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congenital lung lesions

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https://www.readbyqxmd.com/read/28535027/arsenite-binds-to-the-ring-finger-domain-of-fancl-e3-ubiquitin-ligase-and-inhibits-dna-interstrand-cross-link-repair
#1
Ji Jiang, Marina Bellani, Lin Li, Pengcheng Wang, Michael M Seidman, Yinsheng Wang
Human exposure to arsenic in drinking water is known to be associated with the development of bladder, lung, kidney, and skin cancers. The molecular mechanisms underlying the carcinogenic effects of arsenic species remain incompletely understood. DNA interstrand cross-links (ICLs) are among the most cytotoxic type of DNA lesions that block DNA replication and transcription, and these lesions can be induced by endogenous metabolism and by exposure to exogenous agents. Fanconi anemia (FA) is a congenital disorder manifested with elevated sensitivity toward DNA interstrand cross-linking agents...
May 23, 2017: ACS Chemical Biology
https://www.readbyqxmd.com/read/28493304/cpam-type-2-derived-mesenchymal-stem-cells-malignancy-risk-study-in-a-14-month-old-boy
#2
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...
May 10, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28438750/intradiaphragmatic-hybrid-lesion-surgical-decision-making-and-value-of-minimal-invasive-surgery
#3
Blanca Schuster, Stefan Gerhard Holland-Cunz, Peter Zimmermann
Hybrid lesions (HLs) have elements of congenital pulmonary airway malformation and extrapulmonary sequestration (EPS) and belong to the congenital lung lesions. EPS usually arises in the thorax or the abdomen but rarely in the diaphragm. The preoperative diagnostic work-up based on chest radiograph, ultrasound (US) and CT often shows imprecise results. Therefore, the exact localisation of the lesion can only be ascertained intraoperatively. Here we present a patient, with an intradiaphragmatic HL, and demonstrate the difficulties of surgical decision making regarding the localisation of the lesion and discuss the value of minimal invasive surgery...
April 23, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28391172/large-bronchogenic-cyst-of-stomach-a-case-report
#4
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/28293001/-congenital-bronchial-atresia-requiring-differentiation-from-intralobar-sequestration
#5
Yusuke Tanaka, Isao Matsumoto, Daisuke Saito, Syuhei Yoshida, Seiichi Kakegawa, Masaya Tamura, Hirofumi Takemura, Kaori Yoshimura, Hiroko Ikeda
We reported a case of bronchial atresia requiring differentiation from the intralobar sequestration. A 42-year-old man was referred to our institution with suspicion of intralobar sequestration, based on a 3-dimensional computed tomography (CT) angiography that showed abnormal blood vessels from the right inferior phrenic artery flowing into the right lower lobe. CT revealed a lesion between S9 and S10 wherein there were refluxed blood vessels from A9 without an accompanying bronchus, with polycysts and emphysematous changes...
March 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28243740/atypical-pulmonary-sequestration-causing-respiratory-distress-in-a-2-month-old-male-infant
#6
Julio Bauza Rossi
Pulmonary sequestrations are non-functional masses of lung tissue lacking normal communication with the tracheobronchial tree and systemic-circulation-derived blood supply. The disconnection between the normal airway and pulmonary arterial supply prevents pulmonary sequestrations from participating in respiration while their aberrant circulation increases the potential for hemodynamic imbalance. Extralobar sequestrations are prenatally diagnosed, congenital lesions that may become symptomatic before or after birth, whereas intralobar sequestrations are usually identified during adulthood...
