspected ILD, chest high resolution computed tomography and genetic tests play a central role. Then, if the diagnosis remains uncertain, laryngotracheal-bronchoscopy and lung biopsy are needed. Pharmacological treatment is mostly empiric and based on anti-inflammatory and immunomodulatory drugs including corticosteroids, hydroxychloroquine and azithromycin. Despite chILD overall rarity, pediatric pulmonologists must be familiar with these diseases in order to carry out a timely diagnosis and patient treatment.

scholarly journals Differential diagnosis of granulomatous lung disease: clues and pitfalls

2017 ◽  
Vol 26 (145) ◽  
pp. 170012 ◽  
Author(s):  
Shinichiro Ohshimo ◽  
Josune Guzman ◽  
Ulrich Costabel ◽  
Francesco Bonella
Keyword(s):  
Differential Diagnosis ◽  
Lung Diseases ◽  
Granulomatosis With Polyangiitis ◽  
Wide Spectrum ◽  
Clinical Manifestations ◽  
Needle Aspiration ◽  
Endobronchial Ultrasound ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
High Resolution Computed Tomography ◽  
Transbronchial Needle Aspiration

Granulomatous lung diseases are a heterogeneous group of disorders that have a wide spectrum of pathologies with variable clinical manifestations and outcomes. Precise clinical evaluation, laboratory testing, pulmonary function testing, radiological imaging including high-resolution computed tomography and often histopathological assessment contribute to make a confident diagnosis of granulomatous lung diseases. Differential diagnosis is challenging, and includes both infectious (mycobacteria and fungi) and noninfectious lung diseases (sarcoidosis, necrotising sarcoid granulomatosis, hypersensitivity pneumonitis, hot tub lung, berylliosis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, rheumatoid nodules, talc granulomatosis, Langerhans cell histiocytosis and bronchocentric granulomatosis). Bronchoalveolar lavage, endobronchial ultrasound-guided transbronchial needle aspiration, transbronchial cryobiopsy, positron emission tomography and genetic evaluation are potential candidates to improve the diagnostic accuracy for granulomatous lung diseases. As granuloma alone is a nonspecific histopathological finding, the multidisciplinary approach is important for a confident diagnosis.

scholarly journals CHILDREN IN CORONAVIRUSES’ WONDERLAND: WHAT CLINICIANS NEED TO KNOW

2020 ◽  
Vol 12 (1) ◽  
pp. e2020042
Author(s):  
Giuseppe Lassandro ◽  
Valentina  Palladino ◽  
Anna Amoruso ◽  
Viviana Palmieri ◽  
Giovanna Russo ◽  
...  
Keyword(s):  
Respiratory Infections ◽  
Multiorgan Failure ◽  
Clinical Manifestations ◽  
Severe Pneumonia ◽  
Signs And Symptoms ◽  
Upper Respiratory Tract ◽  
Respiratory Tract Illnesses ◽  
Life Threatening ◽  
Human Coronaviruses ◽  
Coronavirus Infections

Human coronaviruses (HCoVs) commonly cause mild upper-respiratory tract illnesses but can lead to more severe and diffusive diseases. A variety of signs and symptoms may be present, and infections can range in severity from common cold and sore throat to more serious laryngeal or tracheal infections, bronchitis, and pneumonia. Among the seven coronaviruses that affect humans, (SARS)-CoV, the Middle East respiratory syndrome (MERS)-CoV and the most recent coronavirus disease 2019 (COVID-19) represent potential life-threatening diseases worldwide. In adults they may cause severe pneumonia that evolve in distress respiratory syndrome and multiorgan failure with a high mortality rate. Children appear to be less susceptible to develop severe clinical disease and present usually with mild and aspecific symptoms similar to other respiratory infections typical of childhood. However, some children such as infants, adolescents or those with underlying diseases may be more at-risk categories and require greater caution from clinicians. Available data on pediatric coronavirus infections are rare and scattered in the literature. The purpose of this review is to provide to clinicians a complete and updated panel useful to recognize and characterize the broad spectrum of clinical manifestations of coronavirus infections in the pediatric age.

