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Transient d-penicillamine-Induced Nephrogenic diabetes Insipidus during Treatment of a Patient with Cystinuria —d-penicillamine-Induced Nephrogenic diabetes Insipidus

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Open Journal of Nephrology 2023年第4期13卷 378-384页

作者： Kamel El-Reshaid Shaikha Al-Bader department of Medicine Faculty of Medicine Kuwait University Kuwait City Kuwait department of Medicine Nephrology Unit Amiri Hospital Ministry of Health Kuwait City Kuwait

Background: diabetes insipidus (dI) is a rare disorder characterized by inappropriate polyuria and hypo-osmolar urine. It is caused by inadequate production of antidiuretic hormone, in response to hypothalamic osmoreceptor-stimulation, from the pituitary gland (central dI) or resistance to its action at terminal distal convoluted tubules and collecting ducts (nephrogenic dI). Most cases of nephrogenic dI are caused by drugs, especially chronic lithium use. The Case: A 46-year-old man manifested such a disorder 8 months following d-penicillamine (d-P) therapy for cystinuria. The drug was discontinued and the patient was managed conservatively with high fluid intake, diet low in protein and salt as well as alkalization of urine with Urolyte U to a pH > 7.5. Six weeks later, such side effect disappeared. Our patient had developed such phenomenon: a) without significant liver or renal disease to account for cumulative toxicity, and b) with a conventional dosage range of d-P. Such isolated toxicity indicates inherited a predisposition to this side effect. Conclusion: dI is a potential side effect of d-P therapy that is nephrogenic in site, transient in prognosis and an isolated phenomenon likely to reflect genetic predisposition.

关键词： diabetes Insipidus d-penicillamine Cystinuria Genetic Side Effect

Wilson's disease in two siblings from Ecuador:Two case reports

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World Journal of Clinical Cases 2025年第3期13卷 50-60页

作者： Enrique Carrera Jonathan Alvarado Martina Astudillo Galo Pillajo department of Gastroenterology and Hepatology Hospital de Especialidades Eugenio EspejoQuito 170136PichinchaEcuador Faculty of Medicine Pontificia Universidad Catolica del EcuadorQuito 170143PichinchaEcuador department of Radiology Hospital de Especialidades Eugenio EspejoQuito 170136PichinchaEcuador

BACKGROUNd Wilson's disease(Wd)is a rare metabolic disorder of copper accumulation in organs such as liver,brain,and *** and treatments are challenging in settings,where advanced diagnostic tests are unavailable,copper chelating agents are frequently scarce,healthcare professionals lack disease awareness,and medical follow-ups are *** diagnoses and treatments help prevent complications,improve patients’quality of life,and ensure a normal life *** clinical presentations and outcomes of Wd can vary within a single *** SUMMARY We present the cases of two siblings(19 and 27 years)from a consanguineous family in rural Ecuador,diagnosed as having Wd during a family *** male patient,diagnosed at age 19 after his brother’s death from acute liver failure,presented with compensated cirrhosis,neurological symptoms,and bilateral Kayser-Fleischer *** developed progressive neurological deterioration during an irregular treatment with d-penicillamine due to medication *** condition improved upon switching to trientine tetrahydrochloride,and his neurological symptoms improved over an 8-year period of *** female patient,diagnosed at age 10,exhibited only biochemical *** treatment history was similar;however,she remained asymptomatic without disease progression over the same follow-up *** discuss the potential influence of epigenetic mechanisms and modifier genes on the various phenotypes,emphasizing the need for research in these areas to optimize therapeutic *** Our patients’medical histories show how early diagnosis and treatment can prevent disease progression;and,how suboptimal treatments impact disease outcomes.

