Hand Function Scale Research Articles

Social conditions impact functional outcome in patients with hand osteoarthritis: the low-income hand osteoarthritis (LIHOA) cohort

BackgroundHand osteoarthritis (HOA) is a highly prevalent disease that may be impacted by social inequalities. Few studies in HOA are from underdeveloped regions. We intend to contribute to fill this gap presenting clinical characteristics of our low-income HOA cohort (LIHOA).MethodsData from 119 patients with a HOA diagnosis fulfilling ACR criteria seen between August 2019 and May 2023 in Fortaleza/Brazil. Evaluations included pain (VAS, visual analogue scale), X-ray (KL, Kellgren-Lawrence), grip and pinch strength (KgF), Cochin hand functional scale (CHFS), FIHOA, and SF-12 scores. Social data included monthly (<1, 1≥/<3, ≥3 MW) minimum wage earnings, occupation, and literacy [</≥ 9 school-years (SY)].Results107 out of the 119 patients were included. Mean age was 61.9 (±10.3) years with 94 (92%) women. Systemic arterial hypertension (48%), metabolic syndrome (42.8%), dyslipidemia (28.4%), and obesity (25%) were the most common comorbidities. Mean disease duration was 7.5 ± 7.1 years. Median VAS values at rest and activity were 3 (3–5) and 8 (5–9), respectively (p < 0.001). Fifty-seven (56.4%) patients had ≥4 symptomatic joints with a median of 4 (2–8) painful joints at activity. The 2nd distal interphalangeal (IF), joint was the most symptomatic (21; 23.3%) and most had >4 IF nodes. OA in other joints: 37 (36.2%) spine, 28 (29.4%) knee, 21 (20.5%) bunions. Functional impairment was mild [8 (5–14) median FIHOA]. Median serum CRP was 0.2 mg/dL (0.1–0.4) with 14 (20%) patients above reference value. Mean total KL score was 27.6 ± 13.6 with 21 (23%), 38 (41.7%), and 33 (36.2%) KL2, KL3, and KL4, respectively; 51 (54.8%) and 42 (45.2%) patients declared </≥3 MW earnings, respectively. Most declared >9SY including 37.2% with a university degree. Individuals earning <3 MW had lower pinch (p < 0.004) and grip strength (p < 0.01), and higher FIHOA scores (p < 0.007), as compared to ≥3 MW earning group. Literacy or occupation did not impact outcome. SYSADOA were used by 13 (12.7%), 6 used oral and 3 topical anti-inflammatory drugs and 2 used 5 mg/d prednisone.ConclusionClinical characteristics in our LIHOA cohort mirror those reported in affluent regions. Socioeconomic disparities influenced functional outcome in LIHOA cohort.

Relationship Between Hand Strength and Function in Duchenne Muscular Dystrophy and Spinal Muscular Atrophy: Implications for Clinical Trials.

Measurement of muscle strength and motor function is recommended in clinical trials of neuromuscular diseases, but the loss of hand strength at which motor function is impacted is not documented. To establish the relationship between hand strength and function, and to determine the strength threshold that differentiates normal and abnormal hand function in individuals with Duchenne Muscular Dystrophy (DMD) or Spinal Muscular Atrophy (SMA). Maximal handgrip and key pinch strength were measured with the MyoGrip and MyoPinch dynamometers, respectively. Hand function was assessed using the MoviPlate, the Motor Function Measure items for distal upper limb (MFM-D3-UL) and the Cochin Hand Function Scale (CHFS). Data from 168 participants (91 DMD and 77 SMA, age 6-31 years) were analyzed. Relationships between strength and function were significant (P < 0.001). Hand function was generally preserved when strength was above the strength threshold determined by Receiver-Operating Characteristic (ROC) analysis: For MFM-D3-UL, the calculated handgrip strength thresholds were 41 and 13% of the predicted strength for a healthy subject (% pred) and the key pinch strength thresholds were 42 and 26% pred for DMD and SMA, respectively. For the MoviPlate, handgrip strength thresholds were 11 and 8% pred and key pinch strength thresholds were 21 and 11% pred for DMD and SMA, respectively. For participants with sub-threshold strength, hand function scores decreased with decreasing strength. At equal % pred strength, individuals with SMA had better functional scores than those with DMD. Hand function is strength-dependent for most motor tasks. It declines only when strength falls below a disease-specific threshold. Therefore, therapies capable of maintaining strength above this threshold should preserve hand function.

