D-penicillamine / Generic mfg. - LARVOL DELTA

DP04 A case of elastosis perforans serpiginosa in a patient with trisomy 21. (PubMed, Br J Dermatol) - "Regular medications were notable for tofacitinib to treat her psoriatic arthritis, moderately potent topical steroid preparations, and coal tar shampoo to treat her scalp psoriasis...Noted associations with EPS include connective tissue diseases such as cutis laxa, pseudoxanthoma elasticum, Ehlers-Danlos syndrome (EDS), Marfan syndrome and systemic sclerosis; trisomy 21; Rothmund-Thompson syndrome; and medications such as D-penicillamine...Reported treatments include topical corticosteroids, topical retinoids, cryotherapy, topical imiquimod, narrowband ultraviolet B radiation, oral isotretinoin and ablative carbon dioxide laser, with variable results. This case highlights that EPS should be considered in patients with trisomy 21 given the association. It also highlights that when undertaking a biopsy in suspected EPS it is important to take samples from the keratotic papules to ensure diagnostic accuracy."

Journal • Dermatology • Dermatopathology • Genetic Disorders • Hurler Syndrome • Immunology • Inflammatory Arthritis • Psoriasis • Psoriatic Arthritis • Rare Diseases • Rheumatology • Scleroderma • Seronegative Spondyloarthropathies • Systemic Sclerosis

Colorimetric "off-on" sensing of penicillamine via copper-mediated modulation of laccase-mimetic activity of trimanganese tetroxide nanoflowers. (PubMed, Spectrochim Acta A Mol Biomol Spectrosc) - "D-Penicillamine (D-PA) is widely prescribed for Wilson's disease, rheumatoid arthritis, and cystinuria; however, its narrow therapeutic window and risk of severe renal, hematological, and autoimmune complications necessitate precise therapeutic monitoring...The sensor achieved an ultralow detection limit of 4.8 nM, exhibited excellent selectivity even in complex matrices, and demonstrated reliable performance for D-PA quantification in pharmaceutical formulations, serum, and urine. This strategy integrates sensitivity, operational simplicity, and eco-friendliness, positioning it as a practical alternative to conventional chromatographic assays for clinical and pharmaceutical monitoring."

Journal • Genetic Disorders • Hematological Disorders • Immunology • Inflammatory Arthritis • Metabolic Disorders • Movement Disorders • Rheumatoid Arthritis • Rheumatology

Coexisting elastosis perforans serpiginosa and acquired cutis laxa following long-term penicillamine in Wilson disease. (PubMed, Skin Health Dis) - "We report a rare case of coexisting elastosis perforans serpiginosa and acquired cutis laxa in a patient with Wilson's disease following prolonged penicillamine therapy. This represents two rare but important dermatologic complications of long-term chelation therapy that clinicians should be aware of, given the potential for underlying elastin disruption to also affect systemic tissues."

Journal • Genetic Disorders • Metabolic Disorders • Movement Disorders

Engineering luminescent Au(0)@Au(I)-thiolate core-shell nanoclusters by HCl-etching of Au(I)-thiolate complexes. (PubMed, Nanotechnology) - "This convenient one-pot methodology demonstrates remarkable versatility, having been successfully extended beyond the preparation of luminescent Hcy-Au NCs and glutathione stabilized Au NCs (GSH-Au NCs) to include the synthesis of luminescent cysteine stabilized Au NCs (Cys-Au NCs) and D-Penicillamine stabilized Au NCs (DPA-Au NCs). Our work provides valuable insights for developing novel highly luminescent metal NCs."

Journal

Aqueous-phase synthesis of dual-anion CuInSeS quaternary quantum dots for ultrasensitive D-penicillamine detection via inter filter effect. (PubMed, Anal Methods) - "Successful quantification of D-PA in human serum samples achieved spiked recoveries of 97.77%-102.8%, confirming method reliability. This study provides a novel design strategy for dual-cation and dual-anion quaternary QD-based fluorescent probes, demonstrating potential application value in clinical drug monitoring."

