keyword
https://read.qxmd.com/read/38605390/patient-reported-experience-with-fabry-disease-and-its-management-in-the-real-world-setting-results-from-a-double-blind-cross-sectional-survey-of-280-respondents
#1
JOURNAL ARTICLE
Lisa Berry, Jerry Walter, Jack Johnson, Julia Alton, Janet Powers, Xavier Llòria, Irene Koulinska, Meghan McGee, Dawn Laney
BACKGROUND: Fabry disease (FD) is a rare X-linked lysosomal storage disorder with a heterogeneous clinical presentation. Patients with FD may exhibit early signs/symptoms including neuropathic pain, gastrointestinal complaints, and dermatologic manifestations. FD may ultimately progress to renal, neurologic, and cardiac dysfunction. Current treatments for FD have significantly improved the management and outcomes for patients with FD, but important clinical and convenience limitations still exist...
April 11, 2024: Orphanet Journal of Rare Diseases
https://read.qxmd.com/read/38590921/suspected-gluteal-compartment-syndrome-etiology-predicts-clinical-course-outcomes-and-resource-utilization
#2
Alicia M Williams, Suman Medda, Meghan K Wally, Rachel B Seymour, Alexander Hysong, Amber Stanley, Givenchy Manzano, Joseph R Hsu
BACKGROUND: Gluteal Compartment Syndrome (GCS) is a rare subtype of acute compartment syndrome (ACS), complex to diagnose and potentially fatal if left untreated. The incidence of ACS is estimated to be 7.3 per 100,000 in males and 0.7 per 100,000 in females [1-3]. Given its rare occurrence, the incidence of GCS is not well reported. In the case of GCS, the most common etiologies are surgical positioning, prolonged immobilization secondary to substance use or loss of consciousness, and traumatic injury...
June 2024: Trauma Case Reports
https://read.qxmd.com/read/38585254/updated-evaluation-of-agalsidase-alfa-enzyme-replacement-therapy-for-patients-with-fabry-disease-insights-from-real-world-data
#3
REVIEW
Sandro Feriozzi, Cristina Chimenti, Ricardo Claudio Reisin
The clinical use of agalsidase alfa as enzyme replacement therapy (ERT) for Fabry disease (FD) has spread since 2001, and a large body of evidence of its effectiveness has been collected. This review presents the clinical and laboratory results achieved with agalsidase alfa, which has been published in the literature. Agalsidase alfa infusion slows down or stops the progression of renal damage, expressed by reduction or stabilization of the annual decline of the glomerular filtration rate; yearly decrease of glomerular filtration rate (slope) sometimes is reduced until its stabilization...
2024: Drug Design, Development and Therapy
https://read.qxmd.com/read/38458768/dosing-time-dependent-difference-in-the-suppressive-effect-of-empagliflozin-on-the-development-of-mechanical-pain-hypersensitivity-in-diabetic-mice
#4
JOURNAL ARTICLE
Ai Sato, Sai Yasukochi, Naho Iwanaka, Tomoaki Yamauchi, Akito Tsuruta, Satoru Koyanagi, Shigehiro Ohdo
A problem for patients with diabetes is the rise of complications, such as peripheral neuropathy, nephropathy and retinopathy. Among them, peripheral neuropathy, characterized by numbness and/or hypersensitivity to pain in the extremities, is likely to develop in the early stages of diabetes. Empagliflozin (EMPA), a sodium-glucose cotransporter-2 inhibitor, exerts hypoglycemic effects by preventing glucose reabsorption in proximal tubular cells. EMPA can improve cardiovascular and renal outcomes in diabetic patients, but its suppressive effect on the development of diabetic neuropathy remains unclear...
March 8, 2024: Journal of Pharmacology and Experimental Therapeutics
https://read.qxmd.com/read/38435190/etiology-of-drug-induced-edema-a-review-of-dihydropyridine-thiazolidinedione-and-other-medications-causing-edema
#5
REVIEW
Evan S Sinnathamby, Bretton T Urban, Robert A Clark, Logan T Roberts, Audrey J De Witt, Danielle M Wenger, Aya Mouhaffel, Olga Willett, Shahab Ahmadzadeh, Sahar Shekoohi, Alan D Kaye, Giustino Varrassi
Edema is an accumulation of fluid in the body's tissues that affects millions of Americans yearly. It can affect multiple body parts, for example, the brain or eyes, but often occurs in the periphery, including the feet and legs. Medications, such as dihydropyridine and thiazolidinediones (TZDs), can be the etiology of edema. Edema can develop in association with problems in the vasculature or lymphatic flow. In recent years, a better understanding of these drug-induced mechanisms has been appreciated. Specifically, dihydropyridines can increase hydrostatic pressure and cause selective pre-capillary vessel vasodilation...