Clinical trajectories of hand function impairment in systemic sclerosis: an unmet clinical need across disease subsets

BackgroundHand involvement is an early manifestation of systemic sclerosis (SSc), culprit of diagnosis and classification, and recognised major driver of disability. Impairment of hand function burdens both limited and diffuse...

Differential Item Functioning on the Cochin Hand Function Scale Among People With Systemic Sclerosis by Language, Sex, and Disease Subtype: A Scleroderma Patient-Centered Intervention Network (SPIN) Cohort Study.

To evaluate the degree that the Cochin Hand Function Scale (CHFS) generates scores that are comparable across language, sex, and disease subtype. We included participants enrolled in the Scleroderma Patient-centered Intervention Network (SPIN) Cohort who completed the CHFS at their baseline assessment between April 2014 and September 2020. Confirmatory factor analysis (CFA) was used to test unidimensionality, and multiple indicator multiple cause (MIMIC) models were used for differential item functioning (DIF) analysis based on language, sex, and disease subtype. Both intraclass correlation coefficient (ICC) and Pearson's correlation were calculated using factor scores obtained from unadjusted and DIF-adjusted MIMIC models to evaluate agreement and correlation between scores. A total of 2,155 participants were included. CFA with covarying error terms supported a good fit of the model (χ2 [127] = 1,754.671; P < 0.001; Tucker-Lewis index=0.985; comparative fit index=0.987; root mean square error of approximation=0.077). Nine items displayed statistically significant DIF for language of administration, 10 items for sex, and 10 items for disease subtype. However, the overall impact of DIF was negligible when comparing factor scores that did and did not account for DIF (ICC=0.999; r=0.999). The CHFS has score comparability in systemic sclerosis regardless of participants' language, sex, and disease subtype.

The use of transarticular external fixation by arthrodiastasis in complex open proximal humeral fractures at Hospital Militar Central Colombia: A case series

Objective To describe the clinical and surgical characteristics of external fixation technique to manage complex open proximal humerus fractures caused by high-energy firearm injuries at the Hospital Militar Central, Colombia. Methods A retrospective case series of patients with open complex proximal humerus fractures (Gustilo &amp; Anderson III A-B or Neer III and IV) caused by long range or fragmentation weapons treated with external fixation. The variables are demographic data, range of motion, consolidation measurements, and functional outcomes according to the Quick Disabilities of the Arm, Shoulder and Hand (Quick DASH) functional scale. Results Seven male patients, with high-energy injuries causing open complex proximal humerus fractures (Gustilo &amp; Anderson III A-B or Neer III or IV) underwent surgical treatment with external fixation and arthrodiastasis. Median age was 21.0 (19–24) years, mean treatment duration was 5.6 (4–7) months, and all patients completed 15 months of follow-up. Mean postoperative active movement measurements were flexion: 80° (40°–120°), abduction 85.7° (40°–130°), external rotation: 24.7° (10°–45°), and internal rotation: 23.6° (10°–45°). Mean postoperative pain according to visual analog scale was 2.7 (1–4) and mean Quick DASH was 36.2 (15.9–58). Two patients presented postoperative complications, one case each of infection and osteitis. Conclusion Complex open proximal humeral fractures caused by high-energy trauma treated with transarticular external fixation via arthrodiastasis show promising short-term and mid-term results with low complication rates, low levels of postoperative pain, and moderate functional results. This external fixation technique seems to be a valid option for the treatment of polytraumatic patients with humeral injuries. Longer follow ups and larger sample sizes studies must be presented to better characterize the clinical and satisfaction outcomes.

Characteristics of ScleroID highlighting musculoskeletal and internal organ implications in patients afflicted with systemic sclerosis

