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BMC Musculoskeletal Disorders Jun 2024Jaffe-Campanacci syndrome is a rare syndrome, characterized by multiple non-ossifying fibromas (NOF) and cafe-au-lait patches. The name was coined in 1982 by Mirra after... (Review)
Review
BACKGROUND
Jaffe-Campanacci syndrome is a rare syndrome, characterized by multiple non-ossifying fibromas (NOF) and cafe-au-lait patches. The name was coined in 1982 by Mirra after Jaffe who first described the case in 1958. Although it's suggested there is a relation with Neurofibromatosis type 1, there is still no consensus on whether Jaffe-Campanacci syndrome is a subtype or variant of neurofibromatosis-1(NF-1).
CASE PRESENTATION
In this article, we present a case series of 2 patients. The first case is a 13-year-old male with Jaffe-Campanacci syndrome who presented with a distal femur fracture. His father had positive features of both Jaffe-Campanacci syndrome and NF-1, while his sister only had features of NF-1, so we presented both.
CONCLUSION
Jaffe-Campanacci has a clear relationship with type 1 neurofibromatosis, which still has to be genetically established. Due to the presence of several large non-ossifying fibromas of the long bones, it is linked to a significant risk of pathological fractures. We concur with previous authors, that an osseous screening program should be performed for all patients with newly diagnosed type 1 neurofibromatosis, to identify non-ossifying fibromas and assess the potential for pathological fracture. Moreover, siblings of patients with NF-1 should be screened for multiple NOFs that may carry a high risk of pathological fractures.
Topics: Humans; Male; Adolescent; Neurofibromatosis 1; Cafe-au-Lait Spots; Female; Fibroma; Femoral Fractures
PubMed: 38937801
DOI: 10.1186/s12891-024-07581-0 -
BMC Cancer Jun 2024Evaluation publications typically summarize the results of studies to demonstrate the effectiveness of an intervention, but little is shared concerning any changes... (Randomized Controlled Trial)
Randomized Controlled Trial
Process evaluation protocol plan for a home-based physical activity intervention versus educational intervention for persistent taxane-induced peripheral neuropathy (B-HAPI study): a randomized controlled trial.
BACKGROUND
Evaluation publications typically summarize the results of studies to demonstrate the effectiveness of an intervention, but little is shared concerning any changes implemented during the study. We present a process evaluation protocol of a home-based gait, balance, and resistance exercise intervention to ameliorate persistent taxane-induced neuropathy study according to 7 key elements of process evaluation.
METHODS
The process evaluation is conducted parallel to the longitudinal, randomized control clinical trial examining the effects of the home-based gait, balance, and resistance exercise program for women with persistent peripheral neuropathy following treatment with taxanes for breast cancer (IRB approval: Pro00040035). The flowcharts clarify how the intervention should be implemented in comparable settings, fidelity procedures help to ensure the participants are comfortable and identify their individual needs, and the process evaluation allows for the individual attention tailoring and focus of the research to avoid protocol deviation.
CONCLUSIONS
The publication of the evaluation protocol plan adds transparency to the findings of clinical trials and favors process replication in future studies. The process evaluation enables the team to systematically register information and procedures applied during recruitment and factors that impact the implementation of the intervention, thereby allowing proactive approaches to prevent deviations from the protocol. When tracking an intervention continuously, positive or negative intervention effects are revealed early on in the study, giving valuable insight into inconsistent results. Furthermore, a process evaluation adds a participant-centered element to the research protocols, which allows a patient-centered approach to be applied to data collection.
TRIAL REGISTRATION
ClinicalTrials.gov NCT04621721, November 9, 2020, registered prospectively.
PROTOCOL VERSION
April 27, 2020, v2.
Topics: Humans; Peripheral Nervous System Diseases; Female; Breast Neoplasms; Taxoids; Exercise Therapy; Patient Education as Topic; Exercise; Bridged-Ring Compounds; Longitudinal Studies; Research Design; Randomized Controlled Trials as Topic
PubMed: 38937667
DOI: 10.1186/s12885-024-12444-x -
Seminars in Oncology Nursing Jun 2024Approximately 60% of cancer survivors receiving neurotoxic chemotherapy experience chemotherapy-induced peripheral neuropathy (CIPN) (eg, hand and foot numbness,...
