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Nephrogenic fibrosing dermatopathy, cardiac calcification and pulmonary hypertension in an adolescent on chronic hemodialysis.

Sharma J, Mongia A, Schoenaman M, Chang S, D'Angelo A, Rao M - Indian J Nephrol (2008)

Bottom Line: Recent reports have associated the development of NFD with the use of gadolinium-enhanced magnetic resonance imaging (MRI).Only five cases of NFD have been reported in children, all of which were prior to the information regarding the consequences of using gadolinium.Because he succumbed to this disease, we stress on the importance of eliminating the use of gadolinium-enhanced MRI examinations in children with impaired kidney function until the etiology of NFD is clarified.

View Article: PubMed Central - PubMed

ABSTRACT
Nephrogenic fibrosing dermatopathy (NFD) is a systemic disorder of unknown etiology. Recent reports have associated the development of NFD with the use of gadolinium-enhanced magnetic resonance imaging (MRI). Here, we present the case of an adolescent with end-stage renal disease who died of biopsy-proven NFD and also developed cardiac calcification and clinical manifestations of pulmonary fibrosis with pulmonary hypertension. Only five cases of NFD have been reported in children, all of which were prior to the information regarding the consequences of using gadolinium. Here, we report a patient with NFD who received gadolinium while on chronic hemodialysis, 16 months prior to the onset of symptoms. Because he succumbed to this disease, we stress on the importance of eliminating the use of gadolinium-enhanced MRI examinations in children with impaired kidney function until the etiology of NFD is clarified.

No MeSH data available.


Related in: MedlinePlus

Parasternal long-axis view showing discrete small areas of calcification in interventricular septum
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Figure 0002: Parasternal long-axis view showing discrete small areas of calcification in interventricular septum

Mentions: Laboratory data were remarkable with erythrocyte sedimentation rate (ESR), 98 mm/h; C-reactive protein (CRP), 5.89 U; and negative serologic antibody testing for ANA, anti-smith, anti-SSA/SSB, lupus anticoagulant, anticardiolipin and anti-SCL-70. Serum intact parathyroid hormone levels and serum calcium levels over the previous 2-4 years were 580-1160 and 9-11 mg/dl, respectively. Chest x-ray demonstrated mild cardiomegaly, increased pulmonary vascular markings and a right pleural effusion. A two-dimensional-echo Doppler study demonstrated moderate concentric left ventricular hypertrophy, mild mitral regurgitation, small discrete areas of calcification in the interventricular septum and mild tricuspid regurgitation with a gradient of 64 mmHg and an estimated pulmonary artery pressure of 70 mmHg (concomitant blood pressure, 130/80 mmHg) [Fig. 2]. Chest computed tomography (CT) showed a moderate right pleural effusion, large discrete areas of pulmonary calcification and cardiac calcification involving the aortic root, proximal coronary arteries, left atrium and mitral valve ring [Fig. 3].


Nephrogenic fibrosing dermatopathy, cardiac calcification and pulmonary hypertension in an adolescent on chronic hemodialysis.

Sharma J, Mongia A, Schoenaman M, Chang S, D'Angelo A, Rao M - Indian J Nephrol (2008)

Parasternal long-axis view showing discrete small areas of calcification in interventricular septum
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2813120&req=5

Figure 0002: Parasternal long-axis view showing discrete small areas of calcification in interventricular septum
Mentions: Laboratory data were remarkable with erythrocyte sedimentation rate (ESR), 98 mm/h; C-reactive protein (CRP), 5.89 U; and negative serologic antibody testing for ANA, anti-smith, anti-SSA/SSB, lupus anticoagulant, anticardiolipin and anti-SCL-70. Serum intact parathyroid hormone levels and serum calcium levels over the previous 2-4 years were 580-1160 and 9-11 mg/dl, respectively. Chest x-ray demonstrated mild cardiomegaly, increased pulmonary vascular markings and a right pleural effusion. A two-dimensional-echo Doppler study demonstrated moderate concentric left ventricular hypertrophy, mild mitral regurgitation, small discrete areas of calcification in the interventricular septum and mild tricuspid regurgitation with a gradient of 64 mmHg and an estimated pulmonary artery pressure of 70 mmHg (concomitant blood pressure, 130/80 mmHg) [Fig. 2]. Chest computed tomography (CT) showed a moderate right pleural effusion, large discrete areas of pulmonary calcification and cardiac calcification involving the aortic root, proximal coronary arteries, left atrium and mitral valve ring [Fig. 3].

Bottom Line: Recent reports have associated the development of NFD with the use of gadolinium-enhanced magnetic resonance imaging (MRI).Only five cases of NFD have been reported in children, all of which were prior to the information regarding the consequences of using gadolinium.Because he succumbed to this disease, we stress on the importance of eliminating the use of gadolinium-enhanced MRI examinations in children with impaired kidney function until the etiology of NFD is clarified.

View Article: PubMed Central - PubMed

ABSTRACT
Nephrogenic fibrosing dermatopathy (NFD) is a systemic disorder of unknown etiology. Recent reports have associated the development of NFD with the use of gadolinium-enhanced magnetic resonance imaging (MRI). Here, we present the case of an adolescent with end-stage renal disease who died of biopsy-proven NFD and also developed cardiac calcification and clinical manifestations of pulmonary fibrosis with pulmonary hypertension. Only five cases of NFD have been reported in children, all of which were prior to the information regarding the consequences of using gadolinium. Here, we report a patient with NFD who received gadolinium while on chronic hemodialysis, 16 months prior to the onset of symptoms. Because he succumbed to this disease, we stress on the importance of eliminating the use of gadolinium-enhanced MRI examinations in children with impaired kidney function until the etiology of NFD is clarified.

No MeSH data available.


Related in: MedlinePlus