1
40
8
-
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1016/s0363-5023(98)80120-2" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/s0363-5023(98)80120-2</a>
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Surgical treatment of carpal tunnel syndrome and trigger digits in children with mucopolysaccharide storage disorders
Publisher
An entity responsible for making the resource available
Journal of Hand Surgery
Date
A point or period of time associated with an event in the lifecycle of the resource
1998
Subject
The topic of the resource
Orthopedics; Surgery; bone-marrow transplantation; hurler-syndrome; tone and motor problems; MPSI; MPSIII; MPSVI; surgical intervention; annular pulley release; partial flexor digitorum superficialis tendon resection
Creator
An entity primarily responsible for making the resource
Van Heest A E; House J; Krivit W; Walker K
Description
An account of the resource
The role of surgical intervention for carpal tunnel syndrome (CTS) and trigger digits in children with mucopolysaccharide storage disorders (MPSDs) has not been clearly defined, particularly as the treatment of the underlying disease has advanced to include bone marrow transplantation. This study reviews our experience in the treatment of CTS and trigger digits in 22 children With MPSDs who were evaluated for CTS by electromyographic (EMG)/nerve conduction velocity (NCV) testing. Seventeen children were diagnosed with CTS by EMG/NCV testing and were treated with bilateral open surgical release with or without flexor tenosynsvectomy. The EMG/NCV testing revealed normal results in 5 patients who are subsequently being monitored. Forty-five digits in 8 children were diagnosed clinically with trigger digits. Nineteen digits were treated by annular pulley release alone. Twenty-six digits were treated by annular pulley release with partial flexor digitorum superficialis tendon resection. The average age at the time of hand surgery was 6.3 years, and at the lime of follow-up, 9.6 years. Postoperative EMG/NCV testing in 7 children showed 1 with improvement and 6 with normalization. None of the patients undergoing carpal tunnel release went on to develop thenar atrophy or absent sensibility, as has been reported in ii untreated cases. Patients were evaluated for triggering digits both by preoperative tendon palpation and by intraoperative flexor tendon excursion at the time of open carpal tunnel release. All patients undergoing trigger release had improved active digital flexion seen at the final follow-up visit. Because of the very high incidence of CTS and trigger digits in this population, the authors currently recommend routine screening of EMG/NCV for all children with MPSDs. Early surgical intervention for nerve compression and stenosing flexor tenosynovitis can maximize hand function in these children. Copyright (C) 1998 by the American Society for Surgery of the Hand.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1016/s0363-5023(98)80120-2" target="_blank" rel="noreferrer noopener">10.1016/s0363-5023(98)80120-2</a>
Rights
Information about rights held in and over the resource
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
1998
annular pulley release
bone-marrow transplantation
House J
hurler-syndrome
Journal of Hand Surgery
Krivit W
MPSI
MPSIII
MPSVI
Orthopedics
partial flexor digitorum superficialis tendon resection
Surgery
surgical intervention
tone and motor problems
Van Heest A E
Walker K
-
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1016/j.ijporl.2014.06.027" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.ijporl.2014.06.027</a>
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Comparison of treatment modalities in syndromic children with Obstructive Sleep Apnea-A randomized cohort study
Publisher
An entity responsible for making the resource available
International Journal of Pediatric Otorhinolaryngology
Date
A point or period of time associated with an event in the lifecycle of the resource
2014
Subject
The topic of the resource
quality of life; major clinical study; priority journal; follow up; randomized controlled trial; mucopolysaccharidosis; outcome assessment; clinical effectiveness; human; article; child; female; male; controlled study; adenotonsillectomy; apnea hypopnea index; Continuous Positive Airway Pressure; CPAP device; Down syndrome; Epworth sleepiness scale; ess-c; Mucopolysaccharidoses; osa-18; Pediatric obstructive sleep apnea; positive end expiratory pressure; radiofrequency ablation device; sleep disordered breathing/su [Surgery]; sleep disordered breathing/th [Therapy]; treatment outcome; breathing difficulties; MPSI; MPSII; MPSIII; MPSIIIA; MPSIIIB; MPSVI; MPSVII; surgical interventions; physical interventions; adenotonsillectomy