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Fe-Doped TiO2 Hollow Nanospheres as Light-Responsive Peroxidase-Like Nanozymes for the Colorimetric and Fluorescent Dual-Mode Detection of d-Penicillamine in Foodstuffs and the Environment. (PubMed, Langmuir) - "The proposed sensor demonstrated detection limits (LODs) of 0.054 μM (colorimetric) and 0.40 μM (fluorescence), with 92.2-107.4% recovery and relative standard deviation < 4.4% in milk, tap water, and fetal bovine serum, along with outstanding reproducibility and stability. This work provides new insights to synthesize light-responsive nanozymes and integrated multimode sensing platforms."

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Biological Effects of D-Penicillamine on Copper Sulfate-Induced Possible Damage in the Caudate Nucleus: Role of Apoptotic Genes Bax, Bcl2, and Bax-Bcl2 Ratio. (PubMed, Biol Trace Elem Res) - "Apoptotic genes and histopathological images showed a more pragmatic beneficial effect of D-penicillamine on neurons of the caudate nucleus compared to the CuSO₄-treated group. The study highlights the potential role of D-penicillamine as a therapeutic option to counteract CuSO₄-induced toxicity, especially in ecologically affected areas where both humans and animals may be exposed to elevated copper levels."

Journal • BAX • BCL2 • IL6 • TNFA

Safety of penicillamine and trientine in the treatment of Wilson's disease: An analysis of the FDA Adverse Event Reporting System (FAERS) database. (PubMed, PLoS One) - "This study reveals the characteristics of AEs and potential associated risks in the clinical application of penicillamine and trientine, emphasizing individualized medication and vigilant monitoring strategies to provide guidance for safe medication use."

Adverse events • Journal • Alzheimer's Disease • CNS Disorders • Gastroenterology • Gastrointestinal Disorder • Genetic Disorders • Immunology • Metabolic Disorders • Movement Disorders

First Results of the European Cystinuria Registry (KIDNEY WEEK 2025) - "As cystine binding drug, 18.5% received Tiopronin and 5.3% D-Penicillamine...Conclusion Despite alkalizing treatment, only one quarter of patients reach the recommended urinary pH level and less than half have an adequate urinary volume. Our data show for the first time that a urinary pH below 7.5 is associated with an increased risk of new stone."

Chronic Kidney Disease • Nephrology • Renal Disease

Effect of Polyurethane Structure on the Physicochemical, Mechanical, and Biological Properties on their Copper Complexes Composites. (PubMed, Macromol Biosci) - "Considering this, copper complexes of D-penicillamine (DP), L-cysteine (LC), and dopamine (DOP) were incorporated into segmented polyurethanes (SPU) synthesized with either a semi-crystalline (poly-ε-caprolactone, PCL) or an amorphous (polytetramethylene ether glycol, PTMEG) soft segment...Finally, the composites demonstrated neither hemolytic nor procoagulating behavior (hemolysis 20), although they exhibited some degree of impairment in cytocompatibility compared to their respective pristine SPUs. Collectively, these findings suggest that some composites possess promising properties for potential cardiovascular applications."

Journal • Cardiovascular • Hematological Disorders

1690-Fold enhanced electrochemiluminescence of gold nanoclusters via Zn2+ induced aggregation. (PubMed, Chem Sci) - "Herein, a zinc ion (Zn2+)-induced aggregation strategy is proposed for 1690-fold enhanced electrochemiluminescence (ECL) from d-penicillamine (DPA)-capped gold nanoclusters (AuNCs), and the aggregation-induced ECL (AIECL) is a multiplicative effect of two enhancing factors...The other is the promoted electron transfer by Zn2+-DPA bonds, which can enhance charge annihilation with the assistance of energy level alignment between AuNCs and the Zn2+-DPA bond, further amplifying ECL by another ∼100-fold. The dual-enhanced AIECL strategy can be utilized to design even brighter ECL luminophores."