February 27, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28204920/a-review-of-congenital-lung-malformations-with-a-simplified-classification-system-for-clinical-and-research-use
#7
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/28195247/type-ii-congenital-pulmonary-airway-malformation-associated-with-intralobar-pulmonary-sequestration-report-of-a-case-and-review-of-classification-criteria
#8
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/28144070/perpetual-dilemma-pleural-or-parenchymal-congenital-or-acquired-solitary-cystic-lesion-with-fluid-level
#9
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/28079822/successfully-treated-congenital-cystic-adenomatoid-malformation-by-open-fetal-surgery-a-care-compliant-case-report-of-a-5-year-follow-up-and-review-of-the-literature
#10
REVIEW
Dazhi Fan, Shuzhen Wu, Rui Wang, Yi Huang, Yao Fu, Wen Ai, Meng Zeng, Xiaoling Guo, Zhengping Liu
BACKGROUND: Congenital cystic adenomatoid malformation (CCAM) is a rare hamartomatous cystic lesion. Open fetal surgery currently provides a potential therapeutic option for management of a fetus with CCAM diagnosis. CASE SUMMARY: A 22-year-old G2P0 woman presented at (Equation is included in full-text article.)weeks' gestation for evaluation of a fetus with a left lung lesion and diagnosed as CCAM at (Equation is included in full-text article.)weeks' gestation...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28040760/efficacy-of-segmental-resection-in-patients-with-prenatally-diagnosed-congenital-lung-malformations
#11
Sungkwang Lee, Do Hyung Kim, Sang Kwon Lee
OBJECTIVES: Lung segmental resection is a better treatment option than lobectomy for patients with prenatally diagnosed congenital lung malformations (CLMs). However, data are lacking on the effects of this procedure in prenatally diagnosed CLM patients. In this study, we explored whether parenchyma-saving resection was feasible in patients with this condition. METHODS: A retrospective analysis was performed on 27 patients prenatally diagnosed with CLM, who subsequently underwent surgery between March 2011 and September 2015...
December 31, 2016: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28018801/thoracoscopic-approach-for-congenital-diaphragmatic-hernia-a-useful-tool-in-diagnosis-and-treatment-of-unexpected-associated-lung-lesion
#12
Mario Lima, Michela Maffi, Tommaso Gargano, Giovanni Ruggeri, Michele Libri, Francesca Destro
We report the case of a neonate affected by prenatally diagnosed congenital diaphragmatic hernia (CDH), treated by thoracoscopy. During the procedure, we detected an associated extralobar pulmonary sequestration (EPS), which was preoperatively undiagnosed, and we removed it. EPS has been shown to be associated with CDH in up to 15 to 40% of cases. A possible explanation hypothesized is that pulmonary sequestration, which develops at 4 to 5 weeks of gestation, can disturb the fusion of the diaphragm and closure of the pleuroperitoneal canal...
December 2016: European Journal of Pediatric Surgery Reports
https://www.readbyqxmd.com/read/28008200/comparing-surgical-outcomes-of-complete-thoracoscopic-lobectomy-for-congenital-cystic-lung-disease-between-neonatal-and-infantile-patients
#13
Takahisa Tainaka, Hiroo Uchida, Yujiro Tanaka, Chiyoe Shirota, Kazuki Yokota, Naruhiko Murase, Kazuo Oshima, Ryo Shirotsuki, Kosuke Chiba, Akinari Hinoki
Thoracoscopic lobectomy has recently become a widely used surgical treatment for congenital cystic lung disease, but significant issues can arise in some cases, such as a limited working space in neonates, a limited view in cases involving large cystic lesions. We reviewed the treatment outcomes of neonates that underwent complete thoracoscopic lobectomy or segmentectomy and evaluated the operative difficulties. From January 2008 to October 2015, 38 patients under the age of 1 year underwent complete thoracoscopic lobectomy or segmentectomy for cystic lung disease at our institution...
December 2016: Nagoya Journal of Medical Science
https://www.readbyqxmd.com/read/27960159/fetal-congenital-lung-lesions-dicer1-syndrome-and-family-history-as-a-risk-assessment-tool
#14
LETTER
Lea Tuzovic
No abstract text is available yet for this article.
2017: Fetal Diagnosis and Therapy
https://www.readbyqxmd.com/read/27889066/does-thoracoscopy-have-advantages-over-open-surgery-for-asymptomatic-congenital-lung-malformations-an-analysis-of-1626-resections
#15
REVIEW
Stephen Adams, Matthew Jobson, Patarawan Sangnawakij, Adam Heetun, Anthony Thaventhiran, Navroop Johal, Dankmar Böhning, Michael P Stanton
AIM: The apparent incidence of antenatally diagnosed congenital lung malformations (CLM) is rising (1 in 3000), and the majority undergo elective resection even if asymptomatic. Thoracoscopy has been popularized, but early series report high conversion rates and significant complications. We aimed to perform systematic review/meta-analysis of outcomes of thoracoscopic vs open excision of asymptomatic CLMs. METHODS: A systematic review according to PRISMA guidelines was performed...