Creator
An entity primarily responsible for making the resource
Sudarsan S S; Paramasivan V K; Arumugam S V; Murali S; Kameswaran M
Description
An account of the resource
Introduction: Obstructive Sleep Apnea (OSA) is a common medical problem in adults that is becoming increasingly recognized in children. It occurs in the pediatric age group, from newborns to teens. More recently, many specialists have estimated OSA prevalence to be between 5 and 6%. However, in syndromic children, the prevalence of OSA can be from 50 to 100%, having a significant effect on their Quality-of-Life. As they are a challenging population for management, it is essential to evaluate them thoroughly before planning appropriate intervention. Objective: To compare the efficacy of Adenotonsillectomy (T&A) and Continuous Positive Airway Pressure (CPAP) in syndromic children [Down syndrome (DS) and Mucopolysaccharidoses (MPS)] with Obstructive Sleep Apnea (OSA). Materials and methods: In a prospective, randomized, cohort comparative study, 124 syndromic children (DS and MPS) aged between 6 and 12 years were recruited from a private MPS support group and the Down Syndrome Society, Chennai. A standard assessment was performed on all children who entered the study including a full overnight Polysomnogram (PSG), Epworth Sleepiness Scale-Children (ESS-C) and Quality-of-Life (QOL) tool OSA-18. The children with positive PSG who consented for the study (n= 80) were randomly distributed to two groups, T&A group & CPAP group. The children were followed up with repeat PSG, clinical evaluation, ESS-C and Quality-of-Life (QOL) tool OSA-18 for a period of 1 year. Observation and results: Follow-up was available for 73 syndromic children. Both the groups, T&A group and CPAP group, showed statistically significant (p<. 0.05) improvement in Apnea-Hypoapnea Index (AHI), ESS-C, QOL from the intervention. In our study, T&A showed equal outcome compared to CPAP. The contrasting feature between the two groups was that CPAP use gave immediate sustained improvement while T&A gave gradual progressive improvement of symptoms over a period of 1 year. Conclusion: On average, T&A gives equal outcomes as CPAP and it can be suggested as a first-line treatment in this group of syndromic children. © 2014.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1016/j.ijporl.2014.06.027" target="_blank" rel="noreferrer noopener">10.1016/j.ijporl.2014.06.027</a>
Rights
Information about rights held in and over the resource
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2014
adenotonsillectomy
apnea hypopnea index
Article
Arumugam S V
breathing difficulties
Child
Clinical Effectiveness
Continuous Positive Airway Pressure
Controlled Study
CPAP device
Down Syndrome
Epworth sleepiness scale
ess-c
Female
Follow Up
Human
International Journal of Pediatric Otorhinolaryngology
Kameswaran M
Major Clinical Study
Male
MPSI
MPSII
MPSIII
MPSIIIA
MPSIIIB
MPSVI
MPSVII
Mucopolysaccharidoses
Mucopolysaccharidosis
Murali S
osa-18
outcome assessment
Paramasivan V K
Pediatric obstructive sleep apnea
physical interventions
positive end expiratory pressure
Priority Journal
Quality Of Life
radiofrequency ablation device
Randomized Controlled Trial
sleep disordered breathing/su [Surgery]
sleep disordered breathing/th [Therapy]
Sudarsan S S
surgical interventions
Treatment Outcome
-
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1016/j.ymgme.2015.02.008" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.ymgme.2015.02.008</a>
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Quantifying behaviors of children with Sanfilippo syndrome: The Sanfilippo Behavior Rating Scale
Publisher
An entity responsible for making the resource available
Molecular Genetics and Metabolism
Date
A point or period of time associated with an event in the lifecycle of the resource
2015
Subject
The topic of the resource
Medicine; amygdala volume; autism; Behavior phenotype; Behavior rating scale; Endocrinology & Metabolism; Genetics & Heredity; kluver; management; mucopolysaccharidosis type iiia; MPSIII; Research & Experimental; Sanfilippo Syndrome; behavioral problems; trajectory; characteristics
