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Juan M González-Morena, María I Montañez, Giancarlo Aldini, Francisco J Sánchez-Gómez, Dolores Pérez-Sala
Drug hypersensitivity reactions result from the activation of the immune system by drugs or their metabolites. The clinical presentations of drug hypersensitivity can range from relatively mild local manifestations to severe systemic syndromes that can be life-threatening. As in other allergic reactions, the causes are multifactorial as genetic, metabolic and concomitant factors may influence the occurrence of drug hypersensitivity. Formation of drug protein adducts is considered a key step in drug adverse reactions, and in particular in the immunological recognition in drug hypersensitivity reactions...
September 27, 2016: Current Pharmaceutical Design
Arja Heliövaara, Annelise Küseler, Pål Skaare, William Shaw, Kirsten Mølsted, Agneta Karsten, Eli Brinck, Sara Rizell, Agneta Marcusson, Paul Sæle, Kirsti Hurmerinta, Elisabeth Rønning, Midia Najar Chalien, Jeanette Mooney, Phil Eyres, Gunvor Semb
BACKGROUND AND AIM: Good dentofacial growth is a major goal in the treatment of unilateral cleft lip and palate (UCLP). The aim was to evaluate dental arch relationships at age 5 years after four different protocols of primary surgery for UCLP. DESIGN: Three parallel randomised clinical trials were undertaken as an international multi-centre 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, D) were tested against a common procedure (Arm A) in the total cohort of 448 children born with non-syndromic UCLP...
October 25, 2016: Journal of Plastic Surgery and Hand Surgery
Weiwei Zhong, Christopher Mychal Johnson, Yang Wu, Ningren Cui, Hao Xing, Shuang Zhang, Chun Jiang
BACKGROUND: Rett syndrome (RTT) is a neurodevelopmental disorder caused mostly by disruptions in the MECP2 gene. MECP2-null mice show imbalances in neuronal excitability and synaptic communications. Several previous studies indicate that augmenting synaptic GABA receptors (GABAARs) can alleviate RTT-like symptoms in mice. In addition to the synaptic GABAARs, there is a group of GABAARs found outside the synaptic cleft with the capability to produce sustained inhibition, which may be potential therapeutic targets for the control of neuronal excitability in RTT...
2016: Journal of Neurodevelopmental Disorders
Hiroshi Yokota, Yuki Ida, Daisuke Wajima, Fumihiko Nishimura, Hiroyuki Nakase
BACKGROUND: A Rathke cleft cyst (RCC) with non-hemorrhagic rupture mimicking pituitary apoplexy has been rarely reported. CASE DESCRIPTION: A 52-year-old woman, previously diagnosed with asymptomatic RCC, came to us with a severe headache, along with visual dysfunction and symptoms of pituitary insufficiency. Fluid-attenuated inversion recovery magnetic resonance imaging demonstrated diffuse hyper-intensity in the cerebral cisterns, while watery clear cerebrospinal fluid was obtained by lumbar puncture...
October 21, 2016: World Neurosurgery
Doris Koesling, Evanthia Mergia, Michael Russwurm
NO-sensitive guanylyl cyclase (NO-GC) acts as the receptor for nitric oxide and by the increase in cGMP executes most of the NO effects in the cardiovascular and neuronal system. Two isoforms of NO-GC exist whose existence has not been paid much attention to probably because they reveal comparable regulatory and catalytic properties and therefore cannot be differentiated in vivo. Analysis of mice in which either one of the isoforms has been genetically deleted unequivocally establishes the coexpression of NO-GC1 and NOGC2 in any tissue tested to date with the exception of platelets...
2016: Current Medicinal Chemistry
Gregory S Antonarakis, Bryan D Tompson, David M Fisher
OBJECTIVE:   Maxillary growth in patients with cleft lip and palate is highly variable. The authors' aim was to investigate associations between preoperative cleft lip measurements and maxillary growth determined cephalometrically in patients with complete unilateral cleft lip and palate (cUCLP). DESIGN:   Retrospective cross-sectional study. PATIENTS:   Children with cUCLP. METHODS:   Preoperative cleft lip measurements were made at the time of primary cheiloplasty and available for each patient...
