1,354 research outputs found

    Pediatric Sleep Questionnaire Used to Assess Sleep-Related Breathing Disorders in a Western Pennsylvania Private Orthodontic Practice

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    Sleep related breathing disorders (SRBD) in children have a reported prevalence of 4-11% and manifest as behavioral, physical, and/or academic deficiencies. SRBD in children include snoring, obstructive sleep apnea (OSA), and hypopnea. Unlike adults, OSA incidence in pediatric patients occurs equally in males and females, with the greatest incidence from ages 2-8 years old. The greater incidence during this age range is primarily due to larger pharyngeal lymphatic tissues, which regress as the patient progresses into adolescence. After puberty, the prevalence of OSA increases more in boys than girls, which could be due to testosterone-induced changes. The gold standard for measuring SRBD is an overnight polysomnograph, which is burdensome to both the patient and the parent. For this reason, numerous questionnaires have been created to help identify patients at risk for SRBD. The Pediatric Sleep Questionnaire (PSQ) is a 22-point questionnaire which can be scored quickly and easily. When more than one third of the answers are answered positively, there is an increased likelihood that the patient would have positive signs of SRBD on a polysomnograph. With a specificity of 81% and a sensitivity of 85%, the questionnaire is a powerful tool for identifying patients who may be at a higher risk for SRBD. By looking at 1500 consecutive PSQ given to all patients under the age of 18 as part of the routine medical history, the prevalence of SRDB in pediatric orthodontic patients in a private practice in Western Pennsylvania will be estimated in an effort to describe how common this disorder may be in a common orthodontic environment

    Recommendations for the management of MPS IVA: systematic evidence- and consensus-based guidance.

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    IntroductionMucopolysaccharidosis (MPS) IVA or Morquio A syndrome is an autosomal recessive lysosomal storage disorder (LSD) caused by deficiency of the N-acetylgalactosamine-6-sulfatase (GALNS) enzyme, which impairs lysosomal degradation of keratan sulphate and chondroitin-6-sulphate. The multiple clinical manifestations of MPS IVA present numerous challenges for management and necessitate the need for individualised treatment. Although treatment guidelines are available, the methodology used to develop this guidance has come under increased scrutiny. This programme was conducted to provide evidence-based, expert-agreed recommendations to optimise management of MPS IVA.MethodsTwenty six international healthcare professionals across multiple disciplines, with expertise in managing MPS IVA, and three patient advocates formed the Steering Committee (SC) and contributed to the development of this guidance. Representatives from six Patient Advocacy Groups (PAGs) were interviewed to gain insights on patient perspectives. A modified-Delphi methodology was used to demonstrate consensus among a wider group of healthcare professionals with experience managing patients with MPS IVA and the manuscript was evaluated against the validated Appraisal of Guidelines for Research and Evaluation (AGREE II) instrument by three independent reviewers.ResultsA total of 87 guidance statements were developed covering five domains: (1) general management principles; (2) recommended routine monitoring and assessments; (3) disease-modifying interventions (enzyme replacement therapy [ERT] and haematopoietic stem cell transplantation [HSCT]); (4) interventions to support respiratory and sleep disorders; (5) anaesthetics and surgical interventions (including spinal, limb, ophthalmic, cardio-thoracic and ear-nose-throat [ENT] surgeries). Consensus was reached on all statements after two rounds of voting. The overall guideline AGREE II assessment score obtained for the development of the guidance was 5.3/7 (where 1 represents the lowest quality and 7 represents the highest quality of guidance).ConclusionThis manuscript provides evidence- and consensus-based recommendations for the management of patients with MPS IVA and is for use by healthcare professionals that manage the holistic care of patients with the intention to improve clinical- and patient-reported outcomes and enhance patient quality of life. It is recognised that the guidance provided represents a point in time and further research is required to address current knowledge and evidence gaps

    Severe Central Sleep Apnea in Vici Syndrome

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    From Jöbsis to the present day: a review of clinical near-infrared spectroscopy measurements of cerebral cytochrome-c-oxidase