OBJECTIVES
Approximately 60% of cancer survivors receiving neurotoxic chemotherapy experience chemotherapy-induced peripheral neuropathy (CIPN) (eg, hand and foot numbness, tingling, or pain). There is only one recommended pharmacological treatment (duloxetine) and one modestly beneficial nonpharmacological treatment (exercise) for CIPN. However, data suggest national guideline recommendations are not routinely practiced. Further, less is known about nurses' CIPN management practices. The purpose of this convergent mixed methods study was to explore oncology clinicians' self-reported practices and perceptions regarding CIPN prevention and management.
METHODS
Oncology clinicians at three cancer centers completed a survey about their recommendations for CIPN prevention and management in practice. A subset of clinicians also participated in a semi-structured interview to explore their perspectives of and motivations for implementing CIPN assessment, prevention, and management in practice. Quantitative data were described (eg, frequency or median) and qualitative data were analyzed using inductive content analysis.
RESULTS
This study (N = 44 survey responses; n = 9 interviews) resulted in four themes: (1) clinicians primarily recommend gabapentin for CIPN management and often observe cryotherapy used for CIPN prevention, but these interventions are complicated by discomfort, intolerable side effects, and efficacy concerns; (2) clinicians perceive CIPN as troublesome and desire additional information and resources regarding CIPN prevention and management; (3) CIPN-related education provided by clinicians may be limited by patient retention of the amount of education received about cancer treatment and other factors; (4) clinicians use subjective CIPN assessment to screen at each visit for common CIPN symptoms (eg, numbness or tingling) and the impact of symptoms on day-to-day activities.
CONCLUSIONS
Discrepancies persist between evidence-based guidelines on CIPN management and current oncology clinician practices.
IMPLICATIONS FOR NURSING PRACTICE
Clinician involvement is needed when developing education and resources to help oncology clinicians provide the most evidence-based care to potentially prevent and manage their patients' CIPN.
PubMed: 38937199
DOI: 10.1016/j.soncn.2024.151685 -
The Lancet. Haematology Jul 2024Early diagnosis is crucial for the successful treatment of primary CNS lymphoma (PCNSL), a rapidly progressing tumour. Suspicion raised on brain MRI must be confirmed by... (Review)
Review
Early diagnosis is crucial for the successful treatment of primary CNS lymphoma (PCNSL), a rapidly progressing tumour. Suspicion raised on brain MRI must be confirmed by a histopathological diagnosis of a tumour specimen collected by stereotactic biopsy. In rare cases, cerebrospinal fluid (CSF) or vitreous humour might aid in providing a cytological diagnosis. Several disease-related, patient-related, and treatment-related factors affect the timing and accuracy of diagnosis and patient outcome. Some molecules detected in CSF, aqueous and vitreous humour, and peripheral blood were proposed as diagnostic biomarkers for PCNSL; however, detection methods for most of these molecules are not yet standardised, have a long turnaround time, are expensive, and have little reproducibility among labs. By contrast, the MYD88 somatic hotspot mutation, revealed by PCR-based assay, is currently and reliably used during the diagnosis of some lymphomas, and IL-10, measured by enzyme-linked immunosorbent assay, is routinely used to diagnose and monitor different common metabolic and immunological diseases. Several independent studies have shown that MYD88 and IL-10 can be easily assessed in peripheral blood, plasma, aqueous and vitreous humour, and CSF of patients with PCNSL with substantial sensitivity and specificity, especially when evaluated in combination. In this Viewpoint, evidence supporting the routine use of MYD88 and IL-10 in diagnosing PCNSL is considered, and some examples of the frequent difficulties found in the diagnosis of PCNSL are provided, highlighting the role and indications of these two biomarkers to improve the timely recognition of this aggressive tumour.