Creator
An entity primarily responsible for making the resource
Shapiro E G; Nestrasil I; Ahmed A; Wey A; Rudser K R; Delaney K A; Rumsey R K; Haslett P A J; Whitley C B; Potegal M
Description
An account of the resource
The Sanfilippo Behavior Rating Scale (SBRS), a 68 item questionnaire, has been developed to assess the behavioral phenotype of children with Sanfilippo syndrome and its progression overtime. Fifteen scales rate orality, movement/activity, attention/self-control, emotional function including anger and fear, and social interaction. Items within scales intercorrelate; measures of internal consistency are adequate. Twelve scales are grouped into 4 abnormality clusters: Movement, Lack of fear, Social/emotional and Executive Dysfunction. A Loess age-trajectory analysis showed that Lack of Fear, Social/Emotional and Executive Dysfunction increased steadily with age; Orality and Mood/Anger/Aggression leveled off. Movement peaked around 6 years, then declined as children's excessive/purposeless actions stopped. Compared with standard scales, SBRS Movement was appropriately associated with the Vineland Motor scale; SBRS Lack of Fear had significant associations with the Autism Diagnostic Observation Schedule (ADOS), indicating a symptom overlap between Sanfilippo syndrome and autism. This suggests that reduced fearfulness may be the most salient/sensitive SBRS marker of disease progression. Volumetric MRI showed that increased Lack of Fear was significantly associated with reduced amygdala volume, consistent with our hypothesis that the behavior seen in Sanfilippo syndrome is a variant of Kluver-Bucy syndrome. Hippocampal volume loss had twice the effect on Social-Emotional Dysfunction as amygdala loss, consistent with a hippocampal role in attachment and social emotions. In conclusion, the SBRS assesses the Sanfilippo behavioral phenotype; it can measure behavior change that accompanies disease progression and/or results from treatment (C) 2015 Elsevier Inc. All rights reserved.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1016/j.ymgme.2015.02.008" target="_blank" rel="noreferrer noopener">10.1016/j.ymgme.2015.02.008</a>
Rights
Information about rights held in and over the resource
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2015
Ahmed A
amygdala volume
Autism
Behavior phenotype
Behavior Rating Scale
behavioral problems
characteristics
Delaney K A
Endocrinology & Metabolism
Genetics & Heredity
Haslett P A J
kluver
Management
Medicine
Molecular Genetics and Metabolism
MPSIII
mucopolysaccharidosis type iiia
Nestrasil I
Potegal M
Research & Experimental
Rudser K R
Rumsey R K
Sanfilippo syndrome
Shapiro E G
Trajectory
Wey A
Whitley C B
-
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1017/s0012162201002171" target="_blank" rel="noreferrer noopener">http://doi.org/10.1017/s0012162201002171</a>
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Melatonin treatment for sleep disorders in children with neurodevelopmental disorders: an observational study
Publisher
An entity responsible for making the resource available
Developmental Medicine and Child Neurology
Date
A point or period of time associated with an event in the lifecycle of the resource
2002
Subject
The topic of the resource
Male; Nervous System Diseases; Medical Records; Nervous System Diseases/complications; Sex Factors; Dose-Response Relationship Drug; Child; Humans; Adolescent; Female; Child Preschool; Infant; Developmental Disabilities; Epilepsy/complications; Epilepsy; Outpatients; Vision Disorders/complications; Developmental Disabilities/complications; Melatonin/therapeutic use; Sleep Disorders/complications/drug therapy; Q3 Literature Search; Melatonin; Sleep Wake Disorders; child; female; male; Vision Disorders; sleep disturbance/disorders; Lennox-Gastaut syndrome; leukodystrophy; MPSII; MPSIII; pharmacologic intervention; melatonin
Creator
An entity primarily responsible for making the resource
Ross C; Davies P; Whitehouse W
Description
An account of the resource