February 2024: Curēus
https://read.qxmd.com/read/38408341/compressive-myelopathy-from-diffuse-spinal-dural-calcifications-in-a-patient-with-end-stage-renal-disease-illustrative-case
#6
JOURNAL ARTICLE
Alexis Malecki, Jacob Pawloski, Anthony Anzalone, Kelly Shaftel, Hassan Ali Fadel, Ian Lee
BACKGROUND: Diffuse spinal dural calcification is a rare disorder associated with hyperparathyroidism, including the secondary forms associated with renal failure, osteodystrophy, and chronic hypocalcemia. Here, the authors report a rare case of diffuse dural calcification causing spinal cord compression with myelopathy, requiring decompressive surgery with duraplasty to achieve adequate decompression. OBSERVATIONS: A 46-year-old male with a history of renal failure on dialysis presented with 2 months of progressive neuropathic pain, lower-extremity weakness, and nonsustained clonus...
February 26, 2024: J Neurosurg Case Lessons
https://read.qxmd.com/read/38255865/spice-up-your-kidney-a-review-on-the-effects-of-capsaicin-in-renal-physiology-and-disease
#7
REVIEW
Michela Musolino, Mario D'Agostino, Mariateresa Zicarelli, Michele Andreucci, Giuseppe Coppolino, Davide Bolignano
Capsaicin, the organic compound which attributes the spicy flavor and taste of red peppers and chili peppers, has been extensively studied for centuries as a potential natural remedy for the treatment of several illnesses. Indeed, this compound exerts well-known systemic pleiotropic effects and may thus bring important benefits against various pathological conditions like neuropathic pain, rhinitis, itching, or chronic inflammation. Yet, little is known about the possible biological activity of capsaicin at the kidney level, as this aspect has only been addressed by sparse experimental investigations...
January 8, 2024: International Journal of Molecular Sciences
https://read.qxmd.com/read/38021980/gabapentin-induced-myoclonus-in-a-patient-with-chronic-kidney-disease
#8
Ammar N Mohamed, Marie-Alex Michel, Samy I McFarlane
Gabapentin contains a cyclohexyl group and is a form of gamma-aminobutyric acid (GABA). Despite its name, gabapentin does not affect the inhibitory neurotransmitter GABA or its receptors. Instead, it acts as a ligand, binding strongly to the α2δ (Ca) channel subunit and interfering with its regulatory function and the release of excitatory neurotransmitters. Gabapentin is approved by the FDA for treating seizure disorders and neuropathic pain, except for trigeminal neuralgia. However, it is frequently used off-label to treat other pain conditions and psychological disorders, such as anxiety...
October 2023: Curēus
https://read.qxmd.com/read/37525576/new-onset-severe-eosinophilic-granulomatosis-with-polyangiitis-following-the-third-dose-of%C3%A2-mrna-covid-19-vaccine-a-case-report
#9
JOURNAL ARTICLE
Salah Mahdi, Anwar I Joudeh, Krishnamoorthy Sundara Raman, Samia Ait Faqih, Mohammed Ibrahim Alhatou, Muhammad Faisal Wadiwala, Mohammed Akhtar, Abdo Qaid Ahmed Lutf
Eosinophilic granulomatosis with polyangiitis (EGPA) is a complex multifactorial disease that results in multisystemic inflammation of the small- and medium-sized arteries. The exact pathogenesis of this syndrome is poorly understood, but it is postulated to result from a combination of eosinophilic dysfunction, genetic predisposition, and the development of autoantibodies after exposure to an unknown stimulus. We describe a case of new-onset EGPA following the third dose of the Pfizer-BioNTech mRNA vaccine in an infection-naive middle-aged man with a background history of allergic respiratory symptoms...
July 25, 2023: Modern rheumatology case reports
https://read.qxmd.com/read/37493632/neuropathic-pain-in-patients-with-primary-knee-osteoarthritis-a-cross-sectional-study
#10
JOURNAL ARTICLE
Ahmed Mougui, Sara Belouaham, Imane El Bouchti
INTRODUCTION: Neuropathic pain (NP) is believed to be a distinct subtype of pain associated with knee osteoarthritis (KO). This study aimed to determine the prevalence of NP in patients with primary KO and examine its correlation with various factors. METHODS: A cross-sectional study was conducted on 195 patients with primary KO who did not have the following conditions: knee surgery, infection, rheumatic diseases, peripheral and central neurological diseases, patients who received treatment for neuropathic pain in the past 6 months, diabetes, renal failure, and alcoholism...