BackgroundSystemic sclerosis (SSc) is a multi-organ disease with impaired health-related quality of life (HRQoL). The EULAR SSc Impact of Disease (ScleroID) is a newly introduced SSc-specific patient-reported outcome to evaluate HRQoL in SSc.ObjectiveTo investigate the correlation between the ScleroID and the involvement of organ systems as well as disease activity/damage in a SSc cohort from a large tertiary care centre.Patients and methodsThe ScleroID and clinical characteristics including internal organ involvement and hand function were investigated in 160 consecutive patients with SSc (median age 46 (43;56) years; diffuse cutaneous SSc 55%).ResultsA strong correlation was found between the ScleroID and articular disease activity scores (DAS28-CRP, DAS28-ESR, CDAI, SDAI), a hand function performance test, the Hand Anatomy Index and muscle strength tests. Additionally, a strong significant correlation was discovered using instruments representing hand function and musculoskeletal disability including the Cochin Hand Function Scale, the Quick Questionnaire of the Disability of the Hands, Arms and the Shoulders and the Health Assessment Questionnaire Disability Index. A significant negative correlation was found between the ScleroID score and the 6-min walking test (6MWT) (rho − 0.444, p < 0.001). Clinically mild lung/heart disease did not show increased ScleroID values. The Mouth Handicap in the Scleroderma Scale and the University of California Los Angeles Scleroderma Clinical Trials Consortium gastrointestinal tract 2.0 also showed significant positive correlations to the ScleroID score (rho: 0.626, p < 0.001; rho: 0.646, p < 0.001, respectively). Patients experiencing oesophageal difficulties bore a significantly higher score compared to individuals with a normal functioning oesophagus (3.2/1.5;4.5/ vs. 2.2/1.0;3.2/, p = 0.011). Moreover, the ScleroID showed a significant positive correlation to the revised EUSTAR disease activity index and modified activity index.ConclusionIn a large single-centre cohort, the previously described ScleroID-related findings were confirmed. Furthermore, several organ involvement-related functional and performance tests showed a good correlation to the ScleroID including the 6MWT and gastrointestinal-related complaints. Many aspects of musculoskeletal damage, overall disease activity, pain and fatigue were also well represented in the ScleroID, which efficiently reflects the impact of organ involvement, disease activity and functional damage.

Evaluation Tools Developed for Rett Syndrome.

Rett syndrome (RTT) is a complex neurodevelopmental X-linked disorder associated with severe functional impairments and multiple comorbidities. There is wide variation in the clinical presentation, and because of its unique characteristics, several evaluation tools of clinical severity, behavior, and functional motor abilities have been proposed specifically for it. This opinion paper aims to present up-to date evaluation tools which have specifically been adapted for individuals with RTT often used by the authors in their clinical and research practice and to provide the reader with essential considerations and suggestions regarding their use. Due to the rarity of Rett syndrome, we found it important to present these scales in order to improve and professionalize their clinical work. The current article will review the following evaluation tools: (a) the Rett Assessment Rating Scale; (b) the Rett Syndrome Gross Motor Scale; (c) the Rett Syndrome Functional Scale; (d) the Functional Mobility Scale-Rett Syndrome; (e) the Two-Minute Walking Test modified for Rett syndrome; (f) the Rett Syndrome Hand Function Scale; (g) the StepWatch Activity Monitor; (h) the activPALTM; (i) the Modified Bouchard Activity Record; (j) the Rett Syndrome Behavioral Questionnaire; and (k) the Rett Syndrome Fear of Movement Scale. The authors recommend that service providers consider evaluation tools validated for RTT for evaluation and monitoring to guide their clinical recommendations and management. In this article, the authors suggest factors that should be considered when using these evaluation tools to assist in interpreting scores.

Home-based rehabilitation improves functional capacity and quality of life in women with systemic sclerosis: A preliminary study.

Recent initiatives, such as earlier diagnosis and treatment, have enhanced the survival of patients with systemic sclerosis (SSc). Despite these initiatives, there is extreme variability in rehabilitation strategies for these patients. In 2006, the Glittre-ADL test (TGlittre) was developed to evaluate functional capacity using multiple tasks similar to the activities of daily living (ADLs). To evaluate the impact of therapist-oriented home rehabilitation (TOHR) on functional capacity using TGlittre and to examine the effects of TOHR on physical function, hand function, and quality of life (QoL) among women with SSc. This quasi-experimental and longitudinal study included 12 women with SSc who underwent TOHR 3 times per week for 12 weeks. Before and after TOHR, functional capacity was assessed using TGlittre, physical function was examined by the Health Assessment Questionnaire Disability Index (HAQ-DI), hand function was evaluated using the Cochin Hand Functional Scale (CHFS) and handgrip strength (HGS), and QoL was evaluated using the Short Form-36 Health Survey Questionnaire (SF-36). When comparing the pre- and post-TOHR values of TGlittre, a significant reduction was found in total time (p= 0.002) and manual time (p= 0.010). There was a nonsignificant decrease in HAQ-DI scores between pre- and post-TOHR (p= 0.07). Regarding hand function, there was a significant reduction in the CHFS between pre- and post-TOHR (p= 0.036), although no significant difference was observed in HGS between pre- and post-TOHR (p= 0.08). Regarding QoL, there was an increase in all SF-36 categories, although physical function was the only category that was significantly increased (p= 0.008). After TOHR, patients with SSc are able to more quickly perform TGlittre tasks when considering both total and manual times. TOHR also positively affects manual skills and QoL.

