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Bulletin of Experimental Biology and... Jan 2024A comparative analysis of the infarct-limiting activity of δ- and κ-opioid receptors (OR) agonists was carried out on a model of coronary occlusion (45 min) and...
A comparative analysis of the infarct-limiting activity of δ- and κ-opioid receptors (OR) agonists was carried out on a model of coronary occlusion (45 min) and reperfusion (120 min) in male Wistar rats. We used selective δ-OR agonist deltorphin II (0.12 mg/kg), δ-OR agonists BW373U86 and p-Cl-Phe DPDPE (0.1 and 1 mg/kg), selective agonists of δ-OR DPDPE (0.1 and 0.969 mg/kg), κ-OR U-50,488 (0.1 and 1 mg/kg), κ-OR GR-89696 (0.1 mg/kg), and κ-OR ICI-199,441 (0.1 mg/kg). All drugs were administered intravenously 5 min before reperfusion. Deltorphin II, BW373U86 (1 mg/kg), p-Cl-Phe DPDPE (1 mg/kg), U-50,488 (1 mg/kg), and ICI-199,441 had a cardioprotective effect. The most promising compounds for drug development are ICI-199,441 and deltorphin II.
Topics: Rats; Animals; Male; Rats, Wistar; Receptors, Opioid, delta; Enkephalin, D-Penicillamine (2,5)-; Myocardial Reperfusion; Infarction; Benzamides; Piperazines
PubMed: 38340196
DOI: 10.1007/s10517-024-06020-3 -
Clinical and Experimental Dermatology May 2024Lipoid proteinosis (LP), also known as Urbach-Wiethe disease, is a rare autosomal recessive genodermatosis, caused by mutations in the ECM1 gene. This results in the...
BACKGROUND
Lipoid proteinosis (LP), also known as Urbach-Wiethe disease, is a rare autosomal recessive genodermatosis, caused by mutations in the ECM1 gene. This results in the deposition of periodic acid-Schiff (PAS)-positive, hyaline-like material on the skin, mucosae and internal organs.
OBJECTIVES
To present a case report of LP and a systematic review to synthesize the scientific literature on the management of this uncommon and frequently missed diagnosis.
METHODS
We present a case report of a 48-year-old man with LP who exhibited significant improvement after oral acitretin therapy. To address the lack of large case-control studies on LP treatment, we performed a systematic review of the literature following the PRISMA 2020 criteria. The search was conducted in PubMed, Web of Science, Cochrane and Scopus databases from inception until June 2023. To assess the methodological quality of case reports and case series, we used the Joanna Briggs Collaboration critical appraisal tool.
RESULTS
We included 25 studies that met eligibility criteria. Data from 44 patients with a histopathologically confirmed diagnosis were analysed. Treatment ranged from systemic therapies (acitretin, etretinate, dimethyl sulfoxide, corticosteroids, penicillamine) to surgical or laser procedures. Regarding methodological quality, the main discrepancies arose in the reporting of participant characteristics and treatment interventions.
CONCLUSIONS
Low-dose oral acitretin could have potential in managing LP, exhibiting fewer side-effects compared with other therapeutic agents. Further research is needed to establish more comprehensive and evidence-based treatment guidelines.
Topics: Humans; Lipoid Proteinosis of Urbach and Wiethe; Male; Acitretin; Middle Aged; Keratolytic Agents; Treatment Outcome
PubMed: 38308656
DOI: 10.1093/ced/llae039 -
BMC Neurology Jan 2024Wilson's disease (WD) is an inherited disorder of copper metabolism. Agenesis of the corpus callosum is the complete or partial absence of the major united fiber bundles... (Review)
Review
BACKGROUND
Wilson's disease (WD) is an inherited disorder of copper metabolism. Agenesis of the corpus callosum is the complete or partial absence of the major united fiber bundles connecting the cerebral hemispheres. Intracranial lipoma is an adipose tissue tumor resulting from an abnormal embryonic development of the central nervous system. The simultaneous occurrence of these three disorders is rare and has not been reported. This report focuses on the pathogenesis and association between the three disorders and highlights the importance of recognizing and effectively managing their coexistence.
CASE PRESENTATION
The purpose of this study was to present a patient with coexisting WD, intracranial lipoma, and corpus callosum dysplasia. We reviewed a female patient hospitalized in 2023 with clinical manifestations of elevated aminotransferases and decreased ceruloplasmin, as well as genetic testing for an initial diagnosis of Wilson's disease. Subsequently, a cranial MRI showed corpus callosum dysplasia with short T1 signal changes in the cerebral falx, leading to a final diagnosis of Wilson's disease combined with intracranial lipoma and corpus callosum dysplasia. The patient's WD is currently stable after treatment with sodium dimercaptosulfonamide (DMPS) and penicillamine, and the patient's abnormal copper metabolism may promote the growth of intracranial lipoma.
