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https://www.readbyqxmd.com/read/28211263/increased-regurgitant-flow-causes-endocardial-cushion-defects-in-an-avian-embryonic-model-of-congenital-heart-disease
#1
Stephanie M Ford, Matthew T McPheeters, Yves T Wang, Pei Ma, Shi Gu, James Strainic, Christopher Snyder, Andrew M Rollins, Michiko Watanabe, Michael W Jenkins
BACKGROUND: The relationship between changes in endocardial cushion and resultant congenital heart diseases (CHD) has yet to be established. It has been shown that increased regurgitant flow early in embryonic heart development leads to endocardial cushion defects, but it remains unclear how abnormal endocardial cushions during the looping stages might affect the fully septated heart. The goal of this study was to reproducibly alter blood flow in vivo and then quantify the resultant effects on morphology of endocardial cushions in the looping heart and on CHDs in the septated heart...
February 17, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28206901/assessment-of-enamel-bond-strength-of-hypoplastic-primary-teeth
#2
Lisa Scheidt, Mariane Emi Sanabe, Fabrãcio Mezzomo Collares, Vicente Castelo Branco Leitune, Eduardo Bresciani, Michele Baffi Diniz
PURPOSE: The purpose of this study was to compare the enamel bond strength of hypoplastic and healthy primary teeth. METHODS: Twenty-five hypoplastic teeth (experimental group) and 25 healthy primary teeth (control group) were selected and conditioned with 35 percent orthophosphoric acid before a two-layer adhesive system (Single Bond) was applied. Composite resin (Filtek Z350) was inserted in a single application, two-mm high and one-mm in diameter, and then light-cured for 20 seconds...
October 15, 2016: Pediatric Dentistry
https://www.readbyqxmd.com/read/28193110/pontine-tegmental-cap-dysplasia-in-an-extremely-preterm-infant-and-review-of-the-literature
#3
Sylvie Picker-Minh, Sebastian Hartenstein, Hans Proquitté, Sebastian Fröhler, Vera Raile, Nadine Kraemer, Sarah Apeshiotis, Michael Leipoldt, Karim D Kalache, Deborah Morris-Rosendahl, Eugen Boltshauser, Wei Chen, Angela M Kaindl
Pontine tegmental cap dysplasia is a rare hindbrain malformation syndrome with a hypoplastic pons, a tissue protrusion into the fourth ventricle, and cranial nerve dysfunction. We here report clinical, imaging, and genetic findings of the first extremely low-birth-weight preterm infant with pontine tegmental cap dysplasia born at 25 weeks of gestation and provide an overview of 29 sporadic cases. A prenatally diagnosed hypoplastic and rostrally shifted cerebellum was indicative of a hindbrain defect and later identified as an early sign of pontine tegmental cap dysplasia in our patient...
March 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/28191791/urethral-duplication-type-influences-on-the-complications-rate-and-number-of-surgical-procedures
#4
Roberto Iglesias Lopes, Amilcar Martins Giron, Marcos Figueiredo Mello, Cristovao Machado Barbosa Neto, Joana Dos Santos, Paulo Renato Marcelo Moscardi, Victor Srougi, Francisco Tibor Denes, Miguel Srougi
INTRODUCTION: Urethral duplication is rare. Characterized by the presence of two urethral channels. This anomaly presents a great variety of clinical findings that depend on the type of duplication that often is associated with other anomalies. MATERIAL AND METHODS: We report thirteen boys with urethral duplication managed in our institution between 1988-2015. Clinical findings, associated anomalies, treatment of urethral duplication and our results are described...
January 27, 2017: International Braz J Urol: Official Journal of the Brazilian Society of Urology
https://www.readbyqxmd.com/read/28191731/right-ventricular-systolic-function-in-hypoplastic-left-heart-syndrome-a-comparison-of-manual-and-automated-software-to-measure-fractional-area-change
#5
Hanna K Ruotsalainen, Hannah R Bellsham-Revell, Aaron J Bell, Jaana I Pihkala, Tiina H Ojala, John M Simpson
BACKGROUND: Quantitative echocardiographic assessment of right ventricular function is important in children with hypoplastic left heart syndrome (HLHS). The aim of this study was to examine the repeatability of different echocardiographic techniques, both manual and automated, to measure fractional area change (FAC) in patients with HLHS and to correlate these measurements with magnetic resonance imaging (MRI)-derived ejection fraction (EF). METHODS: Fifty-one children with HLHS underwent transthoracic echocardiography and cardiac MRI under the same general anesthetic as part of routine inter-stage assessment...
