Browse Items · Pediatric Palliative Care Library

Behavioural phenotypes of the mucopolysaccharide disorders: a systematic literature review of cognitive, motor, social, linguistic and behavioural presentation in the MPS disorders

The mucopolysaccharide disorders (MPS) are a group of recessively inherited metabolic disorders resulting in progressive physical and cognitive decline. MEDLINE, PsycINFO and Embase databases were searched, alongside manual screening, to identify…

Carpal tunnel syndrome in the mucopolysaccharidoses and mucolipidoses

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…

Tags: 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

Comparison of treatment modalities in syndromic children with Obstructive Sleep Apnea-A randomized cohort study

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…

Tags: 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

Disordered breathing during sleep in patients with mucopolysaccharidoses

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…

Gross motor abilities in children with Hurler syndrome

Hurler syndrome is the most severe form of mucopolysaccharidosis type I. There is a paucity of literature reporting the gross motor abilities of children with untreated Hurler syndrome. The purpose of this case series is to describe the gross motor…

Tags: 2006, characteristics, Developmental Medicine and Child Neurology, Dusing S C, Escolar M L, Female, Humans, Infant, Male, Mercer V, Motor Activity, MPSI, Mucopolysaccharidosis I, Rosenberg A, Thorpe D, tone and motor problems, Trajectory

Respiratory complications of mucopolysaccharide storage disorders

Twenty-one patients with the diagnosis of mucopolysaccharidosis or mucolipidosis and a history of respiratory complaints or thorough respiratory evaluation were studied retrospectively. Anatomic factors affecting respiratory status included: (i)…

Tags: 1988, adenoidectomy, Adolescent, Adult, Airway Obstruction/et [Etiology], Anesthesia General/ae [Adverse Effects], beta adrenergic blockers, breathing difficulties, calcium antagonists, Cardiovascular Diseases/et [Etiology], Child, Child Preschool, digoxin, diuretics, Female, Humans, Infant, Intubation Intratracheal/ae [Adverse Effects], Lung Diseases/et [Etiology], Lung Diseases/pp [Physiopathology], Male, Medicine, MLII, MLIII, MPSI, MPSII, MPSVI, Mucopolysaccharidoses/co [Complications], Mucopolysaccharidoses/mo [Mortality], Mucopolysaccharidoses/pp [Physiopathology], pharmacologic interventions, Pyeritz R E, respiratory problems, Respiratory Tract Diseases/et [Etiology], Respiratory Tract Diseases/pp [Physiopathology], Respiratory Tract Diseases/ra [Radiography], Retrospective Studies, Semenza G L, Sleep Apnea Syndromes/et [Etiology], Spinal Diseases/et [Etiology], Spinal Diseases/ra [Radiography], surgical interventions, theophylline, Tonsillectomy, Tracheostomy

Surgical treatment of carpal tunnel syndrome and trigger digits in children with mucopolysaccharide storage disorders

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…

Tags: 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

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