scholarly journals New coronavirus infection in children in the Moscow region: clinical, epidemiological and treatment aspects

2020 ◽  
Vol 49 ◽  
Author(s):  
E. R. Meskina ◽  
M. K. Khadisova ◽  
T. V. Stashko ◽  
A. V. Bitsueva
Keyword(s):  
Pediatric Patients ◽  
Respiratory Infections ◽  
Severe Disease ◽  
Age Distribution ◽  
Clinical Manifestations ◽  
Treatment Strategies ◽  
Signs And Symptoms ◽  
Generation Cephalosporin ◽  
Moscow Region ◽  
Epidemiological Characteristics

Aim: To assess clinical and epidemiological characteristics, as well as treatment results in COVID-19 pediatric patients hospitalized to the Department of infectious disease in the Moscow region.Materials and methods: We retrospectively analyzed medical records of 124 pediatric patients aged from 1 month to 17 years with confirmed (63.7%) and highly probable (proven epidemiological association with the infection source) COVID-19 infection. The children were hospitalized from March 25, 2020 to August 11, 2020. Among patients that were hospitalized during this time period, 43% had pneumonia and 30% had acute respiratory disorders. All cases of pneumonia were confirmed by computed tomography. The children were treated according to the standard Russian guidelines.Results: The children had been infected with SARS-CoV-2 mostly from family members (75%; 95% confidence interval (CI) 66.4–82.3). In the families of the children with pneumonia, the number of pneumonia cases in their relatives was higher than in the families of the children with acute respiratory infections (1.7±1.0 vs. 1.1±0.5 respectively; р<0.001). The mean age of the hospitalized children was 8 years (7.4–9.6), with an even age distribution. The main COVID-19 signs and symptoms were as follows: fever (75.8%; 95% CI 67.3–83.0), cough (66.1%; 95% CI 57.1–74.4]), fatigues (38.7%; 95% CI 30.1–47.9), and hyposmia (33.9%; 95% CI 25.6–42.9). The severe disease course was rare (2.4%; 95% CI 0.5–6.9). The rates of positive reverse transcriptase polymerase chain reaction tests for SARS-CoV-2 were rapidly decreased at repeated tests: at day 3, 63.7% of the tests were positive, at day 7, 21.8% and at day 14, 5.6% (р<0.001). The prevalence of pneumonia was 56.4% without any age differences, with mostly minimal areas of lung abnormalities (78.6%). The severity of pneumonia and duration of clinical manifestations in the patients treated with a III generation cephalosporin or its combination with a macrolide were similar. Cough duration in those, who were treated with ipratropium bromide /fenoterol inhalations including their combination with budesonide, was higher than in those who did not use inhalation treatment. Pyrexia of>5 days duration can be a predictor of pneumonia in a  pediatric patient with COVID-19 (odds ratio 4.55 (2.1–9.9), sensitivity 61.4%, specificity 74.1%).Conclusion: The results obtained are important to develop further treatment strategies for children with COVID-19.

scholarly journals Ataxia telangiectasia: A diagnostic challenge. Case report

Case reports ◽  
2020 ◽  
Vol 6 (2) ◽  
pp. 109-117
Author(s):  
Natalia Martínez-Córdoba ◽  
Eugenia Espinosa-García
Keyword(s):  
Ataxia Telangiectasia ◽  
Respiratory Infections ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
Autosomal Recessive Disorder ◽  
Signs And Symptoms ◽  
Interdisciplinary Treatment ◽  
Diagnostic Challenge ◽  
Progressive Cerebellar Ataxia ◽  
Repair Mechanisms