Topics: Humans; Myeloid Differentiation Factor 88; Central Nervous System Neoplasms; Interleukin-10; Lymphoma; Biomarkers, Tumor; Mutation
PubMed: 38937027
DOI: 10.1016/S2352-3026(24)00104-2 -
The Journal of Pharmacology and... Jun 2024Chemotherapy-induced peripheral neuropathy (CIPN) is a common side effect of chemotherapy treatment, routinely manifesting as increased pain sensitivity (allodynia) in...
Chronic administration of cannabinoid agonists ACEA (CB1), AM1241 (CB2), and CP55,940 (mixed CB1/CB2) induce sex-specific differences in tolerance and sex hormone changes in a chemotherapy-induced peripheral neuropathy.
Chemotherapy-induced peripheral neuropathy (CIPN) is a common side effect of chemotherapy treatment, routinely manifesting as increased pain sensitivity (allodynia) in distal extremities. Despite its prevalence, effective treatment options are limited. Cannabinoids are increasingly being evaluated for their ability to treat chronic pain conditions, including CIPN. While previous studies have revealed sex differences in cannabinoid-mediated antinociception in acute and chronic pain models, there is a paucity of studies addressing potential sex differences in the response of CIPN to cannabinoid treatment. Therefore, we evaluated the long-term anti-allodynic efficacy of CB-selective (ACEA), CB-selective (AM1241), and CB/CB mixed (CP55,940) agonists in the cisplatin CIPN model, using both male and female mice. CB selective agonism was observed to have sex differences in the development of tolerance to anti-allodynic effects, with females developing tolerance more rapidly than males, while the anti-allodynic effects of selective CB agonism lacked tolerance development. Compound-specific changes to the female estrous cycle and female plasma estradiol levels were noted, with CB selective agonism decreasing plasma estradiol while CB selective agonism increased plasma estradiol. Chronic administration of a mixed CB/CB agonist resulted in increased mRNA expression of proinflammatory cytokines and endocannabinoid regulatory enzymes in female spinal cord tissue. Ovarian tissue was noted to have proinflammatory cytokine mRNA expression following administration of a CB acting compound while selective CB agonism resulted in decreased proinflammatory cytokines and endocannabinoid regulatory enzymes in testes. These results support the need for further investigation into the role of sex and sex hormones signaling in pain and cannabinoid-mediated antinociceptive effects. CIPN is a common side effect of chemotherapy. We have found that both CB1 and CB2 receptor agonism produce antinociceptive effects in a cisplatin CIPN model. We observed that tolerance to CB1-mediated antinociception developed faster in females and did not develop for CB¬2-mediated antinociception. Additionally, we found contrasting roles for CB1/CB¬2 receptors in the regulation of plasma estradiol in females, with CB1 agonism attenuating estradiol and CB¬2 agonism enhancing estradiol. These findings support the exploration of cannabinoid agonists for CIPN.
PubMed: 38936979
DOI: 10.1124/jpet.124.002165 -
BMJ Supportive & Palliative Care Jun 2024Chemotherapy-induced peripheral neuropathy (CIPN) affects patients' quality of life and treatment effectiveness. Gabapentinoids, like gabapentin and pregabalin, are...
INTRODUCTION
Chemotherapy-induced peripheral neuropathy (CIPN) affects patients' quality of life and treatment effectiveness. Gabapentinoids, like gabapentin and pregabalin, are often used for CIPN treatment, but their efficacy and safety remain uncertain. This study reviews and analyses randomised controlled trial data on this topic.
MATERIALS/METHODS
We searched PubMed, Embase and Cochrane CENTRAL until 29 August 2022 for studies on gabapentinoid use in CIPN. Meta-analysis was performed using RevMan V.5.4 and the Metafor package in R. Outcomes included pain scores, quality of life and adverse drug events.
