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https://www.readbyqxmd.com/read/28814498/both-ways-at-once-keeping-small-airways-clean
#1
REVIEW
Paul M Quinton
The small airways of the lungs are under constant assault from the pathogens and debris in the air that they must conduct to alveoli. Although hygiene is of paramount importance for respiratory health, the underlying principles of airway clearance have not been well integrated or established. Newly emerging concepts of simultaneous absorption and secretion of airway surface liquid (ASL) and the role of [Formula: see text] in the maturation of mucins have advanced from experimental evidence as well as observations from the congenital disease cystic fibrosis (CF) to present a novel model that integrates microanatomy with organ physiology to meet the constant challenge of cleaning small airways...
September 2017: Physiology
https://www.readbyqxmd.com/read/28812458/congenital-cystic-lung-lesions-evolution-from-in-utero-detection-to-pathology-diagnosis-a-multidisciplinary-approach
#2
Steven Hardee, Lea Tuzovic, Cicero T Silva, Robert A Cowles, Joshua Copel, Raffaella A Morotti
Congenital cystic lung lesions are a group of rare pathologies that are usually diagnosed in the prenatal period. The majority of these lesions are diagnosed at pathology examination as congenital pulmonary airway malformations (CPAM) and bronchopulmonary sequestration (BPS). These lesions are typically managed by surgical intervention within the first year of life and have an excellent prognosis. We examined the evolution of imaging appearances from prenatal diagnosis to postnatal work-up of these lesions and correlate imaging and pathological findings...
September 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28774324/congenital-cystic-adenomatoid-malformation-dangers-of-misdiagnosis-a-case-report
#3
Wafae El Amraoui, Aziza Bentalha, Hajar Hamri, Salma Es-Chrif El Kettani, Alae El Koraichi
BACKGROUND: Congenital cystic adenomatoid malformation is a rare pulmonary malformation, but is the most common lung malformation observed in children. In developing countries, such as Morocco, prenatal diagnosis is missing. Congenital cystic adenomatoid malformation may occur after birth in the presence of complications and needs a computed tomography scan for confirmation. However, our lack of awareness of this malformation has been responsible for a late and wrong diagnosis along with therapeutic errors...
August 4, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28757838/anesthetic-management-for-lobectomy-of-a-2-month-old-infant-with-bronchogenic-cyst-case-report-along-with-review-of-literature
#4
T Bansal, S Kiran, K Kamal, N Bangarwa
Bronchogenic cyst, a benign congenital cystic lesion of the lung, is a rare cause of respiratory distress in children comprising 7.5% of all mediastinal masses. A thorough preoperative evaluation is crucial to plan for definitive intra- and post-operative management. All patients should be thoroughly evaluated for the presence of compression, deviation or distortion of airways and great veins. The easiest means of providing one lung ventilation in pediatrics is to intubate the main stem bronchus of the nonoperated lung...
July 2017: Saudi Journal of Anaesthesia
https://www.readbyqxmd.com/read/28739257/lack-of-epithelial-ppar%C3%AE-causes-cystic-adenomatoid-malformations-in-mouse-fetal-lung
#5
Jung-Hwan Kim, Satoshi Yamaori, Tomotaka Tanabe, Mitsuhiro Takagi, Tsutomu Matsubara, Minoru Okamoto, Shioko Kimura, Frank J Gonzalez
Peroxisome proliferator-activated receptor-γ (PPARγ) plays an important role in lipid and glucose metabolism. In this study, the function of PPARγ on lung development was investigated. Lung-specific Pparg conditional knockout mice (Pparg(ΔLuEpC)) were developed using Cre-Lox system. Pparg(ΔLuEpC) mice showed abnormal lung development with enlarged airspaces and followed by increase of apoptotic cells at E14.5 to E18.5. Gene analysis revealed that expression of Pmaip1, a gene related to apoptosis, was significantly increased while expression of Retnla, a gene related to anti-apoptosis, was dramatically decreased in the fetal lung (E14...
