Impact of Controlling Nutritional Status Score on Comprehensive Clinical Outcomes in Patients With Chronic Limb-Threatening Ischemia in a Local Area of Super-Aged Society.

PURPOSE: This study aimed to evaluate the relationship between nutritional status and hospital outcomes in patients with chronic limb-threatening ischemia (CLTI) in a local area of contemporary super-aged society of Japan. MATERIALS AND METHODS: We analyzed 131 consecutive patients with 179 lower limb diseases admitted to our hospital for the treatment of CLTI between April 2018 and March 2023. These 131 patients were divided into 3 groups according to hospital outcomes: home discharge (HD), out-of-home discharge (OD), and in-hospital death (ID). Patient and lesion backgrounds were compared among the 3 groups, and a multivariable regression analysis was used to analyze the interaction between malnutrition and composite hard endpoints. RESULTS: The median age was 82.8 years, and non-ambulatory patients comprised 61.8% of the study population. The HD group included more ambulatory and fewer patients with higher CONUT score or inflammation than OD or ID group. The Rutherford classification and Wound, Ischemia, and foot Infection stage were significantly more severe in the ID group than in the HD group. Endovascular treatment (EVT) was more often implemented in the HD (94.9%) and OD (81.7%) groups than in the ID group (60.0%). However, all EVT procedures in the ID group were performed until as distally as possible to achieve the target arterial path success contrary to some EVT procedures in the HD or ID group that targeted lesions only above the knee. Multivariate analysis showed that a non-ambulatory state (hazard ratio [HR]=3.65, 95% confidence interval [CI]=1.48-9.02) and a higher controlling nutritional status (CONUT) score (≥5) (HR=7.46, 95% CI=1.66-33.6) were significant predictors for composite endpoints (major amputation or ID). Patients with lower CONUT scores (≤4) showed better outcomes in all indices including overall survival, major amputation-free survival, and wound healing. CONCLUSION: Condition of the CLTI patients represented by higher CONUT score emerged as the most influential predictor of major amputation or ID. Furthermore, non-ambulatory status or condition of higher CONUT score affects the destination after discharge. Implementing multidisciplinary approaches to address patients' nutritional state and physical disability, in addition to revascularization, may enhance comprehensive prognoses in patients with CLTI. CLINICAL IMPACT: In this single-center retrospective study, we analyzed prognoses of 131 consecutive patients with 179 lower limb diseases admitted for the treatment of chronic limb-threatening ischemia (CLTI) between April 2018 and March 2023. Our main finding was that condition of the CLTI patients represented by higher controlling nutritional status (CONUT) score was the most significant predictor of either major amputation or in-hospital death. Furthermore, condition of higher CONUT score or non-ambulatory status affects the destination after discharge. This suggests that multidisciplinary approaches to address patients' nutritional state and physical disability, in addition to revascularization, may enhance the prognosis in patients with CLTI. This is the first report to evaluate nutritional status associated with comprehensive hospital outcomes in addition to previously reported hard endpoints, such as major amputation or overall survival, and will be of great help in future clinical practice.

The role of ataluren in the treatment of ambulatory and non-ambulatory children with nonsense mutation duchenne muscular dystrophy - a consensus derived using a modified Delphi methodology in Eastern Europe, Greece, Israel and Sweden.

BACKGROUND: This paper details the results of an evaluation of the level of consensus amongst clinicians on the use of ataluren in both ambulatory and non-ambulatory patients with nonsense mutation Duchenne muscular dystrophy (nmDMD). The consensus was derived using a modified Delphi methodology that involved an exploration phase and then an evaluation phase. METHODS: The exploration phase involved 90-minute virtual 1:1 interviews of 12 paediatric neurologists who cared for 30-120 DMD patients each and had patient contact every one or two weeks. The respondents managed one to ten nmDMD patients taking ataluren. The Discussion Guide for the interviews can be viewed as Appendix A. Following the exploration phase interviews, the interview transcripts were analysed by an independent party to identify common themes, views and opinions and developed 43 draft statements that the Steering Group (authors) reviewed, refined and endorsed a final list of 42 statements. Details of the recruitment of participants for the exploration and evaluation phases can be found under the Methods section. RESULTS: A consensus was agreed (> 66% of respondents agreeing) for 41 of the 42 statements using results from a consensus survey of healthcare professionals (n = 20) experienced in the treatment of nmDMD. CONCLUSIONS: The statements with a high consensus suggest that treatment with ataluren should be initiated as soon as possible to delay disease progression and allow patients to remain ambulatory for as long as possible. Ataluren is indicated for the treatment of Duchenne muscular dystrophy that results from a nonsense mutation in the dystrophin gene, in ambulatory patients aged 2 years and older (see Summary of Product Characteristics for each country).

