Abstract：
:The mucopolysaccharidoses (MPS) are a family of related inherited metabolic disorders where, due to specific lysosomal enzyme deficiencies, partially degraded glycosaminoglycans (GAGs) accumulate in the body's cells. Due to the ubiquitous nature of GAGs in the body this deposition can occur in many tissue types and may interfere with cellular function. Although these conditions are rare, there is a propensity for the disease process to cause problems with the function of the ears, noses and throats of affected patients. In this review, we present an overview of the clinical manifestations of MPS in general and highlight the problems specifically presenting in the field of otorhinolaryngology.
journal_name
Int J Pediatr Otorhinolaryngoljournal_title
International journal of pediatric otorhinolaryngologyauthors
Simmons MA,Bruce IA,Penney S,Wraith E,Rothera MPdoi
10.1016/j.ijporl.2005.01.017subject
Has Abstractpub_date
2005-05-01 00:00:00pages
589-95issue
5eissn
0165-5876issn
1872-8464pii
S0165-5876(05)00022-4journal_volume
69pub_type
杂志文章,评审abstract：OBJECTIVE:To assess outcome in pediatric patients after treatment for retraction pockets of pars tensa in relation to retraction grade, site, occurrence of complications, and patient age. METHODS:Outcomes in 45 ears of 37 children medically or surgically treated for retraction pockets were compared to a control group ...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/j.ijporl.2009.11.004
更新日期：2010-02-01 00:00:00
abstract：INTRODUCTION:Swallowing problems in children with a tracheostomy tube seem to be a common problem, although exact prevalence is not known. The aim of this study is to identify the prevalence and type of swallowing problems in children with a tracheostomy tube. METHODS:We retrospectively included 44 children having a t...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/j.ijporl.2019.05.003
更新日期：2019-09-01 00:00:00
abstract：OBJECTIVE:Radiological and genetic examination has recently advanced for diagnosis of congenital hearing loss. The aim of this study was to elucidate the prevalence of inner-ear and/or internal auditory canal malformations in children with unilateral sensorineural hearing loss (USNHL) for better management of hearing l...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/j.ijporl.2012.11.001
更新日期：2013-02-01 00:00:00
abstract：OBJECTIVES:To analyze the demographics, survival, and treatment efficacy of pediatric sarcomas of the facial skeleton and skull. METHODS:Retrospective study of cases from the US National Cancer Institute's Surveillance, Epidemiology, and End Results database. Pediatric patients between the ages of 0 and 18 diagnosed w...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/j.ijporl.2017.06.003
更新日期：2017-09-01 00:00:00
abstract：BACKGROUND:Otitis media, a disease highly prevalent among children worldwide, manifests clinically in both acute and chronic forms. The manner and time at which chronicity develops among Indian children is unknown. AIM:To study the prevalence, manifestations and risk factors for otitis media in a birth cohort aged 8 y...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/j.ijporl.2020.110201
更新日期：2020-10-01 00:00:00
abstract：OBJECTIVE:Voice disorders in children are common but ways of their analysis are limited. We conducted a prospective feasibility study of rigid stroboscopy in children. METHODS:All children referred for voice analysis during the years 2002-2003 were evaluated including subjective perception of voice, voice recording, f...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/j.ijporl.2005.03.004
更新日期：2005-08-01 00:00:00
abstract：OBJECTIVE:To evaluate the usefulness of radiofrequency myringotomy (RFM) and the closure time of the myringotomy site in comparison to incisional myringotomy (IM). METHODS:We performed conventional surgical myringotomy on the right ears and RFM on the left ears of 40 rabbits. In order to investigate the effect of the ...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/j.ijporl.2008.06.020
更新日期：2008-10-01 00:00:00
abstract：OBJECTIVES:To assess the effectiveness of nurse-led triage of outpatient referrals in an academic pediatric otolaryngology practice. METHODS:Three hundred consecutive outpatient referrals were reviewed and triaged by two otolaryngology registered nurses and two attending pediatric otolaryngologists. The nurses receive...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/j.ijporl.2015.01.031
更新日期：2015-04-01 00:00:00
