Effect of D-Penicillamine on Liver Fibrosis and Inflammation in Wilson Disease.

Kazemi, Kourosh; Geramizadeh, Bita; Kakaei, Farzad; Nikeghbalian, Saman; Malek-Hosseini, S.A.

doi:10.1097/00007890-201007272-01551

Cited by 12 publications

(11 citation statements)

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“…Furthermore, all patients were on longterm treatment for WD, and the majority of subjects had a stable course of liver disease after initial clinical improvement. Successful therapy can reduce the degree of fibrosis in WD, and therefore may modulate the results of liver stiffness measurement and fibrosis indices [28]. One of our patients, for whom sequential ARFI evaluations were available, initially presented with CHILD B cirrhosis and a median ARFI value of 2.46 m/s.…”

Section: Discussionmentioning

confidence: 99%

Non-invasive evaluation of hepatic manifestation in Wilson disease with transient elastography, ARFI, and different fibrosis scores

Karlas

Hempel

Tröltzsch

et al. 2012

Scandinavian Journal of Gastroenterology

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Section: Discussionmentioning

confidence: 99%

Non-invasive evaluation of hepatic manifestation in Wilson disease with transient elastography, ARFI, and different fibrosis scores

Karlas

Hempel

Tröltzsch

et al. 2012

Scandinavian Journal of Gastroenterology

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“…This drug binds hepatic Cu which subsequently is eliminated in urine . In addition, D‐Pen increases metallothionein in hepatocytes (detoxifying intracellular Cu) and enterocytes (facilitating fecal elimination) and has mild anti‐inflammatory and anti‐fibrotic properties . D‐penicillamine is given PO on an empty stomach because food substantially decreases bioavailability …”

Section: Treatmentmentioning

confidence: 99%

ACVIM consensus statement on the diagnosis and treatment of chronic hepatitis in dogs

Webster

Cullen

Penninck

et al. 2019

Veterinary Internal Medicne

184

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This consensus statement on chronic hepatitis (CH) in dogs is based on the expert opinion of 7 specialists with extensive experience in diagnosing, treating, and conducting clinical research in hepatology in dogs. It was generated from expert opinion and information gathered from searching of PubMed for manuscripts on CH, the Veterinary Information Network for abstracts and conference proceeding from annual meetings of the American College of Veterinary Medicine and the European College of Veterinary Medicine, and selected manuscripts from the human literature on CH. The panel recognizes that the diagnosis and treatment of CH in the dog is a complex process that requires integration of clinical presentation with clinical pathology, diagnostic imaging, and hepatic biopsy. Essential to this process is an index of suspicion for CH, knowledge of how to best collect tissue samples, access to a pathologist with experience in assessing hepatic histopathology, knowledge of reasonable medical interventions, and a strategy for monitoring treatment response and complications.

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“…Conventional copper chelators include, for example, d-penicillamine, tetrathiomolybdate, disulfiram, dithiocarbamates, and 2-mercaptopyridine-N-oxide, largely being nonspecific and broad-spectrum LOX inhibitors, with unwanted off-target effects. (62) d-penicillamine is widely used in patients with Wilson's disease, (63,64) pulmonary fibrosis, (65) and Indian childhood cirrhosis, (66,67) with clear clinical benefits in preventing organ toxicity and fibrosis progression. Inhibition of LOX activity and collagen accumulation was implicated as a major underlying mechanism.…”

Section: Small-molecule Inhibitorsmentioning

confidence: 99%

Lysyl Oxidase (LOX) Family Members: Rationale and Their Potential as Therapeutic Targets for Liver Fibrosis

et al. 2020

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The cross‐linking of structural extracellular matrix (ECM) components, especially fibrillar collagens and elastin, is strongly implicated in fibrosis progression and resistance to fibrosis reversal. Lysyl oxidase family members (LOX and LOXL1 [lysyl oxidase‐like 1], LOXL2 [lysyl oxidase‐like 2], LOXL3 [lysyl oxidase‐like 3], and LOXL4 [lysyl oxidase like 4]) are extracellular copper‐dependent enzymes that play a key role in ECM cross‐linking, but have also other intracellular functions relevant to fibrosis and carcinogenesis. Although the expression of most LOX family members is elevated in experimental liver fibrosis of diverse etiologies, their individual contribution to fibrosis is incompletely understood. Inhibition of the LOX family as a whole and of LOX, LOXL1, and LOXL2 specifically has been shown to suppress fibrosis progression and accelerate its reversal in rodent models of cardiac, renal, pulmonary, and liver fibrosis. Recent disappointing clinical trials with a monoclonal antibody against LOXL2 (simtuzumab) in patients with pulmonary and liver fibrosis dampened enthusiasm for LOX family member inhibition. However, this unexpected negative outcome may be related to the inefficient antibody, rather than to LOXL2, not qualifying as a relevant antifibrotic target. Moreover, LOX family members other than LOXL2 may prove to be attractive therapeutic targets. In this review, we summarize the structural hallmarks, expression patterns, covalent cross‐linking activities, and modes of regulation of LOX family members and discuss the clinical potential of their inhibition to treat fibrosis in general and liver fibrosis in particular.

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Effect of D-Penicillamine on Liver Fibrosis and Inflammation in Wilson Disease.

Cited by 12 publications

References 0 publications

Non-invasive evaluation of hepatic manifestation in Wilson disease with transient elastography, ARFI, and different fibrosis scores

Non-invasive evaluation of hepatic manifestation in Wilson disease with transient elastography, ARFI, and different fibrosis scores

ACVIM consensus statement on the diagnosis and treatment of chronic hepatitis in dogs

Lysyl Oxidase (LOX) Family Members: Rationale and Their Potential as Therapeutic Targets for Liver Fibrosis

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