Development of algorithms for estimating the Child Health Utility 9D from Caregiver Priorities and Child Health Index of Life with Disability.

PURPOSE: The primary aim was to determine Child Health Utility 9D (CHU9D) utilities from the Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD) for non-ambulatory children with cerebral palsy (CP). METHODS: One hundred and eight surveys completed by Australian parents/caregivers of children with CP were analysed. Spearman's coefficients were used to investigate the correlations between the two instruments. Ordinary least square, robust MM-estimator, and generalised linear models (GLM) with four combinations of families and links were developed to estimate CHU9D utilities from either the CPCHILD total score or CPCHILD domains scores. Internal validation was performed using 5-fold cross-validation and random sampling validation. The best performing algorithms were identified based on mean absolute error (MAE), concordance correlation coefficient (CCC), and the difference between predicted and observed means of CHU9D. RESULTS: Moderate correlations (ρ 0.4-0.6) were observed between domains of the CHU9D and CPCHILD instruments. The best performing algorithm when considering the CPCHILD total score was a generalised linear regression (GLM) Gamma family and logit link (MAE = 0.156, CCC = 0.508). Additionally, the GLM Gamma family logit link using CPCHILD comfort and emotion, quality of life, and health domain scores also performed well (MAE = 0.152, CCC = 0.552). CONCLUSION: This study established algorithms for estimating CHU9D utilities from CPCHILD scores for non-ambulatory children with CP. The determined algorithms can be valuable for estimating quality-adjusted life years for cost-utility analysis when only the CPCHILD instrument is available. However, further studies with larger sample sizes and external validation are recommended to validate these findings.

Factors associated with Ontario dairy producers' management and care of down dairy cattle.

The objective of this study was to better understand current management practices for down cows in Ontario, Canada, and to identify factors associated with the adoption of acceptable practices. An online survey was distributed to all dairy producers in Ontario, Canada (n = 3,367) and was available from November 2020 to March 2021, inclusive. Dairy producers were identified through their provincial dairy organization and contacted via email, and the survey was also promoted via social media. The survey was comprised of 134 questions, 31 of which were related to down cow management. Descriptive statistics were evaluated, and 2 logistic regression models were generated using Stata 17, exploring factors associated with 1) relocating down cows with hip lifters and 2) assisting cows to stand within an hour after discovering a down cow. A total of 226 producers responded (7.4%). Participants were predominantly male (68%), farm owners (78%), and 30-39 years old (29%). Producers reported relocating down cows with a boat or sled (32.6%), front-end loader bucket (31.4%), hip lifters (28.0%), or 'other' (with a text box to further describe) (8.0%). The median time to relocating a down cow after identifying her was 1 h (range 0 - 17h). Farms that relocated a down cow sooner after identifying her as down, were more likely to use appropriate methods to move the cow. However, we also found that farms that provided feed and water sooner to down cows, were more likely to use an inappropriate method (hip lifters) to move her. Farms that used hip lifters to move cows had higher odds of assisting a cow to stand within an hour following the discovery of recumbency. Additionally, producers who waited longer to relocate a down cow were less likely to assist the cow to stand within one hour of finding them down. Research has identified effective management practices for down cows, yet there remains a gap in understanding the implementation and the decision-making process of producers. Data from this study will be helpful in designing future research that further explores the barriers and motivations of producers when implementing evidence-based management plans to care for down dairy cows and may help inform current industry extension efforts.

Factors influencing how Canadian dairy producers respond to a downer cow scenario.