CONCLUSION
The pathogenesis of WD combined with intracranial lipoma and corpus callosum dysplasia is complex and clinically rare. The growth of intracranial lipomas may be associated with abnormal copper metabolism in WD. Abnormal copper metabolism affects lipid metabolism and triggers inflammatory responses. Therefore, early diagnosis and treatment are beneficial for improvement. Each new case of this rare co-morbidity is important as it allows for a better assessment and understanding of these cases' more characteristic clinical manifestations, which can help estimate the course of the disease and possible therapeutic options.
Topics: Pregnancy; Humans; Female; Hepatolenticular Degeneration; Corpus Callosum; Copper; Penicillamine; Lipoma; Brain Neoplasms
PubMed: 38273263
DOI: 10.1186/s12883-024-03541-2 -
Analytical Chemistry Jan 2024The commercialized electrochemiluminescence (ECL) immunoassay is carried out by holding luminophore Ru(bpy) at a given potential. Designing an electrochemiluminophore...
The commercialized electrochemiluminescence (ECL) immunoassay is carried out by holding luminophore Ru(bpy) at a given potential. Designing an electrochemiluminophore with a narrow triggering potential window is strongly anticipated to decrease the electrochemical cross-talk and improve the flux of the commercialized ECL immunoassay in a potential-resolved way. Herein, L-penicillamine-capped silver nanoclusters (LPA-AgNCs) are facilely synthesized and utilized as tags to perform the ECL immunoassay with a sole and narrow triggering potential window of 0.24 V by employing hydrazine (NH) as a coreactant. The maximum ECL emission of the LPA-AgNCs/NH system is located ca. +1.27 V. Upon immobilizing LPA-AgNCs onto the electrode surface via forming a sandwich immunocomplex, the ECL of LPA-AgNCs/NH can be utilized to sensitively and selectively determine human carcinoembryonic antigen from 0.5 to 1000 pg/mL with a low limit of detection of 0.1 pg/mL (S/N = 3). This work might open a way to screen electrochemiluminophores for the multiple ECL immunoassay in a potential-resolved way.
Topics: Humans; Silver; Electrochemical Techniques; Immunologic Tests; Immunoassay; Luminescent Measurements; Biosensing Techniques; Limit of Detection
PubMed: 38235596
DOI: 10.1021/acs.analchem.3c04816 -
Cureus Dec 2023Wilson's disease (WD) is an autosomal recessive disorder affecting the metabolism of copper that can present with a variety of clinical symptoms. Low levels of serum...
Wilson's disease (WD) is an autosomal recessive disorder affecting the metabolism of copper that can present with a variety of clinical symptoms. Low levels of serum copper and ceruloplasmin, increased excretion of copper in the urine, and/or increasing quantities of copper in the liver are diagnostic indicators. The gold standard for diagnosis is genetic testing. The care approach includes the utilization of liver transplants as a therapeutic option in advanced patients and the use of copper-chelating medications. We describe a unique case of WD in a 14-year-old girl who presented with ascites, hemolytic anemia, and liver dysfunction. There was no indication of abdominal TB, and her viral, autoimmune, and hemolytic profiles were all normal. Low serum ceruloplasmin, elevated urine copper, and distinctive liver histology all supported the WD diagnosis. After starting penicillamine medication, the patient's symptoms improved, but her blood counts did not. This example emphasizes how crucial it is to rule out WD in patients with chronic liver disease, hemolytic anemia, and unexplained ascites, particularly in younger age groups.
PubMed: 38234952
DOI: 10.7759/cureus.50724 -
BioMed Research International 2024[This retracts the article DOI: 10.1155/2022/3619308.].
[This retracts the article DOI: 10.1155/2022/3619308.].
PubMed: 38230034
DOI: 10.1155/2024/9810690 -
Heliyon Jan 2024It is well known that the chiral materials combined with metal ion's structure have been identified as promising candidate for the nursing Alzheimer Disease (AD)...
Novel fabrication of Cu(II)-incorporated chiral d-penicillamine-chitosan nanocomposites enantio-selectively inhibit the induced amyloid β aggregation for Alzheimer's disease therapy.
It is well known that the chiral materials combined with metal ion's structure have been identified as promising candidate for the nursing Alzheimer Disease (AD) treatment, particularly to inhibit amyloid (Aβ) due to their significant pharmacological effect on the living bodies. In the present study, Cu(II)/Chitosan nanocomposite caped with chiral penicillamine (Cu@D-PEN/Chitosan) have been synthesized and used as an effective amyloid-β (Aβ) inhibitor. The composite formations of the samples were confirmed from the FTIR and XRD, studies. FE-SEM, TEM and AFM studies have been carried out to depict the morphological analysis of the nanocomposites. The prepared samples have also been subjected to various studies such as encapsulation efficiency, drug loading capacity, drug release and biodegrading or compatibility of the nanocomposites to support the Aβ aggregation inhibiting ability investigations. It was observed that the increase in the concentration of the Cu@D-PEN/Chitosan enhancing the Aβ inhibiting ability. Thus, the Cu(II)@D-PEN/Chitosan showed improving memory effect suggesting that Cu(II)@D-PEN/Chitosan nanocomposites may be a potential candidate for inhibiting the Aβ aggregation in nursing AD treatment.
