Multicentric Carpal-Tarsal Osteolysis With Nephropathy Treated Successfully With Cyclosporine A: A Case Report and Literature Review

Connor, Andrew; Highton, John; Hung, Noelyn; Dunbar, John; MacGinley, Robert; Walker, Robert

doi:10.1053/j.ajkd.2007.06.014

Cited by 22 publications

(24 citation statements)

References 12 publications

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“…One patient had a three-allelic mutation (compound heterozygous mutations in the NPHS2 -gene and one mutation in the NPHS1 -gene), two related patients had homozygous mutations in the NPHS2 -gene and one patient had a mutation in the WT1 -gene. A secondary cause was identified in 10 patients (group II): renal agenesis (n=3), traumatic kidney injury (n=1), hypoplasia of one kidney (n=1), reflux nephropathy (n=2), chronic pyelonephritis (n=1), a history of intravenous heroin abuse (n=1), and Hajdu-Cheney syndrome (n=1) [26]. Idiopathic FSGS was diagnosed in 66 patients (group III).…”

Section: Resultsmentioning

confidence: 99%

A retrospective study of focal segmental glomerulosclerosis: clinical criteria can identify patients at high risk for recurrent disease after first renal transplantation

et al. 2013

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BackgroundFocal segmental glomerulosclerosis (FSGS) is a frequent cause of end-stage renal disease. Renal transplantation in patients with FSGS is often complicated by disease recurrence, which is associated with poor outcome. There are no tests that reliably predict recurrence of FSGS after transplantation. The aim of this study was to evaluate if clinical criteria can identify patients at high risk for recurrent disease.MethodsWe retrospectively studied 94 patients who received a first renal transplant at a median age of 37 years (range 5–69 years). Patients were assigned to one of three groups: familial or genetic FSGS (group I; n=18), secondary FSGS (group II; n=10) and idiopathic FSGS (group III; n=66). Pretransplant clinical characteristics were analyzed to determine predictors of a recurrence after transplantation.ResultsFSGS only recurred in patients with idiopathic FSGS (group III; 42%). Patients with a recurrence had a significantly lower serum albumin, higher 24-hour proteinuria and higher estimated glomerular filtration rate at diagnosis. Serum albumin at diagnosis was the only independent predictor of a recurrence in patients with idiopathic FSGS. Patients with recurrent FSGS had more acute rejection episodes (54% vs. 27%, P =0.02) and lower five year graft survival compared to patients without a recurrence (50 vs. 82%, P <0.01).ConclusionsClinical criteria allow identification of patients at high risk of recurrent FSGS after renal transplantation. This information can be used in the counseling and management of patients with FSGS.

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Section: Resultsmentioning

confidence: 99%

A retrospective study of focal segmental glomerulosclerosis: clinical criteria can identify patients at high risk for recurrent disease after first renal transplantation

et al. 2013

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“…Patients with MCTO often progress to end stage kidney disease. 11 Because some of the cases showed FSGS, 27 renal failure might be attributed to podocyte dysfunction caused by MAFB mutations. 11 The Mafb gene has been reported to localize to the vicinity of the albuminuria-susceptible locus in diabetic mice.…”

Section: Discussionmentioning

confidence: 99%

Overexpression of Mafb in Podocytes Protects against Diabetic Nephropathy

Morito¹,

Yoh²,

Ojima³

et al. 2014

Journal of the American Society of Nephrology

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We previously showed that the transcription factor Mafb is essential for podocyte differentiation and foot process formation. Podocytes are susceptible to injury in diabetes, and this injury leads to progression of diabetic nephropathy. In this study, we generated transgenic mice that overexpress Mafb in podocytes using the nephrin promoter/enhancer. To examine a potential pathogenetic role for Mafb in diabetic nephropathy, Mafb transgenic mice were treated with either streptozotocin or saline solution. Diabetic nephropathy was assessed by renal histology and biochemical analyses of urine and serum. Podocytespecific overexpression of Mafb had no effect on body weight or blood glucose levels in either diabetic or control mice. Notably, albuminuria and changes in BUN levels and renal histology observed in diabetic wild-type animals were ameliorated in diabetic Mafb transgenic mice. Moreover, hyperglycemia-induced downregulation of Nephrin was mitigated in diabetic Mafb transgenic mice, and reporter assay results suggested that Mafb regulates Nephrin directly. Mafb transgenic glomeruli also overexpressed glutathione peroxidase, an antioxidative stress enzyme, and levels of the oxidative stress marker 8-hydroxydeoxyguanosine decreased in the urine of diabetic Mafb transgenic mice. Finally, Notch2 expression increased in diabetic glomeruli, and this effect was enhanced in diabetic Mafb transgenic glomeruli. These data indicate Mafb has a protective role in diabetic nephropathy through regulation of slit diaphragm proteins, antioxidative enzymes, and Notch pathways in podocytes and suggest that Mafb could be a therapeutic target.

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“…In a patient with sporadic ICTO, Connor et al found that the renal lesion was focal segmental glomerular sclerosis in early and subsequent renal biopsies. This patient was successfully treated with cyclosporin A. Proteinuria was the first sign of renal dysfunction [6].…”

Section: Discussionmentioning

confidence: 95%