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https://www.readbyqxmd.com/read/27922961/costochondral-graft-in-young-children-with-hemifacial-microsomia
#1
Bin-Zhang Wu, Lian Ma, Yang Li, Shuo Chen, Biao Yi
Patients with severely hypoplastic mandibles usually require condylar reconstruction. This study aimed to describe costochondral graft (CCG) for condylar reconstruction and report subsequent outcomes of these grafts in young children with Pruzansky/Kaban type IIB and type III mandibular hypoplasia. This study included 4 young children with type IIB and type III hemifacial microsomia treated with CCG to reconstruct the condyle at the Department of Oral and Maxillofacial Surgery in our hospital from March 2008 to March 2014...
December 5, 2016: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/27920266/variability-of-ponto-cerebellar-fibers-by-diffusion-tensor-imaging-in-diverse-brain-malformations
#2
Nancy K Rollins, Timothy N Booth, Maria H Chahrour
To describe pontine axonal anomalies across diverse brain malformations. Institutional review board-approved review of magnetic resonance imaging (MRI) and genetic testing of 31 children with brain malformations and abnormal pons by diffusion tensor imaging. Anomalous dorsal pontocerebellar tracts were seen in mid-hindbrain anomalies and in diffuse malformations of cortical development including lissencephaly, gyral disorganization with dysplastic basal ganglia, presumed congenital fibrosis of extraocular muscles type 3, and in callosal agenesis without malformations of cortical development...
December 5, 2016: Journal of Child Neurology
https://www.readbyqxmd.com/read/27913534/myelodysplastic-and-myeloproliferative-disorders-of-childhood
#3
Henrik Hasle
Myelodysplastic syndrome (MDS) and myeloproliferative disorders are rare in children; they are divided into low-grade MDS (refractory cytopenia of childhood [RCC]), advanced MDS (refractory anemia with excess blasts in transformation), and juvenile myelomonocytic leukemia (JMML), each with different characteristics and management strategies. Underlying genetic predisposition is recognized in an increasing number of patients. Germ line GATA2 mutation is found in 70% of adolescents with MDS and monosomy 7. It is challenging to distinguish RCC from aplastic anemia, inherited bone marrow failure, and reactive conditions...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913517/treatment-of-low-risk-myelodysplastic-syndromes
#4
Valeria Santini
The majority of myelodysplastic syndrome (MDS) patients belong to the International Prognostic Scoring System (IPSS) and IPSS-revised (IPSS-R) lower-risk categories. Their precise diagnostics and prognostic stratification is often a challenge, but may ensure the optimization of therapy. The availability of diverse treatment options has significantly improved the quality of life and survival of this group of patients. Anemia is the most relevant cytopenia in terms of frequency and symptoms in lower-risk MDS, and may be treated successfully with erythropoietic stimulating agents, provided a careful selection is performed on the basis of IPSS-R, endogenous erythropoietin levels, and transfusion independence...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27909861/surgical-reconstruction-for-fibular-hemimelia
#5
REVIEW
Dror Paley
Fibular hemimelia presents with foot deformity and leg length discrepancy. Previous classifications have focused on the degree of fibular deficiency rather than the type of foot deformity. Published methods of surgical reconstruction have often failed due to residual or recurrent foot deformity. The purpose of this report is to introduce new classification and reconstruction methods. The Paley SHORDT procedure is used to stabilize the ankle when there is a hypoplastic distal fibula with a dynamic valgus deformity...
December 1, 2016: Journal of Children's Orthopaedics
https://www.readbyqxmd.com/read/27908945/norwood-procedure-for-palliation-of-hypoplastic-left-heart-syndrome-right-ventricle-to-pulmonary-artery-conduit-vs-modified-blalock-taussig-shunt
#6
Dorothy M Beke
Patients with hypoplastic left heart syndrome undergo a series of operations to separate the pulmonary and systemic circulations. The first of at least 3 operations occurs in the newborn period, with a stage I palliation. The goal of stage I palliation is to provide pulmonary blood flow and create an unobstructed systemic outflow tract. Advances in surgical techniques and intraoperative and postoperative care have helped decrease morbidity and mortality for patients with hypoplastic left heart syndrome who have the stage I Norwood operation, but the patients continue to be at increased risk for hemodynamic collapse and adverse outcomes...