RESULTS
For the prevention setting, our meta-analysis shows that pregabalin did not significantly improve average pain (standardised mean difference (SMD) -0.14, 95% CI -0.51 to 0.23; I=26% (95% CI 0% to >98%)) or quality of life (mean difference (MD) 2.5, 95% CI -4.67 to 9.67; p=0.49) in preventing CIPN compared with placebo. However, it showed a potential trend towards reducing the worst pain (SMD -0.28, 95% CI -0.57 to 0.01; I=0% (95% CI 0% to 98%; p=0.06)). For the treatment setting, some studies have shown a potential therapeutic effect of gabapentinoids. However, the results are not consistent between studies. Given the studies' heterogeneity, a meta-analysis in treatment setting was not performed.
CONCLUSION
There is limited evidence to support the use of gabapentinoids in CIPN. In prevention setting, gabapentinoids do not significantly prevent CIPN. In treatment setting, studies have been inconsistent in their conclusions, lacking definitive benefits over placebo. More comprehensive and higher quality research is needed in the future.
PROSPERO REGISTRATION NUMBER
CRD42022361193.
PubMed: 38936970
DOI: 10.1136/spcare-2023-004362 -
In Vivo (Athens, Greece) 2024Treatment with taxanes can result in chemotherapy-induced peripheral neuropathy (CIPN). We investigated the efficacy and safety of mirogabalin for the treatment of CIPN...
BACKGROUND/AIM
Treatment with taxanes can result in chemotherapy-induced peripheral neuropathy (CIPN). We investigated the efficacy and safety of mirogabalin for the treatment of CIPN in patients who had been administered perioperative chemotherapy including taxane-based agents for breast cancer.
PATIENTS AND METHODS
We retrospectively analyzed the case of 43 patients with early breast cancer who received a taxane as perioperative chemotherapy and were administered mirogabalin at the diagnosis of CIPN.
RESULTS
Thirty-six patients (83.7%) had grade 1 CIPN and the other seven patients (16.3%) had grade 2 CIPN. The median mirogabalin dose was 10 mg (5-30 mg). CIPN improved from grade 1 to 0 in 12 patients (27.9%) and from grade 2 to 1 in one patient (2.3%); 13 (30.2%) patients thus had an objective therapeutic response. There were no cases in which chemotherapy was reduced or discontinued due to CIPN. Adverse events were evaluated by Common Terminology Criteria for Adverse Events and included five cases of dizziness (11.7%), three of somnolence (7.0%), and two of nausea (4.7%), all of which were grade ≤2. There were no cases of serious (grade ≥3) adverse effects.
CONCLUSION
Mirogabalin may be effective and safe for treating CIPN of patients who receive a taxane in a perioperative breast cancer setting.
Topics: Humans; Female; Breast Neoplasms; Peripheral Nervous System Diseases; Middle Aged; Taxoids; Aged; Adult; Treatment Outcome; Antineoplastic Combined Chemotherapy Protocols; Retrospective Studies; Bridged Bicyclo Compounds; Neoplasm Staging; Perioperative Care; Antineoplastic Agents; Bridged-Ring Compounds
PubMed: 38936921
DOI: 10.21873/invivo.13649 -
Voprosy Kurortologii, Fizioterapii, I... 2024Search and development of new physiotherapeutic technologies of regenerative medicine for the treatment of patients with different diseases is an urgent task of modern... (Review)
Review
UNLABELLED
Search and development of new physiotherapeutic technologies of regenerative medicine for the treatment of patients with different diseases is an urgent task of modern medicine.
OBJECTIVE
To analyze scientific data on the effectiveness of the peripheral magnetic stimulation (PMS) application in patients with different diseases.
MATERIAL AND METHODS
An analysis of publications in databases of electronic resources (PEDro, PubMed, Embase, eLibrary, Cochrane Library) over the past 20 years was carried out, the results of PMS application in patients with different nosologies were presented.
RESULTS
The majority of the presented articles confirm the clinical effectiveness of PMS application mainly in patients with diseases of the nervous system, spine and genitourinary system.
CONCLUSION
Further research to confirm the effectiveness of the therapeutic impact of magnetic stimulation in patients with other nosologies is needed.