February 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27867946/advances-in-fetal-surgery
#16
REVIEW
Kathryn M Maselli, Andrea Badillo
Historically, the gold standard for the treatment of congenital malformations has been planned delivery at tertiary care center with attempted post-natal repair or amelioration of the lesion. Over the last few decades however, rapid advances in imaging and instrumentation technology combined with superior knowledge of fetal pathophysiology has led to the development of novel intrauterine interventions for most common fetal anomalies. Great success has already been seen the treatment of previous devastating anomalies such as myelomeningocele (MMC), congenital cystic malformations of the lung, twin-twin transfusion, and sacrococcygeal teratomas...
October 2016: Annals of Translational Medicine
https://www.readbyqxmd.com/read/27867411/ultrasound-as-a-screening-tool-in-the-follow-up-of-asymptomatic-congenital-cystic-adenomatoid-malformation
#17
Mehmet Emin Adin
Congenital cystic adenomatoid malformation accounts for the vast majority of congenital cystic lung pathologies. The clinical spectrum of congenital cystic adenomatoid malformation is wide and ranges from silent incidental lesions to severe congenital respiratory distress or stillbirths. Management of clinically silent lesions is still a matter of debate, with conservative follow-up being a popular approach during the last decade. However, an optimal methodology of follow-up is yet to be determined. In this study, ultrasound is proposed as a follow-up tool for clinically silent lesions based on the findings in a case with known congenital cystic adenomatoid malformation from the antenatal period, which has been followed up for four years...
August 2016: Ultrasound: Journal of the British Medical Ultrasound Society
https://www.readbyqxmd.com/read/27781131/cystic-lung-disease-in-down-syndrome-a-case-report-and-literature-review
#18
Mathew George, John Amodio, Haesoon Lee
Subpleural lung cysts (SPC) are seen in children with Down syndrome (DS). The incidence and the long term course of these lesions are not known. It is important for pediatricians and pediatric radiologists to be aware of these lung lesions since the DS patients' longevity has increased and they have greater frequency to encounter the clinicians. Autopsy and the radiology series have shown that these lesions are often found in association with congenital heart disease, particularly the endocardial cushion defect and prematurity...
2016: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/27723920/antenatally-detected-congenital-pulmonary-airway-malformations-the-oxford-experience
#19
Hemanshoo Sudhir Thakkar, Jonathan Durell, Subhasis Chakraborty, Bianca-Lea Tingle, Arnwald Choi, Darren J Fowler, Steve J Gould, Lawrence Impey, Kokila Lakhoo
Congenital airway pulmonary malformations are increasingly being diagnosed, but their management continues to remain controversial. Our approach has been to offer surgery to mitigate the risk of infection and possible malignancy. All patients routinely undergo a CT scan of the chest postnatally and once the diagnosis is confirmed, minimal access surgery is offered. Our anesthetists provide single-lung ventilation to enhance the operative view. We conducted a retrospective review over a 10-year period, during which 91 patients were prenatally suspected to have a cystic lung lesion...
October 10, 2016: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27717396/viable-phenotype-of-ilneb-syndrome-without-nephrotic-impairment-in-siblings-heterozygous-for-unreported-integrin-alpha3-mutations
#20
Elisa Adele Colombo, Luigina Spaccini, Ludovica Volpi, Gloria Negri, Davide Cittaro, Dejan Lazarevic, Salvatore Zirpoli, Andrea Farolfi, Cristina Gervasini, Maria Vittoria Cubellis, Lidia Larizza
BACKGROUND: Integrin α3 (ITGA3) gene mutations are associated with Interstitial Lung disease, Nephrotic syndrome and Epidermolysis bullosa (ILNEB syndrome). To date only six patients are reported: all carried homozygous ITGA3 mutations and presented a dramatically severe phenotype leading to death before age 2 years, from multi-organ failure due to interstitial lung disease and congenital nephrotic syndrome. The involvement of skin and cutaneous adnexa was variable with sparse hair and nail dysplasia combined or not to skin lesions ranging from skin fragility to epidermolysis bullosa-like blistering...
October 7, 2016: Orphanet Journal of Rare Diseases
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