关键词： Wilson disease Kayser-Fleischer rings Copper chelator d-penicillamine Trientine tetrahydrochloride Case report

Kinetically controlled synthesis of atomically precise Ag nanoclusters for the catalytic reduction of 4-nitrophenol

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International Journal of Minerals,Metallurgy and Materials 2021年第10期28卷 1716-1725页

作者： Xian-hu Liu Fei-hong Wang Cong-ying Shao Gang-feng du Bing-qing Yao department of Chemistry Huaibei Normal UniversityHuaibei 235000China Henan Key Laboratory of High-temperature Structural and Functional Materials National Joint Engineering Research Center for Abrasion Control and Molding of Metal MaterialsHenan University of Science and TechnologyLuoyang 471003China department of Public Teaching Nanyang Medical CollegeNanyang 473061China School of Materials Science and Engineering Nanyang Technological UniversitySingapore 639798Singapore

Synthesizing atomically precise Ag nanoclusters(NCs),which is essential for the general development of NCs,is quite *** this study,we report the synthesis of high-purity atomically precise Ag NCs via a kinetically controlled *** Ag NCs were prepared using a mild reducing agent via a one-pot *** as-prepared Ag NCs were confirmed to be Ag_(49)(d-pen)_(24)(d-pen:d-penicillamine)on the basis of their matrix-assisted laser desorption ionization time-of-flight mass spectrometric and thermogravimetric *** interfacial structures of the Ag NCs were illustrated by proton nuclear magnetic resonance and Fourier-transform infrared *** Ag NCs were supported on activated carbon(AC)to form Ag NCs/AC,which displayed excellent activity for the catalytic reduction of 4-nitrophenol with a kinetic reaction rate constant k of 0.21 min^(-1).Such a high k value indicates that the composite could outperform several previously reported ***,the catalytic activity of Ag NCs/AC remained nearly constant after six times of recycle,which suggests its excellent stability.

关键词： Ag nanoclusters d-penicillamine sodium cyanoborohydride catalysis 4-nitrophenol

Pediatric Wilson disease presenting as acute liver failure:Prognostic indices

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World Journal of Clinical Cases 2021年第14期9卷 3273-3286页

作者： Wei-Yuan Fang Kuerbanjiang Abuduxikuer Peng Shi Yi-Ling Qiu Jing Zhao Yu-Chuan Li Xue-Yuan Zhang Neng-Li Wang Xin-Bao Xie Yi Lu A S Knisely Jian-She Wang Center for Pediatric Liver diseases Children’s Hospital of Fudan UniversityShanghai 201102China Medical Statistics department Children’s Hospital of Fudan UniversityShanghai 201102China Institut für Pathologie Medizinische Universität GrazGraz 8010Austria

BACKGROUNd Acute liver failure(ALF)can be a primary presentation of Wilson disease(Wd).Mortality rates are high in Wd with ALF(WdALF).Predictions of mortality in WdALF vary by model and are sometimes contradictory,perhaps because few patients are studied or Wd diagnoses are *** To determine the outcomes among well-documented WdALF patients and assess mortality model performance in this *** We reviewed the medical records of our pediatric WdALF patients(n=41 over 6-years-old,single-center retrospective study)and compared seven prognostic models(King’s College Hospital Criteria,model for end-stage liver disease/pediatric end-stage liver disease scoring systems,Liver Injury Unit[LIU]using prothrombin time[PT]or international normalized ratio[INR],admission LIU using PT or INR,and devarbhavi model)with one *** Among the 41 Han Chinese patients with ALF,Wd was established by demonstrating ATP7B variants in *** 5 others,Kayser-Fleischer rings and Coombs-negative hemolytic anemia permitted *** died during hospitalization and three underwent liver transplantation(LT)within 1 mo of presentation and survived(7.3%each);35(85.4%)survived without LT when given enteral d-penicillamine and zinc-salt therapy with or without urgent *** significantly correlated with mortality included encephalopathy,coagulopathy,and gamma-glutamyl transpeptidase activity,bilirubin,ammonia,and serum sodium *** under the receiver operating curves varied among seven prognostic models from 0.981 to 0.748 with positive predictive values from 0.214 to *** WdALF children can survive and recover without LT when given d-penicillamine and Zn with or without plasmapheresis,even after enlisting for LT.

