<abstract xmlns="http://www.w3.org/1999/xhtml"><p> Wilson’s disease is an inherited autosomal recessive disorder of copper balance leading to accumulation of copper mainly in liver and brain result from absent or reduced function of copper-transporting P-type ATPase. Copper is an essential trace element but in Wilson’s disease it accumulate to the point of toxicity. D-penicillamine is a classic drug for treatment of Wilson’s disease. Its major effect is to promote the urinary copper excretion. The use of D-penicillamine in the therapy of Wilson’s disease is known to be complicated by the development of various glomerular diseases. In this report we describe the development of nephrotic syndrome after 2 years treatment with D-penicillamine in a 31-year-old male undergoing treatment for Wilson’s disease, with a prompt regression at the discontinuation of the drug. We present this case to draw attention to the rare complication as nephrotic syndrome in patients with Wilson’s disease under D-penicillamine treatment and possible underlying causes. It is strongly necessary the therapy and clinical condition of patients with Wilson’s disease to be monitoring regularly - we recommended monthly.</p></abstract>

Wilson’s disease is an inherited autosomal recessive disorder of copper balance leading to accumulation of copper mainly in liver and brain result from absent or reduced function of copper-transporting P-type ATPase. Copper is an essential trace element but in Wilson’s disease it accumulate to the point of toxicity. D-penicillamine is a classic drug for treatment of Wilson’s disease. Its major effect is to promote the urinary copper excretion. The use of D-penicillamine in the therapy of Wilson’s disease is known to be complicated by the development of various glomerular diseases. In this report we describe the development of nephrotic syndrome after 2 years treatment with D-penicillamine in a 31-year-old male undergoing treatment for Wilson’s disease, with a prompt regression at the discontinuation of the drug. We present this case to draw attention to the rare complication as nephrotic syndrome in patients with Wilson’s disease under D-penicillamine treatment and possible underlying causes. It is strongly necessary the therapy and clinical condition of patients with Wilson’s disease to be monitoring regularly - we recommended monthly.

Nephrotic syndrome after treatment with D-penicillamine in a patient with Wilson’s disease

St. Ivan Rilski University Hospital, Internal department, Medical University, Sofia, Bulgaria

St. Ivan Rilski University Hospital, Department of clinical laboratory and immunology, Sofia, Bulgaria

Revista Romana de Medicina de Laborator

This article is distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Wilson’s disease is an inherited autosomal recessive disorder of copper balance leading to accumulation of copper mainly in liver and brain result from absent...

Nephrotic syndrome after treatment with D-penicillamine in a patient with Wilson’s disease

Pubblicato online: 21 giu 2014

Pagine: 181 - 189

Ricevuto: 13 set 2013

Accettato: 03 mag 2014

DOI: https://doi.org/10.2478/rrlm-2014-0016

Parole chiave
Wilson’s disease, D-penicillamine, Urinary copper excretion

© by Irena D. Ivanova

This article is distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Nephrotic syndrome after treatment with D-penicillamine in a patient with Wilson’s disease

Pubblicato online: 21 giu 2014

Pagine: 181 - 189

Ricevuto: 13 set 2013

Accettato: 03 mag 2014

DOI: https://doi.org/10.2478/rrlm-2014-0016

Parole chiaveWilson’s disease, D-penicillamine, Urinary copper excretion

© by Irena D. Ivanova

This article is distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Parole chiave
Wilson’s disease, D-penicillamine, Urinary copper excretion