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https://www.readbyqxmd.com/read/28231640/mind-the-gap-mechanisms-regulating-the-endothelial-barrier
#1
REVIEW
Mariya Y Radeva, Jens Waschke
The endothelial barrier consists of intercellular contacts localized in the cleft between endothelial cells, which is covered by the glycocalyx in a sieve-like manner. Both types of barrier-forming junctions, i.e. the adherens junction (AJ) serving mechanical anchorage and mechanotransduction and the tight junction (TJ) sealing the intercellular space to limit paracelullar permeability, are tethered to the actin cytoskeleton. Under resting conditions, the endothelium thereby builds a selective layer controlling the exchange of fluid and solutes with the surrounding tissue...
February 23, 2017: Acta Physiologica
https://www.readbyqxmd.com/read/28230602/subcutaneous-c-shape-muscular-flap-for-correcting-the-depression-of-alar-base-in-affected-side-in-patients-with-unilateral-complete-cleft-lip-palate-during-primary-surgery
#2
Dandan Wu, Guomin Wang, Ningjuan Ouyang, Yuhen Lin, Yang Chen, Jiewen Dai
PURPOSE: The depression of alar base in affected side in patients with unilateral complete cleft lip/palate (CL/P) is one of common clinical features. In this study, the authors try to explore the effect of subcutaneous C shape muscular flap for correcting the depression of alar base in affected side in patients with unilateral complete CL/P during primary surgery. METHODS: A total of 30 patients with unilateral complete CL/P who received primary correction of the lip nose deformity were included in this study...
February 22, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28230601/ankyloblepharon-ectodermal-defects-cleft-lip-palate-syndrome
#3
Siyuan Guo, Renji Chen, Yi Xu, Yue Mu, Linfeng Chen
Ankyloblepharon-ectodermal defects-cleft lip/palate (AEC) syndrome, also known as Hay-Wells syndrome, is an autosomal genetic disease with the main features of ankyloblepharon filiforme adnatum, ectodermal defects, and cleft lip/palate. The authors report a patient with 17 months old girl with AEC syndrome having ankyloblepharon, cleft and palate, and ectrodactyly with some associated features. Etiology, clinical features, differential diagnosis, and treatment have been elaborated in this clinical report.
February 22, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28230599/a-targeted-next-generation-genetic-sequencing-study-on-tetralogy-of-fallot-combined-with-cleft-lip-and-palate
#4
Lin Liu, Haisong Bu, Yifeng Yang, Zhiping Tan, Fei Zhang, Shijun Hu, Tianli Zhao
BACKGROUND: Congenital heart disease (CHD), plus cleft lip and palate (CLP) are currently the most common types of structural malformation in infants. Many genes have been investigated for their involvement in CHD with CLP. Targeted next-generation sequencing can analyze large amounts of genetic information rapidly, and thus address this question. METHODS: The authors designed a targeted, next-generation sequencing gene panel for 455 genes previously implicated in CHD or CLP...
February 22, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28230594/alveolar-molding-effect-in-infants-with-unilateral-cleft-lip-and-palate-comparison-of-two-and-three-dimensional-measurements
#5
Won Hee Lim, Eun Woo Park, Hwa Sung Chae, Soon Man Kwon, Hoi-In Jung, Seung-Hak Baek
The purpose of this study was to compare the results of two- (2D) and three-dimensional (3D) measurements for the alveolar molding effect in patients with unilateral cleft lip and palate. The sample consisted of 23 unilateral cleft lip and palate infants treated with nasoalveolar molding (NAM) appliance. Dental models were fabricated at initial visit (T0; mean age, 23.5 days after birth) and after alveolar molding therapy (T1; mean duration, 83 days). For 3D measurement, virtual models were constructed using a laser scanner and 3D software...