The study aim was to quantify melatonin-associated improvement in sleep by means of a parent-completed sleep diary during routine outpatient activity. An investigation into sleep disturbance was made at neurology outpatient appointments. Those parents who identified a problem were asked to complete a sleep diary, after which treatment was initiated. The first week of the diary was completed before treatment, the second when established on the maximum dose of melatonin required. Forty-nine patients (26 males, 23 females) aged from one to 13 years, were treated between 1997 and 1998: 28 of these returned interpretable diaries. In a further 18 patients, an assessment could be made of the usefulness of the treatment. Patients were fairly typical of those attending a tertiary centre, the most common primary diagnosis being epilepsy (n=26). Only seven patients were visually impaired. Of the 46 patients who were assessed, 34 showed an improvement. No adverse effects were attributed to the treatment.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1017/s0012162201002171" target="_blank" rel="noreferrer noopener">10.1017/s0012162201002171</a>
Rights
Information about rights held in and over the resource
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2002
Adolescent
Child
Child Preschool
Davies P
Developmental Disabilities
Developmental Disabilities/complications
Developmental Medicine and Child Neurology
Dose-Response Relationship Drug
Epilepsy
Epilepsy/complications
Female
Humans
Infant
Lennox-Gastaut syndrome
Leukodystrophy
Male
Medical Records
melatonin
Melatonin/therapeutic use
MPSII
MPSIII
Nervous System Diseases
Nervous System Diseases/complications
Outpatients
pharmacologic intervention
Q3 Scoping Review Results
Ross C
Sex Factors
Sleep Disorders/complications/drug therapy
sleep disturbance/disorders
Sleep Wake Disorders
Vision Disorders
Vision Disorders/complications
Whitehouse W
-
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1111/j.1365-2788.1983.tb00291.x" target="_blank" rel="noreferrer noopener">http://doi.org/10.1111/j.1365-2788.1983.tb00291.x</a>
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Developmental and degenerative patterns associated with cognitive, behavioural and motor difficulties in the Sanfilippo syndrome: an epidemiological study
Publisher
An entity responsible for making the resource available
Journal of Mental Deficiency Research
Date
A point or period of time associated with an event in the lifecycle of the resource
1983
Subject
The topic of the resource
Parents; Family; Male; Child; Humans; Adult; Adolescent; Child Development; Stress Psychological; Female; Child Preschool; Motor Skills; Infant; Child Behavior Disorders; Cognition; Mucopolysaccharidoses; Mucopolysaccharidosis III; behavioral problems; feeding difficulties; urinary incontinence; MPSII; MPSIII; trajectory; characteristics; hyperactivity; sleep disturbance; sleep problems; physical aggression; aggression
Creator
An entity primarily responsible for making the resource
Nidiffer F D; Kelly T E
Description
An account of the resource
The results of a survey given to parents with Sanfilippo syndrome (MPS III) children (n = 30) are described with an emphasis in several areas. Developmental and degenerative patterns are outlined in detail and contrasted with developmental milestones of normal children. An essentially uniform pattern of behaviour problems was discovered and is discussed along with the efficacy of pharmacological and non-pharmacological approach to remediation. Finally, practical suggestions are offered for physicians and parents which are encountered by the daily management problems of children with this disorder.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1111/j.1365-2788.1983.tb00291.x" target="_blank" rel="noreferrer noopener">10.1111/j.1365-2788.1983.tb00291.x</a>
Rights
Information about rights held in and over the resource
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
1983
Adolescent
Adult
Aggression
behavioral problems
characteristics
Child
Child Preschool
Child Behavior Disorders
Child Development
Cognition
Family
feeding difficulties
Female
Humans
hyperactivity
Infant
Journal of Mental Deficiency Research
Kelly T E
Male
Motor Skills
MPSII
MPSIII
Mucopolysaccharidoses
Mucopolysaccharidosis III
Nidiffer F D
Parents
physical aggression
sleep disturbance
sleep problems
Stress Psychological
Trajectory
urinary incontinence
-
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1097/MPG.0000000000001543" target="_blank" rel="noreferrer noopener">http://doi.org/10.1097/MPG.0000000000001543</a>
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Feed-induced Dystonias in Children with Severe Central Nervous System Disorders
Publisher
An entity responsible for making the resource available
Journal of Pediatric Gastroenterology and Nutrition
Date
A point or period of time associated with an event in the lifecycle of the resource