Introduction: Ataxia-telangiectasia (AT) is a neurodegenerative syndrome with low incidence and prevalence worldwide, which is caused by a mutation of the ATM gene. It is an autosomal recessive disorder that is associated with defective cell regeneration and DNA repair mechanisms. It is characterized by progressive cerebellar ataxia, abnormal eye movements, oculocutaneous telangiectasias and immunodeficiency. Early diagnosis is critical to initiate a timely interdisciplinary treatment, improve acute symptoms, and control the multiple comorbidities of the disease. The following is the case of a patient who presented with the aforementioned characteristics and had an adequate response to the established medical treatment.Case presentation: A 7-year-old female patient from Bogotá, who presented clinical signs of global neurodevelopmental delay, cerebelar ataxia, frequent respiratory infections and ocular telangiectasias. Symptoms were associated with elevation of alpha fetoprotein and immunodeficiency, which allowed for a diagnosis of AT and the initiation of a timely interdisciplinary treatment.Conclusion: AT is a chromosomal instability syndrome with characteristic signs and symptoms. It is essential to know the etiopathogenesis, clinical manifestations, diagnostic criteria, and therapeutic options, emphasizing that early detection and clinical suspicion could favor the proper management of the comorbidities and improve the progressive course of the disease.

scholarly journals Which triggers could support timely identification of primary antibody deficiency? A qualitative study using the patient perspective

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Lisanne M. A. Janssen ◽  
Kim van den Akker ◽  
Mohamed A. Boussihmad ◽  
Esther de Vries
Keyword(s):  
Respiratory Infections ◽  
Diagnostic Delay ◽  
Signs And Symptoms ◽  
Negative Influence ◽  
Primary Antibody ◽  
Emotional Impact ◽  
Structured Interviews ◽  
Timely Treatment ◽  
The Way

Abstract Background Patients with predominantly (primary) antibody deficiencies (PADs) commonly develop recurrent respiratory infections which can lead to bronchiectasis, long-term morbidity and increased mortality. Recognizing symptoms and making a diagnosis is vital to enable timely treatment. Studies on disease presentation have mainly been conducted using medical files rather than direct contact with PAD patients. Our study aims to analyze how patients appraised their symptoms and which factors were involved in a decision to seek medical care. Methods 14 PAD-patients (11 women; median 44, range 16-68 years) were analyzed using semi-structured interviews until saturation of key emergent themes was achieved. Results Being always ill featured in all participant stories. Often from childhood onwards periods of illness were felt to be too numerous, too bad, too long-lasting, or antibiotics were always needed to get better. Recurrent or persistent respiratory infections were the main triggers for patients to seek care. All participants developed an extreme fatigue, described as a feeling of physical and mental exhaustion and thus an extreme burden on daily life that was not solved by taking rest. Despite this, participants tended to normalize their symptoms and carry on with usual activities. Non-immunologists, as well as patients, misattributed the presenting signs and symptoms to common, self-limiting illnesses or other ‘innocent’ explanations. Participants in a way understood the long diagnostic delay. They know that the disease is rare and that doctors have to cover a broad medical area. But they were more critical about the way the doctors communicate with them. They feel that doctors often don’t listen very well to their patients. The participants’ symptoms as well as the interpretation of these symptoms by their social environment and doctors had a major emotional impact on the participants and a negative influence on their future perspectives. Conclusions To timely identify PAD, ‘pattern recognition’ should not only focus on the medical ‘red flags’, but also on less differentiating symptoms, such as ‘being always ill’ and ‘worn out’ and the way patients cope with these problems. And, most important, making time to really listen to the patient remains the key.

scholarly journals Regulatory Effects of Nur77 on Airway Remodeling and ASMC Proliferation in House Dust Mite-Induced Asthma

2020 ◽  
Vol 2020 ◽  
pp. 1-14
Author(s):  
Kun Wang ◽  
Muyun Wang ◽  
Yan Shang ◽  
Yanan He ◽  
Qiang Li ◽  
...  
Keyword(s):  
Chronic Inflammation ◽  
Lung Diseases ◽  
Airway Remodeling ◽  
Vital Role ◽  
Cell Hyperplasia ◽  
Asthma Model ◽  
Dust Mite ◽  
Asthma Patients ◽  
Antioxidant Gene Expression ◽  
Regulatory Effects