February 22, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28229453/chondrodysplasia-with-multiple-dislocations-comprehensive-study-of-a-series-of-30-cases
#6
E Ranza, C Huber, N Levin, G Baujat, C Bole-Feysot, P Nitschke, C Masson, Y Alanay, L Al-Gazali, P Bitoun, O Boute, P Campeau, C Coubes, M McEntagart, N Elcioglu, L Faivre, A Gezdirici, D Johnson, E Mihci, B G Nur, L Perrin, C Quelin, P Terhal, B Tuysuz, V Cormier-Daire
The group of chondrodysplasia with multiple dislocations includes several entities, characterized by short stature, dislocation of large joints, hand and/or vertebral anomalies. Other features, such as epiphyseal or metaphyseal changes, cleft palate, intellectual disability are also often part of the phenotype. In addition, several conditions with overlapping features are related to this group and broaden the spectrum. The majority of these disorders have been linked to pathogenic variants in genes encoding proteins implicated in the synthesis or sulfation of proteoglycans (PG)...
October 13, 2016: Clinical Genetics
https://www.readbyqxmd.com/read/28228961/concurrent-van-der-woude-syndrome-and-turner-syndrome-a-case-report
#7
Evan Los, Hayley Baines, Ines Guttmann-Bauman
Most cases of Van der Woude syndrome are caused by a mutation to interferon regulatory factor 6 on chromosome 1. Turner syndrome is caused by complete or partial absence of the second sex chromosome in girls. We describe a unique case of the two syndromes occurring concurrently though apparently independently in a girl with Van der Woude syndrome diagnosed at birth and Turner syndrome at 14 years 9 months. Short stature was initially misattributed to Van der Woude syndrome and pituitary insufficiency associated with clefts before correctly diagnosing Turner syndrome...
2017: SAGE open medical case reports
https://www.readbyqxmd.com/read/28223522/conformational-dynamics-of-a-neurotransmitter-sodium-symporter-in-a-lipid-bilayer
#8
Suraj Adhikary, Daniel J Deredge, Anu Nagarajan, Lucy R Forrest, Patrick L Wintrode, Satinder K Singh
Neurotransmitter:sodium symporters (NSSs) are integral membrane proteins responsible for the sodium-dependent reuptake of small-molecule neurotransmitters from the synaptic cleft. The symporters for the biogenic amines serotonin (SERT), dopamine (DAT), and norepinephrine (NET) are targets of multiple psychoactive agents, and their dysfunction has been implicated in numerous neuropsychiatric ailments. LeuT, a thermostable eubacterial NSS homolog, has been exploited as a model protein for NSS members to canvass the conformational mechanism of transport with a combination of X-ray crystallography, cysteine accessibility, and solution spectroscopy...
February 21, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28222964/commentary-treating-submucous-clefts-with-a-double-opposing-z-plasty
#9
C C Breugem
No abstract text is available yet for this article.
February 9, 2017: Journal of Plastic, Reconstructive & Aesthetic Surgery: JPRAS
https://www.readbyqxmd.com/read/28221772/desleucyl-oritavancin-with-a-damaged-d-ala-d-ala-binding-site-inhibits-the-transpeptidation-step-of-cell-wall-biosynthesis-in-whole-cells-of-staphylococcus-aureus
#10
Sung Joon Kim, Manmilan Singh, Shasad Sharif, Jacob Schaefer
We have used solid-state NMR to characterize the exact nature of the dual mode of action of oritavancin in preventing cell-wall assembly in Staphylococcus aureu. Measurements performed on whole cells labeled selectively in vivo have established that des-N-methylleucyl-N-4-(4-fluorophenyl)benzyl-chloroeremomycin, an Edman degradation product of [19F]oritavancin, which has a damaged D-Ala-D-Ala binding aglycon, is a potent inhibitor of the transpeptidase activity of cell-wall biosynthesis. The desleucyl-drug binds to partially cross-linked peptidoglycan by a cleft formed between the drug aglycon and its biphenyl hydrophobic sidechain...
