Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 7.251
Filtrar
1.
Anal Chim Acta ; 1184: 339017, 2021 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-34625257

RESUMO

Penicillamine (Pen) is a common chiral drug that is obtained from penicillin. Between the two enantiomers of Pen, only D-Pen can be used to treat cystinuria and rheumatoid arthritis while L-Pen is toxic. Therefore, it requires great efforts for the research of the rigorous analysis and distinction of the two enantiomers. The non-covalent combination of chiral molecules and chiral selectors (CSs) has been proved as a unique strategy for chiral distinction by ion mobility spectrometry in coupling with -mss spectrometry (IM-MS). Here, we developed a simple method to distinguish D, L-Pen by using special CSs for IM-MS separation. The CSs utilized here include cyclodextrins (CD) and linear chain oligosaccharides plus metal ions. We found that non-covalent complexes [Pen+ß-CD + Li]+ could be easily formed by electrospray ionization of the mixture of the solution, and the chirality of Pen could be effectively recognized by measuring their mobilities due to the different collision cross collision sections of [D-Pen+ß-CD + Li]+ and [L-Pen+ß-CD + Li]+. A detailed analysis of [Pen+ß-CD + Li]+ was then conducted by the optical rotation measurements and NMR experiments to reveal their structural differences. Furthermore, DFT calculation showed the differences of molecular conformation between the complexes. The results provide a new powerful method for fast analysis and recognition of chirality of Pen compounds by IM-MS.


Assuntos
Ciclodextrinas , Espectrometria de Mobilidade Iônica , Íons , Espectroscopia de Ressonância Magnética , Espectrometria de Massas , Penicilamina
2.
Phys Chem Chem Phys ; 23(38): 22119-22132, 2021 Oct 06.
Artigo em Inglês | MEDLINE | ID: mdl-34580687

RESUMO

The investigation of the structural characteristics of chiral drugs in physiological environments is a challenging research topic, which may lead to a better understanding of how the drugs work. Raman optical activity (ROA) spectroscopy in combination with density functional theory (DFT) calculations was exploited to inspect the structural changes in penicillamine under different acid-base states in aqueous solutions. The B3LYP/aug-cc-PVDZ method was employed and the implicit solvation model density (SMD) was considered for describing the solvation effect in H2O. The conformations of penicillamine varied with pH, but penicillamine was liable to stabilize in the form of the PC conformation (the sulfur atom is in a trans orientation with respect to carboxylate) in most cases for both D- and L-isomers. The relationship between the conformations of penicillamine and the ROA peaks, as well as peak assignments, were comprehensively studied and elucidated. In the fingerprint region, two ROA couplets and one ROA triplet with different patterns were recognized. The intensity, sign and frequency of the corresponding peaks also changed with varying pH. Deuteration was carried out to identify the vibrational modes, and the ROA peaks of the deuterated amino group in particular are sensitive to change in the ambient environment. The results are expected not only to serve as a reference for the interpretation of the ROA spectra of penicillamine and other chiral drugs with analogous structures but also to evaluate the structural changes of chiral molecules in physiological environments, which will form the basis of further exploration of the effects of structural characteristics on the pharmacological and toxicological properties of chiral drugs.


Assuntos
Teoria da Densidade Funcional , Penicilamina/química , Concentração de Íons de Hidrogênio , Conformação Molecular , Análise Espectral Raman , Estereoisomerismo
3.
Analyst ; 146(17): 5280-5286, 2021 Sep 07.
Artigo em Inglês | MEDLINE | ID: mdl-34342310