February 13, 2017: Echocardiography
https://www.readbyqxmd.com/read/28190605/the-importance-of-age-and-weight-on-cavopulmonary-shunt-stage-ii-outcomes-after-the-norwood-procedure-planned-versus-unplanned-surgery
#6
David J Barron, Intisar Ul Haq, Adrian Crucean, John Stickley, Phil Botha, Natasha Khan, Timothy J Jones, William J Brawn
OBJECTIVE: The study objective was to evaluate the outcomes of the cavopulmonary shunt after the Norwood procedure with a particular focus on age, weight, and whether surgery was planned or expedited by clinical findings. METHODS: We studied 297 consecutive patients with hypoplastic left heart syndrome undergoing the cavopulmonary shunt operation between 2002 and 2014. All patients underwent the Norwood procedure with a right ventricle to pulmonary artery conduit...
January 17, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28188961/high-serum-progesterone-associated-with-infertility-in-a-woman-with-nonclassic-congenital-adrenal-hyperplasia
#7
Yoshimasa Kawarai, Hiroshi Ishikawa, Tomoya Segawa, Shokichi Teramoto, Tomoaki Tanaka, Makio Shozu
Nonclassic congenital adrenal hyperplasia (NCAH) is an autosomal-recessive disorder caused by 21-hydroxylase deficiency and manifests as hirsutism and oligomenorrhea due to excess adrenal androgen and progesterone. We report a case of a woman with NCAH who showed continuous high serum progesterone levels in the follicular phase associated with impaired folliculogenesis. NCAH was diagnosed based on high 17-hydroxyprogesterone levels after rapid adrenocorticotropic hormone loading, and three heterozygous missense mutations in CYP21A2, encoding 21-hydroxylase, were identified...
February 11, 2017: Journal of Obstetrics and Gynaecology Research
https://www.readbyqxmd.com/read/28188034/follistatin-like-1-expression-is-decreased-in-the-alveolar-epithelium-of-hypoplastic-rat-lungs-with-nitrofen-induced-congenital-diaphragmatic-hernia
#8
Toshiaki Takahashi, Julia Zimmer, Florian Friedmacher, Prem Puri
BACKGROUND/PURPOSE: Pulmonary hypoplasia (PH), characterized by incomplete alveolar development, remains a major therapeutic challenge associated with congenital diaphragmatic hernia (CDH). Follistatin-like 1 (Fstl1) is a crucial regulator of alveolar formation and maturation, which is strongly expressed in distal airway epithelium. Fstl1-deficient mice exhibit reduced airspaces, impaired alveolar epithelial cell differentiation, and insufficient production of surfactant proteins similar to PH in human CDH...
January 28, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28185787/progression-of-renal-fibrosis-in-congenital-ckd-model-rats-with-reduced-number-of-nephrons
#9
Hidenori Yasuda, Yuki Tochigi, Kentaro Katayama, Hiroetsu Suzuki
A congenital reduction in the number of nephrons is a critical risk factor for both onset of chronic kidney disease (CKD) and its progression to end-stage kidney disease (ESKD). Hypoplastic kidney (HPK) rats have only about 20% of the normal number of nephrons and show progressive CKD. This study used an immunohistological method to assess glomerular and interstitial pathogenesis in male HPK rats aged 35-210days. CD68 positive-macrophages were found to infiltrate into glomeruli in HPK rats aged 35 and 70days and to infiltrate into interstitial tissue in rats aged 140 and 210days...
February 6, 2017: Experimental and Toxicologic Pathology: Official Journal of the Gesellschaft Für Toxikologische Pathologie
https://www.readbyqxmd.com/read/28184977/lateral-atrial-tunnel-fontan-operation-predisposes-to-the-junctional-rhythm
#10
Katarzyna Januszewska, Anna Schuh, Anja Lehner, Robert Dalla-Pozza, Edward Malec
The goal of the study was to compare the early postoperative course after fenestrated lateral atrial tunnel (LT) and non-fenestrated extracardiac conduit (EC) Fontan operation (FO) in a single center where both techniques were parallelly used. Between 2004 and 2012, 56(32.7%) children underwent LT and 113(66.1%) EC FO. The mean age was 3.7 ± 2.9 years and mean weight was 14.6 ± 7.3 kg. The most common malformation was hypoplastic left heart syndrome (50.9%). The general approach was to perform LT in children after hemi-Fontan operation and EC in patients after Glenn anastomosis...
