Limb Involvement Research Articles

Short-term and long-term prognoses in AChR-Ab positive very-late-onset myasthenia gravis patients.

Very-late-onset myasthenia gravis (VLOMG) refers to myasthenia gravis (MG) with onset at age 65 or older. Current research on VLOMG prognosis remains limited, especially regarding factors influencing outcomes. To identify the clinical factors that affect the short- and long-term prognosis of MG patients with an onset age ⩾65 years. This was a single-center, retrospective cohort study of AChR-ab positive VLOMG patients, classified into two subgroups based on age of onset: sub-very-late-onset MG (S-VLOMG, onset age ⩾65 and <75 years), and super-late-onset MG (SLOMG, onset age ⩾75 years). A total of 93 patients were included, including 75 in the S-VLOMG group and 18 in the SLOMG group. Clinical, therapeutic, and prognosis data were reviewed, and the Cox regression model was used to identify factors influencing short- and long-term prognosis. Patient characteristics were well balanced between the groups. Overall, 49.5% of patients achieved minimal symptom expression (MSE) within 6 months and 86% within 24 months. There was no significant difference between the groups in the proportion achieving MSE at 6 months (p = 0.635) or 24 months (p = 0.714). The median time to achieve MSE was also comparable between the S-VLOMG and SLOMG groups (199.0 days vs 280.5 days, p = 0.463). Low baseline MG-ADL score and steroid therapy were associated with better short-term prognosis (p = 0.007 and p = 0.002, respectively). For long-term prognosis, baseline bulbar and limb involvement, time to treatment initiation, and use of immunosuppressants were significant factors (p = 0.025, p = 0.004, p = 0.025, and p < 0.0001, respectively). There were no significant differences in side effects or drug withdrawal rates between two groups. This study demonstrated that AChR-ab positive VLOMG patients have a favorable prognosis and responded well to medication, with age and comorbidities showing no significant impact.

Paw pressure and gait in middle-aged client-owned cats with and without naturally-occurring musculoskeletal disease.

Musculoskeletal disease (MSD) is common in ageing cats, resulting in chronic pain and mobility impairment, but diagnosis can be challenging. We hypothesised that there would be differences between cats with and without MSD in paw pressure and spatiotemporal and kinetic gait metrics. A cohort of 53 cats, aged between 7 and 10 years from the North West of the United Kingdom, underwent an orthopaedic examination and walked on a pressure sensitive walkway. Thirty-one of the cats (58%) were determined to be apparently-healthy, based on a normal orthopaedic examination and having no history of MSD, whilst the remaining 22 cats (42%) had findings consistent with MSD; 13/22 cats (59%) had multiple limb involvement, 7/22 (32%) had forelimb involvement and 2/22 (9%) had hindlimb involvement. Bodyweight (P = 0.048) and body condition score (BCS; P = 0.015) were both greater in cats with MSD (mean bodyweight 5.4 ± 1.35 kg; median BCS 6, IQR 6-7.75) compared with apparently-healthy cats (mean bodyweight 4.7 ± 0.94 kg; median BCS 5, IQR 4.5-6.5). There was a relatively large intra-cat variation in spatiotemporal and kinetic gait variables (coefficient of variation >3.0%), whilst a linear mixed-effects model suggested no significant difference in spatiotemporal or kinetic gait variables between apparently-healthy cats and those with MSD. Palmar and plantar pressure asymmetry was assessed by pedobarographic statistical parametric mapping (pSPM) within each individual cat, with no significant difference (P = 0.353) between the apparently heathy cats and those with MSD as to the presence or absence of asymmetry. Given the marked intra-cat variation and the 'multi-limb' nature of MSD in this cohort, it was not possible to differentiate healthy cats from those with MSD based on spatiotemporal and kinetic gait metrics or paw pressure asymmetry. Future work should examine gait in cats with defined musculoskeletal disorders (e.g. hip dysplasia) and also to track longitudinal changes within individual cats to better establish age-related trends.

Our Experience in Treating Infantile Hemangioma: Prognostic Factors for Relapse After Propranolol Discontinuation.