February 21, 2017: Biochemistry
https://www.readbyqxmd.com/read/28220515/challenges-in-conducting-multicentre-multidisciplinary-longitudinal-studies-in-children-with-chronic-conditions
#11
Hillary L Broder, Canice E Crerand, Ryan R Ruff, Alexandre Peshansky, David B Sarwer, Lacey Sischo
OBJECTIVES: Conducting longitudinal, multicentre, multidisciplinary research for individuals with chronic conditions can be challenging. Despite careful planning, investigative teams must adapt to foreseen and unforeseen problems. Our objective is to identify challenges encountered and solutions sought in a recently completed observational, longitudinal study of youth with cleft lip and palate as well as their caregivers. METHODS: Data for analysis were derived from a 6-year, multicentre, prospective, longitudinal study of youth with cleft conducted from 2009 to 2015 that examined oral health-related quality of life and other related clinical observations over time in youth who had cleft-related surgery compared to those who did not...
February 21, 2017: Community Dentistry and Oral Epidemiology
https://www.readbyqxmd.com/read/28218671/human-scfvs-that-counteract-bioactivities-of-staphylococcus-aureus-tsst-1
#12
Thunchanok Rukkawattanakul, Nitat Sookrung, Watee Seesuay, Nattawat Onlamoon, Pornphan Diraphat, Wanpen Chaicumpa, Nitaya Indrawattana
Some Staphylococcus aureus isolates produced toxic shock syndrome toxin-1 (TSST-1) which is a pyrogenic toxin superantigen (PTSAg). The toxin activates a large fraction of peripheral blood T lymphocytes causing the cells to proliferate and release massive amounts of pro-inflammatory cytokines leading to a life-threatening multisystem disorder: toxic shock syndrome (TSS). PTSAg-mediated-T cell stimulation circumvents the conventional antigenic peptide presentation to T cell receptor (TCR) by the antigen-presenting cell (APC)...
February 17, 2017: Toxins
https://www.readbyqxmd.com/read/28218561/scandcleft-randomised-trials-of-primary-surgery-for-unilateral-cleft-lip-and-palate-3-descriptive-study-of-postoperative-nursing-care-following-first-stage-cleft-closure
#13
Patricia Bannister, Nina Lindberg, Karin Jeppesen, Ulla Elfving-Little, Ann-Margritt Semmingsen, Anna Paganini, Annica Gustavsson, Emma Slevin, Gry Jacobsen, Phil Eyres, Gunvor Semb
BACKGROUND: Cleft lip and palate is one of the most common congenital anomalies requiring surgical treatment in children, normally commenced in the first year of life. Following the initiation of a group of multicentre surgical trials of primary surgery, variations in postoperative recovery and management became apparent. An agreement was made for a nurse-led survey in eight surgical centres to document postoperative care and recovery. MATERIALS AND METHODS: A postoperative recovery clinical report form was developed to capture relevant data for the children participating in the four arms of the trials...
February 2017: Journal of Plastic Surgery and Hand Surgery
https://www.readbyqxmd.com/read/28218559/a-scandcleft-randomised-trials-of-primary-surgery-for-unilateral-cleft-lip-and-palate-1-planning-and-management
#14
Gunvor Semb, Hans Enemark, Hans Friede, Gunnar Paulin, Jan Lilja, Jorma Rautio, Mikael Andersen, Frank Åbyholm, Anette Lohmander, William Shaw, Kirsten Mølsted, Arja Heliövaara, Stig Bolund, Jyri Hukki, Hallvard Vindenes, Peter Davenport, Kjartan Arctander, Ola Larson, Anders Berggren, David Whitby, Alan Leonard, Erik Neovius, Anna Elander, Elisabeth Willadsen, R Patricia Bannister, Eileen Bradbury, Gunilla Henningsson, Christina Persson, Philip Eyres, Berit Emborg, Mia Kisling-Møller, Annelise Küseler, Birthe Granhof Black, Antje Schöps, Anja Bau, Maria Boers, Helene Søgaard Andersen, Karin Jeppesen, Dorte Marxen, Marjukka Paaso, Elina Hölttä, Suvi Alaluusua, Leena Turunen, Kirsti Humerinta, Ulla Elfving-Little, Inger Beate Tørdal, Lillian Kjøll, Ragnhild Aukner, Øydis Hide, Kristin Billaud Feragen, Elisabeth Rønning, Pål Skaare, Eli Brinck, Ann-Magritt Semmingsen, Nina Lindberg, Melanie Bowden, Julie Davies, Jeanette Mooney, Haydn Bellardie, Nina Schofield, Jill Nyberg, Maria Lundberg, Agneta Linder-Aronson Karsten, Margareta Larson, Anders Holmefjord, Sigvor Reisæter, Nina-Helen Pedersen, Therese Rasmussen, Rolf Tindlund, Paul Sæle, Reidunn Blomhoff, Gry Jacobsen, Christina Havstam, Sara Rizell, Lars Enocson, Catharina Hagberg, Midia Najar Chalien, Anna Paganini, Inger Lundeborg, Agneta Marcusson, Anna-Britta Mjönes, Annica Gustavsson, Christine Hayden, Eilish McAleer, Emma Slevan, Terry Gregg, Helen Worthington
BACKGROUND AND AIMS: Longstanding uncertainty surrounds the selection of surgical protocols for the closure of unilateral cleft lip and palate, and randomised trials have only rarely been performed. This paper is an introduction to three randomised trials of primary surgery for children born with complete unilateral cleft lip and palate (UCLP). It presents the protocol developed for the trials in CONSORT format, and describes the management structure that was developed to achieve the long-term engagement and commitment required to complete the project...