RESUMO

Herein, we designed a diversified sensing platform for d-penicillamine based on amino-functionalized Zr-based metal-organic frameworks (UiO-66-NH2 MOFs) and 3-aminophenylboronic acid (APBA)@Alizarin Red (ARS). The boronic acid group of 3-aminophenylboronic acid could react with Alizarin Red to form an APBA@ARS complex with a yellow fluorescence emission at 580 nm and ultraviolet absorption at 435 nm. APBA@ARS can greatly quench the fluorescence of UiO-66-NH2 MOFs at 450 nm via fluorescence resonance energy transfer (FRET). When copper ions were present in the reaction system of APBA and Alizarin Red, the copper ions could complex with Alizarin Red to prevent the generation of APBA@ARS, and the absorption of Cu@ARS at 530 nm occurred. Thus, the absorbance of APBA@ARS at 435 nm declined, restoring the fluorescence of UiO-66-NH2 MOFs. Nevertheless, when d-penicillamine and copper ions coexist in the APBA and Alizarin Red reaction system, the copper ions would complex with the sulfhydryl group of d-penicillamine and no longer hinder the generation of APBA@ARS, and the fluorescence of UiO-66-NH2 MOFs is quenched again. Meanwhile, the absorbance of APBA@ARS at 435 nm enhanced and the absorbance at 530 nm decreased. Thus, a fluorescence and colorimetric dual-signal sensing platform was constructed for d-penicillamine detection, which could detect d-penicillamine in the 1-20 µM and 2-50 µM ranges with the limit of detection (LOD) values of 0.46 µM and 1.38 µM, respectively. Furthermore, this sensing platform could also realize the intelligent RGB detection via mobile phones due to the obvious color change of the reaction system.


Assuntos
Ácidos Borônicos , Penicilamina , Antraquinonas
4.
Expert Opin Drug Saf ; 20(11): 1443-1450, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34259127

RESUMO

BACKGROUND: D-penicillamine (D-pen) is a copper-chelating drug and has immune-modulatory properties. D-pen is used to treat rheumatoid arthritis, Wilson's disease, and kidney stones (cystinuria). However, associated adverse events (AEs) of D-pen treatment are frequent and often serious. Therefore, a comprehensive assessment of the safety profile of D-pen is urgently needed. RESEARCH DESIGN AND METHODS: We identified and analyzed AEs associated with D-pen between April-1970 to July-2020 from the U.S. Food and Drug Administration Adverse Event Reporting System (FAERS) databases and calculated the reported odds ratio (ROR) with 95% confidence intervals (CI) using the disproportionality analysis. RESULTS: A total of 9,150,234 AEs related to drugs were reported in the FAERS database, of which 542 were related to D-Pen. We report that D-pen was associated with dystonia (ROR: 20.52; 95%CI: 12.46-33.80), drug hypersensitivity (ROR: 5.42; 95%CI: 3.72-7.90), pancytopenia (ROR: 10.20; 95%CI: 5.61-18.56), joint swelling (ROR: 9.07; 95%CI: 5.51-14.94), renal-impairment (ROR: 6.68; 95%CI: 3.67-12.15), dysphagia (ROR: 5.05; 95%CI: 2.76-8.89), aggravation of condition (ROR: 4.16; 95%CI: 2.60-6.67), congestive cardiac failure (ROR: 4.04; 95%CI: 2.22-7.35), peripheral edema (ROR: 3.77; 95%CI: 2.17-6.55), tremor (ROR: 3.46; 95%CI: 2.00-6.01), pyrexia (ROR: 3.46; 95%CI: 2.00-6.01), and gait disturbance (ROR: 2.41; 95%CI: 1.29-4.52). CONCLUSIONS: Patients taking D-pen require close monitoring of renal function, blood counts, immunity, liver, cardiac function, and neurological function. D-pen suppresses immune system which maximizes the risk of infection.


Assuntos
Sistemas de Notificação de Reações Adversas a Medicamentos/estatística & dados numéricos , Quelantes/efeitos adversos , Penicilamina/efeitos adversos , Adolescente , Adulto , Idoso , Bases de Dados Factuais , Monitoramento de Medicamentos/métodos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Farmacovigilância , Estudos Retrospectivos , Estados Unidos , United States Food and Drug Administration , Adulto Jovem
5.
J Pediatr Gastroenterol Nutr ; 73(4): e80-e86, 2021 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-34091542