abstract：:PHACE syndrome consists of the constellation of manifestations including Posterior fossa anomalies of the brain (most commonly Dandy-Walker malformations), Hemangiomas of the face and scalp, Arterial abnormalities, Cardiac defects, and Eye anomalies. We present the case of a patient who presented with respiratory dist...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/j.ijporl.2005.07.014
更新日期：2006-03-01 00:00:00
abstract：OBJECTIVE:Waardenburg syndrome is clinically and genetically heterogeneous. The SOX10 mutation related with Waardenburg syndrome type II is rare in Chinese. This study aimed to uncover the genetic causes of Waardenburg syndrome type II in a three-generation family to improve genetic counseling. METHODS:Complete clinic...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/j.ijporl.2015.03.006
更新日期：2015-05-01 00:00:00
abstract：:In a series of 163 ears with chronic, mucoid effusion, free or immune complex bound pneumococcal capsular polysaccharide antigens were demonstrated with Omniserum and counterimmunoelectrophoresis (CIEP) in 37 ears (23%). In crushed adenoid tissue this figure was 39% (42/107). Using type- or group-specific sera pneumoc...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/s0165-5876(83)80115-3
更新日期：1983-11-01 00:00:00
abstract：:The neck is divided into multiple compartments by cervical fasciae. The deep space compartments, which all have a potential for becoming infected, are interconnected, providing a mechanism for the spread of infections. Thus, infections of the deep spaces can lead to devastating consequences, especially when there is e...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/j.ijporl.2016.02.016
更新日期：2016-04-01 00:00:00
abstract：:Isolated failure to thrive in an infant caused by chronic hypoventilation due to hypertrophic adenoids and tonsils, has not been previously described. A 9 month-old infant presented with weight loss and mild clinical respiratory signs. Hypoxemia and CO2 retention, however, were documented by transcutaneous gas measure...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/s0165-5876(85)80019-7
更新日期：1985-07-01 00:00:00
abstract：OBJECTIVE:Children with sensorineural hearing impairment (SNHI) and concomitant vestibular hypofunction demonstrate deficits in gaze stability that may affect reading. The objective of this study was to develop a reliable, valid clinical test of dynamic visual acuity (DVA) for children. METHODS:Seventy-six typically d...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/j.ijporl.2003.07.004
更新日期：2003-11-01 00:00:00
abstract：:It has been reported that intranasal immunization can induce mucosal immune responses. However, the efficacy of intranasal immunization on otitis media caused by non-typeable Haemophilus influenzae (NTHi) is not yet elucidated. Mice were intranasally, orally, intratracheally or intraperitoneally immunized with outer m...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/s0165-5876(99)00165-2
更新日期：1999-10-05 00:00:00
abstract：OBJECTIVE:OSD-6 is a disease specific questionnaire for pediatric obstructive sleep apnea (OSA). The aims of this study were to validate OSD-6 in Greek language and correlate OSD-6 with polysomnography results. STUDY DESIGN:Prospective study. SETTING:Tertiary referral center. SUBJECTS AND METHODS:OSD-6 questionnaire...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/j.ijporl.2014.05.024
更新日期：2014-08-01 00:00:00
abstract：OBJECTIVE:This study aimed to explore and make an understanding of absorption existed in children with intracapsular fractures. METHODS:The sample was composed of all children (less than 12 years) who presented with intracapsular fractures within a 5-year period (January 2011 to April 2016). Data about age, gender, da...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/j.ijporl.2019.05.013
更新日期：2019-08-01 00:00:00
abstract：:To assist medical and hearing-science professionals in supporting parents of deaf children, we have identified common questions that parents may have and provide evidence-based answers. In doing so, a compassionate and positive narrative about deafness and deaf children is offered, one that relies on recent research e...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章,评审
doi：10.1016/j.ijporl.2018.12.036
更新日期：2019-03-01 00:00:00
abstract：:Early diagnosis, evaluation and treatment of childhood deafness are essential for a child's normal growth. Etiological diagnosis of hearing loss makes prevention, family scheduling and more effective therapy feasible goals. Etiological assessment of sensorineural deafness still remains difficult although recently with...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/j.ijporl.2004.11.007
更新日期：2005-04-01 00:00:00