July 21, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28649564/primary-ciliary-dyskinesia-an-update-on-clinical-aspects-genetics-diagnosis-and-future-treatment-strategies
#6
REVIEW
Virginia Mirra, Claudius Werner, Francesca Santamaria
Primary ciliary dyskinesia (PCD) is an orphan disease (MIM 244400), autosomal recessive inherited, characterized by motile ciliary dysfunction. The estimated prevalence of PCD is 1:10,000 to 1:20,000 live-born children, but true prevalence could be even higher. PCD is characterized by chronic upper and lower respiratory tract disease, infertility/ectopic pregnancy, and situs anomalies, that occur in ≈50% of PCD patients (Kartagener syndrome), and these may be associated with congenital heart abnormalities...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28631156/diagnosis-of-secondary-pulmonary-lymphangiectasia-in-congenital-heart-disease-a-novel-role-for-chest-ultrasound-and-prognostic-implications
#7
Christopher Z Lam, Tanmay Anant Bhamare, Tamadhir Gazzaz, David Manson, Tilman Humpl, Mike Seed
BACKGROUND: Secondary pulmonary lymphangiectasia is a complication of congenital heart disease that results from chronic pulmonary venous obstruction. OBJECTIVES: We aimed to evaluate the performance of chest ultrasound (US) in diagnosing secondary pulmonary lymphangiectasia and to review the clinical course of children with secondary pulmonary lymphangiectasia. MATERIALS AND METHODS: Chest US was performed on 26 children with hypoplastic left heart syndrome, total anomalous pulmonary venous connection or cor triatriatum in a prospective observational study...
June 19, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28607884/rigid-bronchoscopic-placement-of-fogarty-catheter-as-a-bronchial-blocker-for-one-lung-isolation-and-ventilation-in-infants-and-children-undergoing-thoracic-surgery-a-single-institution-experience-of-27-cases
#8
Sunil Kant Kamra, Ashwin Ashok Jaiswal, Amrish Kumar Garg, Manoj Kumar Mohanty
One-lung ventilation (OLV) is a challenging task in infants and children as few techniques are possible because of narrow anatomy. The aim of this study is to evaluate and experience lung isolation with Fogarty catheters as a bronchial blocker placed by rigid bronchoscope for OLV in infants and children with lung pathologies requiring surgical management in an industrial hospital. This study is a prospective study carried out in J.L.N. Hospital and Research Centre, Bhilai (CG), from January 2011 to December 2014...
June 2017: Indian Journal of Otolaryngology and Head and Neck Surgery
https://www.readbyqxmd.com/read/28584070/the-natural-history-of-prenatally-diagnosed-congenital-cystic-lung-lesions-long-term-follow-up-of-119-cases
#9
James Cook, Lyn S Chitty, Paolo De Coppi, Michael Ashworth, Colin Wallis
BACKGROUND: A paucity of evidence regarding the natural history of congenital pulmonary airway malformations (CPAMs) and pulmonary sequestration (PS) has resulted in a divergence in management strategy of asymptomatic cases. METHODS: We describe the long-term clinical course of 119 children diagnosed with these lesions treated at Great Ormond Street Hospital (GOSH). Cases were identified via the GOSH patient database. Study entry required the identification of a cystic lung lesion on prenatal ultrasound and confirmation of CPAM/PS on postnatal CT imaging...
June 5, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28493304/cpam-type-2-derived-mesenchymal-stem-cells-malignancy-risk-study-in-a-14-month-old-boy
#10
Gloria Pelizzo, Maria A Avanzini, Marco Folini, Rossana Bussani, Melissa Mantelli, Stefania Croce, Gloria Acquafredda, Paola Travaglino, Graziella Cimino-Reale, Marina Boni, Irene Dambruoso, Valeria Calcaterra
INTRODUCTION: The association between congenital pulmonary airway malformations (CPAM) and malignancy is reported in the literature. Interactions between the tumor, immune, and mesenchymal stromal/stem cells (MSCs) have been recognized as crucial for understanding tumorigenesis. We characterized MSCs isolated from CPAM lesions in order to define potential malignancy risks. METHODS: CPAM II pulmonary tissue was used for MSC expansion; a "healthy" lung section from the same child was used as a comparator...