RESUMO

OBJECTIVES: To describe a cohort of Wilson disease (WD) pediatric cases, and to point out the diagnostic particularities of this age group and the long-term outcome. METHODS: Clinical data of 182 pediatric patients included in the French WD national registry from 01/03/1995 to 01/06/2019 were gathered. RESULTS: Diagnosis of WD was made at a mean age of 10.7 ±â€Š4.2 years (range 1-18 years). At diagnosis, 154 patients (84.6%) had hepatic manifestations, 19 (10.4%) had neurological manifestations, and 9 patients (4.9%) were asymptomatic. The p.His1069Gln mutation was the most frequently encountered (14% of patients).Neurological patients were diagnosed at least 1 year after they presented their first symptoms. At diagnosis, the median urinary copper excretion (UCE) was 4.2 µmol/24 hours (0.2-253). The first-line treatment was d-penicillamine (DP) for 131 (72%) patients, zinc salts for 24 (13%) patients, and Trientine for 17 (9%) patients. Liver transplantation was performed in 39 (21.4%) patients, for hepatic indications in 33 of 39 patients or for neurological deterioration in 6 of 39 patients, mean Unified Wilson's Disease Rating Scale of the latter went from 90 ±â€Š23.1 before liver transplantation (LT) to 26.8 ±â€Š14.1 (P < 0.01) after a mean follow-up of 4.3 ±â€Š2.5 years. Overall survival rate at 20 years of follow-up was 98%, patient and transplant-free combined survival was 84% at 20 years. CONCLUSION: Diagnosis of WD can be challenging in children, particularly at the early stages of liver disease and in case of neurological presentation; hence the support of clinical scores and genetic testing is essential. Diagnosis at early stages and proper treatment ensure excellent outcomes, subject to good long-term treatment compliance. LT is a valid option for end-stage liver disease not responding to treatment and can be discussed for selected cases of neurological deterioration.


Assuntos
Degeneração Hepatolenticular , Adolescente , Criança , Pré-Escolar , Cobre , França/epidemiologia , Degeneração Hepatolenticular/diagnóstico , Degeneração Hepatolenticular/genética , Degeneração Hepatolenticular/terapia , Humanos , Lactente , Penicilamina/uso terapêutico , Estudos Retrospectivos , Resultado do Tratamento
6.
Mymensingh Med J ; 30(2): 559-561, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33830143

RESUMO

Hepatitis A virus (HAV) is the most common cause of acute viral hepatitis in the world. Infection with hepatitis A virus can cause severe or even fatal illness in patients with chronic liver disease. Here we present a case which seems to be an isolated acute viral hepatitis A infection at the beginning but later found to be coexisted with Wilson's disease. A 14-year-old girl presented in the Department of Gastroenterology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh on 11th April 2019 with progressive jaundice with prodrome, dark urine, itching, hepatomegaly and thyromegaly. She was found positive for serum IgM HAV antibody. Her jaundice was increasing along with prolonged prothrombin time and low albumin. She had coexisting Wilson's disease evidenced by increased 24 hours urinary copper (138µgm/day). She was treated with D-Penicillamine and Zinc acetate. Hepatitis A can be considered as a factor for acute decompensation in undiagnosed patients with Wilson's disease. So it is very crucial to investigate Wilson's disease in appropriate clinical setting of prolonged jaundice and liver dysfunction.


Assuntos
Vírus da Hepatite A , Hepatite A , Degeneração Hepatolenticular , Icterícia , Adolescente , Bangladesh , Cobre , Feminino , Hepatite A/complicações , Hepatite A/diagnóstico , Degeneração Hepatolenticular/complicações , Degeneração Hepatolenticular/diagnóstico , Degeneração Hepatolenticular/tratamento farmacológico , Humanos , Icterícia/diagnóstico , Icterícia/etiologia , Penicilamina
7.
Nephrol Ther ; 17S: S100-S107, 2021 Apr.
Artigo em Francês | MEDLINE | ID: mdl-33910689

RESUMO

Cystinuria is the most common monogenic nephrolithiasis disorder. Because of its poor solubility at a typical urine pH of less than 7, cystine excretion results in recurrent urinary cystine stone formation. A high prevalence of high blood pressure and of chronic kidney disease has been reported in these patients. Alkaline hyperdiuresis remains the cornerstone of the preventive medical treatment. To reach a urine pH between 7.5 and 8 and a urine specific gravity less than or equal to 1.005 should be the goal of medical treatment. D-penicillamine and tiopronin, two cysteine-binding thiol agents, should be considered as second line treatments with frequent adverse events that should be closely monitored.