November 2016: Cleft Palate-craniofacial Journal
Yasuhiro Sasaki, Ayumi Kanamaru, Hisae Uchida, Masataka Yano, Hiroshi Tada
An 51-year-old man had undergone surgery for Rathke's cleft cyst by transsphenoidal approach on October 2015. After the surgery, cerebrospinal fluid (CSF) rhinorrhea arose. Surgical treatment of CSF rhinorrhea was performed by the same approach. Ten days after reoperation, he suffered from severe headache, high fever, and nuchal rigidity. CSF findings indicated bacterial meningitis. CSF culture showed methicillin-resistant Staphylococcus lugdunensis. He received vancomycin intravenously for 19 days and recovered from the bacterial meningitis with no serious complication...
October 21, 2016: Rinshō Shinkeigaku, Clinical Neurology
Iram Naaz, James Scurry, Hui Yin
No abstract text is available yet for this article.
February 2016: Pathology
Rawn Salenger, Xavier Diao, Murtaza Y Dawood, Daniel L Herr, George A Sample, Augusto Pichard, James S Gammie
We report a case of catastrophic hemodynamic compromise secondary to pannus ingrowth and severe mitral stenosis occurring years after repair of a nonrheumatic mitral valve. The initial repair included closure of a posterior leaflet cleft and implantation of an annuloplasty ring. We describe a hybrid treatment strategy for this severely compromised patient, which included initial placement of a right ventricular assist device followed by percutaneous balloon mitral valvuloplasty and, eventually, a definitive mitral valve reoperation...
November 2016: Annals of Thoracic Surgery
Goran Cuturilo, Danijela Drakulic, Ida Jovanovic, Aleksandar Krstic, Milan Djukic, Dejan Skoric, Marija Mijovic, Igor Stefanovic, Milena Milivojevic, Milena Stevanovic
OBJECTIVE: The incidence of the 22q11.2 microdeletion among children who have at least two out of five major clinical criteria for 22q11.2 deletion syndrome. DESIGN: Prospective study. SETTING: University Childrens Hospital in Belgrade, Serbia between 2005 and 2014. PARTICIPANTS: 57 patients with clinical characteristics of 22q11.2 deletion syndrome. METHODS: Standard G-banding cytogenetic analysis was performed in all children, and the 22q11...
September 8, 2016: Indian Pediatrics
Kinga Sałat, Adrian Podkowa, Natalia Malikowska, Felix Kern, Jörg Pabel, Ewelina Wojcieszak, Katarzyna Kulig, Klaus T Wanner, Beata Strach, Elżbieta Wyska
BACKGROUND AND PURPOSE: Since GABAergic dysfunction underlies a variety of neurological and psychiatric disorders, numerous strategies leading to the augmentation of GABAergic neurotransmission have been introduced. One of them is the inhibition of GABA reuptake from the synaptic cleft mediated by four plasma membrane GABA transporters (GAT1-4). GAT1 which is exclusively expressed in the brain is an interesting target for centrally acting drugs. In this research, pharmacological properties of a novel, highly potent and selective inhibitor of GAT1, the guvacine derivative named DDPM-2571, were assessed in vivo...
October 19, 2016: Neuropharmacology
Yuanxin Liang, Tao Zuo
Diagnosis of cystic papillary thyroid carcinoma (PTC) lymph node metastasis at head neck region can be a challenge in the absence of known PTC history. The congenital cystic lesions of head neck, especially thyroglossal duct cyst (TGDC) and branchial cleft cyst (BCC), are major differential diagnoses in this clinicopathological scenario. The location of cyst and morphology of lining epithelium are critical clues for reaching correct diagnosis. However it is not uncommon that the flattened bland epithelial lining can be seen in both cystic metastases and congenital cystic lesions...
October 21, 2016: Head and Neck Pathology
Yasuhiko Hayashi, Masahiro Oishi, Issei Fukui, Yasuo Sasagawa, Yasuhiro Aida, Mitsutoshi Nakada
BACKGROUND: In Rathke cleft cysts (RCCs), inflammation by the cyst contents infrequently spreads to the surrounding structures. Calcification, which is regarded as a consequence of chronic inflammation of the cyst wall, can rarely be found in RCCs, and moreover, ossification is extremely rare. CLINICAL PRESENTATION: A 60-year-old woman presented with headaches, fatigue and weight loss due to panhypopituitarism. Magnetic Resonance imaging revealed a mass lesion in the sellar region, which was comprised of two different parts, with hypointensity anteriorly and hyperintensity posteriorly on T1-weighted image (WI), and the rim with significant hypointensity entirely on T2-WI...
October 18, 2016: World Neurosurgery
P Ha, C Li, B Shi
The purpose of this study was to investigate the key factors in relation to parent satisfaction with the primary repair of paediatric cleft lip. One hundred and ninety-five children born with cleft lip and/or palate aged between 3 months and 1 year were recruited, along with their caregivers. All patients underwent primary cleft lip repair, and a telephone interview was held with their main caregivers at 3 months postoperative. The level of satisfaction with each item included in a simplified Cleft Evaluation Profile was ascertained and recorded...