Hyperspectral imaging in systemic sclerosis-associated Raynaud phenomenon

Background/purposeLack of robust, feasible, and quantitative outcomes impedes Raynaud phenomenon (RP) clinical trials in systemic sclerosis (SSc) patients. Hyperspectral imaging (HSI) non-invasively measures oxygenated and deoxygenated hemoglobin (oxyHb and deoxyHb) concentrations and oxygen saturation (O2 sat) in the skin and depicts data as oxygenation heatmaps. This study explored the potential role of HSI in quantifying SSc-RP disease severity and activity.MethodsPatients with SSc-RP (n = 13) and healthy control participants (HC; n = 12) were prospectively recruited in the clinic setting. Using a hand-held camera, bilateral hand HSI (HyperMed™, Waltham, MA) was performed in a temperature-controlled room (22 °C). OxyHb, deoxyHb, and O2 sat values were calculated for 78-mm2 regions of interest for the ventral fingertips and palm (for normalization). Subjects underwent a cold provocation challenge (gloved hand submersion in 15 °C water bath for 1 min), and repeated HSI was performed at 0, 10, and 20 min. Patients completed two patient-reported outcome (PRO) instruments: the Raynaud Condition Score (RCS) and the Cochin Hand Function Scale (CHFS) for symptom burden assessment. Statistical analyses were performed using the Mann-Whitney U test and a mixed effects model (Stata, College Station, TX).ResultsNinety-two percent of participants were women in their 40s. For SSc-RP patients, 69% had limited cutaneous SSc, the mean ± SD SSc duration was 11 ± 5 years, and 38% had prior digital ulcers—none currently. Baseline deoxyHb was higher, and O2 sat was lower, in SSc patients versus HC (p < 0.05). SSc patients had a greater decline in oxyHb and O2 sat from baseline to time 0 (after cold challenge) with distinct rewarming oxyHb, O2 sat, and deoxyHb trajectories versus HCs (p < 0.01). There were no significant correlations between oxyHb, deoxyHb, and O2 sat level changes following cold challenge and RCS or CHFS scores.ConclusionHyperspectral imaging is a feasible approach for SSc-RP quantification in the clinic setting. The RCS and CHFS values did not correlate with HSI parameters. Our data suggest that HSI technology for the assessment of SSc-RP at baseline and in response to cold provocation is a potential quantitative measure for SSc-RP severity and activity, though longitudinal studies that assess sensitivity to change are needed.

Effectiveness of early rehabilitation in hand burns.

Hand burn trauma occurs quite commonly and the outcome of hand burns can significantly impact self-care daily function, work and employment, leisure activities, and overall health-related quality of life. The overall goal of the management of hand burn trauma is to optimize hand function. Rehabilitation and restoration of hand function are critical for the patient's independence and re-integration into society and work. The purpose of this study is to present our experience with 105 hand burn trauma patients admitted and treated in our burn center and to show the efficacy of early rehabilitation on their ability to return to their prior social life and work. In our study, we included that 105 patients with acute severe hand burn trauma were hospitalized in Gulhane burn center between 2017 and 2021. They underwent rehabilitation program daily sessions. Patients with hand burns are evaluated by ranges of motions (ROM), grip strength, Cochin Hand Function Scale (CHFS), and Michigan Hand Questionnaire (MHQ) 12 months after the injury. Overall, mean digital total active motion were >180°. The mean values for grip strength of dominant hand for men were 27.2±9.3 kg, for women were 22.0±8.8 kg and non-dominant hand for men were 24.05±13.8 kg, for women were 17.8±10.3 kg. Total score of 5 items was 19.0 in CHFS. The mean overall score on the MHQ was 62.3±27.4. All obtained data were within normal or accepted functional ranges. Spearman correlation coefficient indicates a negative correlation between MHQ and CHFS (p≤0.01). A comprehensive rehabilitation program is essential in helping patients to regain optimal function after hand burn trauma. Physiotherapy and occupational therapy is most beneficial when started at the time of admission.

