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https://www.readbyqxmd.com/read/29110827/long-term-outcomes-of-congenital-lung-malformations
#1
Nigel J Hall, Michael P Stanton
Congenital lung malformations comprise a group of anatomical abnormalities of the respiratory tree including congenital cystic malformations, bronchopulmonary sequestrations, bronchogenic cyst, bronchial atresia, and congenital lobar emphysema. These anomalies are detected with increasing frequency by pre-natal sonography, but may also present for the first time with symptoms in childhood or later life. When symptomatic, there is little controversy that resection is indicated, which is usually curative. When a lesion is asymptomatic there is greater debate regarding the benefit of resection versus continued observation...
October 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/29046943/congenital-pulmonary-airway-malformations-state-of-the-art-review-for-pediatrician-s-use
#2
REVIEW
Claire Leblanc, Marguerite Baron, Emilie Desselas, Minh Hanh Phan, Alexis Rybak, Guillaume Thouvenin, Clara Lauby, Sabine Irtan
Congenital pulmonary airway malformations or CPAM are rare developmental lung malformations, leading to cystic and/or adenomatous pulmonary areas. Nowadays, CPAM are diagnosed prenatally, improving the prenatal and immediate postnatal care and ultimately the knowledge on CPAM pathophysiology. CPAM natural evolution can lead to infections or malignancies, whose exact prevalence is still difficult to assess. The aim of this "state-of-the-art" review is to cover the recently published literature on CPAM management whether the pulmonary lesion was detected during pregnancy or after birth, the current indications of surgery or surveillance and finally its potential evolution to pleuro-pulmonary blastoma...
December 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/29026365/incidental-finding-of-bronchopulmonary-sequestration-in-a-64-year-old-female
#3
Pichapong Tunsupon, Ayesha Arshad, Sumit Patel, M Jeffery Mador
BACKGROUND: Bronchopulmonary sequestration is a congenital abnormality of the primitive foregut. In adults, the typical age at presentation is 20-25 years. CASE REPORT: A 64-year-old female was referred for evaluation of an 8 × 6-cm right lower lobe cystic lesion. Her medical history was significant for recurrent right lower lobe pneumonia requiring multiple hospitalizations. Her physical examination was significant for crackles at the right lung base. Computed tomography (CT) of the chest with contrast showed cystic changes with thickened septation of the medial segment of the right lower lobe lacking distinct visceral pleura and with arterial supply from the anomalous branch of the thoracic aorta arising near the celiac trunk...
2017: Ochsner Journal
https://www.readbyqxmd.com/read/28891233/fetoscopic-tracheal-occlusion-for-treatment-of-non-isolated-congenital-diaphragmatic-hernia
#4
Viola Seravalli, Eric B Jelin, Jena L Miller, Aylin Tekes, Luca Vricella, Ahmet A Baschat
Fetoscopic endotracheal occlusion (FETO) is a prenatal treatment that may increase survival in severe congenital diaphragmatic hernia (CDH). In the USA, FETO is offered for isolated severe left-sided CDH in the context of an FDA-approved feasibility study. FETO in non-isolated cases of severe CDH is only performed with a compassionate use exemption from US regulatory bodies. Anomalies frequently associated with CDH include congenital cystic lesions of the lung and cardiac defects. We describe two cases of non-isolated severe left-sided CDH that underwent prenatal FETO, survived after birth and underwent postnatal surgical repair...
October 2017: Prenatal Diagnosis
https://www.readbyqxmd.com/read/28884206/neonatal-congenital-lung-tumors-the-importance-of-mid-second-trimester-ultrasound-as-a-diagnostic-clue
#5
Stephan L Waelti, Laurent Garel, Dorothée Dal Soglio, Françoise Rypens, Michael Messerli, Josée Dubois
BACKGROUND: The differential diagnosis for primary lung masses in neonates includes a variety of developmental abnormalities; it also consists of the much rarer congenital primary lung tumors: cystic pleuropulmonary blastoma (cystic PPB), fetal lung interstitial tumor (FLIT), congenital peribronchial myofibroblastic tumor (CPMT), and congenital fibrosarcoma. Radiologic differentiation between malformations and tumors is often very challenging. OBJECTIVE: The objective was to establish distinctive features between developmental pulmonary abnormalities and primary lung tumors...
December 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28814498/both-ways-at-once-keeping-small-airways-clean
#6
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
#7
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
#8
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
#9
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
#10
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...
September 16, 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
#11
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
#12
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...
October 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
#13
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
#14
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...
September 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
#15
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
#16
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
#17
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
#18
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...
July 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28324617/rare-localized-extralobar-sequestration-with-congenital-cystic-adenomatoid-malformation-a-case-report
#19
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
#20
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
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