Journal

Neuropsychiatric Unmasking of Wilson's Disease: A Diagnostic Challenge With Hepatic Implications (ACG 2025) - "D-penicillamine and zinc acetate were initiated...Early initiation of chelation therapy can reverse symptoms and prevent irreversible liver damage or need for transplantation. This case reinforces the need for high clinical suspicion and interdisciplinary collaboration in atypical psychiatric presentations."

CNS Disorders • Genetic Disorders • Hepatology • Mental Retardation • Metabolic Disorders • Movement Disorders • Psychiatry • Schizophrenia • ATP7B • CP

Toxins and Ticks: Acute Encephalopathy in a Farmer With Wilson Disease (ACG 2025) - "We report the case of ACLF in a patient with stable Wilson disease, triggered by drug-induced liver injury (DILI) and acute tick-borne infections, where the New Wilson Index (NWI) guided timely transplant referral.Case Description/ A 61-year-old male farmer with compensated Wilson disease on d-penicillamine presented with jaundice, confusion, and abdominal distention. Five weeks prior, he began daily amoxicillin for dental issues...His presentation was concerning for ACLF, and he was started on lactulose and doxycycline for Lyme infection and transferred to a tertiary academic center...Lumbar puncture was deferred due to coagulopathy, so doxycycline was changed to intravenous ceftriaxone for empiric CNS Lyme treatment...The NWI facilitated urgent transplant referral, underscoring its value in prognostication. Clinicians should maintain a broad differential and consider early transplant evaluation in patients with decompensated Wilson disease.Figure: Case TimelineFigure:..."

CNS Disorders • Cough • Dermatology • Genetic Disorders • Hematological Disorders • Hepatic Encephalopathy • Hepatology • Infectious Disease • Inflammation • Inflammatory Arthritis • Liver Failure • Lyme Disease • Metabolic Disorders • Movement Disorders • Pruritus • Psychiatry • Renal Disease • Respiratory Diseases

Behavioral and Quality of Life Challenges in Pediatric Wilson Disease: Insights From a Multisite International Registry (AACAP 2025) - "Participants on chelating agents (D-penicillamine or trientine) reported lower physical functioning (77.7 + 20.93, p = .004) and physical health (77.59 + 21.28, p = .005) than those on zinc (94.06 ± 7.35), possibly due to selection bias of using zinc for less ill patients. Children with WD experience internalizing symptoms (anxiety, depression, and somatic complaints) that impact their emotional well-being, social interactions, and academic performance. Children with WD experience internalizing symptoms (anxiety, depression, and somatic complaints) that impact their emotional well-being, social interactions, and academic performance. Females exhibiting more emotional distress may benefit from gender-sensitive interventions. The relationship between internalizing symptoms and academic struggles underscores the need to address mental health challenges early."

Clinical • HEOR • CNS Disorders • Depression • Genetic Disorders • Hepatology • Metabolic Disorders • Mood Disorders • Movement Disorders • Pediatrics • Psychiatry

COMPARISON OF RITUXIMAB AND IMMUNOSUPPRESSANTS IN THERAPY OF INTERSTITIAL LUNG DISEASE ASSOCIATED WITH SYSTEMIC SCLEROSIS (CHEST 2025) - "Cyclophosphamide (CP) received 45% of pts. The median of cumulative dose of CP was 3,2 [2; 14] g. Other patients was on mycophenolate mofetil (15%), methotrexate (5%) and D-penicillamine (6%) therapy... The results of our study confirm the data on the positive effect of RTX in complex therapy in patients with SSc (decrease of skin induration, increase of FVC, stabilization of DLCO). Meantime, patients on IS and prednisolone therapy shown the worsening of lung function and insignificant changes of other clinical parameters. CLINICAL IMPLICATIONS: RTX could be considered as a more effective option in complex treatment of SSc in comparison with IS therapy, but further studies are required."