The effects of motor imagery on trapeziometacarpal osteoarthritis in women during the post-surgical immobilisation period: A protocol for a randomised clinical trial

Background: Trapeziometacarpal osteoarthritis is the second most frequent degenerative hand disease and is the most functionally debilitating. The condition presents in 66% of women over 55. Motor imagery (MI) training post-surgery could help reduce rehabilitation times. Method: It is an experimental, prospective, longitudinal, parallel arm randomised clinical trial. Participants were women over 50 years old on the surgical waiting list. The experimental group will undergo MI training during the 3-week post-surgical immobilisation period. The control group will receive standard rehabilitation treatment. Outcomes will be assessed four times throughout the study using the Disabilities of the Arm, Shoulder and Hand questionnaire, the Cochin Hand Function Scale questionnaire, the Visual Analogue Scale, goniometry, baseline pinch gauge, circumferential measurement (cm), the Modified Kapandji Index and the Kinaesthetic and Visual Imagery questionnaire. Discussion: Early MI could improve hand function leading to improvements in recovery times. Trial registration: Clinical Trials registration: NCT03815734. Ethics Committee approval: 17155. Project funded in 2021.

Randomized controlled trial of an internet-based self-guided hand exercise program to improve hand function in people with systemic sclerosis: the Scleroderma Patient-centered Intervention Network Hand Exercise Program (SPIN-HAND) trial

BackgroundSystemic sclerosis (scleroderma; SSc) is a rare autoimmune connective tissue disease. Functional impairment of hands is common. The Scleroderma Patient-centered Intervention Network (SPIN)-HAND trial compared effects of offering access to an online self-guided hand exercise program to usual care on hand function (primary) and functional health outcomes (secondary) in people with SSc with at least mild hand function limitations.MethodsThe pragmatic, two-arm, parallel-group cohort multiple randomized controlled trial was embedded in the SPIN Cohort. Cohort participants with Cochin Hand Function Scale (CHFS) scores ≥ 3 and who indicated interest in using the SPIN-HAND Program were randomized (3:2 ratio) to an offer of program access or to usual care (targeted N = 586). The SPIN-HAND program consists of 4 modules that address (1) thumb flexibility and strength; (2) finger bending; (3) finger extension; and (4) wrist flexibility and strength. The primary outcome analysis compared CHFS scores 3 months post-randomization between participants offered versus not offered the program. Secondary outcomes were CHFS scores 6 months post-randomization and functional health outcomes (Patient-Reported Outcomes Measurement Information System profile version 2.0 domain scores) 3 and 6 months post-randomization.ResultsIn total, 466 participants were randomized to intervention offer (N = 280) or usual care (N = 186). Of 280 participants offered the intervention, 170 (61%) consented to access the program. Of these, 117 (69%) viewed at least one hand exercise instruction video and 77 (45%) logged into the program website at least 3 times. In intent-to-treat analyses, CHFS scores were 1.2 points lower (95% CI − 2.8 to 0.3) for intervention compared to usual care 3 months post-randomization and 0.1 points lower (95% CI − 1.8 to 1.6 points) 6 months post-randomization. There were no statistically significant differences in other outcomes.ConclusionThe offer to use the SPIN-HAND Program did not improve hand function. Low offer uptake, program access, and minimal usage among those who accessed the program limited our ability to determine if using the program would improve function. To improve engagement, the program could be tested in a group format or as a resource to support care provided by a physical or occupational therapist.Trial registrationNCT03419208. Registered on February 1, 2018.

Investigating performance-based hand functions in patients with systemic sclerosis.

