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A case of tuberous sclerosis complex that progressed to end-stage renal disease

Nature Reviews Nephrology volume 5, pages 172–176 (2009)Cite this article

Abstract

Background A 31-year-old woman with tuberous sclerosis complex presented with a 1 week history of subjective fever, chills, rigors, poor appetite and dizziness.

Investigations Physical examination, urine and blood analysis, CT of the abdomen, chest and brain, and chest X-ray.

Diagnosis End-stage renal disease, septic shock and urinary tract infection secondary to huge bilateral angiomyolipomas of the kidney associated with tuberous sclerosis complex.

Management Antibiotic therapy, vasopressor treatment and bilateral nephrectomy, followed by hemodialysis while awaiting renal transplantation.

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Figure 1: Physical features of the patient with tuberous sclerosis complex described in this article.
Figure 2: Contrast-enhanced CT scan that shows large bilateral kidney masses and the compressed mesentery in the center.
Figure 3: A comparison of the gross morphology of the patient's kidney with that of a normal kidney.
Figure 4: Renal biopsy sample, showing the histopathology of angiomyolipoma (hematoxylin–eosin stain, magnification ×40).
Figure 5: Non-contrast-enhanced CT scan of the chest, showing lymphangioleiomyomatosis of the patient's lungs.

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Authors and Affiliations

  1. A Masoumi is a Research Fellow in the Division of Nephrology, FJ Castro-Silva is a Resident in the Division of Pathology, JB Myers is a Resident in the Division of Urology, SS Wilson is Assistant Professor in the Division of Urology and RW Schrier is a Professor of Medicine at the University of Colorado Health Sciences Center, M Sarraf is a Research Fellow in Cardiology, Denver, CO, USA.,

    Mohammad Sarraf, Amirali Masoumi, Fernando J Castro-Silva, Jeremy B Myers, Shandra S Wilson & Robert W Schrier

Authors
  1. Mohammad Sarraf
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  2. Amirali Masoumi
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  3. Fernando J Castro-Silva
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  4. Jeremy B Myers
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  5. Shandra S Wilson
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  6. Robert W Schrier
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Correspondence to Mohammad Sarraf.

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The authors declare no competing financial interests.

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Sarraf, M., Masoumi, A., Castro-Silva, F. et al. A case of tuberous sclerosis complex that progressed to end-stage renal disease. Nat Rev Nephrol 5, 172–176 (2009). https://doi.org/10.1038/ncpneph1033

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