2017
Subject
The topic of the resource
infant; adolescent; cerebral palsy; percutaneous endoscopic gastrostomy; retrospective study; priority journal; practice guideline; case study; school child; preschool child; intestine obstruction; human; article; child; female; male; clinical article; Rett syndrome; gastroesophageal reflux; infantile spasm; intestinal dysmotility; baclofen/dt [Drug Therapy]; dystonia/dt [Drug Therapy]; motor dysfunction; onset age; central nervous system disease; dystonia/dt [Drug Therapy]; feed induced dystonia; 1134-47-0 (baclofen); acquired brain injury; baclofen/tl [Intrathecal Drug Administration]; feed induced dystonia/dt [Drug Therapy]; gastroscopy; hydrocephalus; hyperglycinemia; intestine motility; microcephaly; peristalsis; reflux esophagitis; Sanfilippo syndrome; total parenteral nutrition; constipation; feeding difficulties; tone and motor problems; MPSIII; West syndrome; pharmacologic intervention; parenteral nutrition; dystonia; GERD; intestinal dysmotility
Creator
An entity primarily responsible for making the resource
Mordekar S R; Velayudhan M; Campbell D I
Description
An account of the resource
Dystonias can arise from any painful stimuli in neurologically disabled children. Classically, feed-induced dystonias from mediastinal pain due to severe gastroesophageal reflux disease are described as Sandifer spasm. We report a case series of 12 severely neurologically impaired children with enteral feed-induced dystonias. Intestinal dysmotility was demonstrated in several. Improvements are seen with jejunal feeds or gut rest with total parenteral nutrition. Use of parenteral nutrition in children with severe neurodisability requires thorough discussion with patient groups and commissioners to give clinicians guidelines to standardize care.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1097/MPG.0000000000001543" target="_blank" rel="noreferrer noopener">10.1097/MPG.0000000000001543</a>
Rights
Information about rights held in and over the resource
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
1134-47-0 (baclofen)
2017
acquired brain injury
Adolescent
Article
Baclofen/dt [drug Therapy]
baclofen/tl [Intrathecal Drug Administration]
Campbell D I
Case Study
central nervous system disease
Cerebral Palsy
Child
Clinical Article
Constipation
Dystonia
dystonia/dt [Drug Therapy]
feed induced dystonia
feed induced dystonia/dt [Drug Therapy]
feeding difficulties
Female
Gastroesophageal Reflux
Gastroscopy
GERD
Human
Hydrocephalus
Hyperglycinemia
Infant
infantile spasm
intestinal dysmotility
intestine motility
Intestine Obstruction
Journal Of Pediatric Gastroenterology And Nutrition
Male
microcephaly
Mordekar S R
motor dysfunction
MPSIII
onset age
Parenteral Nutrition
Percutaneous Endoscopic Gastrostomy
peristalsis
pharmacologic intervention
Practice Guideline
Preschool Child
Priority Journal
reflux esophagitis
Retrospective Study
Rett syndrome
Sanfilippo syndrome
School Child
tone and motor problems
total parenteral nutrition
Velayudhan M
West syndrome
-
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1016/S0165-5876(01)00417-7" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/S0165-5876(01)00417-7</a>
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Disordered breathing during sleep in patients with mucopolysaccharidoses
Publisher
An entity responsible for making the resource available
International Journal of Pediatric Otorhinolaryngology
Date
A point or period of time associated with an event in the lifecycle of the resource
2001
Subject
The topic of the resource
breathing difficulties; sleep disturbance; Galactosialidosis; MPSI; MPSII; MPSIII; MPSIIIA; MPSIIIB; MPSVI; MPSVII; trajectory; characteristics; obstructive sleep apnea; OSA; frequent awakenings; poor sleep quality
Creator
An entity primarily responsible for making the resource
Leighton SEJ; Papsin B; Vellodi A; Dinwiddie R; Lane R
Description
An account of the resource
