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congenital lung lesions

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
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
Ya-Ting Chang, Christina K Chan, Inger Eriksson, Pamela Y Johnson, Xiaofang Cao, Christian Westöö, Christian Norvik, Annika Andersson-Sjöland, Gunilla Westergren-Thorsson, Staffan Johansson, Ulf Hedin, Lena Kjellén, Thomas N Wight, Karin Tran-Lundmark
Pulmonary arterial hypertension (PAH) is a lethal condition for which there is no effective curative pharmacotherapy. PAH is characterized by vasoconstriction, wall thickening of pulmonary arteries, and increased vascular resistance. Versican is a chondroitin sulfate proteoglycan in the vascular extracellular matrix that accumulates following vascular injury and promotes smooth-muscle cell proliferation in systemic arteries. Here, we investigated whether versican may play a similar role in PAH. Paraffin-embedded lung sections from patients who underwent lung transplantation to treat PAH were used for immunohistochemistry...
September 2016: Pulmonary Circulation
Ambrin Gull Shamas, Karishma Bohara
This work was a retrospective audit of CCAM - 1994 to 2008. A total of 26 cases were identified. Mean gestational age at diagnosis was 20 weeks. All were unilateral and had serial scans. In 31% the lesion resolved, 8% decreased, 42% were unchanged and 4% increased in size. Only one foetus developed hydrops. All were born alive. Of 8 foetuses where the CCAM was thought to have resolved, 6 had persistent lesions (overall sensitivity and PPV of US 64% and 69%, respectively). Computerised tomography performed better than chest X-ray in detecting lesions postnatally (sensitivity 100% vs 88%, PPV 95% vs 78%)...
September 25, 2016: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
Gopal Singh, Amy Coffey, Robert Neely, Daniel Lambert, Joshua Sonett, Alain C Borczuk, Lyall Gorenstein
Congenital pulmonary airway malformation (CPAM) is a developmental abnormality of the lung, which results from an abnormality of branching during fetal development of the lung. We report the case of an 18 year-old woman who developed Kirsten rat sarcoma virus (KRAS) mutation positive mucinous adenocarcinoma of the lung (AC) in association with mixed CPAM type 1 and 2. This case is unique as KRAS mutation positive AC is present in a setting of both CPAM 1 and 2 in the same lesion.
October 2016: Annals of Thoracic Surgery
Jane Jackie David, Smilu Mohanlal, Punam Sankhe, Radha Ghildiyal
We report a 2.5-year-old girl who presented with hoarseness of voice since 3 months of age and failure to thrive. Chest X-ray showed cardiomegaly with a deviation of the trachea and mediastinum to the right side. Two-dimensional echocardiography showed decreased flow across the right pulmonary artery, a small atrial septal defect (ASD) with a right-to-left shunt, and a dilated right atrium and right ventricle with severe tricuspid regurgitation suggestive of severe pulmonary hypertension. A silent large patent ductus arteriosus was also seen...
September 2016: Lung India: Official Organ of Indian Chest Society
S Birch, D Casamian-Sorrosal, S Fonfara, G Chanoit, C Warren-Smith
This case report describes a combination of congenital cardiopulmonary abnormalities found in a 1-year-old Labrador Retriever. To the authors' knowledge this combination of cardiopulmonary abnormalities has not been previously reported in veterinary medicine. Unilateral absence of the right pulmonary artery associated with unilateral right-sided aortopulmonary collaterals was observed. These aortopulmonary collaterals preserved the blood supply to the right lung lobes but led to left ventricular volume overload...
September 7, 2016: Journal of Veterinary Cardiology: the Official Journal of the European Society of Veterinary Cardiology
Steven Hardee, Lea Tuzovic, Cicero T Silva, Robert A Cowles, Joshua Copel, Raffaella A Morotti
Congenital cystic lung lesions (CCLL) are a group of rare pathologies that are usually diagnosed in the pre-natal 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...
