AA Amyloidosis and Atypical Familial Mediterranean Fever with Exon 2 and 3 Mutations
A 54-year-old Japanese man presented with recurrent abdominal pain, fever lasting #x3e;5 days, and renal failure. AA amyloidosis was proven by renal and gastric biopsy. Symptoms subsided with the administration of colchicine, but a subsequent recurrence of symptoms did not respond to colchicine. Mediterranean fever gene (MEFV) analysis showed that he was heterozygous for mutations in exon 2 (E148Q/R202Q) and exon 3 (P369S/R408Q), although he had none of the exon 10 mutations known to be closely related to AA amyloidosis. He did not respond to infliximab, but tocilizumab therapy was successful. The present case is a rare re...
Source: Case Reports in Nephrology and Dialysis - July 11, 2017 Category: Urology & Nephrology Source Type: research

Hemodialysis Arteriovenous Access Occlusion Using the Amplatzer Vascular Plug in Patients with Intractable Arm Edema
Conclusion: This report suggests that the use of AVP for embolization of complicated AVA is a safe and reasonable alternative to open surgery in selected patients.Case Rep Nephrol Dial 2017;7:63 –72 (Source: Case Reports in Nephrology and Dialysis)
Source: Case Reports in Nephrology and Dialysis - June 23, 2017 Category: Urology & Nephrology Source Type: research

A Toddler Presenting with Pulmonary Renal Syndrome
In this report, we discuss the differential diagnosis, diagnostic studies, and treatment options to consider when facing a young child presenting with a pulmonary renal syndrome.Case Rep Nephrol Dial 2017;7:73 –80 (Source: Case Reports in Nephrology and Dialysis)
Source: Case Reports in Nephrology and Dialysis - June 23, 2017 Category: Urology & Nephrology Source Type: research

Case Report of Spontaneous Remission of Biopsy-Proven Idiopathic Immune Complex-Mediated Membranoproliferative Glomerulonephritis
Membranoproliferative glomerulonephritis (MPGN) is a histopathologic diagnosis causing microscopic hematuria, nephrotic range proteinuria, and chronic renal failure. Current understanding divides pathogenesis into two broad categories: immune complex mediated and complement mediated (now termed C3 glomerulopathy). The term idiopathic immune complex-mediated MPGN would apply to a patient without an identifiable source of immune complex production and no evidence of C3 glomerulopathy. Presented is a patient with idiopathic immune complex mediated MPGN and her clinical course. The patient opted for conservative therapy with l...
Source: Case Reports in Nephrology and Dialysis - June 23, 2017 Category: Urology & Nephrology Source Type: research

Tubuloreticular Inclusions in the Absence of Systemic Lupus Erythematosus and HIV Infection: A Report of Three Pediatric Cases
In conclusion, renal biopsy with TRIs in the absence of underling SLE and HIV remains obscure. We propose a possible role for excess IFN triggered by an abnormal immune response to common viral infections in the formation of TRIs and renal injury.Case Rep Nephrol Dial 2017;7:91 –101 (Source: Case Reports in Nephrology and Dialysis)
Source: Case Reports in Nephrology and Dialysis - June 23, 2017 Category: Urology & Nephrology Source Type: research

Renal-Limited “Lupus-Like” Nephritis: How Much of a Lupus
Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by development of autoantibodies to nuclear and cytoplasmic antigens. A small subset of SLE patients who had the typical clinical features of SLE was reported to show persistently negative antinuclear antibody tests. Our report describes a 5-year-old male who presented with histopathological findings suggestive of lupus nephritis with no clinical signs or symptoms of SLE and negative autoantibodies. He was treated with corticosteroids, mycophenolate mofetil, and monthly intravenous cyclophosphamide. During the 2-year follow-up period, the proteinuria...
Source: Case Reports in Nephrology and Dialysis - June 2, 2017 Category: Urology & Nephrology Source Type: research

Erdheim-Chester Disease Presenting with Secondary Hypertension as a Result of Bilateral, Proximal Renal Artery Stenosis: A Case Report
We present a known case of ECD, which was referred for secondary hypertension workup and diagnosed with severe, proximal, bilateral renal artery stenosis.Case Rep Nephrol Dial 2017;7:55 –62 (Source: Case Reports in Nephrology and Dialysis)
Source: Case Reports in Nephrology and Dialysis - May 23, 2017 Category: Urology & Nephrology Source Type: research

Renal-Limited “Lupus-Like” Nephritis: How Much of a Lupus?
Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by development of autoantibodies to nuclear and cytoplasmic antigens. A small subset of SLE patients who had the typical clinical features of SLE was reported to show persistently negative antinuclear antibody tests. Our report describes a 5-year-old male who presented with histopathological findings suggestive of lupus nephritis with no clinical signs or symptoms of SLE and negative autoantibodies. He was treated with corticosteroids, mycophenolate mofetil, and monthly intravenous cyclophosphamide. During the 2-year follow-up period, the proteinuria...
Source: Case Reports in Nephrology and Dialysis - May 9, 2017 Category: Urology & Nephrology Source Type: research