Systemic sclerosis (SSc) is aconnective tissue disorder characterized by collagen deposits in various organs. Skin involvement is one of the most common symptoms and along with vascular damage, may deteriorate hand functions. However, the status of hand functions has generally been investigated using patient-reported measures in patients with SSc. The aim of the present study was to investigate performance-based hand functions in patients with SSc using the Sollerman Hand Function Test (SHFT). A total of 39patients with SSc (33females) were included in the study. Twenty-four patients were classified as limited cutaneous SSc (lcSSc), while 15patients were classified as diffuse cutaneous SSc (dcSSc). Hand-related physical characteristics were evaluated using the Modified Hand Mobility in Scleroderma Test, grip strength, and pinch strengths. The Duruoz Hand Index (Cochin Hand Functional Disability Scale), Disability of Arm, Shoulder, and Hand Questionnaire, Health Assessment Questionnaire, and Scleroderma Health Assessment Questionnaire were used as patient-reported measures. Performance-based hand functions were evaluated using SHFT. No significant differences were observed between lcSSc and dcSSc subtypes regarding performance-based and patient-reported hand functions (p > 0.05). SHFT scores significantly correlated with hand-related physical characteristics and patient-reported hand functions (p < 0.05). The highest correlation was determined between SHFT and the Duruoz Hand Index (rho: -0.652, p < 0.001). According to our results, performance-based hand functions seem not to be affected by disease subtype. Performance-based hand functions may partially be captured by the patient-reported outcomes, especially the Duruoz Hand Index, in patients with SSc.

Adipose‐Derived Regenerative Cell Transplantation for the Treatment of Hand Dysfunction in Systemic Sclerosis: A Randomized Clinical Trial

ObjectiveHand dysfunction is common in systemic sclerosis (SSc). We undertook this study to evaluate the capacity of autologous adipose‐derived regenerative cells (ADRCs) to improve hand function in SSc patients.MethodsThe Scleroderma Treatment with Celution Processed Adipose Derived Regenerative Cells Trial was a prospective, randomized, double‐blind trial of ADRCs, in which ADRCs were obtained from patients with SSc by small‐volume adipose tissue harvest, and the fingers of each patient were injected with ADRCs. The primary end point was change in hand function at 24 and 48 weeks, assessed using the Cochin Hand Function Scale (CHFS). One of the secondary end points included the change in Health Assessment Questionnaire disability index (HAQ DI) at 48 weeks. Separate prespecified analyses were performed for patients with diffuse cutaneous SSc (dcSSc) and those with limited cutaneous SSc (lcSSc).ResultsEighty‐eight patients were randomized to receive ADRCs (n = 48 [32 patients with dcSSc and 16 with lcSSc]) or placebo (n = 40 [19 patients with dcSSc and 21 with lcSSc]). Change in hand function according to CHFS score was numerically higher for the ADRC group compared to the placebo group but did not achieve statistical significance (mean ± SD improvement in the CHFS score at 48 weeks 11.0 ± 12.5 versus 8.9 ± 10.5; P = 0.299). For patients with dcSSc, the between‐group difference in the CHFS at 48 weeks was 6.3 points (nominal P = 0.069). For the secondary end point, the dcSSc group exhibited a between‐group difference of 0.17 points in the HAQ DI (nominal P = 0.044) at 48 weeks. Of the ADRC‐treated patients with dcSSc, 52% reported improvement greater than the minimum clinically important difference for both CHFS and HAQ DI compared to 16% in the placebo group (nominal P = 0.016). Small‐volume adipose tissue harvest and ADRC treatment were well tolerated.ConclusionWhile the primary end point of this trial was not achieved, efficacy trends were observed in patients with dcSSc. Adipose tissue harvest and ADRC injection were demonstrated to be feasible. Further clinical trials of this intervention in the setting of dcSSc are warranted.

Initial Validation and Reliability of the CDKL5 Deficiency Disorder Hand Function Scale (CDD-Hand).

Pathogenic variants in the CDKL5 gene result in CDKL5 deficiency disorder (CDD), which is characterized by early-onset epilepsy, severe developmental delay, and often, cortical visual impairment. Validated clinical outcome measures are needed for future clinical trials to be successful. This study aimed to adapt the Rett Syndrome Hand Function Scale for CDKL5 deficiency disorder and evaluate its feasibility, acceptability, content validity, and reliability. Consultation with a cortical visual impairment experienced specialist and the Consumer Reference Group informed modifications to the instructions of the Rett Syndrome Hand Function Scale for children with CDKL5 deficiency disorder (CDD-Hand). Eighty-six families registered with the International CDKL5 Disorder Database provided video clips of their child's hand function and provided feedback about the measure. Video data were coded by 2 researchers to evaluate intra- and interrater reliability. This study provides initial evidence of validation and reliability. The scale appears to be suitable for a range of ages and functional abilities for CDKL5 deficiency disorder.