October 18, 2016: International Journal of Oral and Maxillofacial Surgery
F Li, H-T Wang, Y-Y Chen, W-L Wu, J-Y Liu, J-S Hao, D-Y Luo
This study was performed to investigate the incidence of and risk factors for postoperative cleft relapse and oronasal fistula after Furlow palatoplasty in infants. Sixty-two infants with cleft palate, aged 6-12 months (mean 8.25 months), who underwent cleft repair by Furlow double opposing Z-plasty between March 2012 and August 2014, were enrolled in the study. Risk factors for postoperative cleft relapse and oronasal fistula after Furlow palatoplasty were identified by logistic regression analysis. The incidence rates of cleft relapse at 1 week and oronasal fistula at 3 months after surgery were 24...
October 18, 2016: International Journal of Oral and Maxillofacial Surgery
Breanna L Alman, Evan Coffman, Anna Maria Siega-Riz, Thomas J Luben
BACKGROUND: Water and water-based beverages constitute a major part of daily fluid intake for pregnant women, yet few epidemiologic studies have investigated the role of water consumption on birth outcomes. METHODS: We used data from the National Birth Defects Prevention Study to conduct a case-control study investigating associations between maternal water consumption during pregnancy and birth defects (BD). We used interview data on water consumption during the first trimester of pregnancy in 14,454 cases (major BDs n ≥ 50) and 5,063 controls...
October 21, 2016: Birth Defects Research. Part A, Clinical and Molecular Teratology
K Malkiewicz, E Malkiewicz, K A Eaton, E Widström
Poland is one of the largest European countries in terms of area and population. The country's economic situation does not allow for the allocation of sufficient public funds for healthcare in general and oral healthcare in particular. The health policy of the state focuses primarily on prophylaxis and treatment of diseases, directly threatening the health and lives of the inhabitants. Currently, expenditure on oral health accounts for only 2.7% of the public funds allocated to healthcare. In this context, providing oral care financed from public funds at an appropriate level constitutes a challenge for state institutions, centres providing medical and dental services and private practices...
October 21, 2016: British Dental Journal
Ádám Berniczei-Roykó, Jan-Hendrik Tappe, Axel Krinner, Tomasz Gredes, András Végh, Katona Gábor, Kamila Linkowska-Świdzińska, Ute Ulrike Botzenhart
BACKGROUND Cleft defects are one of the most frequent birth-deformities of the orofacial region and they are commonly associated with anomalies of the tooth structure, size, shape, formation, eruption, and tooth number. The aim of our study was to evaluate the prevalence, distribution, and potential association of combined hypodontia in cleft-affected patients with regard to all types of teeth in both jaws in the permanent dentition. MATERIAL AND METHODS This retrospective radiographic analysis included patients with various types of clefts treated orthodontically in the Department of Orofacial Orthopedics and Orthodontics at Heim Pàl Children's Hospital, Budapest...
October 21, 2016: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
Eva Meia Rüegg, Paul Gniadek, Ali Modarressi, Denise Baratti-Mayer, Brigitte Pittet-Cuénod
INTRODUCTION: Reconstruction of facial bone defects in children is challenging. The use of well-vascularized bone is mandatory to obtain stable lasting results. This study reports our experience of facial bone reconstruction using prefabricated vascularized calvarium flaps. METHODS: Retrospective case series of 50 patients who underwent 52 maxillary, malar, and mandibular reconstructions between 1988 and 2014 using prefabricated vascularized calvarium flaps. Forty-nine patients suffered from noma sequels; one patient had craniofacial cleft Tessier 3-11...
September 10, 2016: Journal of Cranio-maxillo-facial Surgery
S L Huang, B Zhang, L S Chen, L Liang, X N Luo, Z M Lu, S Y Zhang
Objective: To report rare cases of congenital neck cutaneous sinus with an orifice near the sternoclavicular joint and to investigate their origins and managements. Methods: A total of ten patients with congenital neck cutaneous sinus having an orifice near the sternoclavicular joint treated in the Guangdong General Hospital from January 2010 to June 2015 were retrospectively analyzed. Results: There four boys and six girls, aging from 11 months to 96 months with an average of 33.4 months, and they had a common feature showing a congenital cutaneous sinus with an orifice near sternoclavicular joint...
October 7, 2016: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
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