Infantile hemangioma (IH) is a common benign tumor in infants. While most cases exhibit a self-limiting nature, some require medical treatment to avoid complications. Propranolol is the first-line therapy for IH, it has a high success rate, and is safe to use. Unfortunately, some patients might experience rebound growth after propranolol discontinuation. Currently, it is unclear which factors predict this phenomenon. This study aimed to identify factors affecting the rebound growth of IH after propranolol cessation. We also aimed to identify predictors for an excellent response to oral propranolol. We performed a retrospective cohort study using clinical data from all patients referred to our clinic with IH and placed on systemic oral propranolol between January 2009 to December 2023 in the dermatology outpatient clinic of Rambam Healthcare Campus. Out of a total of 552 patients with IH, 301 received oral propranolol for at least 6months. A relapse phenomenon was observed in 38 (12.6%) patients. We found a significant association between limb involvement and the least likelihood of hemangioma rebound (p < 0.001). An excellent response to oral propranolol was observed in 57.8% of patients. Younger age at initiation of oral propranolol was associated with an excellent response (p = 0.015). Also, IHs located on the limbs (67) showed a higher rate of excellent response to oral propranolol compared to other anatomical sites (p = 0.02). Interestingly, patients who were treated with a dose of 2mg/kg/day were associated with excellent response to treatment (p = 0.007). IHs located on the limbs demonstrated less rebound growth and a better treatment response. When oral propranolol was initiated earlier or when the target dose was 2mg/kg/day, there was a higher rate of excellent response to treatment.

Benign Evolution of Complex Regional Pain Syndrome (CRPS) Type 1 in Patients Treated with Intravenous Neridronate: A Single-Center Real-Life Experience.

To investigate the long-term effects of intravenous neridronate treatment in patients with complex regional pain syndrome type 1 (CRPS) in a real-life setting. We conducted a retrospective study on consecutive CRPS patients treated at our hospital from February 2018 to July 2023. All were treated within three months of the onset of CRPS symptoms. The Patient-Reported Outcomes Measurement Information System 29-Item Health Profile (PROMIS-29) version 2.1 was administered. The main outcome of interest was the evolution of the PROMIS-29 scores from baseline to the last follow-up visit. Patients were categorized as "complete responders" or "non-complete responders". The association of clinical and demographic variables with a complete response was analyzed using chi-square tests and univariate logistic regression. Thirty-six patients were included, with a median follow-up time of 4.8 years. A significant improvement was noted in the mean numerical pain rating scale (from 6.4 ± 1.9 to 3.1 ± 2.4, p < 0.001), as well as across all PROMIS-29 domains. Physical function improved from 34.2 ± 4.9 to 49.2 ± 9.9, p < 0.001; anxiety from 58.0 ± 6.7 to 49.6 ± 6.9, p < 0.001; depression from 55.3 ± 6.3 to 47.7 ± 6.6, p < 0.001; fatigue from 55.7 ± 7.7 to 50.9 ± 8.7, p < 0.001; sleep disturbance from 53.8 ± 6.8 to 51.3 ± 6.6, p = 0.034; social roles and activities from 41.8 ± 5.2 to 51.8 ± 8.9, p < 0.001; and pain interference from 64.1 ± 5.9 to 52.4 ± 9.9, p < 0.001. The likelihood of achieving a complete response was associated with the male sex, foot or ankle injuries (compared to hand and wrist injuries), and a younger age. No association was found with the type of inciting event or with the body mass index. Our real-life data indicate that early treatment with neridronate leads to substantial benefits in patients affected by CRPS type 1. The strongest responses are seen in young patients, males, and those with lower limb involvement.

Melanoma in Adolescents and Young Adults (AYAs): An Italian Multi-Centric Retrospective Experience.