February 10, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28174392/-heart-transplantation-allograft-and-xenograft
#11
Norihide Fukushima
Prior to starting clinical cardiac allotransplantation, cardiac xenotransplantation was performed in human in 1960s. In 1964, Hardy performed cardiac transplantation using a chimpanzee heart and Bailey performed cardiac transplantation using a baboon heart to an infant with hypoplastic left heart. The use of cyclosporine has greatly improved the outcome of clinical cardiac transplantation and cardiac allotransplantation became an established treatment strategy for the patients with end-stage heart failure. Although concordant cardiac xenotransplantation from a primate to a human may be successfully performed using current immunosuppressive regimen, a primate heart is not a good candidate for cardiac xenograft due to animal light issues and its size...
January 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28169036/identification-of-risk-factors-for-poor-feeding-in-infants-with-congenital-heart-disease-and-a-novel-approach-to-improve-oral-feeding
#12
Gitanjali Indramohan, Tiffany P Pedigo, Nicole Rostoker, Mae Cambare, Tristan Grogan, Myke D Federman
Many infants with complex congenital heart disease (CHD) do not develop the skills to feed orally and are discharged home on gastrostomy tube or nasogastric feeds. We aimed to identify risk factors for failure to achieve full oral feeding and evaluate the efficacy of oral motor intervention for increasing the rate of discharge on full oral feeds by performing a prospective study in the neonatal and cardiac intensive care units of a tertiary children's hospital. 23 neonates born at ≥37weeks gestation and diagnosed with single-ventricle physiology requiring a surgical shunt were prospectively enrolled and received oral motor intervention therapy...
February 3, 2017: Journal of Pediatric Nursing
https://www.readbyqxmd.com/read/28161406/glans-duplication-in-depth-review-and-proposal-of-a-new-classification
#13
REVIEW
Lisieux E Jesus, Samuel Dekermacher, Erica Lopes, Andreia P Bacon
BACKGROUND: Diphallia is a very uncommon malformation, and glans duplication (GD) is its rarest form. In this last group, patients normally present with esthetic/sexual complaints or obstructed voiding late in life after pubertal genital development. Associated malformations are uncommon and relatively mild in those cases. METHODS: After a case presented, we present herein an extensive review of GD, as the disease is rare, and there is very little about its treatment and there are diverse approaches to address the condition...
January 18, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28149076/knock-and-drill-technique-a-simple-tips-for-the-instrumentation-in-complex-craniovertebral-junction-anomalies-without-using-fluoroscopy
#14
Arun Srivastava, Jayesh Sardhara, Sanjay Behari, Sindgikar Pavaman, Jeena Joseph, Kuntal Das, Anant Mehrotra, Awadhesh K Jaiswal, Kamlesh Bhaishora
CONTEXT: Existence of complex variable bony and vertebral artery (VA) anomalies at craniovertebral junction (CVJ) in subset of complex CVJ anomalies demands individualized instrumentation policy and placing screws in each bone requires strategic preoperative planning and intraoperative skills. AIM: To evaluate the clinical accuracy of knock and drill (K and D) technique for the screw placement in complex CVJ anomalies. SETTINGS AND DESIGN: Prospective study and operative technical note...
January 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28148317/echocardiography-of-coarctation-of-the-aorta-aortic-arch-hypoplasia-and-arch-interruption-strategies-for-evaluation-of-the-aortic-arch
#15
REVIEW
Suma P Goudar, Sanket S Shah, Girish S Shirali
: Aim Echocardiography is the modality of choice for the diagnosis and serial follow-up of aortic arch pathology. In this article, we review the types of obstruction of the aortic arch, various classification schemes of coarctation of the aorta and interrupted aortic arch, methodology for optimal echocardiographic imaging of the aortic arch, and key echocardiographic measurements for accurate diagnosis of obstruction and hypoplasia of the aortic arch. Finally, we will discuss the limitations of echocardiography in optimal imaging of the aortic arch and the use of other non-invasive imaging modalities such as CT or MRI to provide additional information in these cases...