July 26, 2023: Romanian Journal of Internal Medicine
https://read.qxmd.com/read/37480023/management-of-pain-in-fabry-disease-in-the-uk-clinical-setting-consensus-findings-from-an-expert-delphi-panel
#11
JOURNAL ARTICLE
Karolina M Stepien, Alexander Broomfield, Duncan Cole, Patrick B Deegan, Stuart Forshaw-Hulme, Derralynn Hughes, Ana Jovanovic, Liz Morris, Alison Muir, Uma Ramaswami
BACKGROUND: Fabry disease is a rare, X-linked inherited lysosomal storage disorder, that manifests as a heterogeneous disease with renal, cardiac and nervous system involvement. The most common pain experienced by people with Fabry disease are episodes of neuropathic pain reported in up to 80% of classical hemizygous male patients and up to 65% of heterozygous female patients. No clear consensus exists within UK clinical practice for the assessment and management of pain in Fabry disease based on agreed clinical practice and clinical experience...
July 21, 2023: Orphanet Journal of Rare Diseases
https://read.qxmd.com/read/37317726/aetiology-pathogenesis-and-management-of-neuropathic-itch-a-narrative-review-with-recent-updates
#12
REVIEW
Shreya Poddar, Himel Mondal, Indrashis Podder
Neuropathic itch is a relatively common yet under-reported cause of systemic pruritus. It is a debilitating condition often associated with pain, which impairs the patient's quality of life. Although much literature exists about renal and hepatic pruritus, there is a dearth of information and awareness about neuropathic itch. The pathogenesis of neuropathic itch is complex and can result from an insult at any point along the itch pathway, ranging from the peripheral receptors and nerves until the brain. There are several causes of neuropathic itch, many of which do not produce any skin lesions and are thus, often missed...
May 3, 2023: Indian Journal of Dermatology, Venereology and Leprology
https://read.qxmd.com/read/36999581/simultaneous-ovarian-and-renal-vein-thrombosis-as-a-rare-cause-of-postoperative-pain-after-sigmoid-colectomy
#13
JOURNAL ARTICLE
Tara Chandran, Murad Bani Hani, Jon Hain
Post operative pain is often the cause of many ED visits. When patients are discharged and return with postoperative abdominal pain, common etiologies include incisional pain, neuropathic pain, musculoskeletal pain from immobility, ileus, and more sinister causes including adhesive bowel obstruction, abscess formation, and anastomotic leak. We present a 62 year-old female without any hereditary thrombophilia or other prothrombotic factors who presented to the ED after a sigmoid colectomy and diverting ileostomy for perforated diverticulitis and subsequent ileostomy reversal with abdominal pain...
March 31, 2023: American Surgeon
https://read.qxmd.com/read/36994274/factors-affecting-pain-in-hemodialysis-and-non-pharmacological-management
#14
REVIEW
Georgia Gerogianni
Pain is a frequent problem among hemodialysis patients, mostly caused by painful procedures, acute complications of hemodialysis, and painful syndromes, such as musculoskeletal and neuropathic syndromes. Pain can frequently lead to sleep disturbances, reduced adherence to hemodialysis treatment, frequent hospital admissions, decreased quality of life, and high mortality rates. Non-pharmacological management of pain in the hemodialysis population includes aerobic and resistance exercises, music therapy, and cognitive behavioral therapy...
February 2023: Curēus
https://read.qxmd.com/read/36964497/efficacy-of-surgical-treatment-on-polypharmacy-of-elderly-patients-with-lumbar-spinal-canal-stenosis-retrospective-exploratory-research
#15
JOURNAL ARTICLE
Sota Nagai, Risa Inagaki, Takehiro Michikawa, Soya Kawabata, Kaori Ito, Kurenai Hachiya, Hiroki Takeda, Daiki Ikeda, Shinjiro Kaneko, Shigeki Yamada, Nobuyuki Fujita
BACKGROUND: Polypharmacy is a growing public health problem occurring in all healthcare settings worldwide. Elderly patients with lumbar spinal canal stenosis (LSS) who manifest low back and neuropathic pain and have a high frequency of comorbidity are predicted to take many drugs. However, no studies have reported polypharmacy in elderly patients with LSS. Thus, we aimed to review the polypharmacy among elderly LSS patients with elective surgeries and examine how the surgical treatment reduces the polypharmacy...
March 24, 2023: BMC Geriatrics
https://read.qxmd.com/read/36790683/safety-tolerability-and-pharmacokinetics-of-single-and-multiple-doses-of-mirogabalin-in-healthy-chinese-participants-a-randomized-double-blind-placebo-controlled-study
#16
JOURNAL ARTICLE
Yi Li, Kaoru Toyama, Takafumi Nakatsu, Hitoshi Ishizuka, Hailan Wu, Guoying Cao, Jicheng Yu, Yu Wang, Xiaofen Liu, Beining Guo, Jufang Wu, Peimin Yu, Zhen Hong, Jing Zhang, Xiaojie Wu
INTRODUCTION: Mirogabalin is a treatment option for patients with neuropathic pain; however, safety, tolerability, and pharmacokinetics (PK) data specifically for Chinese individuals are limited to a single-dose study. We aimed to assess these for both single- and multiple-dose mirogabalin in healthy Chinese participants. METHODS: In this randomized, double-blind, placebo-controlled, phase I study, 54 healthy Chinese men and women aged 18-45 years were randomly allocated to receive single- (5, 10, or 15 mg, daily) or multiple-dose (5 mg titrated to 15 mg, twice-daily, over 22 days) oral mirogabalin or placebo...