Background: Melanoma is currently the most prevalent malignant neoplasm among adults and represents the second most common cancer in both sexes among individuals aged 0 to 39 years. This retrospective multicenter study delves into the distinctive clinical, anamnestic, histopathologic, and prognostic attributes of melanoma in Adolescent and Young Adults (AYA), defined as patients diagnosed at ≤40 years, across four Italian centers. Methods: Through a computer-based review of clinical records from 1 January 2010 to 30 September 2023, AYA melanomas were contrasted with non-AYA melanomas (>40 years) among 1452 patients. Data on demographics, melanoma localization, histological type, Breslow thickness, ulceration, and sentinel lymph node (SLN) biopsy status were meticulously collected and analyzed. Results: Our analysis revealed a female predominance in the AYA group and a male predominance in the non-AYA group, with significant differences in anatomical localization and histological types between the two. AYA melanomas showed nearly equal trunk and limb involvement, contrasting with the trunk predominance in non-AYA melanomas. While Breslow thickness was similar across both groups, the presence of ulceration and total number of nevi showed no significant difference. Survival analysis indicated a marginally higher Disease-Free Survival (DFS) in AYA patients compared to non-AYA patients, without a significant difference in Overall Survival (OS). Conclusions: This study highlights demographic and clinical distinctions between AYA and non-AYA melanoma patients, underscoring the need for tailored follow-up and treatment strategies. Despite these insights, the heterogeneity of melanoma among young adults calls for further research, including genetic analyses, to fully understand this unique melanoma subgroup. Indeed, AYA melanoma patients could represent a different and specific target for both follow-up and treatments.

Autosomal recessive VWA1-related disorder: comprehensive analysis of phenotypic variability and genetic mutations.

A newly identified subtype of hereditary axonal motor neuropathy, characterized by early proximal limb involvement, has been discovered in a cohort of 34 individuals with biallelic variants in von Willebrand factor A domain-containing 1 (VWA1). This study further delineates the disease characteristics in a cohort of 20 individuals diagnosed through genome or exome sequencing, incorporating neurophysiological, laboratory and imaging data, along with data from previously reported cases across three different studies. Newly reported clinical features include hypermobility/hyperlaxity, axial weakness, dysmorphic signs, asymmetric presentation, dystonic features and, notably, upper motor neuron signs. Foot drop, foot deformities and distal leg weakness followed by early proximal leg weakness are confirmed to be initial manifestations. Additionally, this study identified 11 novel VWA1 variants, reaffirming the 10 bp insertion-induced p.Gly25ArgfsTer74 as the most prevalent disease-causing allele, with a carrier frequency of ∼1 in 441 in the UK and Western European population. Importantly, VWA1-related pathology may mimic various neuromuscular conditions, advocating for its inclusion in diverse gene panels spanning hereditary neuropathies to muscular dystrophies. The study highlights the potential of lower quality control filters in exome analysis to enhance diagnostic yield of VWA1 disease that may account for up to 1% of unexplained hereditary neuropathies.

When Giant Lymphangioma Meets Hydrops Fetalis: An Uncommon Case Report of Lower Extremity Involvement

Abstract Approximately 75% of lymphangiomas manifest in the head and neck region, with limb and bone involvement observed in only 2% of cases. This case report presents a rare instance of fetal lymphangioma associated with hydrops fetalis, affecting the left lower extremity and abdomen. A 22-year-old secundigravida woman was referred to the gynecology department at 31.2 weeks of gestation. Initial fetal ultrasound revealed a live fetus with a large lymphangioma involving the left lower extremity, gluteal region, and abdomen. Additional ultrasound findings included subcutaneous edema, pericardial effusion, ascites, placentomegaly, and tricuspid regurgitation. Postnatal clinical examination confirmed the diagnosis of lymphatic malformation. Notably, only one other similar case has been reported in the English-language PubMed database. This report adds to the medical literature as a rare instance of prenatally diagnosed lymphangioma-associated hydrops fetalis.

Clinical and histological impact of diffuse inflammation at pouchoscopy

PurposeThe anatomical location of inflammation in and around the ileal pouch affects the pouch survival rate, and diffuse inflammation has poor pouch survival rates. We aimed to clarify the symptoms and histological findings of diffuse inflammation of the pouch.MethodsWe evaluated the symptoms, treatment, and histological findings according to the endoscopic phenotypes of diffuse inflammation, focal inflammation, and normal as the pouch body phenotype and afferent limb involvement, inlet involvement, cuffitis, and fistula as the peripheral findings.ResultsOf the 318 pouchoscopies, 47 had diffuse inflammation, 201 had focal inflammation, and 70 were normal. Symptomatic patients had diffuse inflammation more frequently (46.8%) than focal inflammation (13.4%) and normal (14.2%), with no difference between focal inflammation and normal. Antibiotics and steroids were higher rate administered in cases of diffuse inflammation, but not in cases of focal inflammation or in normal cases. Histological inflammation, inflammatory bowel disease (IBD)–specific finding, and colonic metaplasia showed severity in the order of diffuse inflammation > focal inflammation > normal. The number of peripheral inflammatory findings overlapped in the following order: diffuse inflammation > focal inflammation > normal. The number of symptomatic patients increased as the number of peripheral inflammatory findings increased.ConclusionPouches with diffuse inflammation are more symptomatic, have a higher use of therapeutic agents, and have more severe histological inflammation, IBD-specific finding, and colonic metaplasia accompanying peripheral inflammatory findings than the other groups. The higher the overlap of inflammatory findings in the surrounding tissues, the more symptomatic the patients will appear.