Airway remodeling played a vital role in the development of asthma, and airway smooth muscle (ASM) mass was its hallmark. However, few strategies targeting ASM remodeling were developed in treating asthma. Nur77 was the transcription factor nuclear receptor involved in the pathogenesis of several lung diseases. Nur77 distribution and expression were determined in an HDM-mediated allergic asthma model. Its effect on airway hyperresponsiveness (AHR), chronic inflammation, and ASM remodeling in asthmatic mice was evaluated using a lentivirus-mediated shRNA. Possible mechanisms were explored by examining Nur77 actions and its underlying pathways in primary human AMC cells (ASMCs). In this study, we reported that Nur77 expression was mainly distributed along ASM and increased in lungs of HDM-challenged mice. Nur77 depletion by lentivirus-mediated shRNA ameliorated AHR, chronic inflammation, goblet cell hyperplasia, and airway remodeling in the asthmatic mouse model. By means of primary human ASMC, we discovered that Nur77 upregulation by HDM stimulation promoted cell proliferation and ROS production, as well as reduced antioxidant gene expression. These alterations might associate with MFN2/MAPK/AKT pathways. These findings broadened our understanding of airway remodeling and ASMC proliferation, which might provide a novel therapeutic target for asthma patients.

scholarly journals The Role of Transthoracic Ultrasound in the Study of Interstitial Lung Diseases: High-Resolution Computed Tomography Versus Ultrasound Patterns: Our Preliminary Experience

Diagnostics ◽  
2021 ◽  
Vol 11 (3) ◽  
pp. 439
Author(s):  
Donato Lacedonia ◽  
Giulia Scioscia ◽  
Angelamaria Giardinelli ◽  
Carla Maria Irene Quarato ◽  
Ennio Vincenzo Sassani ◽  
...  
Keyword(s):  
Computed Tomography ◽  
High Resolution ◽  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
High Resolution Computed Tomography ◽  
Pleural Line ◽  
Complementary Role ◽  
Number Of Patients ◽  
Significant Difference ◽  
Transthoracic Ultrasound

Transthoracic ultrasound (TUS) is a readily available imaging tool that can provide a quick real-time evaluation. The aim of this preliminary study was to establish a complementary role for this imaging method in the approach of interstitial lung diseases (ILDs). TUS examination was performed in 43 consecutive patients with pulmonary fibrosis and TUS findings were compared with the corresponding high-resolution computed tomography (HRCT) scans. All patients showed a thickened hyperechoic pleural line, despite no difference between dominant HRCT patterns (ground glass, honeycombing, mixed pattern) being recorded (p > 0.05). However, pleural lines’ thickening showed a significant difference between different HRCT degree of fibrosis (p < 0.001) and a negative correlation with functional parameters. The presence of >3 B-lines and subpleural nodules was also assessed in a large number of patients, although they did not demonstrate any particular association with a specific HRCT finding or fibrotic degree. Results allow us to suggest a complementary role for TUS in facilitating an early diagnosis of ILD or helping to detect a possible disease progression or eventual complications during routine clinical practice (with pleural line measurements and subpleural nodules), although HRCT remains the gold standard in the definition of ILD pattern, disease extent and follow-up.

scholarly journals Clinical Features and Maternal-fetal Results of Pregnant Women in COVID-19 Times

2021 ◽  
Vol 43 (05) ◽  
pp. 384-394
Author(s):  
Ana Paula Nogueira Godoi ◽  
Gilcelia Correia Santos Bernardes ◽  
Leilismara Sousa Nogueira ◽  
Patrícia Nessralla Alpoim ◽  
Melina de Barros Pinheiro
Keyword(s):  
Pregnant Women ◽  
Vertical Transmission ◽  
Postpartum Period ◽  
Scientific Literature ◽  
Respiratory Infections ◽  
Clinical Manifestations ◽  
Fetal Distress ◽  
Medical Subject Headings ◽  
Substantial Evidence ◽  
The Subject