PubMed: 38223723
DOI: 10.1016/j.heliyon.2023.e23563 -
Bioorganic Chemistry Mar 2024FK228 is a potent natural pan HDAC inhibitor approved by the FDA for the treatment of cutaneous T-cell lymphoma as well as peripheral T-cell lymphoma. It is generally...
FK228 is a potent natural pan HDAC inhibitor approved by the FDA for the treatment of cutaneous T-cell lymphoma as well as peripheral T-cell lymphoma. It is generally believed that the mechanism of FK228 acting on HDACs is by reducing its disulfide bond after entering the cell, and the dithiol group may chelate with Zn and form a weak reversible covalent bond with cysteine in the catalytic pocket of HDACs, therefore inhibiting the activity of HDACs. However, due to the weak stability of the disulfide bond in FK228, it has been difficult to obtain direct evidence for the above conjecture. Thus, improving the stability of the FK228 disulfide bond will help to explore the exact mechanism of FK228. In this study, based on the stability and target-induced covalent properties of the Cysteine-Penicillamine (Cys-Pen) disulfide bond reported previously, the Pen was introduced into the modification of FK228. Specifically, the d-Cys in FK228 was replaced by d-Pen, the total synthetic pathway was optimized, and the novel synthetic FK228 analogue (FK-P) stability was verified. FK-P can also be used as a new drug molecule in the future to participate in the research of related biological mechanisms or the treatment of diseases.
Topics: Cysteine; Depsipeptides; Histone Deacetylase Inhibitors; Disulfides
PubMed: 38219481
DOI: 10.1016/j.bioorg.2024.107119 -
JPMA. the Journal of the Pakistan... Jan 2024Wilson's disease is arare inherited disorder of copper met abolism. If le f t untre ated, i t can turn into a multi systemic disease with copper deposition in the liver,...
Wilson's disease is arare inherited disorder of copper met abolism. If le f t untre ated, i t can turn into a multi systemic disease with copper deposition in the liver, brain, a nd other tissues. Diagnosi s of Wilson's is delayed in Pak ist an by many ye a rs on average due to va riabl e presen tations. In ad olescents, the initial s igns a re more likely to b e neuropsychiatric. Here we present a case of Wilso n's disease that pre sented initially with he patic symptoms and did not have signs specific to the di sea s e such as Kayser-Fleischer rings. Our case was diagnosed to be Wilson's Disease on ly on further investigat ions and s ubsequently the patient was treated with chela tion therapy using D-Penicillamine.Wilson's Disease should be kept in mind as a differential diagno sis in adolesce nt patients that present with unexplained acute liver failure and cytopenias without any neurological symptoms, as a missed diagnosis can prove to be fatal.
Topics: Male; Humans; Hepatolenticular Degeneration; Copper; Penicillamine; Brain
PubMed: 38219193
DOI: 10.47391/JPMA.9637 -
JAMA Dermatology Feb 2024While several medications are known to induce dermatomyositis (DM), most existing studies are case reports or small case series from a single institution. There is also...
IMPORTANCE
While several medications are known to induce dermatomyositis (DM), most existing studies are case reports or small case series from a single institution. There is also limited information on DM induced by immune checkpoint inhibitors, which are increasingly used in oncologic therapy.
OBJECTIVE
To characterize causes and clinical presentation of drug-induced DM based on the current literature.
EVIDENCE REVIEW
A systematic review was performed in PubMed according to Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) reporting guidelines, from inception to August 22, 2022. Articles meeting preestablished inclusion criteria (written in English and classified as original articles, case reports, literature reviews, and observation letters) were selected and data abstracted. Articles that met the scope of the review were also added from reference lists. When possible, study results were quantitatively combined.
FINDINGS
In 134 studies (114 from the literature search and 20 additional studies pulled from reference lists) describing 165 cases, 88 patients (53.3%) were female, and the median (IQR) age was 61 (49-69) years. Among the cases of drug-induced DM, the most common associated medications were hydroxyurea (50 [30.3%]), immune checkpoint inhibitors (27 [16.4%]), statins (22 [13.3%]), penicillamine (10 [6.1%]), and tumor necrosis factor inhibitors (10 [6.1%]). Histopathologic testing, when undertaken, helped establish the diagnosis. There was a median (IQR) of 60 (21-288) days between drug initiation and drug-induced DM onset. History of cancer was reported in 85 cases (51.6%).
CONCLUSIONS AND RELEVANCE
In this systematic review, drug-induced DM was associated with multiple types of medications, including chemotherapies and immunotherapies. It is essential that dermatologists promptly recognize and diagnose drug-induced DM so that they can guide management to minimize interruption of therapy when possible.
Topics: Humans; Female; Middle Aged; Aged; Male; Dermatomyositis; Immune Checkpoint Inhibitors; Neoplasms; Hydroxyurea
PubMed: 38198130
DOI: 10.1001/jamadermatol.2023.5418