Topics: Humans; Magnetic Field Therapy; Physical Therapy Modalities; Nervous System Diseases
PubMed: 38934960
DOI: 10.17116/kurort202410103162 -
Zhurnal Nevrologii I Psikhiatrii Imeni... 2024To determine the prevalence of insomnia and the effectiveness of its treatment in patients with a painful form of diabetic polyneuropathy (DPN). (Randomized Controlled Trial)
Randomized Controlled Trial
OBJECTIVE
To determine the prevalence of insomnia and the effectiveness of its treatment in patients with a painful form of diabetic polyneuropathy (DPN).
MATERIAL AND METHODS
Fifty patients with the painful form of DPN were randomly divided into 2 groups: the standard therapy group (ST) and the extended therapy group (ET). In the ST group, a single lesson on sleep hygiene was conducted, in the ET group there were 3-4 face-to-face individual sessions for the treatment of insomnia for two weeks. Both groups were interviewed at the time of hospitalization, after 3 and 6 months. The severity of polyneuropathy and the nature of neuropathic pain were assessed using the Neuropathic Neuropathy Impairment Score in the Lower Limbs (NIS-LL) and the Neuropathy Total Symptom Score - 9 (NTSS-9); the intensity of pain was assessed using a Visual Analog Scale (VAS). Sleep disorders were analyzed using the Pittsburgh Sleep Quality Index (PSQI) and the Insomnia Severity Index (ISI).
RESULTS
Sleep disorders of varying severity were observed in 82% of patients in the initial survey. In both groups, improvement in sleep quality was noted during treatment, but significantly better results were in the ET group, the ISI score after 6 months was 7.15±2.08 for the ST group and 3.07±2.49 for the ET group (<0.0001). In the ST group, there was no significant decrease in the intensity of pain and the severity of polyneuropathy in dynamics. In the ET group, a significant decrease in NTSS-9 and VAS scores was found during the initial survey and after 6 months (<0.0001). The intensity of pain also significantly decreased in the ET group compared with the ST group (<0.0001) at the end of follow-up, which indicates the importance of sleep normalization in the treatment of neuropathic pain.
CONCLUSION
Most patients with the painful form of DPN have insomnia. Treatment of insomnia has shown its effectiveness as part of a multimodal approach to the managing of neuropathic pain in DPN and improving the quality of life of patients.
Topics: Humans; Diabetic Neuropathies; Male; Female; Sleep Initiation and Maintenance Disorders; Middle Aged; Neuralgia; Severity of Illness Index; Aged; Pain Measurement; Adult; Treatment Outcome; Sleep Quality
PubMed: 38934671
DOI: 10.17116/jnevro202412405287 -
Neural Regeneration Research Jun 2024Studies have shown that chitosan protects against neurodegenerative diseases. However, the precise mechanism remains poorly understood. In this study, we administered...
Chitosan alleviates symptoms of Parkinson's disease by reducing acetate levels, which decreases inflammation and promotes repair of the intestinal barrier and blood-brain barrier.
Studies have shown that chitosan protects against neurodegenerative diseases. However, the precise mechanism remains poorly understood. In this study, we administered chitosan intragastrically to an MPTP-induced mouse model of Parkinson's disease and found that it effectively reduced dopamine neuron injury, neurotransmitter dopamine release, and motor symptoms. These neuroprotective effects of chitosan were related to bacterial metabolites, specifically short-chain fatty acids, and chitosan administration altered intestinal microbial diversity and decreased short-chain fatty acid production in the gut. Furthermore, chitosan effectively reduced damage to the intestinal barrier and the blood-brain barrier. Finally, we demonstrated that chitosan improved intestinal barrier function and alleviated inflammation in both the peripheral nervous system and the central nervous system by reducing acetate levels. Based on these findings, we suggest a molecular mechanism by which chitosan decreases inflammation through reducing acetate levels and repairing the intestinal and blood-brain barriers, thereby alleviating symptoms of Parkinson's disease.
PubMed: 38934394
DOI: 10.4103/NRR.NRR-D-23-01511