A Comparative Analysis of Clinical Symptoms and Modified Pouchitis Disease Activity Index Among Endoscopic Phenotypes of the J Pouch in Patients With Inflammatory Bowel Disease.

The modified pouchitis disease activity index (mPDAI) based on clinical symptoms and endoscopic findings is used to diagnose pouchitis, but validated instruments to monitor pouchitis are still lacking. We recently established an endoscopic classification that described 7 endoscopic phenotypes with different outcomes. We assessed symptoms and compared mPDAIs among phenotypes in inflammatory bowel disease (IBD). We retrospectively reviewed pouchoscopies and classified them into 7 main phenotypes: normal (n = 25), afferent limb (AL) involvement (n = 4), inlet involvement (n = 14), diffuse (n = 7), focal inflammation of the pouch body (n = 25), cuffitis (n = 18), and pouch-related fistulas (n = 10) with a single phenotype were included. Complete-case analysis was conducted. One hundred and three IBD patients were included. The median mPDAI was 0 (IQR 0-1.0) in patients with a normal pouch. Among inflammatory phenotypes, the highest median mPDAI was 4.0 (IQR 2.25-4.75) in cuffitis, followed by 3.0 (IQR 2.5-4.0) in diffuse inflammation, 2.5 (IQR 1.25-4.0) in inlet involvement, 2.5 (IQR 2.0-3.5) in AL involvement, 2.0 (IQR 1.0-3.0) in focal inflammation, and 1.0 (IQR 0.25-2.0) in the fistula phenotype. Perianal symptoms were frequently observed in pouch-related fistulas (8/10, 80%) and cuffitis (13/15, 87%). Among patients with cuffitis, all had incomplete emptying (6/6, 100%). We correlated the mPDAI with the endoscopic phenotypes and described the limited utility of symptoms in distinguishing between inflammatory phenotypes. Further studies are warranted to understand which symptoms should be monitored for each phenotype and whether mPDAI can be minimized after pouch normalization.

Impact of Stroke on the Quality of Life of its Survivors

Introduction: Ischemic Stroke is due to sudden loss of blood circulation to an area of the brain, resulting in loss of neurologic function. The incidence in India ranges between 105-152/100,000 people/year. Despite all the advancements in care, stroke continues to be the 2nd leading cause of mortality and the 1st cause of long-term disability. The activities of daily living of patients with chronic stroke affect their Quality of Life (QoL). The majority of stroke survivors continue to live with disabilities, and this can have a profound impact on their QoL. Aims &amp; Objectives: To assess the impact of stroke on the various domains of the quality of life of its survivors, who are treated by the Department of Neurological Sciences, Christian Medical College, Vellore. Methods: A descriptive study design employing a non-probability purposive sampling technique was used to recruit 90 stroke survivors. Written consent was obtained, demographic and clinical variables were collected using interviews, and the impact of stroke on its survivors was assessed using the Stroke Specific Quality of Life (SS-QoL) scale. Results: The participant's mean age group was 48.92±12.97 ranging between 26 -78 years respectively; 67.8% were males, 34.4% of them were unskilled workers, 66.7% had a thrombotic type of ischemic stroke, 56.7% had a moderate National Institutes of Health Stroke Scale grade at admission, 26.7% and 25.6% had a modified Rankin Score of 2 and 1, respectively. Among the participants, 47.8% of them had a stroke at the ages of 31-50 years, and the majority (91.1%) of them had comorbidities. The majority (44.77%) of them had a moderate QoL. Among the SS-QoL domains, the mean scores were the highest in the vision domain (4±1.26) and the lowest in the energy domain (2.47±1.25). There was a significant association between the QoL of stroke survivors and their socioeconomic status (p=0.033), type of ischemic stroke (p=0.047), and limb involvement (p=0.034). A positive correlation (p=0.00) was observed among various QoL domains. Conclusion: These findings provided not just insight into the impact of stroke on the QoL of its survivors but also emphasized the need to sensitize the healthcare care professionals to provide care during both the acute period and also continue to ensure all the domains of a good QoL is addressed during the follow-up and rehabilitation. Keywords: Ischemic Stroke, Quality of Life, Stroke Survivor