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    Near-infrared spectroscopy (NIRS) measurements of cytochrome-c-oxidase (CCO) have the potential to yield crucial information about cerebral metabolism at the patient bedside. Developments in instrumentation and the analytical methods used to resolve changes in CCO have led to many clinical applications of the measurement since its first demonstration in 1977 by Jöbsis. There is a substantial literature of work on measures of CCO in animal and in vitro studies; however, this review focuses on translational studies. Almost 40 years from the advent of the first measurement of CCO using NIRS, this signal continues to hold significant interest in our understanding of the human brain in health and disease. We discuss methodologies for obtaining NIRS measurements of CCO in the clinic and review studies in neonates and adults

    ACVIM consensus statement guidelines for the diagnosis, classification, treatment, and monitoring of pulmonary hypertension in dogs.

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    Pulmonary hypertension (PH), defined by increased pressure within the pulmonary vasculature, is a hemodynamic and pathophysiologic state present in a wide variety of cardiovascular, respiratory, and systemic diseases. The purpose of this consensus statement is to provide a multidisciplinary approach to guidelines for the diagnosis, classification, treatment, and monitoring of PH in dogs. Comprehensive evaluation including consideration of signalment, clinical signs, echocardiographic parameters, and results of other diagnostic tests supports the diagnosis of PH and allows identification of associated underlying conditions. Dogs with PH can be classified into the following 6 groups: group 1, pulmonary arterial hypertension; group 2, left heart disease; group 3, respiratory disease/hypoxia; group 4, pulmonary emboli/pulmonary thrombi/pulmonary thromboemboli; group 5, parasitic disease (Dirofilaria and Angiostrongylus); and group 6, disorders that are multifactorial or with unclear mechanisms. The approach to treatment of PH focuses on strategies to decrease the risk of progression, complications, or both, recommendations to target underlying diseases or factors contributing to PH, and PH-specific treatments. Dogs with PH should be monitored for improvement, static condition, or progression, and any identified underlying disorder should be addressed and monitored simultaneously

    The Boston University Photonics Center annual report 2016-2017

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    This repository item contains an annual report that summarizes activities of the Boston University Photonics Center in the 2016-2017 academic year. The report provides quantitative and descriptive information regarding photonics programs in education, interdisciplinary research, business innovation, and technology development. The Boston University Photonics Center (BUPC) is an interdisciplinary hub for education, research, scholarship, innovation, and technology development associated with practical uses of light.This has undoubtedly been the Photonics Center’s best year since I became Director 10 years ago. In the following pages, you will see highlights of the Center’s activities in the past year, including more than 100 notable scholarly publications in the leading journals in our field, and the attraction of more than 22 million dollars in new research grants/contracts. Last year I had the honor to lead an international search for the first recipient of the Moustakas Endowed Professorship in Optics and Photonics, in collaboration with ECE Department Chair Clem Karl. This professorship honors the Center’s most impactful scholar and one of the Center’s founding visionaries, Professor Theodore Moustakas. We are delighted to haveawarded this professorship to Professor Ji-Xin Cheng, who joined our faculty this year.The past year also marked the launch of Boston University’s Neurophotonics Center, which will be allied closely with the Photonics Center. Leading that Center will be a distinguished new faculty member, Professor David Boas. David and I are together leading a new Neurophotonics NSF Research Traineeship Program that will provide $3M to promote graduate traineeships in this emerging new field. We had a busy summer hosting NSF Sites for Research Experiences for Undergraduates, Research Experiences for Teachers, and the BU Student Satellite Program. As a community, we emphasized the theme of “Optics of Cancer Imaging” at our annual symposium, hosted by Darren Roblyer. We entered a five-year second phase of NSF funding in our Industry/University Collaborative Research Center on Biophotonic Sensors and Systems, which has become the centerpiece of our translational biophotonics program. That I/UCRC continues to focus on advancing the health care and medical device industries