August 4, 2016: Pediatric and Developmental Pathology
Magdalena Litwińska, Ewelina Litwińska, Katarzyna Janiak, Anna Piaseczna-Piotrowska, Ewa Gulczyńska, Krzysztof Szaflik
OBJECTIVE: To evaluate the efficiency of thoracoamniotic shunts for drainage of macrocystic-type congenital cystic adenomatoid malformation (CCAM). SUBJECTS AND METHODS: This was a retrospective study of 12 fetuses with a large thoracic cyst treated with thoracoamniotic shunting between 2004 and 2014 in a tertiary fetal therapy center. Medline was searched to identify cases of CCAM treated with thoracoamniotic shunting. RESULTS: In all cases the thoracic cyst was associated with major mediastinal shift, the CCAM volume ratio (CVR) was >1...
August 5, 2016: Fetal Diagnosis and Therapy
Brenda Hiu Yan Law, Ioana Bratu, Venu Jain, Marc-Antoine Landry
Antenatally, congenital pulmonary airway malformation (CPAM) causing fetal hydrops can be palliated with thoracoamniotic shunts, which may become displaced in utero. We report a case of an infant born at 34 weeks gestational age with an antenatally diagnosed macrocystic lung lesion, fetal hydrops and an internally displaced thoracoamniotic shunt. The infant suffered refractory pneumothoraces despite multiple chest drains, and stabilised only after surgical resection of the lesion. Intraoperatively, the shunt was noted to form a connection between a type I CPAM and the pleural space...
2016: BMJ Case Reports
Francesco Emanuele Praticò, Michele Corrado, Giovanni Della Casa, Raffaele Parziale, Giuseppe Russo, Silvia Eleonora Gazzani, Enrica Rossi, Daniele Borgia, Maurizio Mostardi, Emanuele Bacchini, Simone Cella, Massimo De Filippo
Congenital pulmonary malformations represent a broad spectrum of anomalies that may result in varied clinical and pathologic pictures, ranging from recurrent pulmonary infections and acute respiratory distress syndrome, which require timely drug therapy, up to large space-occupying lesions needing surgical treatment. This classification includes three distinct anatomical and pathological entities, represented by Congenital Cystic Adenomatoid Malformation, Bronchopulmonary Sequestration and Congenital Lobar Emphysema...
2016: Acta Bio-medica: Atenei Parmensis
Katsunari Matsuoka, Mitsuhiro Ueda, Yoshihiro Miyamoto
Bronchial atresia is a rare congenital malformation that is often complicated by infectious disease; however, mycobacterial infection is rare. Here we report a case of Mycobacterium avium infection complicating congenital bronchial atresia. A 50-year-old woman consulted our institution for further investigation of a nodular lesion in an emphysematous lung due to congenital bronchial atresia. Because we were unable to rule out malignant disease, we performed video-assisted thoracoscopic surgery. After left S8 segmentectomy, the nodular lesion were diagnosed as Mycobacterial avium infection...
July 27, 2016: Asian Cardiovascular & Thoracic Annals
Tatsuo Kuroda, Eiji Nishijima, Kosaku Maeda, Yasusih Fuchimoto, Seiichi Hirobe, Yuko Tazuke, Toshihiko Watanabe, Noriaki Usui
PURPOSE: The current study aimed to assess the perinatal risk and clinical features of congenital cystic lung diseases (CCLD). MATERIALS AND METHODS: Of the 874 CCLD patients identified in a nationwide survey, 428 patients born between 1992 and 2012 and treated at 10 high-volume centers, were retrospectively reviewed. RESULTS: Fetal hydrops was visualized using MRI in 9.2 % of the patients. Prenatal interventions were described for 221 of the 428 patients, including the maternal administration of steroid and pleuro-amniotic shunting...
September 2016: Pediatric Surgery International
Dayu Huang, Qinyun Ma, Shaohua Wang, Qi Ouyang, Xiaofeng Chen
Tracheal diverticulum is a clinical entity rarely reported in the literature. It can be congenital or acquired in origin, and most cases are asymptomatic, usually being discovered incidentally at CT scan. Therefore, treatment has not been widely reported. We present the case of a 30-year-old man with a 6-month history of repeatedly coughing and tracheobronchitis. CT scanning revealed the lesion. The patient underwent surgical resection of the tracheal diverticulum via a transcervical approach. Symptoms resolved after surgical treatment...