Understanding how downer cattle are managed allows for the evaluation of strengths and weaknesses in these practices, which is an important step toward improving the care these animals receive. The objective of this cross-sectional study was to analyze factors associated with the care and management of downer cattle by Canadian dairy producers. Data were obtained from the 2015 National Dairy Study, and analysis was limited to the 371 respondents completing the downer cow scenario. The scenario described a downer cow that the producer wanted to keep in their herd but must be moved, and was followed by questions addressing the cow's care and management. Using multivariable logistic regression models, associations between respondent demographics and farm characteristics, and the presence of downer cow protocols, we assessed decisions regarding euthanasia and use of behavioral prognostic indicators. Written downer cow protocols were reported by 18.2% of respondents, 67% indicated that they had a nonwritten protocol, and 14.8% reported that they did not have a protocol (either written or nonwritten). Respondents from western provinces were more likely to have a written protocol than those from Ontario. Nineteen percent of the respondents with a written or unwritten protocol reported veterinary involvement in developing their downer cow protocol, which occurred more commonly on farms with more frequent herd health visits and a good producer-veterinarian relationship. An area to move a downer cow to was present on 88% of farms, with respondents who were farm staff being less likely to report having knowledge of a designated area than respondents who were the farm owner. In addition, approximately half (45%) of respondents reported moving downer cattle with hip lifters as their most common method. Behavioral prognostic indicators chosen by respondents were associated with the respondent's geographic region, age, farm size, and education. Most notably, older respondents were more likely to use appetite, and less likely to use attitude, as a prognostic indicator compared with younger respondents. Using perceived pain as a prognostic indicator was more common among respondents from western and Atlantic provinces compared with respondents from Ontario, and more common among respondents with a college or university education. These results highlighted herd and farmer demographics that were associated with how Canadian dairy producers managed downer cattle in 2015 and could be used as a benchmark for evaluating how these management practices compare with those currently implemented.

Long term treatment with ataluren-the Swedish experience.

INTRODUCTION: Ataluren is a relatively new treatment for male patients with Duchenne muscular dystrophy (DMD) due to a premature stop codon. Long-term longitudinal data as well as efficacy data on non-ambulant patients are still lacking. Here we present the results from a long-term follow-up study of all DMD patients treated with ataluren and followed at the Queen Silvia Children's Hospital in Gothenburg, Sweden, with focus on the evolution of patients' upper motor and respiratory function over time. METHODS: This is a retrospective longitudinal case-series study of all male DMD patients treated with ataluren and followed at the Queen Silvia Children's Hospital in Gothenburg, Sweden, since 2008. RESULTS: Our eleven patients had a median exposure to ataluren of 2312 days which is almost a fourfold higher than previous studies. Loss of ambulation occurred at a median age of 13.2 years. Patients who lost ambulation prior to 13.2 years of age had received ataluren for 5 years, whereas patients who continued to be ambulatory after 13.2 years of age had received ataluren for 6.5 years until loss of ambulation or last follow-up if still ambulatory. Four of six non ambulatory patients had Performance of the Upper Limb scores above the expected mean values over time. All but one patient maintained a pulmonary decline above the expected over time. All ambulatory patients increased in their predicted forced vital capacity (FVC) with 2.8 to 8.2% annually. Following loss of ambulation, 5 of 6 patients declined in predicted FVC (%), with annual rate of decline varying from 1.8 to 21.1%. The treatment was safe and well tolerated throughout the follow-up period. CONCLUSIONS: This is the first study to present long-term cumulative treatment outcomes over a median period of 6.3 years on ataluren treatment. Our results indicate a delay in loss of ambulation, as well as a slower decline in FVC and upper limb motor function even after loss of ambulation. We suggest that treatment with ataluren should be initiated as soon as the diagnosis is confirmed, closely monitored and, in case of sustainable benefit, continued even after loss of ambulation.

Combined upper limb and breathing exercise programme for pain management in ambulatory and non-ambulatory multiple sclerosis individuals: part II analyses from feasibility study.