    Juvenile parotitis

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    In parotitis, one or both of the parotid glands swell, causing pain while eating, reduced mouth opening, and in some cases fever. Before the vaccination era, mumps was the commonest cause of childhood parotitis. Nowadays acute pediatric parotitis is rare, and the causative agent(s) are not fully known. It is assumed that other viruses in addition to mumps virus are capable of causing similar symptoms. Some children develop recurrent symptoms i.e. juvenile recurrent parotitis (JRP). If symptoms are frequent, this condition can be quite life-disruptive. Fortunately, JRP often resolves in puberty. The etiology and pathophysiology of this juvenile recurrent parotid inflammation are other aspects currently not completely understood. The aim of the present study was to assess the epidemiology, etiology, clinical picture, and outcome for pediatric parotitis at present. In addition, it addresses differential diagnosis and complications. A group of 41 children aged ≤17 with acute parotid inflammation was collected prospectively for this study that reported clinical characteristics, treatment, outcome and complications. Another group of 133 children was collected retrospectively with the clinical picture of their disease reported, as well. The serine protease inhibitor Kazal-type 1 (SPINK-1) genotype was tested in 88 parotitis patients, since mutation of this gene disposes to pancreatitis, and salivary glands bear some resemblance to the pancreas in function. To map the etiology of parotitis, a questionnaire about history of parotitis, and parotid gland -related symptoms went to 1,000 adolescents randomly selected. In addition, human herpes viruses (HHVs) from saliva samples of children with acute parotid inflammation, and from healthy controls were tested. To assess the differential diagnosis and complications of parotitis, the database of Helsinki University Central Hospital, Department of Otorhinolaryngology - Head and Neck Surgery, was searched according to ICD-10 codes in order to find all children diagnosed and treated due to osteomyelitis or parotid abscess. All prospectively studied children with acute parotitis were in good general condition, and most episodes of parotitis in childhood seem to have a benign course. Half these children were treated only with non-steroidal anti-inflammatory drugs. However, parotid symptoms have a tendency to recur in about half the cases. About 1% of the respondents to the epidemiologic survey had suffered from parotitis. Heredity similar to pancreatitis could not be shown, since no difference emerged in SPINK-1 genotype in children with parotitis compared to controls. HHVs seem to play no role in acute juvenile parotitis, but are instead common findings in saliva. Osteomyelitis of the head and neck region is rare, but important in differential diagnosis of children with recurrent parotid symptoms. Parotitis-related complications are rare. Parotid abscesses are multi-bacterial infections with intravenous antibiotic therapy being the cornerstone of treatment. Surgical drainage assists in recovery and does not lead to fistula formation. In conclusion, according to this study juvenile parotitis has a frequency close to 1%, it has a tendency to recur, and in most cases the overall condition of the child is good during the infection. Osteomyelitis as a differential diagnosis must be kept in mind when treating recurrent symptoms of the parotid area. Abscesses related to parotitis are rare. The full etiology of juvenile parotitis still remains to be discovered.Korvasylkirauhastulehduksessa poski on kipeä ja turvonnut. Suun aukaiseminen on usein rajoittunutta ja syöminen pahentaa kipua. Tautiin voi liittyä kuumetta. Ennen kattavia kansallisia rokotuksia sikotauti oli yleinen ja tavallisin korvasylkirauhastulehduksen aiheuttaja. Nykyään lasten korvasylkirauhastulehdukset ovat harvinaisia eikä kaikkia taudinaiheuttajia tunneta. Sikotautiviruksen lisäksi myös muut virukset voivat aiheuttaa korvasylkirauhastulehduksen. Osa