关键词： Acute liver failure ATP7B d-penicillamine Liver transplant Wilson disease Zinc

Quantum Chemical Studies of Host-guest Nanostructures of PAMAM dendrimers in drug deliver

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Chinese Journal of Structural Chemistry 2020年第5期39卷 838-848页

作者： HAGHIGHI Fatemeh MORSALI Ali BOZORGMEHR Mohammad Reza BEYRAMABAdI SAli department of Chemistry Mashhad BranchIslamic Azad UniversityMashhadIran Research Center for Animal development Applied Biology Mashhad BranchIslamic Azad UniversityMashhad 917568Iran

Using B3LYP and M06-2X functionals,eight noncovalent configurations for the adsorption of d-penicillamine drug(dPA)drug on poly(amidoamine)G0 generation dendrimer(PAMAMG0)carrier have been *** quantum molecular descriptors and the binding and solvation energies were examined in aqueous solution and gas *** binding energies demonstrated the energetic stability of non-bonded species(PAMAMG0/dPA1-8).The solvation energies showed that solubility of dPA rises in the vicinity of PAMAMG0 carrier which is a fundamental factor for applicability of a *** quantum molecular descriptors such as electrophilicity power and global hardness,the toxicity of dPA drug in the vicinity of PAMAMG0 carrier decreases and drug release is *** AIM analysis for all PAMAMG0/dPA1-8 structures indicated that the hydrogen and pseudo-hydrogen bonds play important roles in the functionalization of PAMAMG0 with dPA *** configuration in which dPA drug interacts simultaneously with two-NH2 functional groups of PAMAMG0 is the most stable configuration.

关键词： dFT d-penicillamine hydrogen bonding nanomedicine poly(amidoamine)dendrimer

Practical insights into chronic management of hepatic Wilson’s disease

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World Journal of Clinical Cases 2022年第14期10卷 4334-4347页

作者： Erica Nicola Lynch Claudia Campani Tommaso Innocenti Gabriele dragoni Paolo Forte Andrea Galli Gastroenterology Research Unit Department of Experimental and Clinical Biomedical Sciences“Mario Serio”University of FlorenceFlorence 50134Italy department of Experimental and Clinical Medicine University of FlorenceFlorence 50134Italy department of Medical Biotechnologies University of SienaSiena 53100Italy division of Gastroenterology University Hospital“Careggi”Florence 50134Italy

Wilson’s disease(Wd)is a rare inherited disorder of human copper metabolism,with an estimated prevalence of 1:30000-1:50000 and a broad spectrum of hepatic and neuropsychiatric *** healthy individuals,the bile is the main route of elimination of *** Wd patients,copper accumulates in the liver,it is released into the bloodstream,and is excreted in *** can also be accumulated in the brain,kidneys,heart,and osseous matter and causes damage due to direct toxicity or oxidative *** Wd is commonly but not exclusively diagnosed in childhood or young ***,non-cirrhotic Wd patients seem to have a normal life ***,chronic management of patients with Wilson’s disease is challenging,as available biochemical tests have many limitations and do not allow a clear identification of non-compliance,overtreatment,or treatment *** provide optimal care,clinicians should have a complete understanding of these limitations and counterbalance them with a thorough clinical *** aim of this review is to provide clinicians with practical tools and suggestions which may answer doubts that can arise during chronic management of patients with hepatic *** particular,it summarises current knowledge on Wilson’s disease clinical and biochemical monitoring and *** also analyses available evidence on pregnancy and the role of low-copper diet in *** research should focus on trying to provide new copper metabolism tests which could help to guide treatment adjustments.

关键词： Wilson’s disease Urinary copper excretion Non-ceruloplasmin-bound copper d-penicillamine Trientine Zinc salts

Chelation therapy in liver diseases of childhood: Current status and response

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World Journal of Hepatology 2021年第11期13卷 1552-1567页

作者： Jayendra Seetharaman Moinak Sen Sarma department of Pediatric Gastroenterology Sanjay Gandhi Post-graduate Institute of Medical SciencesLucknow 226014Uttar PradeshIndia