PURPOSE: The present small semi-controlled feasibility study investigated a possible efficacy of a combined upper limb and breathing exercise programme in managing pain in ambulatory and non-ambulatory patients with EDSS from 0.0-8.0. METHOD: People with MS (N = 19) were enrolled in this single-blind randomized controlled study and divided into 2 groups: exercise group (5 ambulatory, 5 non-ambulatory; Expanded Disability Status Scale (EDSS), 1.0-8.0) and related control group that performed no exercise (4 ambulatory, 5 non-ambulatory; EDSS, 1.0-7.5). The exercise group performed combined upper limb and breathing exercises in a group led by a physiotherapist (2 days/week, 60 min/session) accompanied by independent home exercises (3 days/week, ≥ 20 min/session). Participants underwent measures of pain level (visual analogue scale) for physical pain, functional independence of daily activities (Barthel index) and handgrip strength (HGS) for dominant (D) and non-dominant (ND) hand evaluated by a dynamometer before and after the 4-week period by the blinded assessor. RESULTS: The VAS for pain showed statistically significant group-by-time interaction only in non-ambulatory (p = .049) individuals, but with large intervention effects on both subgroups (ambulatory, p = .159; non-ambulatory, d = 0.97). Functional independence in daily activities (Barthel index) showed statistically non-significant group-by-time interaction in ambulatory (p = .195, d = 0.89) and non-ambulatory (p = .102, d = 1.64) individuals, but despite the absence of statistical significance, there were large intervention effects. Handgrip strength was significantly improved for both hands in ambulatory (D, p = .012; d = 2.07; ND, p = .025, d = 1.77) and only non-dominant hand in non-ambulatory individuals (D, p = .288, d = 0.83; ND, p = .012, d = 2.21). CONCLUSION: This small pilot study provides preliminary proof-of-concept data supporting low-intensity upper limb and breathing exercise programme for potential reduction of pain and improvement of functional independence in both ambulatory and non-ambulatory individuals with MS in a larger sample and that strengthening the upper limbs might be an additional pain relief mechanism. TRIAL REGISTRATION: NTC03222596.

Exploring the feasibility of a mild and short 4-week combined upper limb and breathing exercise program as a possible home base program to decrease fatigue and improve quality of life in ambulatory and non-ambulatory multiple sclerosis individuals.

PURPOSE: To evaluate the feasibility of a combined upper limb and breathing exercise for a home-based program and to explore its effect on primary fatigue and quality of life in ambulatory and non-ambulatory individuals with multiple sclerosis (MS) in a short time. METHOD: Nineteen individuals with MS were assigned into semi-controlled pre-post feasibility study based on Expanded Disability Status Scale (EDSS) status and divided into two groups: exercise (five ambulatory, five non-ambulatory; EDSS 1.0-8.0) and related control with no exercise (four ambulatory, five non-ambulatory; EDSS 1.0-7.5). Exercise group performed combined upper limb and breathing exercise in a controlled group (2 days/week, 60 min/session) accompanied by independent home exercise (3 days/week, ≥ 20 min/session). Participants underwent measures of fatigue impact (Modified Fatigue Impact Scale (MFIS) and quality of life (RAND Medical outcomes study 36-item short-form health survey (SF-36)) before and after a 4-week period. RESULTS: The MFIS (physical, psychosocial, total) showed statistically significant group-by-time interaction in ambulatory (p = 0.033, d = 1.60; p = 0.039, d = 1.59; p = 0.033, d = 1.62) and non-ambulatory individuals (p = 0.009, d = 2.42; p = 0.018, d = 1.96; p = 0.0008, d = 3.92). Physical functioning (SF-36) showed statistically significant group-by-time interaction in ambulatory (p = 0.014, d = 2.14) but no significance in non-ambulatory (p = 0.368, d = 0.68) individuals. Despite the absent statistical significance, there were large intervention effects on MFIS cognitive scores for ambulatory (d = 1.28) and non-ambulatory (d = 1.47), and on other SF-36 scores for ambulatory (general health: d = 1.76 and pain: d = 1.02) and non-ambulatory (physical limitation: d = 1.03 and emotional well-being: d = 0.94) individuals. CONCLUSION: Our 4-week program reduced some aspects of fatigue and improved some aspects of quality of life in a small group of ambulatory and non-ambulatory individuals with MS. Good feasibility and significant positive changes from baseline warrant further exploratory work. TRIAL REGISTRATION: Name of the registry: The Impact of Exercise Training on Living Quality in Multiple Sclerosis. Registration: The study was registered at www.clinicaltrial.gov on July 14, 2017. First participant enrollment: August 28, 2017. URL: 602-01/17-01-147; Trial registration ID: NTC03222596.

Clinical trial readiness in non-ambulatory boys and men with duchenne muscular dystrophy: MDA-DMD network follow-up.