Assuntos
Cistinúria , Cálculos Renais , Cistina , Cistinúria/diagnóstico , Cistinúria/epidemiologia , Cistinúria/terapia , Humanos , Penicilamina , Tiopronina
8.
Reprod Domest Anim ; 56(6): 864-871, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33715248

RESUMO

The study investigated the effects of sperm sorting, capacitation treatment and co-cultivation on sexed bovine in vitro embryo production. The effect of treatment and co-culture on production of embryos of the preferred sex from unsorted sperm was also studied. Sperm from five breeding bulls was used for fertilization of mature oocytes as follows: Experiment 1, sorted and unsorted sperm (bulls A-E) treated only with heparin in standard co-cultures; Experiment 2, sorted sperm (bulls A-E) treated with heparin-PHE (penicillamine, hypotaurine, and epinephrine) or heparin-caffeine in drop co-cultures; and Experiment 3, unsorted sperm (bull E) treated with either heparin-PHE or heparin-caffeine in both standard and drop co-cultures. In all bulls, treatment with heparin resulted in significantly (p < .05) reduced cleavage and blastocyst rates from sorted sperm, as compared with those from unsorted sperm. In bulls A, B, D and E, treatment of sorted sperm with heparin-PHE in drops significantly increased the blastocyst rate (p < .05). In unsorted sperm of bull E, heparin-PHE treatment in drops resulted in the XX/XY sex ratio inverse to that obtained by heparin-caffeine treatment in standard co-cultures (32.3%/67.7% and 66.7%/33.3%, respectively). In conclusion, the treatment of sorted sperm with heparin-PHE in modified drop co-cultures can be recommended for production of in vitro sexed embryos. The use of unsorted sperm for production of embryos of the preferred sex by selected capacitation treatment and co-culture can be the method of choice in bulls with low IVF yields from sorted sperm.


Assuntos
Técnicas de Cocultura/veterinária , Pré-Seleção do Sexo/veterinária , Espermatozoides/efeitos dos fármacos , Animais , Bovinos , Técnicas de Cocultura/métodos , Técnicas de Cultura Embrionária/veterinária , Epinefrina/farmacologia , Feminino , Fertilização In Vitro/métodos , Fertilização In Vitro/veterinária , Heparina/farmacologia , Masculino , Oócitos , Penicilamina/farmacologia , Pré-Seleção do Sexo/métodos , Taurina/análogos & derivados , Taurina/farmacologia
9.
Zhonghua Gan Zang Bing Za Zhi ; 29(1): 79-82, 2021 Jan 20.
Artigo em Chinês | MEDLINE | ID: mdl-33541028

RESUMO

Wilson Disease is kind of an autosomal recessive genetic disease. Early diagnosis and timely treatment are very important for prognosis. This article reviews the treatment of Wilson Disease, focusing on penicillamine, sodium dimercaptopropane sulfonate, ammonium tetrathiomolybdate and zinc, liver transplantation and gene therapy. At the same time, the problems of medication adherence and follow-up evaluation in patients with Wilson Disease are also discussed.


Assuntos
Degeneração Hepatolenticular , Transplante de Fígado , Terapia Genética , Degeneração Hepatolenticular/tratamento farmacológico , Degeneração Hepatolenticular/genética , Humanos , Penicilamina/uso terapêutico , Zinco
10.
Spectrochim Acta A Mol Biomol Spectrosc ; 253: 119584, 2021 May 15.
Artigo em Inglês | MEDLINE | ID: mdl-33636492

RESUMO

Herein, fluorescent DNA-templated silver nanoclusters (DNA-AgNCs) with red emission were synthesized and utilized as novel probe to detect D-penicillamine (D-Pen) for the first time. D-Pen molecules contain a thiol which can combine with Ag to form a non-fluorescent ground state complex, inducing the aggregation of DNA-AgNCs followed by the fluorescence quenching. The quenching mechanism is well-studied and found to be a static quenching process. This method can detect D-Pen in the range of 0.025-0.7 µM with the detection limit as low as 8 nM, which is 1-3 orders of magnitude more sensitive than those based on other fluorescent nanoprobes. More importantly, the preparation procedure for DNA-AgNCs is fast and without the requirement of heavy metal ions. Thus, this detection strategy is time-saving and eco-friendly. Satisfactory recoveries have been acquired for monitoring D-Pen in human serum samples and pharmaceutical samples owing to the high sensitivity.