February 2017: Journal of Plastic Surgery and Hand Surgery
https://www.readbyqxmd.com/read/28218558/scandcleft-randomised-trials-of-primary-surgery-for-unilateral-cleft-lip-and-palate-10-parental-perceptions-of-appearance-and-treatment-outcomes-in-their-5-year-old-child
#15
Kristin Billaud Feragen, Gunvor Semb, Arja Heliövaara, Anette Lohmander, Emma Christine Johannessen, Betty Marie Boysen, Christina Havstam, Inger Lundeborg, Jill Nyberg, Nina-Helen Pedersen, Joan Bogh-Nielsen, Philip Eyres, Eileen Bradbury, Nichola Rumsey
BACKGROUND AND AIM: Few studies have explored children's emotional and behavioural reactions to cleft surgery and treatment-related stress. The objective was to investigate parents' evaluations of appearance and treatment outcomes in their 5-year-old child with unilateral cleft lip and palate (UCLP), and their perceptions of how their child was coping with treatment, comparing this information with recorded postsurgical complications. DESIGN: Three parallel group randomised clinical trials were undertaken as an international multicentre study by 10 cleft teams in five countries: Denmark, Finland, Sweden, Norway, and the UK...
February 2017: Journal of Plastic Surgery and Hand Surgery
https://www.readbyqxmd.com/read/28218557/scandcleft-randomised-trials-of-primary-surgery-for-unilateral-cleft-lip-and-palate-7-occlusion-in-5-year-olds-according-to-the-huddart-and-bodenham-index
#16
Agneta Karsten, Agneta Marcusson, Kirsti Hurmerinta, Arja Heliövaara, Annelise Küseler, Pål Skaare, Haydn Bellardie, Elisabeth Rønning, William Shaw, Kirsten Mølsted, Paul Sæle, Eli Brinck, Sara Rizell, Midia Najal Chalier, Philip Eyres, Gunvor Semb
BACKGROUND AND AIM: Good dentofacial development and good occlusion are main goals in the treatment of UCLP. The aim was to evaluate dental occlusion at age 5 years with the Huddart and Bodenham index after four different protocols of primary surgery for UCLP. DESIGN: Three parallel randomised controlled trials were undertaken as an international multicentre study by 10 cleft teams in five countries: Denmark, Finland, Sweden, Norway, and the UK. METHODS: Three different surgical procedures for primary palatal repair (Arms B, C, and D) were tested against a common procedure (Arm A) in the total cohort of 448 children born with non-syndromic UCLP...