INTRODUCTION: Outcomes sensitive to change over time in non-ambulatory boys/men with Duchenne muscular dystrophy (DMD) are not well-established. METHODS: Subjects (n = 91; 16.8 ± 4.5 years old) were assessed at baseline and 6-month intervals for 2 years. We analyzed all subjects using an intent-to-treat model and a subset of stronger subjects with Brooke Scale score ≤4, using repeated measures. RESULTS: Eight patients (12-33 years old) died during the study. Sixty-six completed 12-month follow-up, and 51 completed 24-month follow-up. Those taking corticosteroids performed better at baseline, but rates of decline were similar. Forced vital capacity percent predicted (FVC% predicted) declined significantly only after 2 years. However, Brooke and Egen Klassifikation (EK) Scale scores, elbow flexion, and grip strength declined significantly over both 1 and 2 years. CONCLUSION: Brooke and EK Scale scores, elbow flexion, and grip strength were outcomes most responsive to change. FVC% predicted was responsive to change over 2 years. Corticosteroids benefited non-ambulatory DMD subjects but did not affect decline rates of measures tested here. Muscle Nerve 54: 681-689, 2016.

Changes in quadriceps muscle thickness in acute non-ambulatory stroke survivors.

BACKGROUND: Loss of skeletal muscle mass is one of the main reasons for disability in patients with stroke. However, lower leg muscle wasting has not been studied in acute stroke patients. OBJECTIVE: To investigate the changes in quadriceps muscle thickness in acute non-ambulatory stroke survivors. METHODS: A total of 16 consecutive acute non-ambulatory stroke survivors who were in acute inpatient rehabilitation, with a mean age of 72.1 years, were included in the study. Quadriceps muscle thickness was examined in their paretic and non-paretic limbs within the first week from admission (first week), 1 week after the first examination (second week), and 1 week after the second week examination (third week) using ultrasonography. RESULTS: Quadriceps muscle thickness in the paretic limb decreased every week (mean% difference between the first and second weeks, 12.8, 95% confidence interval (CI) 5.3-20.2%; mean% difference between the second and third weeks, 10.1, 95% CI 5.2-14.9%). Quadriceps muscle thickness in the non-paretic limb was lower in the second and third weeks than the first week, but there was no difference between the second and third weeks (mean% difference between the first and second weeks, 9.3, 95% CI 2.5-16.1%; mean% difference between the second and third weeks, 5.3, 95% CI - 1.6 to 12.1%). CONCLUSION: Quadriceps muscle thickness decreased in acute non-ambulatory stroke survivors not only in the paretic limb but also in the non-paretic limb, particularly during the period from admission to the second week.

Outcome reliability in non-ambulatory boys/men with Duchenne muscular dystrophy.

INTRODUCTION: Therapeutic trials in Duchenne muscular dystrophy (DMD) often exclude non-ambulatory individuals. Here we establish optimal and reliable assessments in a multicenter trial. METHODS: Non-ambulatory boys/men with DMD (N = 91; 16.7 ± 4.5 years of age) were assessed by trained clinical evaluators. Feasibility (percentage completing task) and reliability [intraclass correlation coefficients (ICCs) between morning and afternoon tests] were measured. RESULTS: Forced vital capacity (FVC), assessed in all subjects, showed a mean of 47.8 ± 22% predicted (ICC 0.98). Brooke Upper Extremity Functional Rating (Brooke) and Egen Klassifikation (EK) scales in 100% of subjects showed ICCs ranging from 0.93 to 0.99. Manual muscle testing, range of motion, 9-hole peg test, and Jebsen-Taylor Hand Function Test (JHFT) demonstrated varied feasibility (99% to 70%), with ICCs ranging from 0.99 to 0.64. We found beneficial effects of different forms of corticosteroids for the Brooke scale, percent predicted FVC, and hand and finger strength. CONCLUSIONS: Reliable assessment of non-ambulatory boys/men with DMD is possible. Clinical trials will have to consider corticosteroid use.

A radiolucent chair for sitting-posture radiographs in non-ambulatory children: use in biplanar digital slot-scanning.

EOS imaging (EOS System; EOS imaging, Paris, France) enables fast 2-D/3-D imaging of children in standing load-bearing position. Non-ambulatory children with neuromuscular scoliosis need evaluation of their spinal balance while in a normal daily position. We designed a customized chair fitting the EOS patient-area dimensions to obtain images in natural sitting postures. The chair is a 360° rotating orthopaedic chair made of fully radiolucent polyethylene and equipped with an adjustable headrest and three-point belts. Out of 41 consecutive patients, 36 (88%, 95% confidence interval 74-96%) had successful imaging. In most patients with severe neuromuscular trunk deformities, the EOS system combined with our chair was useful for assessing preoperative trunk collapse, pelvic obliquity and postoperative corrections in all planes. This specific device changed our daily practice for the assessment of spinal deformities in non-ambulatory patients.