Objective: Obstructive sleep apnoea (OSA) has been reported as a feature of children with mucopolysaccharidoses (MPS). However, the incidence and severity of OSA with respect to disease type is poorly defined. The aim of the present study was to measure objectively the degree of OSA in a group of children with a range of MPS syndromes. Methods: In a cross-sectional study, cardiopulmonary sleep studies were performed during unsedated sleep in 26 children with MPS over a period of 2 years. Scores of OSA severity based upon clinical history and upon objective sleep study data were made in each case and compared. Results: OSA was present in 24/26 patients, and ranged in severity from mild to severe. OSA was most marked in MPS type IH (Hurler syndrome) followed by types IHS (Hurler–Scheie syndrome) and II (Hunter syndrome). Frequent arousals and poor sleep quality, not suspected clinically, were noted in several patients. There was agreement between the clinical and objective scoring systems in only 17/26 patients (65%) with clinical history scores tending to underestimate the most severe cases (5/26 cases) and overestimate the severity in the mild cases (4/26 cases). Conclusions: Obstructive respiratory problems are frequent in MPS patients and there are differences in severity of OSA between the different MPS types. Assessments of the severity of OSA based upon clinical history alone are inadequate. Our results suggest that objective sleep studies are necessary to evaluate these cases, to monitor clinical outcome and to assess the effects of therapeutic intervention. Prospective studies in larger numbers of patients are needed to validate these observations.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1016/S0165-5876(01)00417-7" target="_blank" rel="noreferrer noopener">10.1016/S0165-5876(01)00417-7</a>
Rights
Information about rights held in and over the resource
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2001
breathing difficulties
characteristics
Dinwiddie R
frequent awakenings
Galactosialidosis
International Journal of Pediatric Otorhinolaryngology
Lane R
Leighton SEJ
MPSI
MPSII
MPSIII
MPSIIIA
MPSIIIB
MPSVI
MPSVII
obstructive sleep apnea
OSA
Papsin B
poor sleep quality
sleep disturbance
Trajectory
Vellodi A
-
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1302/0301-620X.79B4.7547" target="_blank" rel="noreferrer noopener">http://doi.org/10.1302/0301-620X.79B4.7547</a>
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Carpal tunnel syndrome in the mucopolysaccharidoses and mucolipidoses
Publisher
An entity responsible for making the resource available
Journal of Bone and Joint Surgery - Series B
Date
A point or period of time associated with an event in the lifecycle of the resource
1997
Subject
The topic of the resource
tone and motor problems; MLIII; MPSI; MPSII; MPSIII; MPSVI; surgical interventions; physical interventions; surgery; physiotherapy; exercises; simultaneous tendon release
Creator
An entity primarily responsible for making the resource
Haddad F S; Jones D H A; Vellodi A; Kane N; Pitt M C
Description
An account of the resource
Children with a mucopolysaccharidosis or mucolipidosis suffer progressive disability of the hands, particularly in relation to dysfunction of the median nerve. This is an increasing problem because bone-marrow transplantation has dramatically improved survival without apparently changing the musculoskeletal manifestations. We have reviewed 48 children with these syndromes who required carpal tunnel decompression, recording symptoms, signs, radiological, electrophysiological and operative findings, histology and upper-limb function. In these children the carpal tunnel syndrome differs from that seen in adults. Symptoms are rare but signs such as decreased sweating, pulp atrophy, thenar wasting and manual clumsiness are much more common. At operation, the flexor retinaculum was thickened and a mass of white tenosynovium engulfed the flexor tendons. Most patients had some definite nerve constriction with a thickened epineurium. Functional improvement was seen after early decompression, with some benefit from simultaneous tendon release. Regular physiotherapy helped to maintain increased hand movement. We describe our assessment protocol, the physiotherapy and operative regime and the standard functional review which helps to maximise function in the hands and upper limbs of these children.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1302/0301-620X.79B4.7547" target="_blank" rel="noreferrer noopener">10.1302/0301-620X.79B4.7547</a>
Rights
Information about rights held in and over the resource
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
1997
exercises
Haddad F S
Jones D H A
Journal of Bone and Joint Surgery - Series B
Kane N
MLIII
MPSI
MPSII
MPSIII
MPSVI
physical interventions
Physiotherapy
Pitt M C
simultaneous tendon release
Surgery
surgical interventions
tone and motor problems
Vellodi A