February 2017: Journal of Plastic Surgery and Hand Surgery
https://www.readbyqxmd.com/read/28218556/scandcleft-randomised-trials-of-primary-surgery-for-unilateral-cleft-lip-and-palate-5-speech-outcomes-in-5-year-olds-consonant-proficiency-and-errors
#17
Elisabeth Willadsen, Anette Lohmander, Christina Persson, Inger Lundeborg, Suvi Alaluusua, Ragnhild Aukner, Anja Bau, Maria Boers, Melanie Bowden, Julie Davies, Berit Emborg, Christina Havstam, Christine Hayden, Gunilla Henningsson, Anders Holmefjord, Elina Hölttä, Mia Kisling-Møller, Lillian Kjøll, Maria Lundberg, Eilish McAleer, Jill Nyberg, Marjukka Paaso, Nina Helen Pedersen, Therese Rasmussen, Sigvor Reisæter, Helene Søgaard Andersen, Antje Schöps, Inger-Beate Tørdal, Gunvor Semb
BACKGROUND AND AIM: Normal articulation before school start is a main objective in cleft palate treatment. The aim was to investigate if differences exist in consonant proficiency at age 5 years between children with unilateral cleft lip and palate (UCLP) randomised to different surgical protocols for primary palatal repair. A secondary aim was to estimate burden of care in terms of received additional secondary surgeries and speech therapy. DESIGN: Three parallel group, randomised clinical trials were undertaken as an international multicentre study by 10 cleft teams in five countries: Denmark, Finland, Norway, Sweden, and the UK...
February 2017: Journal of Plastic Surgery and Hand Surgery
https://www.readbyqxmd.com/read/28218555/scandcleft-randomised-trials-of-primary-surgery-for-unilateral-cleft-lip-and-palate-8-assessing-naso-labial-appearance-in-5-year-olds-a-preliminary-study
#18
Kirsten Mølsted, Kirsti Humerinta, Annelise Küseler, Pål Skaare, Haydn Bellardie, William Shaw, Agneta Karsten, Paul Kåre Sæle, Sara Rizell, Agneta Marcusson, Philip Eyres, Gunvor Semb
BACKGROUND AND AIM: Facial appearance is one of the most relevant measures of success in cleft lip and palate treatment. The aim was to assess nasolabial appearance at 5 years of age in all children in the project. In this part of the project the local protocol for lip closure continued to be used because the primary lip and nose operations were not part of the randomisation. The great majority of the surgeons used Millard's technique together with McComb's technique for the nose. One center used Tennison-Randalls technique and in one center the centers own technique as well as nose plugs were used...
February 2017: Journal of Plastic Surgery and Hand Surgery
https://www.readbyqxmd.com/read/28218554/the-scandcleft-randomised-trials-of-primary-surgery-for-unilateral-cleft-lip-and-palate-11-what-next
#19
William Shaw, Gunvor Semb
BACKGROUND AND AIMS: Longstanding uncertainty surrounds the selection of surgical protocols for unilateral cleft lip and palate (UCLP), and randomised trials have only occasionally been performed. The Scandcleft Project consists of three trials initiated in 1997. The results at age 5 have been published in the series of reports that precede this manuscript, which aims to distill some of the issues that future trialists may need to address. METHOD: The shared experience of this group of trials is reviewed with reference to the current literature on trial management and, more specifically, trials of surgical technique and timing...
February 2017: Journal of Plastic Surgery and Hand Surgery
https://www.readbyqxmd.com/read/28218553/scandcleft-randomised-trials-of-primary-surgery-for-unilateral-cleft-lip-and-palate-9-parental-report-of-social-and-emotional-experiences-related-to-their-5-year-old-child-s-cleft-diagnosis
#20
Kristin Billaud Feragen, Nichola Rumsey, Arja Heliövaara, Betty Marie Boysen, Emma Christine Johannessen, Christina Havstam, Agneta Marcusson, Jill Nyberg, Nina-Helen Pedersen, Joan Bogh-Nielsen, Philip Eyres, Eileen Bradbury, Gunvor Semb
BACKGROUND AND AIM: Parents of children with a cleft lip and palate may be emotionally affected by the child's diagnosis. Their experiences and perceptions are important when evaluating the complexity of satisfactory treatment outcomes. The objective was to examine parents' social and emotional experiences related to their child's cleft diagnosis, and their perceptions of the child's adjustment to living with a visible difference. DESIGN: International multicentre study by 10 cleft teams in five countries: Denmark, Finland, Sweden, Norway, and the UK...
February 2017: Journal of Plastic Surgery and Hand Surgery
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