Efficacy and safety of mesenchymal stem cells in the treatment of systemic sclerosis: a systematic review and meta-analysis

BackgroundSystemic sclerosis (SSc) is an autoimmune disease with high morbidity and mortality characterized by fibrosis of the skin and internal organs. Some studies have investigated the use of stem cells to treat SSc. Herein, a systematic review and meta-analysis was conducted to determine the efficacy and safety of mesenchymal stem cells (MSCs) in the treatment of SSc.MethodsPubMed, Embase, Cochrane Library, Web of Science, OVID, China National Knowledge Infrastructure and Wanfang databases were searched up to February 1, 2021. Literature screening, data extraction and quality assessment were conducted independently by two researchers in according to the inclusion and exclusion criteria. The discrepancies were resolved by a third researcher.ResultsA total of 9 studies encompassing 133 SSc patients were included in the study. Compared to the baseline after treatment with MSCs: 1. The modified Rodnan skin score (mRSS) was significantly reduced in patients with SSc (P < 0.00001). 2. MSCs decreased the number of digital ulcer, mouth handicap scale, and visual analog scale of hand pain in SSc patients (P = 0.0007 and P = 0.03, respectively). 3. No statistical differences were detected in Raynaud's condition score and Cochin hand function scale score at 6 months of MSCs therapy (P = 0.5 and P = 0.62). 4. After 12 months of follow-up, MSCs improve carbon monoxide diffusing capacity and forced vital capacity of SSc patients (P < 0.05). 5. Overall, MSCs application was safe; a few cases exhibited swelling at the injection site, diarrhea and arthralgia, which had self-recovery, and no severe adverse events occurred in the included trials.ConclusionsMSC therapy improves the degree of skin thickening, lung function, and mouth opening and relieves finger ulcers and pain in patients with SSc without severe adverse events. Thus, MSCs or MSCs combined with plasma and traditional medicine might be an effective and promising treatment of SSc patients.PROSPERO registration number: CRD42020200350

Functional exercise capacity in rheumatoid arthritis unrelated to lung injury: A comparison of women with and without rheumatoid disease.

Rheumatoid arthritis (RA) mainly affects the joints of the upper and lower limbs, so evaluating functional exercise capacity in individuals with RA via dynamic tests of the locomotor system is essential. To compare functional exercise capacity using the Glittre-activities of daily living (ADL) test (G-AT) in women with and without RA in the absence of RA pulmonary disease (RA-PD) and to correlate the findings with hand functioning, physical functioning, handgrip strength (HGS), and quadriceps strength (QS). This cross-sectional pilot study evaluated 35 women with RA and 25 healthy controls by assessing hand functioning using the Cochin Hand Functional Scale (CHFS), physical functioning with the Health Assessment Questionnaire Disability Index (HAQ-DI), muscle functioning using HGS and QS, and G-AT results. Compared to the women in the control group, the women with RA presented higher scores for the CHFS (p< 0.0001) and HAQ-DI (p< 0.0001) and lower HGS (p< 0.0001) and QS (p= 0.013) values. The median G-AT time was higher in the RA patients than in the healthy controls [300 (295-420) vs. 180 (155-203) s], p< 0.0001), and the greatest difficulty reported by patients after the G-AT was squatting to perform the shelving tasks. G-AT time was positively correlated with the HAQ-DI (rs= 0.668, p< 0.0001) and CHFS (rs= 0.586, p= 0.0007) and negatively correlated with QS (rs=-0.429, p= 0.037). There was no significant correlation between the G-AT time and HGS. Women with RA take longer to perform G-AT tasks. Moreover, G-AT time was associated with hand functioning, physical functioning and QS, but not with HGS.

Development and validation of a patient-reported outcome in systemic sclerosis: the Hand scleroDerma lived Experience (HAnDE) scale.

