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Congenital lung disorder

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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
#1
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
#2
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/28120278/long-term-management-challenges-in-esophageal-atresia
#3
REVIEW
Abby White, Raphael Bueno
Esophageal atresia is a rare congenital anomaly, but improved surgical and critical care has resulted in survival rates exceeding 90%. Long-term survival is associated with numerous management challenges including chronic motility disorders, dysphagia, strictures, reflux, esophagitis and attendant complications, tracheomalacia and chronic restrictive lung disease, and recurrent pulmonary infections. No guidelines for adolescents and younger or older adults exist for the treatment and monitoring of this specialized patient population...
January 24, 2017: Current Treatment Options in Gastroenterology
https://www.readbyqxmd.com/read/28094013/pulmonary-outcome-of-esophageal-atresia-patients-and-its-potential-causes-in-early-childhood
#4
René Dittrich, Philippe Stock, Karin Rothe, Petra Degenhardt
INTRODUCTION: The aim of this study was to illustrate the pulmonary long term outcome of patients with repaired esophageal atresia and to further examine causes and correlations that might have led to this outcome. METHODS: Twenty-seven of 62 possible patients (43%) aged 5-20years, with repaired esophageal atresia were recruited. Body plethysmography and spirometry were performed to evaluate lung function, and the Bruce protocol treadmill exercise test to assess physical fitness...
January 3, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28029591/cardiorespiratory-interactions-in-paediatrics-it-s-almost-always-the-circulation-stupid
#5
REVIEW
M L Rigby, M Rosenthal
The interaction of the heart and lungs is probably the most important aspect of life and survival. Fortunately, it is not difficult to understand the fundamentals. The purpose of the lungs and their ventilation is to present oxygen to the circulation via the alveoli and to receive carbon dioxide from the circulation and then expel it. The relations of the heart and lungs and the matching of blood flow to the various organs with ventilation and lung perfusion may be disrupted by a variety of congenital or acquired heart malformations...
August 12, 2016: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/28012500/familial-diffuse-pulmonary-ossification-a-possible-genetic-disorder
#6
Yoshiaki Kinoshita, Ichiko Mizuguchi, Kouko Hidaka, Hiroshi Ishii, Kentaro Watanabe
Diffuse pulmonary ossification (DPO) is an uncommon disease that is characterized by the widespread formation of ectopic bone in the lungs. Herein, we describe two familial cases of DPO. The patients were a 47-year-old woman and her 76-year-old father. Both patients had a history of recurrent cough, and their chest images showed multiple nodules with ossification. No underlying diseases that might have caused DPO were evident; however, the female patient also had congenital finger hypoplasia and deformity. The present cases indicate the possibility that DPO might be partly associated with genetic disorders...
January 2017: Respiratory Investigation
https://www.readbyqxmd.com/read/27993326/interventional-treatment-of-pulmonary-lymphatic-anomalies
#7
REVIEW
Maxim Itkin
Pulmonary lymphatic diseases have been recognized for many years and have been referred as pulmonary lymphangiectasia, pulmonary lymphangiomatosis, plastic bronchitis, and idiopathic chylothorax or chylopericardium. The lymphatic etiology of these conditions has been determined by detection of cystic lymphatic structures on biopsy or postmortem examination. Development of new imaging techniques such as dynamic contrast-enhanced magnetic resonance lymphangiography has allowed better understanding of pathophysiology of these conditions...
December 2016: Techniques in Vascular and Interventional Radiology
https://www.readbyqxmd.com/read/27875026/disseminated-bcg-pneumonitis-revealing-severe-combined-immunodeficiencyxs-in-charge-syndrome
#8
Hyung Young Kim, Yoo-Mi Kim, Hee Ju Park
CHARGE (coloboma, heart defect, atresia choanae, retarded growth and development, genital hypoplasia, and ear anomalies/deafness) syndrome is a rare genetic disorder caused by CHD7 mutation and is related to immunodeficiency. A 6-month-old girl with right lung agenesis, congenital heart defects, and ear anomalies developed repeated and serious respiratory infection for a short period. She was clinically diagnosed with typical CHARGE syndrome with severe combined immunodeficiency (T-, B+, NK-); however, CHD7 mutation was not detected...
November 22, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27864598/impact-of-fetal-counseling-on-outcome-of-antenatal-congenital-surgical-anomalies
#9
Shilpa Sharma, Ranjana Bhanot, Dipika Deka, Minu Bajpai, Devendra K Gupta
AIM: To analyze the impact of counseling on antenatal congenital surgical anomalies (ACSA). METHODS: Cases presenting with ACSA for fetal counseling and those presenting in post-natal period following diagnosis of ACSA (PACSA) for surgical opinion were analyzed for spectrum, presentation and outcome. RESULTS: 117 cases including ACSA(68);PACSA(49) were analyzed. Gestational age at diagnosis of ACSA;PACSA was 17-37;17-39 weeks (median 24;32 weeks)...