December 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/28145930/combined-endoscopic-and-trans-palpebral-orbital-reconstruction-for-silent-sinus-syndrome
#16
Riccardo Tieghi, Nicola Malagutti, Luisa Valente, Giulia Carnevali, Luigi C Clauser
Silent Sinus Syndrome is defined as a painless spontaneous and progressive enophthalmos and hypoglobus with maxillary sinus hypoplasia and orbital floor resorption. It is caused by maxillary sinus atelectasis in a setting of ipsilateral chronic maxillary sinus hypoventilation. The syndrome was first described in 1964 by Montgomery, but the term "Silent Sinus Syndrome" was not coined until 1994 by Soparkar. The aetiology is still controversial: some authors postulate a basal hypoplastic sinus, other suggest an acquired process due to an obstruction of the ostium in the medium meatus...
January 31, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28142228/notch1-dependent-nitric-oxide-signaling-deficiency-in-hypoplastic-left-heart-syndrome-revealed-through-patient-specific-phenotypes-detected-in-bioengineered-cardiogenesis
#17
Sybil C L Hrstka, Xing Li, Timothy J Nelson
Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect (CHD) attributable to multifactorial molecular underpinnings. Multiple genetic loci have been implicated to increase the risk of disease, yet genotype-phenotype relationships remain poorly defined. Whole genome sequencing complemented by cardiac phenotype from five individuals in an HLHS-affected family enabled the identification of NOTCH1 as a prioritized candidate gene linked to CHD in three individuals with mutant allele burden significantly impairing Notch signaling in the HLHS-affected proband...
January 31, 2017: Stem Cells
https://www.readbyqxmd.com/read/28138333/short-stature-and-hypoparathyroidism-in-a-child-with-kenny-caffey-syndrome-type-2-due-to-a-novel-mutation-in-fam111a-gene
#18
Mary B Abraham, Dong Li, Dave Tang, Susan M O'Connell, Fiona McKenzie, Ee Mun Lim, Hakon Hakonarson, Michael A Levine, Catherine S Choong
BACKGROUND: Hypoparathyroidism in children is a heterogeneous group with diverse genetic etiologies. To aid clinicians in the investigation and management of children with hypoparathyroidism, we describe the phenotype of a 6-year-old child with hypoparathyroidism and short stature diagnosed with Kenny-Caffey syndrome (KCS) Type 2 and the subsequent response to growth hormone (GH) treatment. CASE PRESENTATION: The proband presented in the neonatal period with hypocalcemic seizures secondary to hypoparathyroidism...
2017: International Journal of Pediatric Endocrinology
https://www.readbyqxmd.com/read/28132535/modified-total-cavopulmonary-shunt-as-a-staged-fontan-operation
#19
Hidetsugu Asai, Tsuyoshi Tachibana, Yasushige Shingu, Hiroki Kato, Satoru Wakasa, Yoshiro Matsui
The left superior vena cava became occluded in an infant with hypoplastic left heart syndrome. After a bidirectional Glenn procedure, he presented with severe oxygen desaturation and right ventricular dysfunction; the left superior vena cava drained into the inferior vena cava through collateral veins. As salvage therapy, we created a modified total cavopulmonary shunt using only autologous tissue in which the right hepatic vein and inferior vena cava drained into the pulmonary artery via a lateral tunnel in the right atrium...
January 1, 2017: Asian Cardiovascular & Thoracic Annals
https://www.readbyqxmd.com/read/28130977/a-novel-amelx-mutation-causes-hypoplastic-amelogenesis-imperfecta
#20
Young-Jae Kim, Youn Jung Kim, Jenny Kang, Teo Jeon Shin, Hong-Keun Hyun, Sang-Hoon Lee, Zang Hee Lee, Jung-Wook Kim
OBJECTIVES: Amelogenesis imperfecta (AI) is a hereditary genetic defect affecting tooth enamel. AI is heterogeneous in clinical phenotype as well as in genetic etiology. To date, more than 10 genes have been associated with the etiology of AI. Amelogenin is the most abundant enamel matrix protein, most of which is encoded by the amelogenin gene in the X-chromosome (AMELX). More than 16 alternative splicing transcripts have been identified in the murine Amelx gene. The purpose of this study was to identify the genetic cause of an AI family...
January 12, 2017: Archives of Oral Biology
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