abstract：:Trisomy 22 has multiple physical anomalies, and aural malformations are commonly associated with trisomy 22. However, there has been only one report describing the temporal bone histopathology in trisomy 22. Our case is the second reported temporal bone histopathology of trisomy 22. Aural anomalies in this case were l...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/s0165-5876(01)00459-1
更新日期：2001-06-07 00:00:00
abstract：:The relation between the results of vestibular function tests and gross motor development was examined in 4 children with inner ear anomalies. CT scans demonstrated the absence of lateral semicircular canals in both ears in all 4 cases. None responded to caloric stimulation using 40 ml of icewater. In contrast, the da...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/0165-5876(92)90108-2
更新日期：1992-05-01 00:00:00
abstract：OBJECTIVE:Chloral hydrate (CH) is an oral sedative widely used to sedate infants and young children during auditory brainstem response (ABR) testing. The aim of this study was to record effectiveness, complications and safety of CH as a sedative for ABR. METHODS:From January of 2003 until December of 2007, 1903 childr...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/j.ijporl.2011.02.010
更新日期：2011-06-01 00:00:00
abstract：BACKGROUND:Post-tonsillectomy hemorrhage (PTH) remains a significant complication. There are no guidelines for Pediatric Emergency Department (PED) disposition of children with secondary PTH. OBJECTIVES:To describe the incidence, clinical characteristics and interventions required by children presenting to a PED with ...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/j.ijporl.2014.11.034
更新日期：2015-02-01 00:00:00
abstract：OBJECTIVE:Mental function is considered to affect the post-operative outcomes of deaf children with cochlear implants. The purpose of this study is to evaluate the effect of mental function on the auditory performance in children with cochlear implants. METHODS:In a retrospective review of data, 26 pre-lingual deafene...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/j.ijporl.2004.04.011
更新日期：2004-09-01 00:00:00
abstract：OBJECTIVE:The purpose of this study was to examine the speech perception skills of prelingually deafened French children with preoperative residual hearing who received multichannel cochlear implants. DESIGN:The design of the study incorporated a within-subject, repeated measures design for assessing speech perception...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/s0165-5876(01)00638-3
更新日期：2002-04-25 00:00:00
abstract：:Nager's acrofacial dysostosis is a rare congenital anomaly in which mandibulofacial dysostosis is seen in combination with limb deformities, particularly hypoplasia of the radial aspect of the hand. A boy with a severe manifestation of this syndrome is presented. Deformities include severe bilateral mandibular hypopla...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章,评审
doi：10.1016/0165-5876(90)90353-s
更新日期：1990-12-01 00:00:00
abstract：INTRODUCTION:Previous studies have suggested a direct relationship between the nasopharyngeal carriage of potential middle ear pathogens and the development of middle ear disease. It has been shown that otitis-prone (OP) children tend to be colonized more often than non-OP children. To study the turnover of nontypeable...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/s0165-5876(00)00321-9
更新日期：2000-08-11 00:00:00
abstract：OBJECTIVES:Children with laryngomalacia may present with obstructive sleep apnea (OSA). The role of polysomnography (PSG) in treatment decision making for laryngomalacia is not well defined. We aimed to investigate the prevalence of OSA in children with laryngomalacia and the role of PSG in treatment decision. METHODS...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/j.ijporl.2020.110255
更新日期：2020-10-01 00:00:00
abstract：OBJECTIVES:To provide age-equivalent norms for a 500Hz logon evoked ABR obtained in a group of children ranging 40 weeks-4 years old and compare these 500Hz tonal norms to age-equivalent norms for click-evoked ABR. METHODS:Seventy-seven infants and children ranging from conceptional age of 40 weeks (term babies) to 4 ...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/j.ijporl.2007.01.016
更新日期：2007-05-01 00:00:00
abstract：OBJECTIVE:This study assessed the development of balance function in children using platform stabilometry. METHODS:A total of 251 healthy children aged from 3 to 12 years were enrolled in this study. Each subject underwent stabilometry under four various conditions (A: firm surface with eyes open; B: firm surface with...
journal_title：International journal of pediatric otorhinolaryngology
pub_type： 杂志文章
doi：10.1016/j.ijporl.2009.01.016
更新日期：2009-05-01 00:00:00