Chelation is the mainstay of therapy in certain pediatric liver *** and iron related disorders require ***’s disease(Wd),one of the common causes of cirrhosis in children is treated primarily with copper chelating agents like d-penicillamine and trientine.d-penicillamine though widely used due its high efficacy in hepatic Wd is fraught with frequent adverse effects resulting ***,an alternative drug has comparable efficacy in hepatic Wd but has lower frequency of adverse *** role of ammonium tetra-thiomolybdate is presently experimental in hepatic *** childhood cirrhosis is related to excessive copper ingestion,rarely seen in present *** is effective in the early part of this disease with reversal of clinical *** chelators are commonly used in secondary hemochromatosis of liver in hemolytic *** are strict chelation protocols during bone marrow *** role of iron chelation in neonatal hemochromatosis is presently not in vogue due to its poor efficacy and availability of other modalities of *** hemochromatosis is rare in children and the use of iron chelators in this condition is limited.

关键词： Wilson’s disease d-penicillamine Trientine Indian childhood cirrhosis deferoxamine deferasirox Hemochromatosis

Challenges and dilemmas in pediatric hepatic Wilson’s disease

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World Journal of Hepatology 2023年第10期15卷 1109-1126页

作者： Upasana Ghosh Moinak Sen Sarma Arghya Samanta department of Pediatric Gastroenterology Sanjay Gandhi Post graduate Institute of Medical SciencesLucknow 226014Uttar PradeshIndia department of Pediatric Gastroenterology Sanjay Gandhi Postgraduate Institute of Medical SciencesLucknow 226014India

Wilson disease is an autosomal recessive disorder affecting the ATP7B gene located on chromosome *** leads to copper deposition in various organs,most importantly in the liver and *** genetic mutations are vast,well reported in the West but poorly documented in developing *** the diagnosis is made with a constellation of clinico-laboratory parameters which have significant overlap with other liver diseases and often pose a significant dilemma for *** scoring systems are not *** availability and affordability of chelators in developing countries impact the drug compliance of *** d-penicillamine is a potent drug,its side effects lead to drug *** is cost-prohibitive in developing *** is no single test to assess the adequacy of *** urinary copper is an essential upcoming diagnostic and prognostic *** the presence of cirrhosis,hypersplenism clouds the assessment of myelosuppression of ***,it may be difficult to distinguish disease tubulopathy from druginduced *** worsening due to chelators may appear similar to disease *** as fulminant hepatic failure requires rapid *** is a limited window of opportunity to salvage these patients with the help of plasmapheresis and other liver-assisted *** review addresses the challenges and clinical dilemmas faced at beside in developing countries.

关键词： Wilson’s disease Children Hepatic Wilson disease d-penicillamine Trientine Exchangeable copper

ClinicalandExperimentalStudyofRAMixtureinTreatmentofRhematoidArthritislxl

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Chinese Journal of Integrative Medicine 1996年第3期2卷 178-183页

作者：许得盛沈自尹王文健蔡定芳陈伟华

The RA mixture is composed of Tripterygium wilfordii (TW) and other Chinese medicinalherbs with effect of expelling Wind, activating blood circulation, invigorating the Kidney and Qi . The authorstreated rheumatoid arthritis ( RA) patients with RA mixture and compared it with d-penicillamine as ocntrol .The two group's therapeutical effect is similar ( P >0. 05) , but side effect occuring ratio in treatment groupwas obviously lower than that of control group ( Pdegenerative reaction ( HLA-dR+ ) cell, Cd4/Cd8 ratio were reduced and auto-mixed lymphocytic reaction ( AMLR) level enhanced ( P dthat swelling of rheumatoid arthritis model mouse's joint could be reduced by both RA mixture and TW. Com-paring with TW. RA mixture had stronger effect in controlling the inflammation of synovial cell and fibroid de-generation of fibrocytes. At the same time, RA mixture had stronger effect in protecting immune organ fromatrophy and maintaining functions of cellular immunity. All these suggest that RA mixture can reduce the syn-droms of RA by improving distribution of T Iymphocyte subsets and functions of cellular immunity. The Chinesemedicinal herbs for reinforcing Kidney, supplementing Qi , activating blood circulation and expelling evil windin the RA mixture could enhance TW's therapeutical effect and reduce it's side effect.

关键词： RA mixture Tripterygium wilfordii d-penicillamine rheumatoid arthritis animal model ,cellular immunity joint pathology