Ayurveda in the integrative management of bovine secondary recumbency-a case report.

Downer cow syndrome, or secondary recumbency, is a condition primarily affecting dairy cows, where the animal is unable to rise and stand, due to unknown cause. It is usually associated with poor prognosis. Terminal downers are euthanized in most countries. A four-year old Kasarkode dwarf-cow, post-calving was brought up laterally recumbent with heavy nasal discharge, labored breathing, loss of appetite and signs of dehydration on 6th day of its recumbency. Before this, the patient was diagnosed with milk fever and standard treatment with calcium borogluconate was administered intravenously. The animal was left to succumb under unprotected conditions, due to various constraints on euthanasia. After adopting the cow, Nasya was started immediately to avoid death due to sepsis and shock. The animal was drenched with Ayurvedic fluids containing deepana-pacana herbs. Sternal recumbency, warm and moistened muzzle was observed on the fourth day of commencing ayurvedic treatment. Respiratory distress was minimal. Drastic prognostic shift from "no hope" to "good" was possible within 6 days thanks to Nasya, and the animal was stable. Thereafter, integrative care comprising of antibiotics, rehydrating IV fluids, and supplementations, along with ayurvedic medicines was initiated. Ruminal-fluid obtained from slaughterhouse was used for ruminal-flora replacement. Rumination on 14th day, cow on its feet by 19th day and complete healing of decubital ulcers by approximately 40 days was recorded. A downed cow which did not respond to standard veterinary care was managed with Ayurveda-integrated veterinary care. Ayurveda herbs like bamboo leaves (Bambusa vulgaris), green chiretta (Andrographis paniculata) that cattle prefer eating during certain illness, turn out to be useful for Ayurvedic management. Hence, Ayurveda veterinary medicine might be, a good choice for integrative management of terminal downers, preventing early death in downed dairy cows.

Can postoperative Cobb and pelvic obliquity corrections be predicted using supine traction X-rays in non-ambulatory patients with cerebral palsy fused to L5? A case series study.

PURPOSE: This study aimed to determine whether Cobb and pelvic obliquity corrections can be predicted using supine traction radiographs in patients with cerebral palsy (CP) who underwent posterior spinal fusion (PSF) from T2/3 to L5. METHODS: From January 2010 to January 2020, 167 non-ambulatory patients with CP scoliosis underwent PSF using pedicle screws in two quaternary centers with a minimum of 2 years follow-up (FU). Radiological measurements and chart reviews were performed. RESULTS: A total of 106 patients aged 15.6 ± 0.4 years were included. All patients had significant correction of the Cobb angle (MC), pelvic obliquity (PO), thoracic kyphosis (TK), and lumbar lordosis (LL) without loss of correction at the last FU (LFU). Curve flexibility was significantly correlated with Cobb correction (δMC) immediately postoperatively (p < 0.0001, r = 0.8950), followed by the amount of correction in pelvic obliquity under traction (δPOT) (p = 0.0252, r = 0.2174). For correction in PO (δPO), the most significant variable was δPOT (p < 0.0001, r = 0.7553), followed by curve flexibility (p = 0.0059, r = 0.26) and the amount of correction in Cobb under traction (p = 0.0252, r = 0.2174). CONCLUSIONS: Cobb and PO corrections can be predicted using supine traction films for non-ambulatory CP patients treated with PSF from T2/3 to L5. The variables evaluated were interconnected, reinforcing preoperative planning for these patients. Comparative large-scale studies on patient-related clinical outcomes are required to determine whether this predicted correction is associated with improved surgical outcomes and reduced complication rates. LEVEL OF EVIDENCE: IV.

Distal level in scoliosis surgery for non-ambulatory patients with cerebral palsy: is L5 an option? A case series study.