December 2016: Critical Care Nurse
https://www.readbyqxmd.com/read/27896819/extracorporeal-membrane-oxygenation-outcomes-after-the-comprehensive-stage-ii-procedure-in-patients-with-single-ventricles
#7
Daniel Gomez, Vicky Duffy, Diane Hersey, Carl Backes, Peter Rycus, Patrick McConnell, Jordan Voss, Mark Galantowicz, Clifford L Cua
Outcomes for extracorporeal membrane oxygenation (ECMO) have been described for patients with single ventricle physiology (SVP) undergoing cavopulmonary connection (Glenn procedure). An alternative surgical pathway for patients with SVP consists of an initial hybrid procedure followed by a comprehensive Stage II procedure. No data exist describing the outcomes of patients requiring ECMO after the comprehensive Stage II procedure. The goal of this study is to describe the outcomes for patients who required ECMO after the comprehensive Stage II procedure...
November 29, 2016: Artificial Organs
https://www.readbyqxmd.com/read/27894567/proportion-of-selected-congenital-heart-defects-attributable-to-recognized-risk-factors
#8
Regina M Simeone, Sarah C Tinker, Suzanne M Gilboa, A J Agopian, Matthew E Oster, Owen J Devine, Margaret A Honein
PURPOSE: To assess the contribution of multiple risk factors for two congenital heart defects-hypoplastic left heart syndrome (HLHS) and tetralogy of Fallot (TOF). METHODS: We used data from the National Birth Defects Prevention Study (1997-2011) to estimate average adjusted population attributable fractions for several recognized risk factors, including maternal prepregnancy overweight-obesity, pregestational diabetes, age, and infant sex. RESULTS: There were 594 cases of isolated simple HLHS, 971 cases of isolated simple TOF, and 11,829 controls in the analysis...
December 2016: Annals of Epidemiology
https://www.readbyqxmd.com/read/27891328/abnormal-course-of-right-coronary-artery-together-with-variant-left-coronary-artery-and-partial-atresia-of-cicumflex-artery
#9
Rajani Singh
Heart is supplied by right and left coronary arteries. Therefore, the knowledge of normal and variant anatomy of both these arteries and their configurations is indispensable for proper understanding of diagnosis and treatment of dreaded cardiac diseases. During routine cadaveric dissection, a heart of an adult female with anomalous course of Right Coronary Artery (RCA), new variant of trifurcated Left Coronary Artery (LCA) and partial atresia of circumflex (CX) artery was detected. Trifurcation of LCA into anterior interventricular, CX and a ramus intermedius without giving accessory branches has been reported...
October 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27889377/retinoid-acid-induced-microrna-27b-3p-impairs-c2c12-myoblast-proliferation-and-differentiation-by-suppressing-%C3%AE-dystrobrevin
#10
Nan Li, Yi Tang, Bo Liu, Wei Cong, Chao Liu, Jing Xiao
We previously reported that excess retinoic acid (RA) resulted in hypoplastic and derangement of myofilaments in embryonic tongue by inhibiting myogenic proliferation and differentiation through CamKIID pathway. Our further studies revealed that the expression of a series of miRNAs was altered by RA administration in embryonic tongue as well as in C2C12 cells. Thus, if excess RA impairs myogenic proliferation and differentiation through miRNAs is taken into account. In present study, miR-27b-3p was found up-regulated in RA-treated C2C12 cells as in embryonic tongue, and predicted to target the 3'UTR of α-dystrobrevin (DTNA)...
November 23, 2016: Experimental Cell Research
https://www.readbyqxmd.com/read/27886392/deregulated-expression-of-ezh2-in-congenital-brainstem-disconnection
#11
P G Barth, E Aronica, S Fox, K Fluiter, M A J Weterman, A Poretti, D C Miller, E Boltshauser, B Harding, M Santi, F Baas
Congenital brainstem disconnection (CBSD) is an enigmatic embryo-fetal defect presenting as (sub)total absence of a segment between mesencephalon and lower brainstem. Rostro-caudal limits of the defect vary while the basal pons is always involved and the cerebellum is globally hypoplastic. A recent update and review[1] lists 14 cases, including 3 brain autopsy studies[1-3]. Necrosis and glial- or inflammatory reactions were absent. Inferior olivary nuclei were small or absent, pontine nuclei depleted, and the cerebellar dentate nuclei dysplastic...
