Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Triggering in the mucopolysaccharidoses

F S Haddad1, R A Hill, D H Jones

  • 1Department of Orthopaedics, Hospital for Sick Children, London, United Kingdom.

Journal of Pediatric Orthopedics. Part B
|May 23, 1998
PubMed
Summary
This summary is machine-generated.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Black Hole Spectroscopy and Tests of General Relativity with GW250114.

Physical review letters·2026
Same author

GW250114: Testing Hawking's Area Law and the Kerr Nature of Black Holes.

Physical review letters·2025
Same author

Improving cosmological reach of a gravitational wave observatory using Deep Loop Shaping.

Science (New York, N.Y.)·2025
Same author

SEPN1-related myopathies: clinical course in a large cohort of patients.

Neurology·2011
Same author

Markers for the identification of tumours arising from the neuroectoderm.

Reviews in the neurosciences·2011
Same author

Use of polymerase chain reaction for making recombinant constructs.

Methods in molecular biology (Clifton, N.J.)·2011
Same journal

Risk stratification in conservatively treated pediatric lateral humeral condyle fractures: the prognostic value of articular gap and initial displacement - a retrospective cohort study.

Journal of pediatric orthopedics. Part B·2026
Same journal

Surgical management of unstable juvenile osteochondritis dissecans of the knee: clinical outcomes, return to sport, complications, and reoperation rates with an average follow-up of 6.5 years.

Journal of pediatric orthopedics. Part B·2026
Same journal

Reliability of the Beit CURE classification for the management of chronic hematogenous osteomyelitis of the long bones in children.

Journal of pediatric orthopedics. Part B·2026
Same journal

Surgical outcomes for displaced lateral humeral condyle fractures in children: a retrospective study comparing two types of tension band wiring procedures and pinning.

Journal of pediatric orthopedics. Part B·2026
Same journal

Finding the physeal midpoint for distal femoral hemiepiphysiodesis utilizing novel anatomic measurements.

Journal of pediatric orthopedics. Part B·2026
Same journal

Association between vitamin D deficiency and adolescent idiopathic scoliosis.

Journal of pediatric orthopedics. Part B·2026
See all related articles

Mucopolysaccharidoses and mucolipidoses cause upper limb dysfunction, including carpal tunnel syndrome. Surgical intervention for triggering at the carpal tunnel in these patients showed favorable outcomes, improving hand function.

Area of Science:

  • Orthopedics
  • Genetics
  • Neurology

Background:

  • Mucopolysaccharidoses (MPS) and mucolipidoses (ML) are genetic disorders causing skeletal dysplasia and upper limb dysfunction.
  • Median nerve dysfunction, including triggering and carpal tunnel syndrome, is a known complication in MPS and ML patients.
  • Improved survival due to bone marrow transplantation highlights the need to address persistent musculoskeletal issues.

Purpose of the Study:

  • To investigate triggering at the carpal tunnel in patients with mucopolysaccharidoses and mucolipidoses.
  • To evaluate the efficacy of surgical intervention for carpal tunnel syndrome in this patient population.

Main Methods:

  • Case report of two patients with triggering at the A1 pulley and distally.
  • Identification of four additional patients with triggering at the carpal tunnel and carpal tunnel syndrome.

Related Experiment Videos

  • Surgical intervention for five of the six affected patients.

    • Main Results:

    • Triggering at the carpal tunnel was observed in association with carpal tunnel syndrome in MPS/ML patients.
    • Surgical intervention in five patients resulted in favorable outcomes.
    • The study highlights a significant upper limb manifestation in MPS and ML.

    Conclusions:

    • Carpal tunnel syndrome and triggering are significant upper limb issues in mucopolysaccharidoses and mucolipidoses.
    • Surgical treatment for carpal tunnel syndrome offers positive results in these patients.
    • Addressing musculoskeletal complications is crucial for improving quality of life in MPS/ML survivors.