Immunology • Interstitial Lung Disease • Pulmonary Disease • Respiratory Diseases • Scleroderma • Systemic Sclerosis

Ultrasensitive Circularly Polarized Photon Detectors Based on Chiral Two-Dimensional MoS2. (PubMed, ACS Nano) - "Here we demonstrate chiral 2D MoS2 with sub-nanometer thickness via chirality transfer from l-/d-penicillamine (l-/d-PEN)...Phototransistors with chiral 2D MoS2 channels exhibit gate-tunable circularly polarized light detection with responsivity of >102 A/W and anisotropy g-factor of 1.98, close to the theoretical maximum of 2.0. The reduced dimensionality magnifies the chirality transfer efficiency, allowing realization of ultrasensitive detectors for circularly polarized photons."

Journal

EUROCYS, a European cystinuria registry (ESPN 2025) - "As cystine binding drug, 88 patients (18.5%) were treated with Tiopronin and 25 (5.3%) with D-Penicillamine... The EUROCYS registry shows real life data on cystinuria patients followed in European centers. Despite alkalinizing treatment, only few patients reach the recommended urinary pH level and less than half have an adequate urinary volume. Our data suggest that pH below 7.5 is associated with an increased risk of new stone."

Chronic Kidney Disease • Nephrology • Renal Calculi • Renal Disease

A VERY RARE ASSOCIATION OF WILSON'S DISEASE REVEALED BY AN ABSCESSED PERIANAL FISTULA: A CASE REPORT (UEGW 2025) - "Clinical Case Summary: Introduction: Wilson's disease is a rare inherited liver disorder caused by copper metabolism dysfunction,whereas Crohn's disease is a chronic inflammatory bowel condition.The co-occurrence of both conditions in an adolescent is extremely rare and becomes particularly noteworthy when associated with severe complications.Case presentation: We report a 17-year-old male with cirrhotic Wilson's disease,stable on D-penicillamine, who developed severe anal pain and bloody diarrhea.On proctologic examination,he had hemorrhoids, a s crotal fissure,and a non-draining anal fistula.Laboratory investigations revealed severe anemia (Hb 4.4 g/dL),leukopenia,and elevated C-reactive protein... The association between Wilson's disease and Crohn's disease is exceedingly rare. This case emphasizes the need for a multidisciplinary approach in managing complex patients, particularly when faced with severe complications."

Case report • Clinical • Anemia • Crohn's disease • Gastrointestinal Disorder • Genetic Disorders • Hematological Disorders • Hepatology • Immunology • Inflammation • Inflammatory Bowel Disease • Leukopenia • Metabolic Disorders • Movement Disorders • CRP

Copper-associated Chronic Hepatitis in Dogs. (PubMed, Vet Clin North Am Small Anim Pract) - "Early diagnosis through liver biopsy and copper quantification is critical to prevent cirrhosis. Genetic testing and selective breeding can help manage risk in predisposed breeds."

Journal • Review • Fibrosis • Genetic Disorders • Hepatology • Immunology • Inflammation • Metabolic Disorders • Movement Disorders • ATP7A

MEDICATION ADHERENCE IN WILSON DISEASE PATIENTS TREATED WITH TRIENTINE TETRAHYDROCHLORIDE SUPPLIED BY A SINGLE SOURCE SPECIALTY PHARMACY (AASLD 2025) - "Most frequently reported were trientine hydrochloride capsules, zinc salts and penicillamine in that order. In this real-world analysis of patients with WD receiving TETA-4HCl through a single-source specialty pharmacy, adherence was consistently high with minimal interruption in therapy as described with a gap of 2.7 days per shipment and a low overall discontinuation rate of 17%. The extent to which single-source pharmacy distribution mitigates barriers to access, reduces treatment burden, and facilitates adherence warrants further investigation."