November 25, 2016: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/27885446/post-cardiotomy-rescue-extracorporeal-cardiopulmonary-resuscitation-in-neonates-with-single-ventricle-after-intractable-cardiac-arrest-attrition-after-hospital-discharge-and-predictors-of-outcome
#12
Anastasios C Polimenakos, Vincent Rizzo, Chawki F El-Zein, Michel N Ilbawi
Extracorporeal cardiopulmonary resuscitation (ECPR) in children with cardiac arrest refractory to conventional cardiopulmonary resuscitation (CPR) has been reported with encouraging results. We reviewed outcomes of neonates with functional single ventricle (FSV) surviving post-cardiotomy ECPR after hospital discharge. Fifty-eight patients who required post-cardiotomy extracorporeal membrane oxygenation (ECMO) since the introduction of our ECPR protocol (January 2007-December 2011) were identified. Forty-one were neonates...
November 24, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27884484/ross-hybrid-arch-and-frozen-elephant-trunk-reconstruction-for-late-complications-of-bicuspid-aortic-valve-and-aortopathy
#13
Matthew Valdis, Gaetano DeRose, Linrui Guo, Michael W A Chu
Young patients with bicuspid aortic valve disease and aortopathy remain a clinical challenge, with many requiring multiple corrective operations throughout their lifetimes. Innovative surgical approaches are often required to address complex aortic pathologic conditions but leave patients at risk for reintervention, lifelong anticoagulation, and suboptimal hemodynamics. We describe an active 44-year-old female triathlete with recurrent bicuspid aortic stenosis, a small aortic root, a hypoplastic aortic arch and complex distal arch, and a descending aortic aneurysm, who underwent a single-stage reconstruction with a combined Ross procedure, hybrid arch, and frozen elephant trunk reconstruction...
December 2016: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/27882009/functional-and-structural-abnormalities-of-the-kidney-and-urinary-tract-in-severely-malnourished-children-a-hospital-based-study
#14
Misbah Anjum, Khemchand N Moorani, Ifra Sameen, Muhammad Ayaz Mustufa, Shazia Kulsoom
OBJECTIVES: The association of malnutrition and systemic diseases like chronic kidney disease (CKD) is well known. Various urinary tract abnormalities may be associated with malnutrition. So objective of current study was to determine the frequency of functional and structural urinary tract abnormalities in severely malnourished children admitted in Nutritional Rehabilitation Unit (NRU) of a tertiary care facility, Karachi. METHODS: This descriptive cases series of 78 children was conducted in NRU from October 2014 - March 2015...
September 2016: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/27879470/incidence-and-management-of-thrombotic-and-thromboembolic-complications-following-the-norwood-procedure-a-systematic-review
#15
Arnav Agarwal, Mohammed Firdouse, Nishaan Brar, Andy Yang, Panos Lambiris, Anthony K Chan, Tapas K Mondal
BACKGROUND: The stage 1 Norwood procedure and its variants represent the first step of palliation for hypoplastic left heart syndrome. Although appropriate postoperative thromboprophylaxis is integral, significant variance remains across institutional practices. The purpose of this systematic review is to estimate the incidence of thrombosis and thromboembolism following the Norwood or modified Blalock-Taussig shunt procedure and examine current thromboprophylaxis regimens. METHODS: Ovid MEDLINE and Embase were searched from January 2000 to June 2016 for primary studies explicitly reporting incidence of thrombosis, thromboembolism (strokes and pulmonary embolisms), or shunt occlusion in neonates, infants, and children undergoing the Norwood procedure or any variant...