Assuntos
Nanopartículas Metálicas , Prata , DNA , Corantes Fluorescentes , Humanos , Limite de Detecção , Penicilamina , Espectrometria de Fluorescência
11.
J Hazard Mater ; 412: 125249, 2021 06 15.
Artigo em Inglês | MEDLINE | ID: mdl-33548789

RESUMO

In this work, we innovatively synthesized homochiral fluorescence nano molecularly imprinted polymers (D-MIP) with dual affinity (metal ion affinity and homochiral affinity) for the specific separation and detection of L-penicillamine (L-PA), which is a core-shell structure with a SiO2-covered CDs core and an imprinted layer with L-PA cavities. A switch for fluorescence response was built by chelating grafted Cu2+, what's more, the imprinted L-PA was pre immobilized by Cu2+ to form the directional imprinting with predetermined spatial structure. More importantly, the homochiral affinity of D-galactose in homochiral molecularly imprinted polymers (D-MIP) greatly enhanced the selective adsorption of L-PA, and D-MIP showed a high selectivity factor (α) of 3.45, which is 1.9 times that of the non-homochiral molecularly imprinted polymers (MIP). Meanwhile, D-MIP exhibited a high enantiomeric excess (ee) value of 56% for separation of racemic PA. Additionally, a high sensitive and selective method was established for the detection of L-PA.


Assuntos
Impressão Molecular , Adsorção , Polímeros Molecularmente Impressos , Penicilamina , Dióxido de Silício
12.
Clin Anat ; 34(5): 766-773, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-33580904

RESUMO

Dyshomeostasis of trace elements have been implicated in the progression of Alzheimer's disease (AD), which is characterized by amyloid-ß (Aß) plaques. Trace elements are particularly associated with the Aß plaques. Metal-protein attenuating compounds have been developed to inhibit metals from binding to Aß proteins, which result in Aß termination, in the hope of improving cognitive functioning. However, there are still some contradicting reports. This review aims to first establish which trace elements are increased or decreased in the brains of Alzheimer's patients, and secondly, to review the effectiveness of clinical trials with metal-protein attenuating compounds for AD. Studies have consistently reported unchanged or increased iron, contradicting reports for zinc, decreased copper, unchanged or decreased manganese, inconsistent results for calcium, and magnesium seems to be unaffected. However, varied results have been reported for all trace elements. Clinical trials using metal-protein attenuating compounds to treat AD have also reported varied results. Copper chelators have repeatedly been used in clinical trials, even though few studies report increased brain copper levels in AD patients. Homeostasis of copper levels is important since copper has a vital role in several enzymes, such as cytochrome c, Cu/Zn superoxide dismutase and ceruloplasmin. Dyshomeostasis of copper levels can lead to increased oxidative stress and neuronal loss. Future studies should assess a variety of trace element levels in moderately and severely affected AD patients since there are contradicting reports. This review thus provides some insight into trace element alterations in the brains of individuals with AD.


Assuntos
Doença de Alzheimer/tratamento farmacológico , Doença de Alzheimer/metabolismo , Metais/metabolismo , Oligoelementos/metabolismo , Clioquinol/uso terapêutico , Cobre/uso terapêutico , Desferroxamina/uso terapêutico , Humanos , Penicilamina , Sideróforos/uso terapêutico
13.
BMJ Case Rep ; 14(1)2021 Jan 20.
Artigo em Inglês | MEDLINE | ID: mdl-33472809

RESUMO

Acute abdominal pain is a common presentation to the emergency department (ED). Ruling out life-threatening causes and giving pain relief are the most important tasks in ED. We describe a 32-year-old man who presented to ED with abdominal pain and vomiting which was unrelieved by usual doses of analgesic. Extensive investigations revealed no significant abnormalities. On further probing, he admitted taking traditional medications for infertility. The toxicological panel revealed a high blood lead level, leading to a diagnosis of acute lead toxicity. Chelation therapy with D-penicillamine was initiated and the patient's abdominal pain resolved within 4 days.


