Nephrogenic Systemic Fibrosis

Common Name(s)

Nephrogenic Systemic Fibrosis

Nephrogenic fibrosing dermopathy (NFD) is a condition of unknown cause that affects different parts of the body, particularly the skin. Symptoms of the condition may include progressive swelling and tightening of the skin, sometimes resulting in contractures, and pruritis (itching).  The skin findings are similar to those seen in patients with scleroderma.  NFD was first described in 1997.  To date, NFD has only been reported in patients with kidney disease. 
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Nephrogenic Systemic Fibrosis" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Nephrogenic Systemic Fibrosis" returned 46 free, full-text research articles on human participants. First 3 results:

Gadolinium compounds signaling through TLR4 and TLR7 in normal human macrophages: establishment of a proinflammatory phenotype and implications for the pathogenesis of nephrogenic systemic fibrosis.
 

Author(s): Peter J Wermuth, Sergio A Jimenez

Journal: J. Immunol.. 2012 Jul;189(1):318-27.

 

Nephrogenic systemic sibrosis is a progressive disorder occurring in some renal insufficiency patients exposed to gadolinium-based contrast agents (GdBCA). Previous studies demonstrated that the GdBCA Omniscan upregulated several innate immunity pathways in normal differentiated human ...

Last Updated: 22 Jun 2012

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Positive effect of low-dose imatinib mesylate in a patient with nephrogenic systemic fibrosis.
 

Author(s): Tina Rask Elmholdt, Michael Pedersen, Bettina Jørgensen, Mette Ramsing, Anne Braae Olesen

Journal: Acta Derm. Venereol.. 2011 Jun;91(4):478-9.

 

Last Updated: 20 Jun 2011

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Osseous metaplasia late in the course of nephrogenic systemic fibrosis.
 

Author(s): David R Berk, Alicia Miller, Debra Scarlett, Franz J Wippold, Susan J Bayliss, Dongsi Lu

Journal:

 

Osseous metaplasia has recently been described in several cases of nephrogenic systemic fibrosis, sometimes in association with unusual clinical features such as painful hyperkeratotic spicules, palpable bony masses, and disease regression. Some authors have suggested that it may ...

Last Updated: 31 Aug 2010

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Nephrogenic Systemic Fibrosis" returned 22 free, full-text review articles on human participants. First 3 results:

Nephrogenic systemic fibrosis: concepts and perspectives.
 

Author(s): Ana Carolina de Souza Machado Igreja, Kleyton de Carvalho Mesquita, Shawn Edwin Cowper, Izelda Maria Carvalho Costa

Journal: An Bras Dermatol. ;87(4):597-607.

 

Nephrogenic systemic fibrosis is a chronic, progressive condition that develops in some patients with renal impairment after exposure to gadolinium-based contrast agents used in magnetic resonance imaging. Thickening of the skin is typical, usually affecting the extremities. Visceral ...

Last Updated: 15 Aug 2012

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Linking drugs to obscure illnesses: lessons from pure red cell aplasia, nephrogenic systemic fibrosis, and Reye's syndrome. a report from the Southern Network on Adverse Reactions (SONAR).
 

Author(s): Charles L Bennett, Karen M Starko, Henrik S Thomsen, Shawn Cowper, Oliver Sartor, Iain C Macdougall, Zaina P Qureshi, P Brandon Bookstaver, April D Miller, LeAnn B Norris, Sudha Xirasagar, Alyssa Trenery, Isaac Lopez, Adam Kahn, Alanna Murday, Stefano Luminari, Denis Cournoyer, Francesco Locatelli, Paul Ray, Donald R Mattison

Journal: J Gen Intern Med. 2012 Dec;27(12):1697-703.

 

Identification of serious adverse drug reactions (sADRS) associated with commonly used drugs can elude detection for years. Reye's syndrome (RS), nephrogenic systemic fibrosis (NSF), and pure red cell aplasia (PRCA) among chronic kidney disease (CKD) patients were recognized in 1951, ...

Last Updated: 29 Nov 2012

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Minimizing risk of nephrogenic systemic fibrosis in cardiovascular magnetic resonance.
 

Author(s): Theresa Reiter, Oliver Ritter, Martin R Prince, Peter Nordbeck, Christoph Wanner, Eike Nagel, Wolfgang Rudolf Bauer

Journal:

 

Nephrogenic Systemic Fibrosis is a rare condition appearing only in patients with severe renal impairment or failure and presents with dermal lesions and involvement of internal organs. Although many cases are mild, an estimated 5% have a progressive debilitating course. To date, ...

Last Updated: 1 Aug 2012

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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Use of Plasmapheresis for Nephrogenic Fibrosing Dermopathy (NFD)/Nephrogenic Systemic Fibrosis (NSF) and Understanding Its Etiology
 

Status: Recruiting

Condition Summary: Nephrogenic Fibrosing Dermopathy; Nephrogenic Systemic Fibrosis

 

Last Updated: 1 Jun 2011

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