November 18, 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/27638769/adamts18-deletion-results-in-distinct-developmental-defects-and-provides-a-model-for-congenital-disorders-of-lens-lung-and-female-reproductive-tract-development
#10
Dalya Ataca, Marian Caikovski, Alessandra Piersigilli, Alexandre Moulin, Charaf Benarafa, Sarah E Earp, Yakir Guri, Corinne Kostic, Yvan Arsenivic, Raija Soininen, Suneel S Apte, Cathrin Brisken
The ADAMTS family comprises 19 secreted metalloproteinases that cleave extracellular matrix components and have diverse functions in numerous disease and physiological contexts. A number of them remain 'orphan' proteases and among them is ADAMTS18, which has been implicated in developmental eye disorders, platelet function and various malignancies. To assess in vivo function of ADAMTS18, we generated a mouse strain with inactivated Adamts18 alleles. In the C57Bl6/Ola background, Adamts18-deficient mice are born in a normal Mendelian ratio, and are viable but show a transient growth delay...
November 15, 2016: Biology Open
https://www.readbyqxmd.com/read/27583409/new-guidelines-for-managing-pulmonary-hypertension-what-the-pediatrician-needs-to-know
#11
Steven H Abman
PURPOSE OF REVIEW: Pediatric pulmonary vascular disease contributes to morbidities and death in diverse clinical settings, ranging from idiopathic or heritable forms of pediatric arterial hypertension, congenital heart disease, developmental lung disorders, chronic lung disease, left heart disease, sickle cell disease, oncologic disease, and systemic disorders. Despite its impact on the clinical courses in so many diseases, information is limited on how to best approach the diagnosis and evaluation of pediatric pulmonary hypertension...
October 2016: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/27565037/polygenic-causes-of-congenital-diaphragmatic-hernia-produce-common-lung-pathologies
#12
REVIEW
Patricia K Donahoe, Mauro Longoni, Frances A High
Congenital diaphragmatic hernia (CDH) is one of the most common and lethal congenital anomalies, and significant evidence is available in support of a genetic contribution to its etiology, including single-gene knockout mice associated with diaphragmatic defects, rare monogenetic disorders in humans, familial aggregation, and association of CDH with chromosomal abnormalities. Structural lung defects in the form of lung hypoplasia are almost invariably seen in patients with CDH and frequently in animal models of this condition...
October 2016: American Journal of Pathology
https://www.readbyqxmd.com/read/27486780/ttc25-deficiency-results-in-defects-of-the-outer-dynein-arm-docking-machinery-and-primary-ciliary-dyskinesia-with-left-right-body-asymmetry-randomization
#13
Julia Wallmeier, Hidetaka Shiratori, Gerard W Dougherty, Christine Edelbusch, Rim Hjeij, Niki T Loges, Tabea Menchen, Heike Olbrich, Petra Pennekamp, Johanna Raidt, Claudius Werner, Katsura Minegishi, Kyosuke Shinohara, Yasuko Asai, Katsuyoshi Takaoka, Chanjae Lee, Matthias Griese, Yasin Memari, Richard Durbin, Anja Kolb-Kokocinski, Sascha Sauer, John B Wallingford, Hiroshi Hamada, Heymut Omran
Multiprotein complexes referred to as outer dynein arms (ODAs) develop the main mechanical force to generate the ciliary and flagellar beat. ODA defects are the most common cause of primary ciliary dyskinesia (PCD), a congenital disorder of ciliary beating, characterized by recurrent infections of the upper and lower airways, as well as by progressive lung failure and randomization of left-right body asymmetry. Using a whole-exome sequencing approach, we identified recessive loss-of-function mutations within TTC25 in three individuals from two unrelated families affected by PCD...
August 4, 2016: American Journal of Human Genetics
https://www.readbyqxmd.com/read/27477800/low-grade-disease-activity-in-early-life-precedes-childhood-asthma-and-allergy
#14
REVIEW
Bo Lund Krogsgaard Chawes
Asthma and allergies are today the most common chronic diseases in children and the leading causes of school absences, chronic medication usage, emergency department visits and hospitalizations, which affect all members of the family and represent a significant societal and scientific challenge. These highly prevalent disorders are thought to originate from immune distortion in early childhood, but the etiology and heterogeneity of the disease mechanisms are not understood, which hampers preventive initiatives and makes treatment inadequate...
August 2016: Danish Medical Journal
https://www.readbyqxmd.com/read/27366557/anaesthesia-application-for-cardiac-denervation-in-a-patient-with-long-qt-syndrome-and-cardiomyopathy
#15
Ümit Karadeniz, Aslı Demir, Rabia Koçulu
Long QT syndrome is a congenital disorder that is characterized by a prolongation of the QT interval on electrocardiograms and a propensity to ventricular tachyarrhythmias, which may lead to syncope, cardiac arrest or sudden death. Cardiomyopathy and pulmonary hypertension diseases have additional risks in anaesthesia management. In this study, we emphasize on one lung ventilation, pacemaker-implantable cardioverter-defibrillator and the anaesthesia management process in a patient with long QT syndrome, cardiomyopathy and pulmonary hypertension who underwent thoracic sympathectomy...