Focal shock waves increase efficacy and prolong the effect of botulinum toxin on spasticity in patients with brain injury from stroke and multiple sclerosis.

Assess the effects on spasticity reduction of the association between focal extracorporeal shock wave therapy and botulinum toxin type A, versus the toxin only in brain injury patients. Eighteen patients were included. The study had two phases: the first phase was observational, and botulinum toxin type A was used. The second was a prospective, deliberate intervention phase in which the toxin was injected and focal extracorporeal shock wave treatment was added (1 sessions/week, for three weeks). The patients were followed up in the 1st, 4th and 6th month, the Ashworth Scale criterion was applied and, for those with lower limb involvement and changes in walking the 10-metre walk test was used. Patients treated with toxin only showed a statistically significant improvement in spasticity, with 1 point on the Ashworth Scale from week 5, which disappeared at week 17. However, the combined therapy reduced spasticity by 2 points from week 1 to week 25 (p < 0.001), with a faster result in the 10-meter gait test (p = 0.004). Combined and simultaneous treatment with botulinum toxin and focal extracorporeal shock wave reduced spasticity in a more effective and prolonged way than treatment with botulinum toxin only.

Endoscopic Normalization and Transition of J-Pouch Phenotypes Over Time in Patients With Inflammatory Bowel Disease.

Patients with inflammatory bowel disease (IBD) who undergo proctocolectomy with ileal pouch-anal anastomosis may develop pouchitis. We previously proposed a novel endoscopic classification of pouchitis describing 7 phenotypes with differing outcomes. This study assessed phenotype transitions over time. We classified pouch findings into 7 main phenotypes: (1) normal, (2) afferent limb (AL) involvement, (3) inlet (IL) involvement, (4) diffuse, (5) focal inflammation of the pouch body, (6) cuffitis, and (7) pouch-related fistulas noted more than 6 months after ileostomy takedown. Among 2 endoscopic phenotypes, the phenotype that was first identified was defined as the primary phenotype, and the phenotype observed later was defined as the subsequent phenotype. We retrospectively reviewed 1359 pouchoscopies from 426 patients (90% preoperative diagnosis of ulcerative colitis). The frequency of primary phenotype was 31% for AL involvement, 42% for IL involvement, 28% for diffuse inflammation, 72% for focal inflammation, 45% for cuffitis, 18% for pouch-related fistulas, and 28% for normal pouch. The most common subsequent phenotype was focal inflammation (64.8%), followed by IL involvement (38.6%), cuffitis (37.8%), AL involvement (25.6%), diffuse inflammation (23.8%), normal pouch (22.8%), and pouch-related fistulas (11.9%). Subsequent diffuse inflammation, pouch-related fistulas, and AL or IL stenoses significantly increased the pouch excision risk. Patients who achieved subsequent normal pouch were less likely to have pouch excision than those who did not (8.1% vs 15.7%; P = .15). Pouch phenotype and the risk of pouch loss can change over time. In patients with pouch inflammation, subsequent pouch normalization is feasible and associated with favorable outcome.

