Subject(s)
Ascariasis , Biliary Tract Diseases , Down Syndrome , Ascariasis/diagnosis , Canada , Child , Family , HumansABSTRACT
The ductus arteriosus is important to fetal circulation. Failure to close at birth is a common event. In this educational pictorial essay, we illustrate the association of the ductus arteriosus with a variety of congenital cardiac, vascular and pulmonary lesions. These lesions can impact the systemic circulation, the pulmonary circulation or the airway and include coarctation of the aorta, ductal origin of the pulmonary artery and vascular rings.
Subject(s)
Ductus Arteriosus, Patent , Ductus Arteriosus , Ductus Arteriosus/diagnostic imaging , Ductus Arteriosus, Patent/diagnostic imaging , Heart , Humans , Infant, Newborn , Pulmonary Artery/diagnostic imaging , Pulmonary CirculationABSTRACT
Marden-Walker syndrome is challenging to diagnose, as there is significant overlap with other multi-system congenital contracture syndromes including Beals congenital contractural arachnodactyly, D4ST1-Deficient Ehlers-Danlos syndrome (adducted thumb-clubfoot syndrome), Schwartz-Jampel syndrome, Freeman-Sheldon syndrome, Cerebro-oculo-facio-skeletal syndrome, and Van den Ende-Gupta syndrome. We discuss this differential diagnosis in the context of a boy from a consanguineous union with Van den Ende-Gupta syndrome, a diagnosis initially confused by the atypical presence of intellectual disability. SNP microarray and whole exome sequencing identified a homozygous frameshift mutation (p.L870V) in SCARF2 and predicted damaging mutations in several genes, most notably DGCR2 (p.P75L) and NCAM2 (p.S147G), both possible candidates for this child's intellectual disability. We review distinguishing features for each Marden-Walker-like syndrome and propose a clinical algorithm for diagnosis among this spectrum of disorders. © 2016 Wiley Periodicals, Inc.
Subject(s)
Abnormalities, Multiple/diagnosis , Abnormalities, Multiple/genetics , Arachnodactyly/diagnosis , Arachnodactyly/genetics , Blepharophimosis/diagnosis , Blepharophimosis/genetics , Contracture/diagnosis , Contracture/genetics , Genetic Association Studies , Abnormalities, Multiple/metabolism , Arachnodactyly/metabolism , Blepharophimosis/metabolism , Child , Contracture/metabolism , DNA Copy Number Variations , Exome , Frameshift Mutation , High-Throughput Nucleotide Sequencing , Homozygote , Humans , Male , Multimodal Imaging , Oligonucleotide Array Sequence Analysis , Phenotype , Polymorphism, Single Nucleotide , Scavenger Receptors, Class F/geneticsABSTRACT
Gold kiwifruit pomace extracted using citric acid, water and enzyme (Celluclast 1.5L) were studied in terms of pectin yield, protein, ash, non-starch polysaccharide, galacturonic acid (GalA), neutral sugar composition, molar mass (Mw), viscosity and degree of branching. Water-extracted pectin was considered closest to its native form. Enzyme extracted pectin showed the highest yield (â¼ 4.5%w/w) as compared with the acid and water extraction methods (â¼ 3.6-3.8%w/w). Pectin obtained from different extraction methods showed different degree of branching. The Mw and root mean square (RMS) radius varied with the extraction methods with values of 8.4 × 10(5) g/mol and 92 nm, 8.5 × 10(5)g/mol and 102 nm, 6.7 × 10(5) g/mol and 52 nm for acid, water and enzymatic extraction methods, respectively. Similar trend was observed for pectin viscosity, with water-extracted pectin giving a slightly higher viscosity followed by acid and enzyme-extracted pectin. This study showed that gold kiwifruit pomace pectin has potential application in food products.
Subject(s)
Actinidia/chemistry , Fruit/chemistry , Pectins/analysis , Dietary Proteins/analysis , Hexuronic Acids/analysis , Hydrogen-Ion Concentration , Molecular Weight , Polysaccharides/analysis , Viscosity , Water/analysisABSTRACT
BACKGROUND: Counts are the commonest method used to ensure that all sponges and neuropatties are removed from a surgical site before closure. When the count is not reconciled, plain radiographs of the operative site are taken to determine whether the missing patty has been left in the wound. The purpose of this study was to describe the detectability of commonly used neuropatties in the clinical setting using digital technologies. METHODS: Neuropatties were implanted into the anterior and posterior cranial fossae and the thoracolumbar extradural space of a mature male cadaver. Four neuropatty sizes were used: 3 × 1 in, 2 × ½ in, ½ × ½ in, and » × » in. Neuropatties, with size and location chosen at random, were placed in the surgical sites and anteroposterior/posterior-anterior and lateral radiographs were taken using standard portable digital radiographic equipment. Six clinicians reviewed the digital images for the presence or absence of neuropatties. The readers were not aware of the number and size of the patties that were included in each image. RESULTS: The detectability of neuropatties is dependent on the size of the neuropatty's radiopaque marker and the operative site. Neuropatties measuring 2 × ½ in and 3 × 1 in were detected reliably regardless of the operative site. » × » in neuropatties were poorly detected by neurosurgeons and radiologists in all three operative sites. Readers of various experience and background were similar in their ability to detect neuropatties under these conditions. CONCLUSIONS: Under simulated operating room conditions and using currently available neuropatties and plain radiograph imaging technology, small »-in and ½-in neuropatties are poorly visible/detectable on digital images.
