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Wrist hypermobility

MedGen UID:
340648
Concept ID:
C1850853
Finding
Synonym: Hyperextensibility at wrists
 
HPO: HP:0005072

Definition

The ability of the wrist joints to move beyond their normal range of motion. [from HPO]

Term Hierarchy

Conditions with this feature

Ullrich congenital muscular dystrophy 1A
MedGen UID:
98046
Concept ID:
C0410179
Disease or Syndrome
Collagen VI-related dystrophies (COL6-RDs) represent a continuum of overlapping clinical phenotypes with Bethlem muscular dystrophy at the milder end, Ullrich congenital muscular dystrophy (UCMD) at the more severe end, and a phenotype in between UCMD and Bethlem muscular dystrophy, referred to as intermediate COL6-RD. Bethlem muscular dystrophy is characterized by a combination of proximal muscle weakness and joint contractures. Hypotonia and delayed motor milestones occur in early childhood; mild hypotonia and weakness may be present congenitally. By adulthood, there is evidence of proximal weakness and contractures of the elbows, Achilles tendons, and long finger flexors. The progression of weakness is slow, and more than two thirds of affected individuals older than age 50 years remain independently ambulatory indoors, while relying on supportive means for mobility outdoors. Respiratory involvement is not a consistent feature. UCMD is characterized by congenital weakness, hypotonia, proximal joint contractures, and striking hyperlaxity of distal joints. Decreased fetal movements are frequently reported. Some affected children acquire the ability to walk independently; however, progression of the disease results in a loss of ambulation by age ten to eleven years. Early and severe respiratory insufficiency occurs in all individuals, resulting in the need for nocturnal noninvasive ventilation (NIV) in the form of bilevel positive airway pressure (BiPAP) by age 11 years. Intermediate COL6-RD is characterized by independent ambulation past age 11 years and respiratory insufficiency that is later in onset than in UCMD and results in the need for NIV in the form of BiPAP by the late teens to early 20s. In contrast to individuals with Bethlem muscular dystrophy, those with intermediate COL6-RD typically do not achieve the ability to run, jump, or climb stairs without use of a railing.
Deficiency of hyaluronoglucosaminidase
MedGen UID:
226942
Concept ID:
C1291490
Disease or Syndrome
Mucopolysaccharidosis type IX (MPS9) is a rare progressive lysosomal storage disorder caused by the deficiency of the enzyme hyaluronoglucosaminidase-1, which degrades hyaluronan (summary by Imundo et al., 2011).

Professional guidelines

PubMed

Thissen GCE, van Middelkoop M, Colaris JW, Selles RW, Dziedzic K, Nicholls E, Bierma-Zeinstra SMA
BMJ Open 2022 Jan 17;12(1):e057156. doi: 10.1136/bmjopen-2021-057156. PMID: 35039303Free PMC Article
de Inocencio J
Pediatrics 1998 Dec;102(6):E63. doi: 10.1542/peds.102.6.e63. PMID: 9832591

Recent clinical studies

Etiology

Karagiannopoulos C, Griech SF
J Hand Ther 2024 Apr-Jun;37(2):209-217. Epub 2024 Feb 10 doi: 10.1016/j.jht.2023.10.001. PMID: 38342640

Diagnosis

Karagiannopoulos C, Griech SF
J Hand Ther 2024 Apr-Jun;37(2):209-217. Epub 2024 Feb 10 doi: 10.1016/j.jht.2023.10.001. PMID: 38342640

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