PURPOSE: This study aimed to determine the radiographic outcomes of patients with cerebral palsy (CP) who underwent posterior spinal fusion from T2/3 to L5 at two quaternary hospitals. METHODS: From January 2010 to January 2020, 167 non-ambulatory patients with CP scoliosis underwent posterior spinal fusion using pedicle screws from T2/3 to L5 in both centers, with a minimum of 2 years follow-up (FU). Radiological measurements and chart reviews were performed. RESULTS: A total of 106 patients aged 15.6 ± 0.4 years were included. None of the patients was lost to FU. All patients had significant correction of the Cobb angle (MC) and pelvic obliquity (PO), thoracic kyphosis (TK), and lumbar lordosis (LL), without loss of correction at the last FU (LFU). The mean values for preoperative, immediate postoperative, and LFU were MC 93.4°, 37.5°, and 42.8°; PO 25.8°, 9.9°, and 12.7°; TK 52.2°, 44.3°, and 45°; and LL - 40.9°, - 52.4°, and - 52.9°, respectively. Higher residual PO at LFU was associated with more severe MC and PO baselines, lower implant density, and an apex located at L3. CONCLUSIONS: CP scoliosis and PO can be corrected, and this correction is maintained over time with posterior spinal fusion using pedicle screws, with L5 as the lowest instrumented vertebra. Larger preoperative MC and PO values associated with the apex at L3 appear to be related to residual PO. Comparative large-scale studies of patient-related clinical outcomes are required to determine whether this intervention is associated with improved surgical outcomes and reduced complication rates. LEVEL OF EVIDENCE: IV.

Hip resection arthroplasty for acute femoral neck fractures in the non-ambulator.

OBJECTIVES: Hemiarthroplasty (HA) is the current standard of care for displaced femoral neck fractures (FNFs) in non-ambulators. Despite excellent outcomes, arthroplasty-specific risks remain, including dislocation, implant failure, periprosthetic fracture and infection, and fat embolization syndrome. To eliminate the possibility of these complications, should non-ambulatory patients with acute, native hip FNFs be treated with simple hip resection arthroplasty (HRA) instead of HA? DESIGN: Retrospective case series. SETTING: Large, urban level-1 trauma center. PATIENTS/PARTICIPANTS: Five non-ambulatory patients (6 hips) with acute, native hip FNF underwent femoral head and neck resection. Also, the most recent 10 FNFs treated with HA were also identified for comparison purposes. INTERVENTION: HRA was performed via a Smith-Peterson approach with an oscillating saw or osteotome to complete the fracture or perform a fresh neck cut. MAIN OUTCOME MEASUREMENTS: Outcomes included postoperative vs preoperative VAS pain scores and narcotics usage, and return to baseline functional status (sit up in bed or a chair postoperatively). Procedure time for HRA was compared with the 10 most recent patients with FNF treated with HA. RESULTS: HRA resulted in decreased postoperative vs preoperative VAS pain scores (7.7 vs 3.3, P = .002), and decreased operative times (59.2 minutes for HRA, 111.8 minutes for HA, P < .001). All HRA patients had immediate return of baseline function. CONCLUSION: HRA offers shorter operative times when compared with HA, decreased postoperative VAS pain scores, and immediate return to functional baseline status without possibility of arthroplasty-specific complications. HRA may be an acceptable treatment option for FNFs in the non-ambulator.Level of evidence: IV.

The Developments and Iterations of a Mobile Technology-Based Fall Risk Health Application.

Falls are a prevalent and serious health concern across clinical populations. A critical step in falls prevention is identifying modifiable risk factors, but due to time constraints and equipment costs, fall risk screening is rarely performed. Mobile technology offers an innovative approach to provide personalized fall risk screening for clinical populations. To inform future development, this manuscript discusses the development and testing of mobile health fall risk applications for three unique clinical populations [older adults, individuals with Multiple Sclerosis (MS), and wheeled-device users]. We focus on key lessons learned and future directions to improve the field of fall risk mHealth. During the development phase, we first identified fall risk factors specific to each population that are measurable with mobile technology. Second, we determined whether inertial measurement units within smartphones can measure postural control within the target population. Last, we developed the interface of each app with a user-centered design approach with usability testing through iterative semi-structured interviews. We then tested our apps in real-world settings. Our cumulative work demonstrates that mobile technology can be leveraged to provide personalized fall risk screening for different clinical populations. Fall risk apps should be designed and tailored for the targeted group to enhance usefulness and feasibility. In addition, fall risk factors measured with mobile technology should include those that are specific to the population, are measurable with mobile technology, and can accurately measure fall risk. Future work should improve fall risk algorithms and implement mobile technology into fall prevention programs.