Assuntos
Dor Abdominal/diagnóstico , Medicamentos Falsificados/efeitos adversos , Intoxicação por Chumbo/diagnóstico , Charlatanismo , Vômito/diagnóstico , Dor Abdominal/tratamento farmacológico , Dor Abdominal/etiologia , Doença Aguda , Adulto , Anemia/etiologia , Antieméticos/uso terapêutico , Quelantes/uso terapêutico , Clordiazepóxido/uso terapêutico , Antagonistas Colinérgicos , Constipação Intestinal/etiologia , Medicamentos Falsificados/química , Combinação de Medicamentos , Serviço Hospitalar de Emergência , Humanos , Intoxicação por Chumbo/complicações , Intoxicação por Chumbo/tratamento farmacológico , Masculino , Parassimpatolíticos/uso terapêutico , Penicilamina/uso terapêutico , Fenetilaminas/uso terapêutico , Quinuclidinas/uso terapêutico , Tomografia Computadorizada por Raios X , Vômito/tratamento farmacológico , Vômito/etiologia
14.
ACS Biomater Sci Eng ; 7(2): 517-526, 2021 02 08.
Artigo em Inglês | MEDLINE | ID: mdl-33397083

RESUMO

Bacterial infections have been increasingly recognized as the major reason for the failure of tissue engineering scaffolds. Therefore, there is a need for novel and multifunctional biomaterials that not only enhance tissue regeneration but also can combat infections. An antibacterial and bioactive scaffold was fabricated in this study by incorporation of honey and a nitric oxide (NO) donor, S-nitroso-N-acetyl-penicillamine (SNAP), into polylactic acid (PLA) nanofibers using a single-jet electrospinning method. The morphology of the prepared nanofibers was observed using a scanning electron microscope. PLA/honey/SNAP (PLA/HN/SNAP) nanofibers had an average diameter of 624.92 ± 137.69 nm and showed a sustained release of NO for 48 h. The scaffolds were characterized for their chemical composition via Fourier-transform infrared spectroscopy. Moreover, the tensile properties of nanofibers along with their wettability, water retention ability, and water vapor transmission rate were evaluated. The results of antibacterial studies revealed that the synergistic combination of honey and SNAP significantly reduced the viability of Gram positive Staphylococcus aureus and Gram negative Escherichia coli. In addition, qualitative and quantitative 3T3 fibroblast cell culturing experiments proved that the PLA/HN/SNAP scaffolds supported better cell attachment and proliferation compared to PLA. The promising results obtained in this study indicate that PLA/HN/SNAP nanofibrous scaffolds have great potential for tissue engineering applications.


Assuntos
Mel , Nanofibras , Animais , Antibacterianos/farmacologia , Camundongos , Penicilamina , Tecidos Suporte
15.
Int Immunopharmacol ; 92: 107299, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33418246

RESUMO

Pemphigus encompasses a rare heterogeneous group of autoimmune blistering diseases characterized by cutaneous and/or mucosal blistering. Multiple factors, such as some specific types of drugs, have been found to be involved in the induction of pemphigus. Here, we have designed a systematic review by searching PubMed/Medline and Embase databases to find the drugs, involved in pemphigus induction and exacerbation (updated on 19 August 2019). From 1856 initially found articles, 134 studies (198 patients; 170 patients in the drug-induced patients and 28 in exacerbation group) have been included. Regarding drug-induced cases, the mean age was 57.19 ± 16.9-year-old (ranged 8-105), and patients had developed pemphigus within a mean of 154.27 days. Pemphigus vulgaris (38.9%), pemphigus foliaceus (33.5%), and paraneoplastic pemphigus (3.6%) were the most common subtypes. Furthermore, penicillamine (33.1%), captopril (7.7%), and bucillamine (6.5%) were the most reported drugs related to pemphigus induction; penicillamine was associated with the most persistent disease. Regardless of disease subtype, cutaneous, mucocutaneous, and mucosal involvements were reported in 68.6%, 30.1%, and 1.3% of patients, respectively. In total, the IgG deposition in the pathological studies, being positive for autoreactive antibodies in the serum against desmoglein 3 (Dsg3), and desmoglein 1 (Dsg1), were reported in 93%, 34.9%, and 72.7% of reported patients, respectively. Regarding the management of such patients, in 75% of healed cases, treatment (mainly transient systemic and topical corticosteroids and/or azathioprine) was needed besides stopping the probable pemphigus-inducing culprit drug, while drug cessation was enough to control the disease in 25%. As the outcomes, the lesions in 129 of 147 (87.8%) patients had been healed, while in 18 (12.2%), no healing was reported; fifteen out of 18 had died. In conclusion, some specific groups of treatments can induce pemphigus, including penicillamine, captopril, and bucillamine; despite the similar clinical and pathological manifestations to classical pemphigus, most of the cases are less severe and have a better prognosis.