August 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28405541/it-s-rare-so-be-aware-pleuropulmonary-blastoma-mimicking-congenital-pulmonary-airway-malformation
#11
Fayza Haider, Khulood Al Saad, Fatima Al-Hashimi, Hakima Al-Hashimi
Pleuropulmonary blastoma (PPB) is a rare aggressive malignant tumor of infancy and early childhood. The tumor arises in the lung and pleura and is regarded as a pulmonary dysontogenetic or embryonic neoplasm. Four types are defined in literature. Type I PPB is a rare, cystic lung neoplasm in infants characterized by subtle malignant changes and a good prognosis. Recurrences after type I PPB are usually advanced with a poor prognosis. We report this case to increase awareness about this entity so that the pediatricians, pediatric surgeons, radiologist, and pathologist recognize it early...
January 2017: Thoracic and Cardiovascular Surgeon Reports
https://www.readbyqxmd.com/read/28391172/large-bronchogenic-cyst-of-stomach-a-case-report
#12
Amine Chhaidar, Houssem Ammar, Nihed Abdessayed, Mohamed Azzaza, Rahul Gupta, Nefis Abdennaceur, Ahlem Bdioui, Moncef Mokni, Ali Ben Ali
INTRODUCTION: Bronchogenic cysts are congenital cysts arising as an abnormal budding from primitive tracheobronchial tree. They are lined by pseudostratified columnar or cuboidal ciliated epithelium and contain smooth muscle fibers, submucosal bronchial glands and/or cartilage. They are most frequently located in the mediastinum or the lung parenchyma. Intramural occurrence of bronchogenic cyst in the gastric wall is very rare. PRESENTATION OF CASE: We present a case of 65-year-old lady with a 7×8cm lesion in the gastric cardia suspicious of gastrointestinal stromal tumor...
2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28337699/lung-ultrasound-is-comparable-with-chest-roentgenogram-for-diagnosis-of-community-acquired-pneumonia-in-hospitalised-children
#13
Krishna Kumar Yadav, Shally Awasthi, Anit Parihar
OBJECTIVE: To evaluate the accuracy of lung ultrasound (LUS) in comparison to chest roentgenogram (CXR) in hospitalised children with community-acquired pneumonia (CAP). METHODS: This study was a hospital based prospective observational study, conducted between January 2014 and December 2014. Hospitalised children aged 2 to 59 mo with community-acquired pneumonia were included in the study. The informed written consent was taken from parents (or legal guardian) before recruitment...
March 24, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28324617/rare-localized-extralobar-sequestration-with-congenital-cystic-adenomatoid-malformation-a-case-report
#14
Satoshi Nagasaka, Satsuki Kina, Yoshihito Arimoto, Fumi Yokote, Tsuyoshi Uchida, Hirochika Matsubara
Extralobar sequestrations constitute a rare form of congenital pulmonary airway malformations that are difficult to diagnose. Here, we report a rare case of a localized extralobar sequestration in the right superior portion of the mediastinum accompanied by congenital cystic adenomatoid malformation.A 19-year-old man presented with a right upper mediastinal mass that was detected using chest radiography, had a history of left spontaneous pneumothorax, and had undergone a bullectomy 4 years previously.The initial diagnosis included a mature teratoma and a bronchogenic cyst in the mediastinum; however, the presence of a cystic mass in the right upper lobe of the lung prompted further examination...
December 2017: Surgical Case Reports
https://www.readbyqxmd.com/read/28204920/a-review-of-congenital-lung-malformations-with-a-simplified-classification-system-for-clinical-and-research-use
#15
REVIEW
Michael Seear, Jennifer Townsend, Amy Hoepker, Douglas Jamieson, Deborah McFadden, Patrick Daigneault, William Glomb
PURPOSE: Congenital lung abnormalities are rare malformations increasingly detected early by prenatal ultrasound. Whether management of these frequently asymptomatic lesions should be surgical or conservative is an unresolved issue. The necessary prospective studies are limited by the absence of a widely accepted practical classification system. Our aim was to develop a simple, clinically relevant system for classifying and studying congenital lung abnormalities. MATERIALS AND METHODS: We based our proposed grouping on a detailed analysis of clinical, radiological, and histological data from well-documented cases, plus an extensive review of the literature...
