Morquio syndrome/mucopolysaccharidosis type IV/keratan sulfaturia
William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop in Atlas of Inherited Metabolic Diseases, 2020
The clinical pictures in types A and B are indistinguishable. In both, there is considerable heterogeneity ranging from mild to severe, including even hydrops fetalis phenotypes. The most characteristic features of this syndrome are skeletal deformities and shortness of stature, which is particularly short-trunked, though the long bones are also involved. The neck is short and the head appears to sit directly on the barrel chest, which classically has a very pronounced pectus carinatum (Figures 80.2–80.8). The upper part of the sternum may be almost horizontal. There is also a pronounced genu valgum, and patients often have a semi-crouching stance. The joints are enlarged and prominent (Figures 80.9–80.14). On the other hand, as a result of ligamentous laxity, there is usually extreme hypermobility and hyperextension of the joints, particularly at the wrists, where there may be marked ulnar deviation (Figure 80.10). Joints may become stiff with age. Pes planus is also seen.
Digital Deformities in Rheumatoid Arthritis
J. Terrence Jose Jerome in Clinical Examination of the Hand, 2022
The ulnar interossei and more specifically the abductor digiti minimi of the fifth finger, contribute not only to the MCP flexion but also to its ulnar deviation. At the level of the long and middle fingers, the palmar plate and the neighbouring structures which are detached from it move on the ulnar edge of the finger under the action of the flexors whose path is oblique with respect to the axis of the A1 pulley. Radial tilt of the wrist, collapsed wrist and flexion of the fourth and fifth metacarpals contribute to the aggravation of the ulnar deviation.
Hands
Tor Wo Chiu in Stone’s Plastic Surgery Facts, 2018
Ulnar deviation is caused by normal use/anatomical predispositions: Thumb pressure during pinch gripUlnar inclination of MC headsADM action as a strong ulnar deviator
Functional passive range of motion of individuals with chronic cervical spinal cord injury
Published in The Journal of Spinal Cord Medicine, 2020
Sara Kate Frye, Paula Richley Geigle, Henry S. York, W. Mark Sweatman
The relationship between PROM and function. A Spearman's Rho correlation examined the relationship between functional skills and PROM (P ≤ 0.05). Upper limb: Shoulder horizontal adduction displayed a significant association with the most functional tasks (Table 4). Significant associations were found between horizontal adduction and overall ADL and mobility status as measured by the SCIM-III and the SCI-FI subsections, as well as the SCIM-III feeding, dressing, and bathing domains. Elbow extension was associated with overall SCIM-III scores and the lower body bathing subtest. Shoulder flexion was associated with SCIM-III Feeding (P = 0.029), and shoulder abduction was associated with SCIM-III grooming (P = 0.046). Wrist ulnar deviation was associated with lower body bathing (P = 0.035). Lower limb: The most significant lower extremity correlations arose from hip and knee flexion, where greater PROM was associated with improved performance in ADLs and mobility as measured by the SCIM-III, as well as a large majority of the subtests (see Table 4). Interestingly, hip and knee flexion ROM increased with lower injury levels (Table 2). Additional associations included both straight leg raise, (P = 0.037) and forefoot inversion (P = 0.049) with SCIM-III Grooming.
A new assessment tool for ulnar drift in patients with rheumatoid arthritis using pathophysiological parameters of the metacarpophalangeal joint
Published in Modern Rheumatology, 2019
Shogo Toyama, Ryo Oda, Daisaku Tokunaga, Daigo Taniguchi, Satoru Nakamura, Maki Asada, Hiroyoshi Fujiwara, Toshikazu Kubo
Our pathophysiological parameters of the MP joint were applied to a cluster analysis, in which a total of 297 hands were grouped into five clusters. When these clusters were sorted by duration of RA, the cluster number increased and the parameters had increased grades, meaning that UD worsens as RA duration increases. Bone destruction worsened as the cluster number increased, and it was determined that bone destruction could progress at any stage depending on disease activity control. Subluxation worsened from cluster 1 to cluster 3 and became unreducible after that. Almost no deviation was present in clusters 1 and 2, but the deviation angle worsened moderately in cluster 3. Interestingly, cluster 4 showed no deviation in which the MP joint became unreducible; however, deviation worsened again to the largest degree in cluster 5. The two patterns of change from cluster 2 to cluster 3 or 4 indicate that there are two types of progression in which the deviation worsens earlier, or the MP joint dislocation occurs first. These alterations over time may indicate the natural course of what we call ‘ulnar deviation’ – it starts with bone destruction and subluxation of the MP joint. In some cases, finger deviation worsens even though the MP joints are still reducible, while in other cases, the MP joint becomes unreducible earlier than the finger deviation worsens. Finally, when the condition subsequently progresses, finger deviation and joint dislocation become the most severe.
Surgical fixation techniques in four-corner fusion of the wrist: a systematic review of 1103 cases
Published in Journal of Plastic Surgery and Hand Surgery, 2023
Octavian Andronic, Raffael Labèr, Philipp Kriechling, Daniel Karczewski, Andreas Flury, Ladislav Nagy, Andreas Schweizer
All 29 studies reported ROM either as total flexion–extension arc or with single values for flexion and extension. Two studies reported their values as percentage of the contralateral side and were excluded in the calculation performed but reported in Table 3. Total flexion-extension arc in the locking plate group was 63° (range 48 − 79°), non-locking plates 62° (range 52 − 78°), compression screws 53° (range 49 − 57°), staples 64° (range 60 − 68°) and k-wire 68° (range 45 − 80°), respectively. Radial- and ulnar deviation was reported in 24 studies. Two of them reported values as percentage of contralateral side (Table 3). The mean arc was 31° (range 24 − 53°). Radio-Ulnar-Arc was 28° (range 25 − 30°), 31° (range 27 − 37°), 31° and 36° (range 24 − 53°) for locking plates, non-locking plates, staple fixation and k-wire fixation, respectively (Table 3).
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