Nephrotic syndrome in the course of treatment of Wilson's disease with DL-penicillamine.

Karp, Melvyn P.; Lurie, Miriam; Yonis, Z

doi:10.1136/adc.41.220.684

Cited by 10 publications

(2 citation statements)

References 8 publications

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“…The normal GFR observed in almost all our patients on long-term D-penicillamine treatment indicates that this regimen is usually well tolerated by the kidney. The nephrologist should, however, be aware of the rare patient with WD who manifests as acute renal failure related to a hemolytic crisis and liver failure [33,38], and who may profit from liver transplantation [4]. .…”

Section: Discussionmentioning

confidence: 99%

Proteinuria and other renal functions in Wilson's disease

Sözeri

Feist

Ruder

et al. 1997

Pediatric Nephrology

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Renal lesions have repeatedly been described in Wilson's disease (WD). We investigated the excretion of total protein, albumin, low (LMW) and high molecular weight (HMW) proteins, N-acetyl-beta-D-glucosaminidase (NAG), and calcium, as well as creatinine clearance, in 24-h urine samples of 41 patients with WD aged 6-37 (mean 17) years who had been treated for a period of 0-15 (mean 4.5) years with D-penicillamine (900 mg/day). The amount of all protein excreted was significantly increased compared with controls, 39% of patients presenting with total proteinuria more than two standard deviations from the mean of controls. The changes in protein excretion depended on the duration of treatment. LMW proteinuria was elevated almost exclusively in the first 2 years after the start of treatment, indicating early tubular damage. This is supported by an initially high excretion of beta 2-microglobulin, NAG, and calcium. Increased excretion of HMW proteins, including albumin, persisted over longer periods, which suggests glomerular injury in some patients, possibly related to the use of D-penicillamine. Creatinine clearance remained roughly within normal limits. We propose that renal function should regularly be checked in patients with WD.

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Section: Discussionmentioning

confidence: 99%

Proteinuria and other renal functions in Wilson's disease

Sözeri

Feist

Ruder

et al. 1997

Pediatric Nephrology

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“…However, in case of severe reaction it may be prudent to withdraw penicillamine immediately and substitute with trientine. [59][60][61][62][63][64][65][66] In a small subset of patients though, similar hypersensitivity-related reactions also develop with the use of trientine. In a study, elevated serum antinuclear antibodies did not help predict longterm penicillamine associated immunological reactions.…”

Section: Treatment Optionsmentioning

confidence: 99%

Advances in Treatment of Wilson Disease

Aggarwal

Bhatt

2018

Tremor and Other Hyperkinetic Movements

101

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Background: Wilson disease (WD) is an inherited neurometabolic disorder that results in excessive copper deposition in the liver and the brain, affecting children and young adults. Without treatment the disease is invariably fatal. Though treatments for WD have been available since the 1950s, the disease continues to be associated with considerable morbidity and mortality because of missed diagnosis, and delayed or inadequate treatment. In this paper we survey WD-related literature in order to review recent advances in WD treatment. Methods: We performed a literature search using the PubMed database for articles relating to WD and its medical treatment. We reviewed the articles, and crossreferences of relevant articles, to summarize the current practices for treatment of WD. Results: The survey shows that if WD is properly treated, in most patients the liver can be stabilized, even severe neurological disability reversed, and patients can resume normal lives. Discussion: Medical treatment for WD includes use of copper chelators (penicillamine, trientine, dimercaprol, dimercaptopropane sulfonate, and ammonium tetrathiomolybdate) and drugs that decrease gastrointestinal copper absorption. Our knowledge of the treatment approaches has benefited from the large systematic clinical studies that have been conducted over the last decade. For each drug used to treat WD, we surveyed its development, indication for use, dosing, efficacy, and adverse effects.

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