Kinematic upper limb analysis outperforms electromyography at grading the severity of dystonia in children with cerebral palsy

BackgroundSeverity of dyskinesia in children with cerebral palsy is often assessed using observation-based clinical tools. Instrumented methods to objectively measure dyskinesia have been proposed to improve assessment accuracy and reliability. Here, we investigated the technique and movement features that were most suitable to objectively measure the severity of dystonia in children with cerebral palsy. MethodsA prospective observational study was conducted with 12 participants with cerebral palsy with a predominant motor type of dyskinesia, spasticity, or mixed dyskinesia/spasticity who had upper limb involvement (mean age: 12.6 years, range: 6.7–18.2 years). Kinematic and electromyography data were collected bilaterally during three upper limb tasks. Spearman rank correlations of kinematic or electromyography features were calculated against dystonia severity, quantified by the Dyskinesia Impairment Scale. FindingsKinematic features were more influential compared to electromyography features at grading the severity of dystonia in children with cerebral palsy. Kinematic measures quantifying jerkiness of volitional movement during an upper limb task with a reaching component performed best (|rs| = 0.78–0.9, p < 0.001). InterpretationThis study provides guidance on the types of data, features of movement, and activity protocols that instrumented methods should focus on when objectively measuring the severity of dystonia in children with cerebral palsy.

Prevalence and Associations of Depression in Parents of Children With Congenital Talipes Equinovarus: A Single-Centre Study.

Congenital talipes equinovarus (CTEV) is a congenital deformity that requires weekly visits to the hospital for manipulation and corrective cast application, followed by an intensive bracing regimen requiring multiple visits to the hospital spread over the years. Parents of children with clubfoot are known to undergo a range of negative emotions. The objective of this study was to identify the prevalence of depression and the factors associated with depression in parents of children with idiopathic CTEV. This cross-sectional study consecutively enrolled 190 parents of children with idiopathic CTEV undergoing treatment at King George Medical University. Parents with conditions that preclude the assessment of mental status were not included. These conditions include a history of head injury or psychiatric illness, parents with ongoing treatment of psychiatric illness, ongoing chronic illness, chronic neurological disease, and parents with clinically established intellectual disability. Information was recorded on certain parent-related characteristics and certain child-related characteristics. Parent-related information included age and sex of the parent, religion, area of residence, number of children in the family, degree of perceived social support (using the Multidimensional Scale of Perceived Social Support, MSPSS), level of education, socio-economic status, depression subscale score of DASS 21 (Depression, Depression Anxiety, and Stress Scale -21), chronic pain (visual analogue scale, VAS), family history of clubfoot or depression, and level of stress caused by a major life event during the past year using the Presumptive Stressful Life Event Scale (PSLES). Child-related information included the sex of the child, phase of treatment (casting or bracing), limb involvement (unilateral or bilateral), relapse of the deformity, and Pirani score of the deformity. Bivariate analysis and logistic regression were used to identify factors associated with a score ≥10 on the depression subscale of DASS 21. One hundred forty-five subjects were males (76.3%). The mean age of the enrolled parents was 28.47±4.89 years. The mean score on the depression subscale of DASS-21 was 4.87±6.3. Thirty-two parents (16.8%) had a score of ≥10 on the depression subscale of the DASS-21. On bivariate analysis, female sex, being Hindu, having studied up to class 12th, relapse, MSPSSscore, and PSLES score were found to be associated with a score ≥10 on the depression subscale of the DASS-21. On logistic regression, female sex, lack of graduate education and above, and MSPSS scores were found to be significantly associated with a score of ≥10 on the depression subscale of the DASS 21 score. The prevalence of depression in parents of children with idiopathic clubfoot was 16.8%. Female gender, lack of college education, and the level of perceived social support (MSPSS) are independently associated with a score ≥10 on the depression subscale of DASS 21. We recommend screening parents of children with clubfoot and referring those with abnormal scores to a psychiatrist for a confirmed diagnosis.

Anaesthetic Management for a Dilated Cardiomyopathy Patient, Scheduled for Lumbar Spine Surgery

Background: Dilated cardiomyopathy (DCM) is characterized by dilatation and impaired contraction of left or both ventricles. Diagnostic criteria are left ventricular ejection fraction less than 40% or fractional shortening less than 25%. Perioperative anesthetic management of these patients are very challenging for anesthesiologists and intensive care team. Case presentation: A 55-year-old female, presented with complaints of severe low-back ache with bilateral lower limb involvement without involvement of bladder and bowel. Severe cord compression at the L4-5 level and severe lumbar canal stenosis were detected by magnetic resonance imaging. Echocardiography showed that the left ventricle (LV) had global hypokinesia, with an ejection fraction of 25% to 30%. The planed surgical procedures were L4 lumbar laminectomy and L4-5 microdiscectomy. Conclusion: Continuous monitoring and control of hemodynamic parameters as well as central venous pressure guided fluid therapy is the cornerstone in improving outcome in patients with dilated cardiomyopathy for non-cardiac surgery.