Subject(s)
Foreign Bodies/diagnosis , Neurosurgical Procedures/adverse effects , Skull/diagnostic imaging , Spine/diagnostic imaging , Surgical Sponges , Adult , Cadaver , Foreign Bodies/etiology , Humans , Male , Models, Anatomic , Neurosurgical Procedures/instrumentation , Observer Variation , Radiography , Sensitivity and Specificity , Skull/surgery , Spine/surgeryABSTRACT
Studies on gold kiwifruit pectins are limited. In this work, the characterization of pectin isolated from two different stages of maturity of gold kiwifruit, namely early harvested fruit (EHF) and main harvested fruit (MHF) isolated by three methods (acid, water, enzymatic) was carried out. Pectins isolated from MHF were higher in galacturonic acid content (52-59% w/w) and weight-average molecular weights (Mw, 1.7-3.8 × 10(6)g/mol) compared with EHF pectins (29-49% w/w and 0.2-1.7 × 10(6)g/mol respectively). Enzymatic treatment gave the highest yield but lowest in Mw, viscosity and mechanical spectra for both maturities. The pectin of both maturities was classified as high-methoxyl pectin with the degree of esterification ranged from 82% to 90%. Water-extracted MHF pectin molecules had the highest RMS radius (182.7 nm) and Mw (3.75 × 10(6)g/mol). The water extraction method appeared to retain the native state of pectin molecules compared with acid and enzymatic extraction methods based on the Mw and viscosity data.
Subject(s)
Actinidia/chemistry , Pectins/isolation & purification , Fruit/chemistry , Molecular Weight , Plant Proteins/analysis , ViscosityABSTRACT
The effects of Celluclast 1.5L concentration on the physicochemical characterization of gold kiwifruit pectin was evaluated. Varying the enzyme concentration affected the pectin yield and pectin physicochemical properties. The viscosity of extracted pectin was largely dependent on the enzyme concentration. Celluclast 1.5L with medium concentration exhibited the highest viscosity. Varying the enzyme concentration also influenced the molecular weight distribution. High molecular weight (M(w)) pectin (1.65 × 10(6) g/mol) was obtained when the medium concentration was used. Overall, the study clearly reflects the importance of taking into consideration the amount of cellulytic enzyme added in order to determine the final quality of pectin.
Subject(s)
Actinidia/metabolism , Enzymes/metabolism , Pectins/chemistry , Actinidia/chemistry , Ethanol/chemistry , Fruit/chemistry , Fruit/metabolism , Molecular Weight , Pectins/isolation & purification , Pectins/metabolism , Plant Extracts/chemistry , ViscosityABSTRACT
Sintered stainless steel (SSS) microfiltration membranes, which served as electrode directly, were used for the experiment of separating Alamin, a calcium salt and protein containing particles, found in dairy processing. Fouling and cleaning of the SSS membranes under the application of an external electric field were studied. The imposed electric field was found, diverging the pH of permeate and retentate. This in turn altered the solubility of the calcium salt and impacted the performance of electro microfiltration membrane. Using electric field as an enhanced cleaning-in-place (CIP) method in back flushing SSS membrane was also studied.
ABSTRACT
Following extracorporeal membrane oxygenation (ECMO), two patients subsequently developed carotid aneurysms at the site of cannulation. Given the invasive nature of ECMO, vascular ultrasound and/or computerized tomographic imaging should be considered to rule out cannulation-site complications post-ECMO.
Subject(s)
Carotid Artery Injuries/etiology , Extracorporeal Membrane Oxygenation/adverse effects , Intracranial Aneurysm/etiology , Adolescent , Carotid Artery Injuries/diagnosis , Carotid Artery Injuries/surgery , Diagnosis, Differential , Diagnostic Imaging , Female , Heart Defects, Congenital/surgery , Humans , Imaging, Three-Dimensional , Infant , Intracranial Aneurysm/diagnosis , Intracranial Aneurysm/surgery , MaleABSTRACT
A right aortic arch, with a retroesophageal transverse arch and an aberrant left brachiocephalic artery, is a rare anomaly by itself. When combined with severe mitral stenosis and hypoplasia of the left ventricle, it is rarer still. We describe the diagnosis and treatment of this rare anomaly in an infant using a modification of the Norwood operation.