July 20, 2016: Head & Neck
Tsubasa Aiyoshi, Kouji Masumoto, Toko Shinkai, Yasunari Tanaka, Shunsuke Fujii, Takato Sasaki, Fumiko Chiba, Naoya Sakamoto, Chikashi Gotoh, Yasuhisa Urita, Makoto Nakao, Hajime Takayasu, Hideaki Tanaka, Hironori Imai
Pulmonary interstitial emphysema (PIE) primarily affects premature infants on positive pressure ventilation. PIE is rarely reported in infants and children in the absence of mechanical ventilation and/or associated respiratory infection. We report a case of PIE in a 22-month-old girl who had severe respiratory distress due to respiratory syncytial virus infection. Chest computed tomography showed cystic lung lesions mimicking congenital cystic adenomatoid malformation. The cystic lesions spontaneously resolved after conservative treatment...
September 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
Arianna Casagrande, Federica Pederiva
INTRODUCTION: The appropriate management of asymptomatic congenital pulmonary malformations (CPMs) remains controversial. Prophylactic surgery is recommended to avoid the risk for development of pulmonary infections and to prevent the highly debated development of malignancy. However, the true risk for development of malignancy remains unknown. A systematic review analyzed all cases in which lung tumors associated with CPMs in both the pediatric and adult populations were described...
July 15, 2016: Journal of Thoracic Oncology
Dandan Zong, Ruoyun Ouyang, Jinhua Li, Yan Chen, Ping Chen
Notch signaling is an evolutionarily conserved cell-cell communication mechanism that plays a key role in lung homeostasis, injury and repair. The loss of regulation of Notch signaling, especially Notch1 and Notch3, has recently been linked to the pathogenesis of important lung diseases, in particular, chronic obstructive pulmonary disease (COPD), asthma, pulmonary fibrosis, pulmonary arterial hypertension (PAH), lung cancer and lung lesions in some congenital diseases. This review focuses on recent advances related to the mechanisms and the consequences of aberrant or absent Notch1/3 activity in the initiation and progression of lung diseases...
October 2016: Therapeutic Advances in Respiratory Disease
Alexandria L Irace, Eelam A Adil, Natasha M Archer, Victoria M Silvera, Antonio Perez-Atayde, Reza Rahbar
OBJECTIVES: Sialoblastoma is a rare congenital salivary gland tumor of epithelial origin. The objectives of this study are to review the literature regarding clinical presentation of sialoblastoma, evaluate the effectiveness of various treatment methods, and present guidelines for evaluation and management in the pediatric population. DATA SOURCES: Case presentation and literature review. REVIEW METHODS: A comprehensive search was conducted to identify cases of pediatric sialoblastoma in the English-language literature...
August 2016: International Journal of Pediatric Otorhinolaryngology
Christopher P Barnett, Nathalie J Nataren, Manuela Klingler-Hoffmann, Quenten Schwarz, Chan-Eng Chong, Young K Lee, Damien L Bruno, Jill Lipsett, Andrew J McPhee, Andreas W Schreiber, Jinghua Feng, Christopher N Hahn, Hamish S Scott
Ectrodactyly/split hand-foot malformation is genetically heterogeneous with more than 100 syndromic associations. Acinar dysplasia is a rare congenital lung lesion of unknown etiology, which is frequently lethal postnatally. To date, there have been no reports of combinations of these two phenotypes. Here, we present an infant from a consanguineous union with both ectrodactyly and autopsy confirmed acinar dysplasia. SNP array and whole-exome sequencing analyses of the affected infant identified a novel homozygous Fibroblast Growth Factor Receptor 2 (FGFR2) missense mutation (p...
September 2016: Human Mutation
Yooyoung Chong, Youn Ju Rhee, Sung Joon Han, Hyun Jin Cho, Shin Kwang Kang, Min-Woong Kang
Congenital cystic adenomatoid malformation is a rare, but well-known disease. It can be managed conservatively in patients without symptoms or require surgical removal when symptomatic. The surgical option of choice is en bloc resection of the affected lesion. We report an experience of life-threatening congenital cystic adenoid malformation in a low-birth-weight (1,590 g) premature neonate who was successfully treated with a lobectomy of the lung.
June 2016: Korean Journal of Thoracic and Cardiovascular Surgery
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