Outcomes following interval delayed first rib resection for acute axillosubclavian deep venous thrombosis

BackgroundCurrent management of axillosubclavian deep venous thrombosis (DVT) often uses thrombolysis for the DVT, prompt first rib removal, and occasional venoplasty or stenting. Our institution has increasingly used anticoagulation alone followed by interval first rib resection. We sought to analyze the effectiveness of this simplified technique. MethodsBetween September 2012 and April 2021, 27 patients were identified within the institution's electronic medical record as having undergone first rib resection for upper extremity DVT. Seven of these patients had undergone preoperative thrombolysis before referral and were excluded. Among the remaining 20 patients, preoperative clinic charts were evaluated for age, venous segment involvement, contralateral limb involvement, presence of documented hypercoagulable state, duration of preoperative and postoperative anticoagulation, and postoperative outcomes. ResultsOf the 20 patients (mean age, 26.2 years; 13 males) presenting with acute axillosubclavian DVT, all patients had right (n = 8) or left (n = 12) arm swelling. Five patients had extremity pain and four had extremity discoloration. Ten had axillosubclavian vein involvement, 9 had subclavian vein involvement, and 1 had axillary vein involvement. Two patients were on oral contraceptives and no patients had any other diagnosed hypercoagulable conditions. The mean duration of preoperative and postoperative anticoagulation was 3.2 ± 2.6 months and 2.1 ± 2.1 months, respectively. Nineteen patients underwent supraclavicular first rib resection and 1 patient underwent transaxillary resection. Twelve patients (60%) demonstrated complete DVT resolution by venous duplex examination during the postoperative period and 8 patients (40%) demonstrated partial recanalization/chronic DVT. Complications included one hemothorax and one thoracic duct injury. All 20 patients remain asymptomatic without arm swelling, with a mean follow-up of 55.1 ± 34.7 months. ConclusionsAmong patients presenting with acute axillosubclavian DVT, anticoagulation alone followed by interval first rib resection proved to be successful in providing symptomatic relief in the short to medium term. By eliminating the need for preoperative thrombolysis and postoperative venograms, this potentially cost-saving algorithm simplifies our management for acute venous thoracic outlet syndrome while maintaining good clinical outcomes. Because this study only analyzed our management algorithm's effectiveness in the short to medium term, the long-term effectiveness of this treatment will need to be demonstrated.

Neurologic complications of thiamine (B1) deficiency following bariatric surgery in adolescents

BackgroundThe prevalence of obesity among children and adolescents is rising and poses a major health concern. Bariatric surgery is well established in adults and has become an option for adolescents. Thiamine (B1) deficiency is common following bariatric surgery in adults. It may present as Beri-Beri, Wernicke encephalopathy, or Korsakoff psychosis. ObjectiveOur aim was to describe the clinical features, diagnosis, and treatment of adolescents who presented with B1 deficiency after bariatric surgery at one center, and to summarize the data from the literature. PatientsThree adolescents with morbid obesity (two boys and one girl, aged 15.5 to- 17-years-old), presented at Schneider Children's Medical Center of Israel with progressive lower limb pain and weakness 2–3 month following a bariatric procedure (sleeve gastrectomy or narrowing of a bariatric band). The girl also had upper limb involvement and cerebellar signs. All three were non-compliant with micronutrient supplementation. After admission, they received intravenous B1 and oral multivitamin supplementation, and their symptoms improved considerably. ConclusionsMicronutrient supplementation following bariatric surgery is crucial to prevent deficiencies. In adolescents, compliance with micronutrient supplementation should be assessed before and after such surgery. Thiamine deficiency may cause polyneuropathy, among other symptoms. Treatment reduces the severity of neurological complications.

The burden of upper motor neuron involvement is correlated with the bilateral limb involvement interval in patients with amyotrophic lateral sclerosis: a retrospective observational study.