Long-Term Evolution of the Hip and Proximal Femur after Hip Reconstruction in Non-Ambulatory Children with Cerebral Palsy: A Retrospective Radiographic Review.

BACKGROUND: Hip displacement in children with cerebral palsy (CP) has a higher prevalence in non-ambulatory children. Progression can lead to pain, pelvic obliquity and difficulty with sitting. This can be addressed with hip reconstruction. Our study aims to report the long-term radiological outcomes after hip reconstruction, in particular the evolution of femoral head deformity. METHODS: A total of 58 hips of non-ambulatory children with CP were evaluated retrospectively using pre-operative, early (median 120 days) and late post-operative (median 8.6 years) anteroposterior standardised radiographs. All the hips were treated with femoral shortening varus derotation osteotomy (VDRO), pelvic osteotomy and an open reduction, if indicated. The radiographical indices measured included the migration percentage (MP), sharp angle, acetabular index, centre-edge angle, neck shaft angle, head shaft angle, pelvic obliquity, femoral head sphericity, femoral head deformity (FHD) and growth plate orientation. RESULTS: Improvements in hip congruency and morphology were evident after reconstructive hip surgery. These were maintained at the late post-operative time point. Median MP improved from 56% (IQR 46-85%) to 0% (IQR 0-15%) at early follow-up. This increased to 12% (IQR 0-20%) at late follow-up. Pre-operatively, FHDs of 14 hips (24%) were classified as grade A (spherical femoral head). This increased to 22 hips (38%) at early follow-up and increased further to 44 hips (76%) at late follow-up. CONCLUSIONS: Our study shows that hip reconstruction reduces hip displacement in the long term, indicated by decreased post-operative MP maintained at long-term follow-up. Although non-ambulatory children lack weight-bearing forces promoting bone remodelling, improved femoral head morphology after surgery alters the forces between the acetabulum and the femoral head. Mild femoral head deformity (grades A and B) remained stable and even improved after surgery, postulated to be due to severe osteoporosis allowing remodelling.

Burden and severity of disease of aromatic L-amino acid decarboxylase deficiency: a systematic literature review.

OBJECTIVE: The objective was to investigate the severity of aromatic L-amino acid decarboxylase deficiency (AADCd) as reported in the published literature and to collate evidence of the clinical manifestations of AADCd, and the impact of the disease on patients, caregivers, and healthcare systems. METHODS: Published articles reporting severity of disease or disease impact were eligible for inclusion in this review. Articles were searched in MEDLINE, EMBASE, Cochrane CENTRAL, TRIP medical, and CRD databases in October 2021. The quality of the included studies was investigated using a modified version of the grading system of the Centre for Evidence-Based Medicine (CEBM). Descriptive data of the literature was extracted and a narrative synthesis of the results across studies was conducted. This review is reported according to the PRISMA reporting guidelines for systematic reviews. RESULTS: The search identified 970 unique reports, of which 59 met eligibility criteria to be included in the review. Of these, 48 included reports provided details on the clinical manifestations of AADCd. Two reports explored the disease impact on patients, while four described the impact on caregivers. Five reports assessed the impact on healthcare systems. Individuals with AADCd experience very severe clinical manifestations regardless of motor milestones achieved, and present with a spectrum of other complications. Individuals with AADCd present with very limited function, which, in combination with additional complications, substantially impact the quality-of-life of individuals and their caregivers. The five studies which explore the impact on the healthcare system reported that adequate care of individuals with AADCd requires a vast array of medical services and supportive therapies. CONCLUSIONS: Irrespective of the ambulatory status of individuals, AADCd is a debilitating disease that significantly impacts quality-of-life for individuals and caregivers. It impacts the healthcare system due to the need for complex coordinated activities of a multidisciplinary specialist team.

A new technique for proximal femoral resection in non-ambulatory patients with cerebral palsy.

Proximal femoral resection may be proposed to non-ambulatory patients with cerebral palsy and chronic painful hip dislocation. McCarthy's technique confers good results but does not solve the problems related to femoral reascension (bone migration causing painful osseous or cutaneous conflict). We describe a new technique of resection-interposition of the proximal end of the femur which preserves the greater trochanter by an orthogonal osteotomy below the lesser trochanter while maintaining the gluteal-vastus lateralis strut in continuity. A suture of the remaining joint capsule upon itself and a trans-trochanteric capsulodesis are associated to stabilize the cephalic displacement of the femur.