Subject(s)
Abnormalities, Multiple/diagnosis , Aorta, Thoracic/abnormalities , Brachiocephalic Trunk/abnormalities , Cardiac Surgical Procedures/methods , Hypoplastic Left Heart Syndrome/diagnosis , Abnormalities, Multiple/surgery , Combined Modality Therapy , Echocardiography, Transesophageal , Female , Follow-Up Studies , Heart Defects, Congenital/diagnosis , Heart Defects, Congenital/surgery , Humans , Hypoplastic Left Heart Syndrome/surgery , Infant, Newborn , Magnetic Resonance Angiography , Risk Assessment , Treatment OutcomeABSTRACT
Pneumothorax is a potentially life-threatening condition in the setting of the neonatal special-care nursery (SCN) that may result in rapid deterioration and death. The familiar appearances associated with pneumothorax on AP supine chest radiograph are highly specific, but limited in sensitivity. In this case report, we describe the theory and technique of thoracic ultrasound for detection of pneumothorax in the SCN, providing a viable alternative to the cross-table lateral radiograph without ionising radiation, with highly accurate results, and with minimal patient positioning.
Subject(s)
Infant, Premature, Diseases/diagnostic imaging , Pneumothorax/diagnostic imaging , Chest Tubes , Humans , Infant, Newborn , Infant, Premature , UltrasonographyABSTRACT
BACKGROUND: Magnetic resonance (MR) imaging is frequently used to diagnose arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). However, the reliability of various MR imaging features for diagnosing ARVC/D is unknown. The purpose of this study was to determine which morphologic MR imaging features have the greatest interobserver reliability for diagnosing ARVC/D. METHODS: Forty-five sets of films of cardiac MR images were sent to 8 radiologists and 5 cardiologists with experience in this field. There were 7 cases of definite ARVC/D as defined by the Task Force criteria. Six cases were controls. The remaining 32 cases had MR imaging because of clinical suspicion of ARVC/D. Readers evaluated the images for the presence of (a) right ventricle (RV) enlargement, (b) RV abnormal morphology, (c) left ventricle enlargement, (d) presence of high T(1) signal (fat) in the myocardium, and (e) location of high T(1) signal (fat) on a Likert scale with formatted responses. RESULTS: Readers indicated that the Task Force ARVC/D cases had significantly more (chi(2) = 119.93, d.f. = 10, p < 0.0001) RV chamber size enlargement (58%) than either the suspected ARVC/D (12%) or no ARVC/D (14%) cases. When readers reported the RV chamber size as enlarged they were significantly more likely to report the case as ARVC/D present (chi(2)(= )33.98, d.f. = 1, p < 0.0001). When readers reported the morphology as abnormal they were more likely to diagnose the case as ARVC/D present (chi(2) = 78.4, d.f. = 1, p < 0.0001), and the Task Force ARVC/D (47%) cases received significantly more abnormal reports than either suspected ARVC/D (20%) or non-ARVC/D (15%) cases. There was no significant difference between patient groups in the reported presence of high signal intensity (fat) in the RV (chi(2) = 0.9, d.f. = 2, p > 0.05). CONCLUSIONS: Reviewers found that the size and shape of abnormalities in the RV are key MR imaging discriminates of ARVD. Subsequent protocol development and multicenter trials need to address these parameters. Essential steps in improving accuracy and reducing variability include a standardized acquisition protocol and standardized analysis with dynamic cine review of regional RV function and quantification of RV and left ventricle volumes.
Subject(s)
Arrhythmogenic Right Ventricular Dysplasia/diagnosis , Magnetic Resonance Imaging/methods , Adult , Aged , Case-Control Studies , Electrocardiography , Female , Humans , Male , Middle Aged , Observer Variation , Probability , Reference Values , Registries , Sensitivity and Specificity , Severity of Illness IndexABSTRACT
Coronary artery anomalies are a well recognized feature of many cardiac malformations and have been catalogued in a number of reviews. This overview concentrates on 1) the interplay between congenital heart defects and coronary morphogenesis, examining how some of the embryology fits with the experiments of nature encountered in clinical practice; and 2) the influence of coronary anatomy on patient management. This overview uses, as examples, pulmonary atresia with intact ventricular septum, complete and congenitally corrected transpositions of the great arteries, and tetralogy of Fallot.