Cisplatin Therapy Does Not Worsen Renal Function in Severe Antenatal Bartter Syndrome
A 30-year-old man with severe antenatal Bartter syndrome, diagnosed and treated in infancy, developed testicular carcinoma. Despite the known renal complications of cisplatin, this drug was used for his chemotherapy because of its superior antineoplastic effect. Nonsteroidal anti-inflammatory drug administration was continued during cisplatin therapy. Despite an increase in his oral potassium requirement, renal function was maintained following completion of chemotherapy. In spite of its significant associated nephrotoxicity, cisplatin can be used in patients with severe antenatal Bartter syndrome if required for therapy o...
Source: Case Reports in Nephrology and Dialysis - May 9, 2017 Category: Urology & Nephrology Source Type: research

Extracranial Aneurysms in 2 Patients with Autosomal Recessive Polycystic Kidney Disease
We present 2 ARPKD patients with extracranial vascular abnormalities: a young man with infrarenal aortic and iliac artery aneurysms complicated by dissection and a teenage girl with multiple splenic and gastric artery aneurysms and arterial vascular malformations. These cases raise the question of whether vascular integrity and development may be impaired in ARPKD, perhaps through molecular mechanisms overlapping with ADPKD. This possibility is supported by studies in mice that show ARPKD gene expression in the walls of large blood vessels.Case Rep Nephrol Dial 2017;7:34 –42 (Source: Case Reports in Nephrology and Dialysis)
Source: Case Reports in Nephrology and Dialysis - May 2, 2017 Category: Urology & Nephrology Source Type: research

A case of fibrillary glomerulonephritis with fibril deposition in the arteriolar wall and a family history of renal disease
Case Rep Nephrol Dial 2017;7:26 –33 (Source: Case Reports in Nephrology and Dialysis)
Source: Case Reports in Nephrology and Dialysis - April 5, 2017 Category: Urology & Nephrology Source Type: research

Discontinuation of Peritoneal Dialysis after Late Initiation of Eculizumab in a Case of Familial Atypical Hemolytic-Uremic Syndrome: A Case Report
Conclusions: This case shows that eculizumab treatment for patients with atypical hemolytic-uremic syndrome, even when initiated many months after beginning on dialysis, might offer substantial benefits and improve the patients ’ quality of life.Case Rep Nephrol Dial 2017;7:18 –25 (Source: Case Reports in Nephrology and Dialysis)
Source: Case Reports in Nephrology and Dialysis - March 2, 2017 Category: Urology & Nephrology Source Type: research

Neurotoxicity following the Ingestion of Bilimbi Fruit (Averrhoa bilimbi) in an End-Stage Renal Disease Patient on Hemodialysis
Conclusions: The neurologic status without any other known cause and with clear temporal association with the ingestion of the fruit suggests the diagnosis of neurotoxicity. We propose the hypothesis that the bilimbi fruit has neurotoxic effects similar to those exhibited by the star fruit.Case Rep Nephrol Dial 2017;7:6 –12 (Source: Case Reports in Nephrology and Dialysis)
Source: Case Reports in Nephrology and Dialysis - February 3, 2017 Category: Urology & Nephrology Source Type: research

Neurotoxicity following the Ingestion of Bilimbi Fruit ( < b > < i > Averrhoa bilimbi < /i > < /b > ) in an End-Stage Renal Disease Patient on Hemodialysis
Conclusions: The neurologic status without any other known cause and with clear temporal association with the ingestion of the fruit suggests the diagnosis of neurotoxicity. We propose the hypothesis that the bilimbi fruit has neurotoxic effects similar to those exhibited by the star fruit.Case Rep Nephrol Dial 2017;7:6 –12 (Source: Case Reports in Nephrology and Dialysis)
Source: Case Reports in Nephrology and Dialysis - January 20, 2017 Category: Urology & Nephrology Source Type: research

Tubular Dysfunction Mimicking Dent ’s Disease in 2 Infants Born with Extremely Low Birth Weight
Two preterm infants, with extremely low birth weight born at gestational weeks 24 and 25, showed generalized proximal tubular dysfunction during their stay in the neonatal intensive care unit, including glucosuria, low molecular weight proteinuria, phosphaturia, uricosuria, enzymuria (elevated urine N-acetyl- β-D-glucosaminidase), panaminoaciduria, and hypercalciuria, associated with renal calcification. Renal tubular acidosis was not present in either patient. DNA mutation analysis for Dent’s disease, performed in patient 1, was negative. Although both patients had rickets of prematurity, tubular dys function persisted...
Source: Case Reports in Nephrology and Dialysis - January 20, 2017 Category: Urology & Nephrology Source Type: research