Assuntos
Captopril/efeitos adversos , Cisteína/análogos & derivados , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/patologia , Pênfigo/patologia , Penicilamina/efeitos adversos , Anti-Inflamatórios não Esteroides/efeitos adversos , Anti-Hipertensivos/efeitos adversos , Antirreumáticos/efeitos adversos , Cisteína/efeitos adversos , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/etiologia , Humanos , Pênfigo/induzido quimicamente , Pênfigo/imunologia
16.
Neurol Sci ; 42(9): 3829-3834, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-33474589

RESUMO

Wilson's disease (WD) is an autosomal recessive genetic disorder of copper metabolism, and WD patients can present with neurologic symptoms. We aimed to report the general characteristics and prognosis of a Turkish series of WD patients with neurological manifestations. A total of 12,352 patients were screened from the patient database, and 53 WD patients were included. Patients were classified based on the predominant neurological syndrome type including tremor, dystonia, parkinsonism, or discrete neurological signs and were classified as having "good outcome," "stable," and "poor outcome" according to their treatment response. There were 32 male and 21 female patients, aged 20-66 years. The mean follow-up was 11.3 ± 4.56 years. Sixty-two percent of patients presented predominantly with neurological symptoms. Neurological WD diagnosis was established after a mean time delay of 2.3 years from the WD diagnosis. The most common neurological manifestation was dystonia, followed by tremor and parkinsonism. Fifteen patients had a family history of WD. Consanguinity was present in 20 patients. Patients were treated with D-penicillamine, trientine, zinc salts, or their combinations. Besides the main treatments, 41 patients were on symptomatic treatment for neurologic symptoms. Thirty-six patients had a "good outcome," five patients were stable, and six patients had "poor outcome." Post-chelation neurological worsening was observed in 11 patients. WD should be considered in differential diagnosis in any patient with unexplained neurologic symptoms. Early diagnosis is important, and appropriate treatment should be promptly initiated to prevent progressive and irreversible damage, with good prognosis and stable disease in the majority of the patients with treatment compliance.


Assuntos
Distonia , Degeneração Hepatolenticular , Cobre , Distonia/diagnóstico , Distonia/epidemiologia , Distonia/etiologia , Feminino , Degeneração Hepatolenticular/diagnóstico , Degeneração Hepatolenticular/tratamento farmacológico , Degeneração Hepatolenticular/epidemiologia , Humanos , Masculino , Penicilamina/uso terapêutico , Tremor/diagnóstico , Tremor/epidemiologia , Tremor/etiologia
18.
Clin Rheumatol ; 40(6): 2485-2490, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33057918

RESUMO

Although lupus induced by penicillamine, the first-line medication for Wilson's disease, is well-documented, primary systematic lupus erythematosus (SLE) co-occurring with Wilson's disease has only rarely been reported. Symptom overlap can add to difficulties in making the correct and complete diagnosis of these two systemic diseases. An 18-year-old female was diagnosed with simultaneous Wilson's disease and SLE and was successfully treated with hydroxychloroquine and oral zinc. We also reviewed the literature for cases of Wilson's disease co-occurring with SLE not induced by penicillamine and found six other cases. Clinical presentations, diagnoses, treatments, and outcomes were analyzed and summarized to expand our understanding of this rare condition. The most frequent diagnostic clues to Wilson's disease in patients with SLE included unexplained liver damage despite well-controlled SLE, extrapyramidal symptoms and signs, hyper-intense signals of the basal ganglia bilaterally on T2-weighted and fluid-attenuated inversion recovery (FLAIR) MRI images, and Kayser-Fleischer (K-F) rings on physical examination. Penicillamine should be avoided or used cautiously in Wilson's disease patients complicated by SLE. The overall prognosis is good if treated in a timely manner. Key Points • SLE complicated by Wilson's disease or the co-occurrence of the two conditions in the absence of penicillamine may exist in rare conditions. • The diagnostic clues for identifying Wilson's disease in SLE patients may include unexplained liver damage despite well-controlled SLE, extrapyramidal symptoms and signs, and K-F rings found by physical examination. • Penicillamine should be avoided or used cautiously in Wilson's patients with SLE.


