Nephrogenic fibrosing dermopathy: two pediatric cases

Jan, Farhana; Segal, Jennifer M; Dyer, John A.; LeBoit, Philip E.; Siegfried, Elaine C.; Frieden, Ilona J.

doi:10.1067/s0022-3476(03)00538-9

Cited by 82 publications

(56 citation statements)

References 10 publications

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“…A single center-based study by Leung et al [28] failed to show any benefit with renal transplantation when compared to those patients who remained on dialysis. This observation is contrary to previously published reports [9,32,105]. We found that patients who underwent renal transplantation had a higher chance of a positive response, documented as a clinical improvement, when compared to patients who either continued on maintenance dialysis or those who received alternative forms of treatment.…”

Section: Isrn Transplantationcontrasting

confidence: 57%

Renal Transplantation Is Associated with Improved Clinical Outcomes in Nephrogenic Systemic Fibrosis

Yacoub

Kandukurti

Panesar

2013

ISRN Transplantation

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Nephrogenic systemic fibrosis is a debilitating disorder seen in chronic kidney disease patients and is characterized by stiffening of the joints and thickening of the skin. Treatment options are limited, but some patients have had an improvement of their clinical symptoms after renal transplantation and the use of immunosuppression. Although there is a variable response to renal transplantation, it is currently unknown what factors promote a favorable outcome. Our objective was to evaluate if the response to renal transplantation was superior to other treatment modalities and to determine which characteristics allowed for a positive response to occur. We retrieved the data from the literature of 298 reported patients, compared the response to renal transplantation and to other treatments, and analyzed their characteristics. We found that more patients had a higher response to renal transplantation, as determined by softening of the skin and improved joint mobility, and among those that did respond, they had a shorter dialysis vintage. We suggest that if renal transplantation is to be considered as a treatment modality, it should be initiated at the earliest possible in the course of the disease to achieve maximum clinical benefit.

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Section: Isrn Transplantationcontrasting

confidence: 57%

Renal Transplantation Is Associated with Improved Clinical Outcomes in Nephrogenic Systemic Fibrosis

Yacoub

Kandukurti

Panesar

2013

ISRN Transplantation

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“…Partial (n ¼ 8) or complete (n ¼ 2) improvement followed restoration of renal function in 11 cases. One NSF patient was reported to have substantial improvement after renal transplant and again got worse after rejection (28). In three patients, kidney function improvement did not affect the clinical course of the skin disorder presumably because the kidney function was improving at the time the diagnosis of NSF was made ( Table 5).…”

Section: Discussionmentioning

confidence: 99%

Risk factors for NSF: A literature review

Prince

Zhang

Roditi

et al. 2009

Magnetic Resonance Imaging

109

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Emerging evidence linking gadolinium-based contrast agents (GBCAs) to nephrogenic systemic fibrosis (NSF) has changed medical practice patterns toward forgoing GBCA-enhanced magnetic resonance imaging (MRI) or substituting other imaging methods, which are potentially less accurate and often radiation-based. This shift has been based on reports of high NSF incidence at sites where a confluence of risk factors occurred in patients with severe renal dysfunction. This review article explores the factors that affect NSF risk, compares risks of alternative imaging procedures, and demonstrates how risk can be managed by careful selection of GBCA dose, timing of injection with respect to dialysis, and other factors. Nearly half of NSF cases are a milder form that does not cause contractures or reduce mobility. It appears that eliminating even a single risk factor can reduce NSF incidence/risk at least 10-fold. Elimination of multiple risk factors by using single-dose GBCA, dialyzing dialysis patients quickly following GBCA administration, avoiding GBCA in acute renal failure while serum creatinine is rising, and avoiding nonionic linear GBCA in renal failure patients may reduce NSF risk more than a thousand-fold, thereby allowing safe GBCA-enhanced MRI in virtually all patients.

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“…No treatment modality is absolutely effective. Improving renal function either spontaneously or by renal transplant commonly results in improvement of NSF, but is not a guarantee (14,20). Absence of improved renal function has also been reported in some patients who have had a very gradual but spontaneous improvement.…”

Section: Nephrogenic Systemic Fibrosis In Liver Transplant Recipientsmentioning

confidence: 99%

Nephrogenic Systemic Fibrosis Among Liver Transplant Recipients: A Single Institution Experience and Topic Update

Maloo

Abt

Kashyap

et al. 2006

American Journal of Transplantation

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Nephrogenic systemic fibrosis (NSF) is a recently characterized systemic fibrosing disorder developing in the setting of renal insufficiency. NSF's rapidly progressive nature resulting in disability within weeks of onset makes early diagnosis important. Two reports of NSF after liver transplantation are known of. We present three cases of NSF developing within a few months after liver transplantation and review the current literature. Loss of regulatory control of the circulating fibrocyte, its aberrant recruitment, in a milieu of renal failure and a recent vascular procedure appear important in its development. Known current therapies lack consistent efficacy. Only an improvement in renal function has the greatest likelihood of NSF's resolution. Delayed recognition may pose a significant barrier to functional recovery in the ubiquitously deconditioned liver transplant patient. Early recognition and implementation of aggressive physical therapy appear to have the greatest impact on halting its progression.

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Nephrogenic fibrosing dermopathy: two pediatric cases

Cited by 82 publications

References 10 publications

Renal Transplantation Is Associated with Improved Clinical Outcomes in Nephrogenic Systemic Fibrosis

Renal Transplantation Is Associated with Improved Clinical Outcomes in Nephrogenic Systemic Fibrosis

Risk factors for NSF: A literature review

Nephrogenic Systemic Fibrosis Among Liver Transplant Recipients: A Single Institution Experience and Topic Update

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