Abstract Objective Coronavirus disease 2019 (COVID-19) is a disease caused by a newly discovered coronavirus, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), which usually leads to non-specific respiratory symptoms. Although pregnant women are considered at risk for respiratory infections by other viruses, such as SARS and Middle East respiratory syndrome (MERS), little is known about their vulnerability to SARS-CoV-2. Therefore, this study aims to identify and present the main studies on the topic, including the postpartum period. Methods In this narrative review, articles were searched in various databases, organizations, and health entities using keywords compatible with medical subject headings (MeSH), such as: COVID-19, pregnancy, vertical transmission, coronavirus 2019, and SARS-CoV-2. Results The review of the scientific literature on the subject revealed that pregnant women with COVID-19 did not present clinical manifestations significantly different from those of non-pregnant women; however, there are contraindicated therapies. Regarding fetuses, studies were identified that reported that infection by SARS-CoV-2 in pregnant women can cause fetal distress, breathing difficulties and premature birth, but there is no substantial evidence of vertical transmission. Conclusion Due to the lack of adequate information and the limitations of the analyzed studies, it is necessary to provide detailed clinical data on pregnant women infected with SARS-CoV-2 and on the maternal-fetal repercussions caused by this infection. Thus, this review may contribute to expand the knowledge of professionals working in the area as well as to guide more advanced studies on the risk related to pregnant women and their newborns. Meanwhile, monitoring of confirmed or suspected pregnant women with COVID-19 is essential, including in the postpartum period.

Thoracoabdominal asynchrony associates with exercise intolerance in fibrotic interstitial lung diseases

Respirology ◽  
2021 ◽  
Author(s):  
Pauliane Vieira Santana ◽  
Leticia Zumpano Cardenas ◽  
Jeferson George Ferreira ◽  
Carlos Roberto Ribeiro Carvalho ◽  
André Luis Pereira Albuquerque ◽  
...  
Keyword(s):  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Exercise Intolerance ◽  
Thoracoabdominal Asynchrony

Development and evaluation of a computer-based decision support system for diffuse lung diseases at high-resolution computed tomography

2021 ◽  
pp. 028418512199579
Author(s):  
Simon S Martin ◽  
Delina Kolaneci ◽  
Julian L Wichmann ◽  
Lukas Lenga ◽  
Doris Leithner ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Decision Support ◽  
High Resolution ◽  
Lung Diseases ◽  
Differential Diagnoses ◽  
High Resolution Computed Tomography ◽  
Computer Based ◽  
Diffuse Lung Diseases ◽  
Hrct Patterns ◽  
Diffuse Lung

Background High-resolution computed tomography (HRCT) is essential in narrowing the possible differential diagnoses of diffuse and interstitial lung diseases. Purpose To investigate the value of a novel computer-based decision support system (CDSS) for facilitating diagnosis of diffuse lung diseases at HRCT. Material and Methods A CDSS was developed that includes about 100 different illustrations of the most common HRCT signs and patterns and describes the corresponding pathologies in detail. The logical set-up of the software facilitates a structured evaluation. By selecting one or more CT patterns, the program generates a ranked list of the most likely differential diagnoses. Three independent and blinded radiology residents initially evaluated 40 cases with different lung diseases alone; after at least 12 weeks, observers re-evaluated all cases using the CDSS. Results In 40 patients, a total of 113 HRCT patterns were evaluated. The percentage of correctly classified patterns was higher with CDSS (96.8%) compared to assessment without CDSS (90.3%; P < 0.01). Moreover, the percentage of correct diagnosis (81.7% vs. 64.2%) and differential diagnoses (89.2% vs. 38.3%) were superior with CDSS compared to evaluation without CDSS (both P < 0.01). Conclusion Addition of a CDSS using a structured approach providing explanations of typical HRCT patterns and graphical illustrations significantly improved the performance of trainees in characterizing and correctly identifying diffuse lung diseases.

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