JOURNAL/nrgr/04.03/01300535-202505000-00032/figure1/v/2024-07-28T173839Z/r/image-tiff Amyotrophic lateral sclerosis is a rare neurodegenerative disease characterized by the involvement of both upper and lower motor neurons. Early bilateral limb involvement significantly affects patients' daily lives and may lead them to be confined to bed. However, the effect of upper and lower motor neuron impairment and other risk factors on bilateral limb involvement is unclear. To address this issue, we retrospectively collected data from 586 amyotrophic lateral sclerosis patients with limb onset diagnosed at Peking University Third Hospital between January 2020 and May 2022. A univariate analysis revealed no significant differences in the time intervals of spread in different directions between individuals with upper motor neuron-dominant amyotrophic lateral sclerosis and those with classic amyotrophic lateral sclerosis. We used causal directed acyclic graphs for risk factor determination and Cox proportional hazards models to investigate the association between the duration of bilateral limb involvement and clinical baseline characteristics in amyotrophic lateral sclerosis patients. Multiple factor analyses revealed that higher upper motor neuron scores (hazard ratio [HR] = 1.05, 95% confidence interval [CI] = 1.01-1.09, P = 0.018), onset in the left limb (HR = 0.72, 95% CI = 0.58-0.89, P = 0.002), and a horizontal pattern of progression (HR = 0.46, 95% CI = 0.37-0.58, P < 0.001) were risk factors for a shorter interval until bilateral limb involvement. The results demonstrated that a greater degree of upper motor neuron involvement might cause contralateral limb involvement to progress more quickly in limb-onset amyotrophic lateral sclerosis patients. These findings may improve the management of amyotrophic lateral sclerosis patients with limb onset and the prediction of patient prognosis.

Predictors of Developing a Complex Course of Osteomyelitis in Patients with Sickle Cell Anaemia.

Despite the numerous advances in management strategies, treating osteomyelitis in individuals with sickle cell disease (SCD) remains a significant challenge, leading to severe long-term consequences. This study aimed to assess the key factors potentially linked to a complex progression of osteomyelitis in patients diagnosed with SCD. A cohort of 34 patients was identified and their progress was monitored over a span of 12 months during a 10-year period (2010-2020). The variables under investigation encompassed demographic and clinical traits, laboratory analyses and imaging data, as well as the treatment strategies employed. The risk prediction model pinpointed 5 factors (severity of SCD, involvement of lower limbs, presence of bacteraemia, magnetic resonance image [MRI] findings and utilisation of surgical debridement) that exhibited an area under the curve (AUC) exceeding 0.7. Causative organisms were identified in 9 out of the total 34 patients (26.47%). A total of 17 patients displayed a severe course of SCD (AUC = 7.88), with MRI being highlighted as a valuable contributing factor (AUC = 7.88). Furthermore, 13 patients (38.2%) underwent surgical debridement, a procedure that yielded a statistically significant P value of 0.012 and an AUC of 0.714. Osteomyelitis within the context of severe SCD, particularly when accompanied by lower extremity infection, bacteraemia, positive MRI findings and the need for surgical debridement, emerges as a cluster of risk factors predisposing individuals to osteomyelitis relapse and a more complex disease course.

Arterial involvement of lower limbs in peripheral artery disease - the difference between diabetics and non-diabetics.

To detect peripheral artery disease in diabetic and non-diabetic individuals. The case-control study was conducted from October 2018 to September 2019 at Ruth K.M. Pfau Civil Hospital, Karachi, and comprised diagnosed diabetic patients with random blood sugar ≥200mg/dl in group A, and healthy non-diabetic subjects in group B. Ankle brachial pressure index was measured and mean luminal diameters of lower limb arteries were compared using colour Doppler ultrasonography. Data was analysed using SPSS 21. Of the 82 subjects, 41(50%) were in each of the 2 groups. The sample had 42(51.2%) males and 30(48.8%) females with overall mean age of 53.9±5.07 years (range 44-60 years). There was significant difference in the ankle brachial pressure index values between the groups (p=0.004). There was also a significant difference in the mean luminal diameters of distal arteries (p=0.001), while there was no significant difference in proximal arteries (p>0.05). The diabetics were more prone to developing peripheral arterial disease than nondiabetics.