Adherence • Clinical • Genetic Disorders • Hepatology • Metabolic Disorders • Movement Disorders • Rare Diseases

TDMQ20 as A Drug Candidate for Wilson's Disease: Comparison with D-Penicillamine, Trientine, and Tetrathiomolybdate In Vitro and In Mice. (PubMed, Pharmaceutics) - "Conversely, DPA, TETA, and bcTTM give rise to various complexes with copper ions, often with oligomeric or cluster structures that can be retained in blood circulation or sequestered by proteins. Taking into consideration all the advantages of TDMQ20 compared to other ligands, including its lack of toxicity during long-term administration in mice, the drug candidate TDMQ20 appears to be a first-class challenger to the currently used treatments, i.e., DPA, TETA, and bcTTM."

Journal • Preclinical • Genetic Disorders • Metabolic Disorders • Movement Disorders

Chiral au nanocluster-porphyrin assemblies enhanced photodynamic disinfection for acidophilic Bacteria. (PubMed, J Colloid Interface Sci) - "This study introduces chiral L/D-Au@MTPPS nanoparticles (NPs), synthesized through the spontaneous self-assembly of porphyrin/metalloporphyrin (MTPPS) with L/D-penicillamine-stabilized Au nanoclusters (L/D-Au NCs)...A systematic comparative analysis revealed that D-configurated NPs exhibit superior efficacy, achieving eradication rates exceeding 90 % through optimized photoactivation mechanisms. This research establishes a strategic framework for developing precision antimicrobial platforms targeting extremophilic pathogens, particularly acid-resistant species such as Helicobacter pylori and Acetobacter spp."

Journal • Infectious Disease

Protective Effects of N-Acetylcysteine and its Amide Derivative Against Toxic Metals In Vitro: Potential Alternatives/Adjuncts to Traditional Chelators. (PubMed, Environ Toxicol Pharmacol) - "The protective efficacies of N-acetylcysteine (NAC) and N-acetylcysteine amide (NACA) against the toxicity of compounds containing arsenic, cadmium, cobalt, chromium and mercury, were compared to those of dimercaprol, dimercaptosuccinic acid, 2,3-dimercaptopropanesulphonate, D-penicillamine, and derivatives of ethylenediamine tetraacetic acid and diethylenetriaminepentaacetic in CHO-K1 cells. Given NAC's activity against metal toxicity, widespread clinical use, parenteral or oral routes of administration, high clinical safety, low cost and ease of accessibility, it should be given consideration as a broad-spectrum protectant against toxic metal poisoning as a treatment or adjunct/combination treatment. Its more lipophilic derivative NACA, also warrants attention, especially in those cases where brain toxicity is a concern."

Journal • Preclinical

NCPA-templated red-emitting gold nanoclusters: a turn-off-on fluorescent probe for rapid and selective detection of Cu2+ ions in live cells and evaluation of anticancer activity. (PubMed, Dalton Trans) - "In this study, a turn-OFF-ON red-emitting AuNC fluorescent probe was synthesized using N-cholyl D-penicillamine (NCPA) as a stabilizing and templating agent above its critical micellar concentration (CMC) under UV light irradiation...In an in vitro cellular environment, the quenched fluorescence of the NCPA@AuNCs-Cu2+ complex could also be restored by the EDTA treatment, demonstrating the potential of this system for intracellular Cu2+ sensing and live-cell imaging. Overall, the developed NCPA-stabilized red-emitting AuNCs offer an efficient, reversible fluorescent sensing platform for Cu2+ ions and exhibit significant potential for cancer cell imaging and therapeutic applications."

Journal • Oncology

Lipoid Proteinosis: A Rare Genodermatosis Accompanied By Hoarseness (EADV 2025) - "The patient was initiated on systemic acitretin therapy at a dose of 20 mg/day for 6 months. In conclusion, while LP is a rare disorder, its diverse clinical presentations and treatment challenges have made it a focal point of scientific research."

Ataxia • CNS Disorders • Dermatology • Epilepsy • Genetic Disorders • Mental Retardation • Mood Disorders • Movement Disorders • Otorhinolaryngology • Psychiatry • Rare Diseases • Schizophrenia