February 15, 2023: Advances in Therapy
https://read.qxmd.com/read/36719161/gabapentin-concomitant-prescription-of-opioids-and-benzodiazepines-among-kidney-transplant-recipients
#17
JOURNAL ARTICLE
Yusi Chen, JiYoon B Ahn, Sunjae Bae, Corey Joseph, Mark Schnitzler, Gregory P Hess, Krista L Lentine, Bonnie E Lonze, Dorry L Segev, Mara McAdams-DeMarco
BACKGROUND: Gabapentinoids, commonly used for treating neuropathic pain, may be misused and coprescribed with opioid and benzodiazepine, increasing the risk of mortality and dependency among kidney transplant recipients. METHODS: We identified adult kidney transplant recipients who enrolled in Medicare Part D in 2006-2017 using the United States Renal Data System/Medicare claims database. We characterized recipients' post-transplant concomitant prescription of gabapentinoids, opioids, and benzodiazepine stratified by transplant year and recipient factors (age, sex, race, and diabetes)...
January 1, 2023: Clinical Journal of the American Society of Nephrology: CJASN
https://read.qxmd.com/read/36709533/clinical-outcomes-among-young-patients-with-fabry-disease-who-initiated-agalsidase-beta-treatment-before-30-years-of-age-an-analysis-from-the-fabry-registry
#18
JOURNAL ARTICLE
Robert J Hopkin, Gustavo H Cabrera, John L Jefferies, Meng Yang, Elvira Ponce, Eva Brand, Ulla Feldt-Rasmussen, Dominique P Germain, Nathalie Guffon, Ana Jovanovic, Ilkka Kantola, Amel Karaa, Ana M Martins, Camilla Tøndel, William R Wilcox, Han-Wook Yoo, Alessandro P Burlina, Michael Mauer
BACKGROUND: Clinical manifestations of classic Fabry disease (α-galactosidase A deficiency) usually occur in childhood, while complications involving major organs typically develop in adulthood. Outcomes of Fabry-specific treatment among young patients have not been extensively reported. Our aim was to analyze clinical outcomes among patients aged 5-30 years at initiation of treatment with agalsidase beta using data from the Fabry Registry (NCT00196742, sponsor: Sanofi). METHODS: Reported GLA variants were predicted to be associated with the classic phenotype or not classified in fabry-database...
February 2023: Molecular Genetics and Metabolism
https://read.qxmd.com/read/36703293/ultrasound-guided-cutaneous-nerve-chemo-ablation-for-severe-access-site-neuropathic-pain-a-case-report
#19
Aakanksha Agarwal, Timothy Murray, Nikki Craig, Mercedeh Kiaii
Neuropathic pain at the cannulation site is challenging, both for the patient and the dialysis team. We present a case of a middle-aged man on chronic hemodialysis, who developed excruciating pain at the cannulation area without incident, limiting his dialysis sessions. Multidisciplinary collaboration allowed identification of the cutaneous nerve-the inferior lateral cutaneous nerve of the arm, relaying his pain. Subsequent ultrasound-guided phenol chemoablation resulted in the complete resolution of his pain and allowed continued use of the well-functioning fistula for dialysis...
April 2023: Hemodialysis International
https://read.qxmd.com/read/36651363/synergistic-action-between-a-synthetic-cannabinoid-compound-and-tramadol-in-neuropathic-pain-rats
#20
JOURNAL ARTICLE
Geovanna Nallely Quiñonez-Bastidas, Ulises Osuna-Martínez, Ana Laura Reda-Licea, Manuel López-Ortíz, Ignacio Regla, Andrés Navarrete
In the present study the interaction of cannabinoid, PhAR-DBH-Me [( R , Z )-18-((1 S ,4 S )-5-methyl-2,5-diazabicyclo[2.2.1]heptan-2-yl)-18-oxooctadec-9-en-7-ylphenyl-acetate] and tramadol in two neuropathy models, as well as their possible toxic effects, was analyzed. The anti-allodynic effect of PhAR-DBH-Me, tramadol, or their combination, were evaluated in neuropathic rats. Furthermore, the effective dose 35 (as the 35 % of the anti allodynic effect) was calculated from the maximum effect of each drug. Moreover, the isobolographic analysis was performed to determine the type of interaction between the drugs...
December 1, 2022: Acta Pharmaceutica
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