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https://www.readbyqxmd.com/read/27922232/changing-epidemiology-of-non-cystic-fibrosis-bronchiectasis
#1
Semiha Bahçeci, Sait Karaman, Hikmet Tekin Nacaroğlu, Selçuk Yazıcı, Saniye Girit, Şule Ünsal-Karkıner, Demet Can
Non-cystic fibrosis bronchiectasis again becomes a major health problem due to inappropriate antibiotic use and increasing frequency of protracted bacterial bronchitis. The aim was to determine the changes in etiology of bronchiectasis. Patients who admitted to Behçet Uz Children Hospital between 2005 and 2015 (n=110) were retrospectively examined. The etiology of bronchiectasis was detected as; primary ciliary dyskinesia 26.4%, protracted bacterial bronchitis 22.8%, primary immune deficiency 11.8%, bronchiolitis obliterans 8...
2016: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/27867946/advances-in-fetal-surgery
#2
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
#3
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
#4
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/27780555/relation-of-left-atrial-size-to-atrial-fibrillation-in-patients-aged-%C3%A2-22%C3%A2-years
#5
Douglas Y Mah, Divya Shakti, Kimberlee Gauvreau, Steven D Colan, Mark E Alexander, Dominic J Abrams, David W Brown
Left atrial (LA) dilation has been shown to be associated with atrial fibrillation (AF) in the adult population, with some studies indicating that larger LAs are more prone to AF recurrence. The relation of LA size to AF in the pediatric and young adult population has not been investigated. In this study, all pediatric patients (aged ≤22 years) who presented to Boston Children's Hospital from January 2002 to December 2012 with AF were reviewed. Patients with significant congenital heart disease, cardiomyopathies, proven channelopathies, previous cardiac surgery, end-stage renal disease, or severe lung disease/cystic fibrosis were excluded...
September 30, 2016: American Journal of Cardiology
https://www.readbyqxmd.com/read/27780343/gene-editing-and-genetic-lung-disease-basic-research-meets-therapeutic-application
#6
Deepthi Alapati, Edward E Morrisey
While our understanding of the genetics and pathology of congenital lung diseases such as surfactant protein deficiency, cystic fibrosis and alpha 1 antitrypsin deficiency is extensive, treatment options are lacking. Since the lung is a barrier organ in direct communication with the external environment, targeted delivery of gene corrective technologies to the respiratory system via intra-tracheal or intranasal routes is an attractive option for therapy. CRISPR/Cas9 gene editing technology is a promising approach to repair or inactivate disease causing mutations...
October 25, 2016: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/27723920/antenatally-detected-congenital-pulmonary-airway-malformations-the-oxford-experience
#7
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/27705088/thoracoscopic-lobectomy-in-infants-and-children-utilizing-a-5%C3%A2-mm-stapling-device
#8
Steven Rothenberg
PURPOSE: Thoracoscopic lobectomy for congenital cystic lung disease has become an accepted and in many institutions the preferred technique. However, the technical challenges are many. Previous endoscopic staplers (12 mm) used commonly in adults are too large for use in infants This study evaluates the safety and efficacy of using a 5 mm stapling device to seal and divide major pulmonary structures. METHODS: From July 2014 to March 2016, 26 patients of age 6 weeks to 13 months underwent thoracoscopic lobectomy for CPAM or sequestration...
December 2016: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://www.readbyqxmd.com/read/27671988/congenital-pulmonary-airway-malformation-cpam-with-initial-presentation-in-an-adult-a-rare-presentation-of-a-rare-disease
#9
Mohannad Abu Omar, Emily Tylski, Mouhanna Abu Ghanimeh, Ashraf Gohar
Congenital pulmonary airway malformation (CPAM) is a rare congenital abnormality with unknown exact aetiology or clear genetic association. It is characterised by a failure of bronchial development and localised glandular overgrowth. Typically, it is diagnosed on prenatal ultrasound, only infrequently in children, and even less commonly in adults. We present a case of a 25-year-old man, with no previous lung diseases who presented with right-sided chest pain, fever and cough suggestive of pulmonary infection...
September 26, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27666113/congenital-cystic-adenomatoid-malformation-of-the-lung-ccam-a-retrospective-clinical-audit-and-literature-review-in-a-tertiary-centre-in-scotland-over-a-period-of-14-years
#10
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
https://www.readbyqxmd.com/read/27625455/unilateral-right-pulmonary-artery-agenesis-and-congenital-cystic-adenomatoid-malformation-of-the-right-lung-with-ortner-s-syndrome
#11
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
https://www.readbyqxmd.com/read/27549731/extracorporeal-life-support-in-multisystem-smooth-muscle-dysfunction-syndrome
#12
Sudesh Prabhu, Scott Fox, Adrian Mattke, Jane E Armes, Nelson Alphonso
We describe an infant with congenital mydriasis, patent ductus arteriosus (PDA), pulmonary hypertension, and cystic lung disease. She had all the major components of multisystemic smooth muscle dysfunction syndrome. Due to progressive respiratory deterioration, she required surgical PDA interruption, extracorporeal life support, and subsequent prolonged respiratory support. Genetic testing revealed ACTA2 R179H mutation and cystic lung disease on biopsy.
August 22, 2016: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/27490105/congenital-cystic-lung-lesions-evolution-from-in-utero-to-pathology-diagnosis-a-multidisciplinary-approach
#13
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
https://www.readbyqxmd.com/read/27489957/thoracoamniotic-shunts-in-macrocystic-lung-lesions-case-series-and-review-of-the-literature
#14
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
https://www.readbyqxmd.com/read/27469386/refractory-tension-pneumothorax-as-a-result-of-an-internally-displaced-thoracoamniotic-shunt-in-an-infant-with-a-congenital-pulmonary-airway-malformation
#15
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
https://www.readbyqxmd.com/read/27467867/imaging-of-congenital-pulmonary-malformations
#16
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...
July 28, 2016: Acta Bio-medica: Atenei Parmensis
https://www.readbyqxmd.com/read/27461429/perinatal-features-of-congenital-cystic-lung-diseases-results-of-a-nationwide-multicentric-study-in-japan
#17
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
https://www.readbyqxmd.com/read/27435178/pulmonary-interstitial-emphysema-due-to-respiratory-syncytial-virus-infection
#18
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
https://www.readbyqxmd.com/read/27428024/hyperpolarized-gas-magnetic-resonance-lung-imaging-in-children-and-young-adults
#19
Lucia Flors, John P Mugler, Eduard E de Lange, Grady W Miller, Jaime F Mata, Nick Tustison, Iulian C Ruset, F William Hersman, Talissa A Altes
The assessment of early pulmonary disease and its severity can be difficult in young children, as procedures such as spirometry cannot be performed on them. Computed tomography provides detailed structural images of the pulmonary parenchyma, but its major drawback is that the patient is exposed to ionizing radiation. In this context, magnetic resonance imaging (MRI) is a promising technique for the evaluation of pediatric lung disease, especially when serial imaging is needed. Traditionally, MRI played a small role in evaluating the pulmonary parenchyma...
September 2016: Journal of Thoracic Imaging
https://www.readbyqxmd.com/read/27423390/association-between-congenital-lung-malformations-and-lung-tumors-in-children-and-adults-a-systematic-review
#20
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
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