Assuntos
Degeneração Hepatolenticular , Lúpus Eritematoso Sistêmico , Adolescente , Cobre , Feminino , Degeneração Hepatolenticular/complicações , Degeneração Hepatolenticular/tratamento farmacológico , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Imageamento por Ressonância Magnética , Penicilamina/efeitos adversos
19.
Talanta ; 224: 121886, 2021 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-33379095

RESUMO

D-penicillamine (D-PA) plays an important role in medical and clinical treatment of some diseases. In this work, we designed a sensitive fluorometric and colorimetric dual-mode assay on the basis of fluorescent silicon quantum dots (SiQDs) and 5,5-dithiobis-(2-nitrobenzoic acid) (DTNB) to effectively detect D-PA. The sulfydryl group (-SH) of D-PA can react with DTNB to generate 5-thio-2-nitrobenzoate (TNB), which has a broad absorption peak centered at 407 nm and is capable to absorb excitation light of SiQDs and greatly quench the fluorescence of SiQDs on account of inner filter effect (IFE). Meanwhile, the color of the detection system obviously turned yellow. Consequently, the quantitative determination of D-PA can be achieved through both fluorometric and colorimetric methods. The fluorometric and colorimetric sensing platform can detect D-PA in 1-20 µM and 2-20 µM concentration range, respectively, and the limits of detection (LOD) were 0.48 µM and 0.68 µM, accordingly. Furthermore, the designed sensing platform was utilized to detect D-PA in real biological samples and the experimental results were satisfactory, suggesting the feasibility and potential applications of the sensing platform.


Assuntos
Pontos Quânticos , Colorimetria , Corantes Fluorescentes , Penicilamina , Silício
20.
Spectrochim Acta A Mol Biomol Spectrosc ; 248: 119277, 2021 Mar 05.
Artigo em Inglês | MEDLINE | ID: mdl-33310611

RESUMO

The identification of chiral penicillamine (Pen) is of great significance for clinical medication safety. The host-guest systems formed by enantiomers and macromolecule can be applied to differentiate the chiral drugs and enable the drug delayed release. We hereby performed the dispersion corrected density functional theory (DFT-D) calculation on the complex formed by ß-cyclodextrin(ß-CD) and D/L-penicillamine (D/L-Pen). The diverse encapsulation configurations with different interaction energy show that both D-Pen and L-Pen tend to longitudinally embedded into the narrow aperture of ß-CD with the front part of the sulfur group and the methyl group, and the interaction energy between L-Pen and ß-CD is 5.47 kJ/mol(M062XD3) lower than that between D-Pen and ß-CD. Based on the computed vibration frequency of host, guest, and the most stable complex, it is found that the featured peaks attributed to the vibration of the carboxyl group of guest and the skeleton vibration of complex are the most significant spectral standard to distinguish the ß-CD-D/L-Pen and ß-CD. Moreover, the peaks resulted from the skeleton vibration in terahertz spectra can be also used to distinguish the complex of ß-CD with chiral Pen. Through the topological analysis and the Independent Gradient Model (IGM) analysis, the O-H…O hydrogen bond in ß-CD-D-Pen is stronger than that in ß-CD-L-Pen, and the van der Waals interactions such as C-H…O,C-H…N,C-H…S, O…S and C-H…C-H have the most contributions to the intermolecular interaction in ß-CD-D/L-Pen. It is also noted that the H(-OH) in D-Pen and S in L-Pen contribute the most to the intermolecular interaction with ß-CD in comparison with other atoms in Pen.


Assuntos
Penicilamina , beta-Ciclodextrinas , Ligação de Hidrogênio , Estereoisomerismo , Vibração
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...