February 2016: Turkish Journal of Anaesthesiology and Reanimation
https://www.readbyqxmd.com/read/27225963/the-fetal-circulation-pathophysiology-of-hypoxemic-respiratory-failure-and-pulmonary-hypertension-in-neonates-and-the-role-of-oxygen-therapy
#16
REVIEW
S Lakshminrusimha, O D Saugstad
Neonatal hypoxemic respiratory failure (HRF), a deficiency of oxygenation associated with insufficient ventilation, can occur due to a variety of etiologies. HRF can result when pulmonary vascular resistance (PVR) fails to decrease at birth, leading to persistent pulmonary hypertension of newborn (PPHN), or as a result of various lung disorders including congenital abnormalities such as diaphragmatic hernia, and disorders of transition such as respiratory distress syndrome, transient tachypnea of newborn and perinatal asphyxia...
June 2016: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/26975810/a-global-view-of-pulmonary-hypertension
#17
REVIEW
Marius M Hoeper, Marc Humbert, Rogerio Souza, Majdy Idrees, Steven M Kawut, Karen Sliwa-Hahnle, Zhi-Cheng Jing, J Simon R Gibbs
Pulmonary hypertension is a substantial global health issue. All age groups are affected with rapidly growing importance in elderly people, particularly in countries with ageing populations. Present estimates suggest a pulmonary hypertension prevalence of about 1% of the global population, which increases up to 10% in individuals aged more than 65 years. In almost all parts of the world, left-sided heart and lung diseases have become the most frequent causes of pulmonary hypertension. About 80% of affected patients live in developing countries, where pulmonary hypertension is frequently associated with congenital heart disease and various infectious disorders, including schistosomiasis, HIV, and rheumatic heart disease...
April 2016: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/26932830/nasoethmoidal-meningocele-in-a-child-presenting-bilateral-congenital-cystic-adenomatoid-malformation-evidence-for-a-new-entity-or-consequence-of-gestational-exposures
#18
Ernani B da Rosa, Daniélle B Silveira, Laís G Tsugami, Nathan L Bellé, Izabelle O Matos, Luciano V Targa, Rosilene da S Betat, André C da Cunha, Rolando A R Villacis, Sílvia R Rogatto, Luiza E Dorfman, Rafael F M Rosa, Paulo R G Zen
BACKGROUND: Nasoethmoidal meningocele is considered an uncommon type of cephalocele, and congenital cystic adenomatoid malformation (CCAM) is a rare lung disorder characterized by overgrowth of the terminal bronchioles. CASE: We report the unusual association between a nasoethmoidal meningocele and CCAM type II in a fetus exposed to valproic acid and misoprostol. The mother was an 18-year-old woman on her first pregnancy. She had a history of absence seizures since she was 5 years old...
April 2016: Birth Defects Research. Part A, Clinical and Molecular Teratology
https://www.readbyqxmd.com/read/26927810/vici-syndrome-a-review
#19
REVIEW
Susan Byrne, Carlo Dionisi-Vici, Luke Smith, Mathias Gautel, Heinz Jungbluth
Vici syndrome [OMIM242840] is a severe, recessively inherited congenital disorder characterized by the principal features of callosal agenesis, cataracts, oculocutaneous hypopigmentation, cardiomyopathy, and a combined immunodeficiency. Profound developmental delay, progressive failure to thrive and acquired microcephaly are almost universal, suggesting an evolving (neuro) degenerative component. In most patients there is additional variable multisystem involvement that may affect virtually any organ system, including lungs, thyroid, liver and kidneys...
February 29, 2016: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/26864093/percutaneous-lymphatic-embolization-of-abnormal-pulmonary-lymphatic-flow-as-treatment-of-plastic-bronchitis-in-patients-with-congenital-heart-disease
#20
Yoav Dori, Marc S Keller, Jonathan J Rome, Matthew J Gillespie, Andrew C Glatz, Kathryn Dodds, David J Goldberg, Samuel Goldfarb, Jack Rychik, Maxim Itkin
BACKGROUND: Plastic bronchitis is a potentially fatal disorder occurring in children with single-ventricle physiology, and other diseases, as well, such as asthma. In this study, we report findings of abnormal pulmonary lymphatic flow, demonstrated by MRI lymphatic imaging, in patients with plastic bronchitis and percutaneous lymphatic intervention as a treatment for these patients. METHODS AND RESULTS: This is a retrospective case series of 18 patients with surgically corrected congenital heart disease and plastic bronchitis who presented for lymphatic imaging and intervention...
March 22, 2016: Circulation
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