Hand involvement in systemic sclerosis (SSc) is at the core of the disease, with a substantial impact on both functional aspects and quality of life. There is no patient-reported outcome (PRO) scale globally assessing hand involvement in SSc. To develop and validate a PRO scale, the Hand scleroDerma lived Experience (HAnDE) scale, to assess the lived experience of hand involvement in patients with SSc. This was an exploratory sequential mixed-methods study with two phases: (i) PRO development through an inductive process to analyse the structure of lived experience, involving 21 patients with SSc; and (ii) PRO validation by assessing the psychometric properties of the scale among 105 patients with SSc. Phase 1 enabled us to generate the 18-item provisional scale. From Phase 2, the mean (SD) total score of the scale was 29·16 (16·15). The item reduction process retained 16 items with five levels of answers (range 0-64). Internal consistency of the 16-item version was excellent (Cronbach's alpha = 0·946). Construct validity was very good, principal component analysis pointing towards a unidimensional instrument, with one factor explaining 56% of the variance, and concurrent validity being confirmed: Cochin Hand Function Scale r = 0·66; Health Assessment Questionnaire - Disability index r = 0·58; Hospital Anxiety and Depression Scale, anxiety r = 0·51, depression r = 0·4; Mouth Handicap in Systemic Sclerosis scale r = 0·61; 36-Item Short Form Health Survey, physical component r = -0·48, mental component r = -0·46; and Kapandji score r = -0·46. The correlations were statistically significant (P < 0·05). We propose, for future trials and clinical practice in SSc, a new PRO, the HAnDE scale, that assesses all the dimensions - functional, aesthetic, relational, existential and emotional - of the lived experience of hand involvement in SSc.

Kāiqiàotōngluò acupuncture combined with repetitive transcranial magnetic stimulation for the treatment of hand dysfunction after stroke

ObjectiveTo observe the clinical improvement of Kāiqiàotōngluò(induce resuscitation and remove obstruction in meridians) acupuncture combined with repetitive transcranial magnetic stimulation (rTMS) in patients with hand dysfunction after stroke, and to provide evidence for its extensive application in the future. MethodsSixty patients with the problem of hand dysfunction were divided into the observation group and the control group randomly. The two groups of patients were treated with the basic treatment, the observation group received Kāiqiàotōngluò acupuncture combined with rTMS, and the control group only received the rTMS treatment. Broetz hand function scale, hemiplegic hand function grading, and modified Barthel index were used to evaluate the hand function of the patients in two groups before and after treatment. ResultsAfter treatment, Broetz score of the patients in observation group and control group were significantly lower than that before treatment, and Broetz score of the patients in observation group was lower than that of control group (all P < 0.05); As for the hemiplegic hand function grading, 24 cases in the observation group improved, the improvement rate was 80.0%, and 8 cases in the control group improved, the improvement rate was only 26.7%. The improvement rate of observation group was significantly better than that of control group (P < 0.01); the Barthel index scores of the observation group and the control group were significantly improved after treatment, and the observation group was better than the control group (both P < 0.05). ConclusionThe Kāiqiàotōngluò acupuncture combined with rTMS can improve the hand dysfunction of patients after stroke, and it is worthy of clinical application.

The effect of a 24-week physiotherapy and occupational therapy program in systemic sclerosis: a monocentric controlled study with follow-up.

The structural and functional changes of the hands and face in systemic sclerosis (SSc) can be severely disabling. We aimed to assess the effect of a 24-week supervised physiotherapy and occupational therapy program (POTp) combined with home exercise on the function of hands/mouth of SSc patients, compared to a daily home exercise program in typical outpatient care. Fifty-nine patients with SSc were consecutively and non-selectively enrolled in an intervention (IG, n=27) or control (CG, n=32) group. Only the IG underwent the POTp twice a week for 1.5 hours. At baseline, 12, 24, and 48 weeks, all patients were assessed by a blinded physiotherapist for the hands/mouth function (delta finger-to-palm, handgrip strength, Hand and Mobility in Scleroderma, interincisal/interlabial distance), and self-evaluated their hand (Cochin Hand Function Scale) and mouth function (Mouth Handicap in Systemic Sclerosis scale), disability (Health Assessment Questionnaire [HAQ], SSc HAQ), and quality of life (Short Form-36). At week 24, compared to the significant deterioration in the CG, we found a significant improvement in the IG in the objectively assessed hands/mouth function and in the subjectively evaluated hand function and disability. The improvement was clinically meaningful (by >20%) in a substantial proportion of patients. Although the improvement in most outcomes was still present at week 48, the maximum effect was not sustained. This 24-week POTp not only attenuated the progressive deterioration, but also significantly improved the function of the hands/mouth, which was clinically meaningful in a substantial proportion of patients with SSc.