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A Case of Crescentic Glomerulonephritis Complicated with Hypocomplementemic Urticarial Vasculitis Syndrome and ANCA-Associated Vasculitis
Systemic urticaria in a 64-year-old woman was diagnosed as leukocytoclastic vasculitis by a punch biopsy of the skin. Her physical findings improved after prescription of prednisolone at a dose of 20 mg/day, but the skin rash relapsed with renal dysfunction, proteinuria, and hematuria when the dose of prednisolone was reduced over a period of 9 months to 1 mg/day. She was admitted to our institute for further examination, when urinary protein and plasma creatinine levels were 0.8 g/day and 1.7 mg/dL, respectively. Complement analysis showed that levels of total hemolytic component, component C3 fraction, and component C4 f...
Source: Case Reports in Nephrology and Dialysis - November 27, 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 - October 25, 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 - October 25, 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 - October 25, 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 - October 25, 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 - October 25, 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 - October 25, 2017 Category: Urology & Nephrology Source Type: research

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 - October 25, 2017 Category: Urology & Nephrology Source Type: research

Tolvaptan for the Treatment of Enlarged Polycystic Liver Disease
A 44-year-old Japanese woman with autosomal dominant polycystic kidney disease was admitted to our hospital for evaluation of abdominal distension. Her eGFR was 53.7 mL/min/1.73 m2. Total kidney volume was 2,614 mL. Tolvaptan (60 mg/day) was started to treat renal involvement. The patient ’s abdominal fullness began to improve and liver volume, indicating advanced polycystic liver disease (PLD), decreased from 9,750 mL to 8,345 mL after 17 months of tolvaptan treatment, though there was no significant change in kidney volume. This case indicates that tolvaptan may be a therapeutic option for hepatomegaly in patients with...
Source: Case Reports in Nephrology and Dialysis - October 25, 2017 Category: Urology & Nephrology Source Type: research

A Case of Lipoprotein Glomerulopathy with apoE Chicago and apoE (Glu3Lys) Treated with Fenofibrate
Lipoprotein glomerulopathy (LPG) is characterized by the accumulation of lipoprotein thrombi within glomerular capillaries. This rare disorder is associated with various types of mutations in the apolipoprotein E gene (apoE). Herein, we present a case of LPG with a combination ofapoE Chicago (Arg147Pro) andapoE (Glu3Lys) mutations. A 51-year-old Japanese woman presented with severe (3+) proteinuria. The initial renal biopsy showed glomerular capillary dilation and occlusion with lipid granules, a specific characteristic of LPG. Phenotype, genotype, andapoE DNA sequence analyses detected 2 mutations as described above withi...
Source: Case Reports in Nephrology and Dialysis - October 25, 2017 Category: Urology & Nephrology Source Type: research

Novel de novo AVPR2 Variant in a Patient with Congenital Nephrogenic Diabetes Insipidus
In this study, a Swedish male with no family history was diagnosed with CNDI at 6 months of age during an episode of gastroenteritis. We analyzed the coding regions ofAVPR2 by PCR and direct DNA sequencing and identified an 80-bp duplication in exon 2 (GenBank NM_000054.4; c.800_879dup) in the proband. This variant leads to a frameshift and introduces a stop codon four codons downstream (p.Ala294Profs*4). The variant gene product either succumbs to nonsense-mediated decay or is translated to a truncated nonfunctional vasopressin V2 receptor. This variant was absent in four unaffected family members, including his parents, ...
Source: Case Reports in Nephrology and Dialysis - October 25, 2017 Category: Urology & Nephrology Source Type: research

Novel de novo AVPR2 Variant in a Patient with Congenital Nephrogenic Diabetes Insipidus
In this study, a Swedish male with no family history was diagnosed with CNDI at 6 months of age during an episode of gastroenteritis. We analyzed the coding regions ofAVPR2 by PCR and direct DNA sequencing and identified an 80-bp duplication in exon 2 (GenBank NM_000054.4; c.800_879dup) in the proband. This variant leads to a frameshift and introduces a stop codon four codons downstream (p.Ala294Profs*4). The variant gene product either succumbs to nonsense-mediated decay or is translated to a truncated nonfunctional vasopressin V2 receptor. This variant was absent in four unaffected family members, including his parents, ...
Source: Case Reports in Nephrology and Dialysis - September 27, 2017 Category: Urology & Nephrology Source Type: research

A Case of Lipoprotein Glomerulopathy with apoE Chicago and apoE (Glu3Lys) Treated with Fenofibrate
Lipoprotein glomerulopathy (LPG) is characterized by the accumulation of lipoprotein thrombi within glomerular capillaries. This rare disorder is associated with various types of mutations in the apolipoprotein E gene (apoE). Herein, we present a case of LPG with a combination ofapoE Chicago (Arg147Pro) andapoE (Glu3Lys) mutations. A 51-year-old Japanese woman presented with severe (3+) proteinuria. The initial renal biopsy showed glomerular capillary dilation and occlusion with lipid granules, a specific characteristic of LPG. Phenotype, genotype, andapoE DNA sequence analyses detected 2 mutations as described above withi...
Source: Case Reports in Nephrology and Dialysis - July 27, 2017 Category: Urology & Nephrology Source Type: research

Tolvaptan for the Treatment of Enlarged Polycystic Liver Disease
A 44-year-old Japanese woman with autosomal dominant polycystic kidney disease was admitted to our hospital for evaluation of abdominal distension. Her eGFR was 53.7 mL/min/1.73 m2. Total kidney volume was 2,614 mL. Tolvaptan (60 mg/day) was started to treat renal involvement. The patient ’s abdominal fullness began to improve and liver volume, indicating advanced polycystic liver disease (PLD), decreased from 9,750 mL to 8,345 mL after 17 months of tolvaptan treatment, though there was no significant change in kidney volume. This case indicates that tolvaptan may be a therapeutic option for hepatomegaly in patients with...
Source: Case Reports in Nephrology and Dialysis - July 19, 2017 Category: Urology & Nephrology Source Type: research