June 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28202768/respiratory-morbidity-in-infants-born-with-a-congenital-lung-malformation
#16
MULTICENTER STUDY
Celine Delestrain, Naziha Khen-Dunlop, Alice Hadchouel, Pierrick Cros, Héloïse Ducoin, Michael Fayon, Isabelle Gibertini, André Labbé, Géraldine Labouret, Marie-Noëlle Lebras, Guillaume Lezmi, Fouad Madhi, Guillaume Thouvenin, Caroline Thumerelle, Christophe Delacourt
BACKGROUND AND OBJECTIVES: The actual frequency of respiratory symptoms related to congenital pulmonary malformations (CPMs) remains undetermined. The goal of this study was to prospectively evaluate the respiratory symptoms occurring in infants with prenatally diagnosed CPMs, identify factors associated with the occurrence of these symptoms, and evaluate their resolution after surgery. METHODS: Infectious and noninfectious respiratory symptoms were prospectively collected in a French multicenter cohort of children with CPMs...
March 2017: Pediatrics
https://www.readbyqxmd.com/read/28195247/type-ii-congenital-pulmonary-airway-malformation-associated-with-intralobar-pulmonary-sequestration-report-of-a-case-and-review-of-classification-criteria
#17
M G Mastrogiulio, A Barone, M G Disanto, A Ginori, M R Ambrosio, S F Carbone, D Spina
Pulmonary congenital abnormalities are rare disorders including congenital pulmonary airway malformations (CPAM) and pulmonary sequestration (PS). CPAM is a lesion characterized by the presence of anomalous bronchiolar or acinar structures, variable in size, either cystic or not cystic. PS is generally defined as nonfunctioning lung tissue that is not in normal continuity with the tracheobronchial tree and that derives its blood supply from systemic vessels. We describe a case of a baby girl with a very rare association between CPAM type 2 and intralobar pulmonary sequestration (IPS) focusing on the cystic lesions typical of CPAM and on the lymphatic and blood vessels...
March 2016: Pathologica
https://www.readbyqxmd.com/read/28165443/video-assisted-thoracoscopic-lobectomy-for-congenital-cystic-adenomatoid-malformations
#18
Amina Selimović, Edo Hasanbegović, Ermina Mujičić, Selma Milišić, Emir Haxhija, Kenan Karavdić, Alen Pilav
Aim The aim was to show rare cases of congenital cystic adenomatoid malformation (CCAM) and the manner of its surgical treatment with video-assisted thoracoscopic surgery (VATS). Methods Two male and one female child, 7, 4 and 3 years of age were treated for symptoms of cough and high temperature in district hospitals. In all three children laboratory blood tests and chest radiography were done. Auscultatory findings showed the presence of pneumonia. Children were treated with appropriate doses of antibiotics...
February 1, 2017: Medicinski Glasnik
https://www.readbyqxmd.com/read/28144070/perpetual-dilemma-pleural-or-parenchymal-congenital-or-acquired-solitary-cystic-lesion-with-fluid-level
#19
Deepak Talwar, Onkar Jha, Rahul Kumar Sharma, Rajat Saxena
Congenital cystic adenomatoid malformations (CCAMs) are rare congenital, nonhereditary developmental anomalies of the lung with unknown etiology. CCAM is predominantly a disorder of infancy with the majority of the cases being diagnosed within the first 2 years of life. When CCAM presents in adults, it represents a diagnostic dilemma and requires careful evaluation. We here report a case of large solitary congenital pulmonary cystic adenomatoid malformation with infection and hemorrhage, which was diagnosed as encysted hydropneumothorax on computerized tomography scans but turned out to be infected pulmonary cystic adenomatoid malformation after surgical excision...
January 2017: Lung India: Official Organ of Indian Chest Society
https://www.readbyqxmd.com/read/28138192/a-rare-cause-of-recurrent-chest-infection-in-children-bronchopulmonary-sequestration
#20
Shabbir Hussain, Shoaib Ahmed, Syed Awais Ul Hassan Shah, Saba Haider Tarar
Bronchopulmonary sequestration is one of the rare thoracic congenital anomalies. We report the case of a 6 year old boy with history of recurrent episodes of chest infection and breathing difficulty. This time admitted with fever and cough. Investigations revelaed neutrophilic leucocytosis, raised C-reactive protein and a retrocardiac opacity on chest radiographs. Contrast enhanced Computed Topography(CT scan) revealed a large, well defined, left lower lobe multi-loculated cystic mass with a vessel arising from the descending aorta supplying sequestrated lung portion...
February 2017: JPMA. the Journal of the Pakistan Medical Association
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