lapsista kärsii toistuvasta korvasylkirauhastulehduksesta, jonka oireet helpottavat tai häviävät yleensä murrosiässä. Myöskään toistuvan korvasylkirauhastulehduksen etiologiaa tai patofysiologiaa ei tällä hetkellä tunneta tarkasti. Tämän väitöskirjatyön tarkoituksena oli selvittää lasten korvasylkirauhastulehdusten epidemiologiaa, etiologiaa, taudin kuvaa ja hoitoa. Lisäksi tutkittiin erotusdiagnostiikkaa ja komplikaatioita. Prospektiivinen tutkimusryhmä koostui 41 korvasylkirauhastulehdusta sairastavasta alle 17-vuotiaasta lapsesta. Taudinkuva, hoito ja komplikaatiot selvitettiin. Toinen tutkimusryhmä koostui 133 retrospektiivisesti kerätystä lapsesta, joiden taudinkuva ja hoito rekisteröitiin. Seriiniproteaasin estäjä Kazal tyyppi 1:n (SPINK-1) genotyyppi testattiin 88 lapselta, koska tiedetään, että tämän geenin mutaatiot altistavat haimatulehduksille. Sylkirauhasten ja haiman toiminnassa on paljon samankaltaista. Epidemiologian selvittämiseksi tuhannelle satunnaisesti valitulle nuorelle lähetettiin kysely sairastetuista korvasylkirauhastulehduksista ja muista mahdollisista korvasylkirauhasoireista. Lisäksi korvasylkirauhastulehdusta sairastavien lasten, verrokkilasten ja terveiden aikuisverrokkien syljestä testattiin herpesviruksia osana etiologisia selvittelyjä. Helsingin yliopistollisen keskussairaalan Silmä-korvasairaalan potilastietokannasta etsittiin kaikki lapset ja nuoret, jotka olivat olleet hoidossa pään ja kaulan alueen luutulehduksen tai korvasylkirauhaspaiseen vuoksi. Pään ja kaulan alueen luutulehduksen ja korvasylkirauhastulehduksen oireet voivat muistuttaa toisiaan, ja paise on korvasylkirauhastulehduksen harvinainen komplikaatio. Kaikkien 41 prospektiivisesti tutkitun lapsen yleistila oli korvasylkirauhastulehduksen aikana hyvä. Suurin osa lasten tulehduksista parantui hyvin, vaikka noin puolet lapsista hoidettiin pelkällä tulehduskipulääkityksellä. Noin puolella lapsista korvasylkirauhastulehdus uusi. Noin joka sadannella kyselytutkimukseen osallistuneesta lapsesta oli ollut korvasylkirauhastulehdus. Tässä tutkimuksessa ei pystytty osoittamaan korvasylkirauhastulehduksissa samanlaista SPINK-1 genotyyppiin liittyvää perinnöllisyyttä kuin haimatulehduksissa. Herpesvirukset olivat yleisiä kaikkien testiryhmien syljessä, eikä yhteyttä äkilliseen korvasylkirauhastulehdukseen löydetty. Pään ja kaulan alueen luutulehdus on harvinainen, mutta tärkeä erotusdiagnostinen vaihtoehto. Korvasylkirauhastulehduksiin liittyvät komplikaatiot ovat harvinaisia. Korvasylkirauhasen paiseet ovat usean bakteerin aiheuttamia infektioita, joiden hoidon perusta on suonensisäinen antibioottilääkitys. Paiseen puhkaisu tai avaaminen on usein välttämätöntä, eikä johda sylkifistelin muodostumiseen. Tämän tutkimuksen perusteella lasten korvasylkirauhastulehdukset ovat luultua yleisempiä, niillä on taipumus uusiutua, ja lapsen yleistila on tulehduksen aikana yleensä hyvä. Komplikaatiot ovat harvinaisia. Toistuvia korvasylkirauhastulehduksia hoidettaessa on muistettava myös pään ja kaulan alueen luutulehduksen mahdollisuus. Kaikkia korvasylkirauhastulehduksen aiheuttajia ei tunneta

    Added value of acute multimodal CT-based imaging (MCTI) : a comprehensive analysis

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    Introduction: MCTI is used to assess acute ischemic stroke (AIS) patients.We postulated that use of MCTI improves patient outcome regardingindependence and mortality.Methods: From the ASTRAL registry, all patients with an AIS and a non-contrast-CT (NCCT), angio-CT (CTA) or perfusion-CT (CTP) within24 h from onset were included. Demographic, clinical, biological, radio-logical, and follow-up caracteristics were collected. Significant predictorsof MCTI use were fitted in a multivariate analysis. Patients undergoingCTA or CTA&CTP were compared with NCCT patients with regards tofavourable outcome (mRS ≤ 2) at 3 months, 12 months mortality, strokemechanism, short-term renal function, use of ancillary diagnostic tests,duration of hospitalization and 12 months stroke recurrence
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