November 21, 2016: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/27876863/intestinal-tuft-cells-regulate-the-atm-mediated-dna-damage-response-via-dclk1-dependent-mechanism-for-crypt-restitution-following-radiation-injury
#16
Parthasarathy Chandrakesan, Randal May, Nathaniel Weygant, Dongfeng Qu, William L Berry, Sripathi M Sureban, Naushad Ali, Chinthalapally Rao, Mark Huycke, Michael S Bronze, Courtney W Houchen
Crypt epithelial survival and regeneration after injury require highly coordinated complex interplay between resident stem cells and diverse cell types. The function of Dclk1 expressing tuft cells regulating intestinal epithelial DNA damage response for cell survival/self-renewal after radiation-induced injury is unclear. Intestinal epithelial cells (IECs) were isolated and purified and utilized for experimental analysis. We found that small intestinal crypts of Villin(Cre);Dclk1(f/f) mice were hypoplastic and more apoptotic 24 h post-total body irradiation, a time when stem cell survival is p53-independent...
November 23, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27870251/getting-to-the-core-of-inherited-bone-marrow-failures
#17
Soheir Adam, Dario Melguizo Sanchis, Ghada El-Kamah, Sujith Samarasinghe, Sameer Alharthi, Lyle Armstrong, Majlinda Lako
Bone marrow failure syndromes (BMFS) are a group of disorders with complex pathophysiology characterized by a common phenotype of peripheral cytopenia and/or a hypoplastic bone marrow. Understanding genetic factors contributing to the pathophysiology of BMFS has enabled the identification of causative genes and development of diagnostic tests. To date more than 40 mutations in genes involved in maintenance of genomic stability, DNA repair, ribosome and telomere biology have been identified. In addition, pathophysiological studies have provided insights into several biological pathways leading to the characterization of genotype/phenotype correlations as well as the development of diagnostic approaches and management strategies...
November 21, 2016: Stem Cells
https://www.readbyqxmd.com/read/27869673/3d-visualization-of-developmental-toxicity-of-2-4-6-trinitrotoluene-in-zebrafish-embryogenesis-using-light-sheet-microscopy
#18
Juneyong Eum, Jina Kwak, Hee Joung Kim, Seoyoung Ki, Kooyeon Lee, Ahmed A Raslan, Ok Kyu Park, Md Ashraf Uddin Chowdhury, Song Her, Yun Kee, Seung-Hae Kwon, Byung Joon Hwang
Environmental contamination by trinitrotoluene is of global concern due to its widespread use in military ordnance and commercial explosives. Despite known long-term persistence in groundwater and soil, the toxicological profile of trinitrotoluene and other explosive wastes have not been systematically measured using in vivo biological assays. Zebrafish embryos are ideal model vertebrates for high-throughput toxicity screening and live in vivo imaging due to their small size and transparency during embryogenesis...
November 17, 2016: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/27865477/tracheostomy-among-infants-with-hypoplastic-left-heart-syndrome-undergoing-cardiac-operations-a-multicenter-analysis
#19
Parthak Prodhan, Amit Agarwal, Nahed O ElHassan, Elijah H Bolin, Brandon Beam, Xiomara Garcia, Michael Gaies, Xinyu Tang
BACKGROUND: Less than 2.7% of infants undergoing congenital heart disease operations have difficulty weaning from invasive mechanical ventilation. In such instances, clinicians may choose to perform tracheostomy. Limited literature has examined tracheostomy placement specifically in infants with hypoplastic left heart syndrome (HLHS). This study evaluated the risk factors for tracheostomy placement in infants with HLHS and examined the outcomes of these infants before their first hospital discharge...
November 16, 2016: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/27860551/congenital-variants-and-anomalies-of-the-aortic-arch
#20
Kate Hanneman, Beverley Newman, Frandics Chan
Congenital variants and anomalies of the aortic arch are important to recognize as they may be associated with vascular rings, congenital heart disease, and chromosomal abnormalities, and can have important implications for prognosis and management. The purpose of this article is to review cross-sectional imaging techniques used in the evaluation of the aortic arch, describe the embryology and anatomy of the aortic arch system, discuss aortic arch variants and anomalies, and review other malformations of the aortic arch, including interrupted aortic arch, hypoplastic aortic arch, and aortic coarctation...
November 18, 2016: Radiographics: a Review Publication of the Radiological Society of North America, Inc
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