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Case Reports in Nephrology and Dialysis

Junko Yabuuchi, Tatsuya Suwabe, Toshiharu Ueno, Junichi Hoshino, Akinari Sekine, Noriko Hayami, Masahiko Oguro, Kyohei Kunisawa, Masahiro Kawada, Masayuki Yamanouchi, Keiichi Sumida, Hiroki Mizuno, Eiko Hasegawa, Naoki Sawa, Kenmei Takaichi, Kenichi Ohashi, Takeshi Fujii, Yoshifumi Ubara
We report a case of glomerulopathy in a 36-year-old Japanese woman with primary Sjögren syndrome (pSS). The first renal biopsy suggested membranous glomerulonephritis. However, repeat biopsy was performed after 16 years because of increased proteinuria, revealing membranoproliferative glomerulonephritis with mesangial deposits, subendothelial deposits, and subepithelial deposits. Immunofluorescent studies showed predominant deposition of IgG2 and IgG4. This patient was positive for antinuclear antibody and anti-SS-A antibody...
September 2016: Case Reports in Nephrology and Dialysis
Doriana Ferrara, Michele Di Filippo, Flavia Spalla, Anna Maria Giribono, Emanuela Viviani, Annamaria Santagata, Umberto Bracale, Michele Santangelo, Luca Del Guercio, Umberto Marcello Bracale
The usual manifestation of brachial artery aneurysms is the incidental finding of a swelling of the arm, combined with paresthesia or pain in some cases. The etiology is often traumatic or secondary to drug abuse. Pathophysiology of brachial artery dilation in these cases is not completely clear. We herein describe a case of a 61-year-old male presenting with a giant, painful, pulsatile mass on his left arm. He was submitted to a cadaveric kidney transplant in 2005. He had a functioning arteriovenous fistula (AVF) on his right arm, and a spontaneously thrombosed radiocephalic AVF on his left arm...
September 2016: Case Reports in Nephrology and Dialysis
Valentina Dolcemascolo, Marina Vivarelli, Manuela Colucci, Francesca Diomedi-Camassei, Rossella Piras, Marta Alberti, Francesco Emma
Hemolytic uremic syndrome (HUS) is defined by the simultaneous occurrence of hemolytic anemia, thrombocytopenia, and acute kidney injury due to thrombotic microangiopathy (TMA) mainly occurring in renal and cerebral microvessels. Although the most common cause of HUS in children is Shiga toxin-producing Escherichia coli, atypical forms in which Shiga toxin is not the trigger may occur. Research over the last few years has shown that complement dysregulation secondary to mutations of genes coding for proteins involved in the regulation of the alternative pathway of complement account for most forms of atypical HUS (aHUS)...
September 2016: Case Reports in Nephrology and Dialysis
Varun Agrawal, Jonathan B Wilfong, Christopher E Rich, Pamela C Gibson
Hyperoxaluria after Roux-en-Y gastric bypass (RYGB) increases the risk for kidney injury. Medical therapies for hyperoxaluria have limited efficacy. A 65-year-old female was evaluated for acute kidney injury [AKI, serum creatinine (Cr) 2.1 mg/dl, baseline Cr 1.0 mg/dl]. She did not have any urinary or gastrointestinal symptoms or exposure to nephrotoxic agents. Sixteen months prior to this evaluation, she underwent RYGB for morbid obesity. Her examination was unremarkable for hypertension or edema and there was no protein or blood on urine dipstick...
September 2016: Case Reports in Nephrology and Dialysis
Yoichi Iwafuchi, Tetsuo Morioka, Yuko Oyama, Kandai Nozu, Kazumoto Iijima, Ichiei Narita
Many types of inherited renal diseases have ocular features that occasionally support a diagnosis. The following study describes an unusual example of a 40-year-old woman with granular corneal dystrophy type II complicated by renal involvement. These two conditions may coincidentally coexist; however, there are some reports that demonstrate an association between renal involvement and granular corneal dystrophy type II. Granular corneal dystrophy type II is caused by a mutation in the transforming growth factor-β-induced (TGFBI) gene...
September 2016: Case Reports in Nephrology and Dialysis
Ryan Kunjal, Rabie Adam-Eldien, Raafat Makary, Francois Jo-Hoy, Charles W Heilig
We report the case of a 22-year-old African American female who presented to another facility for routine follow-up in the 34th week of pregnancy with lower extremity swelling and nephrotic-range proteinuria. Although she was normotensive, it was initially thought that she had preeclampsia. She was monitored carefully and delivery was induced at 37 weeks of gestation. She was transferred to our hospital, where she was diagnosed with systemic lupus erythematosus (SLE) based on clinical and laboratory criteria...
September 2016: Case Reports in Nephrology and Dialysis
Takayasu Ito, Eiji Ishikawa, Hiroshi Matsuo, Mika Fujimoto, Tomohiro Murata, Kenichi Isoda, Hitoshi Mizutani, Masaaki Ito
Renal failure and infectious disease are strongly associated with morbidity and mortality in patients with severe generalized recessive dystrophic epidermolysis bullosa (RDEB-sev gen). However, it is reportedly difficult to introduce hemodialysis with an arteriovenous fistula (AVF). We encountered a 32-year-old man with RDEB-sev gen in whom hemodialysis with a native AVF was introduced that favorably affected his long-term survival. This patient eventually died because of cachexia related to the recurrence of cutaneous squamous cell carcinoma 51 months after hemodialysis introduction...
May 2016: Case Reports in Nephrology and Dialysis
Zeyuan Lu, Jianyong Yin, Hongda Bao, Qiong Jiao, Huijuan Wu, Rui Wu, Qin Xue, Niansong Wang, Zhigang Zhang, Feng Wang
INTRODUCTION: IgG4-related disease (IgG4-RD) is a fibroinflammatory disorder that may involve almost each organ or system. IgG4-related kidney disease (IgG4-RKD) refers to renal lesions associated with IgG4-RD. The most frequent morphological type of renal lesions is IgG4-related tubulointerstitial nephritis (IgG4-TIN) which is associated with increased IgG4-positive plasma cell infiltration and interstitial fibrosis. CASE REPORT: Herein, we present a rare case with coexisting IgG4-RKD and acute crescent glomerulonephritis with concomitant severe tubulointerstitial lesions instead of classic IgG4-TIN...
May 2016: Case Reports in Nephrology and Dialysis
Keiichi Kubota, Junichi Hoshino, Toshiharu Ueno, Koki Mise, Ryo Hazue, Akinari Sekine, Junko Yabuuchi, Masayuki Yamanouchi, Tatsuya Suwabe, Koichi Kikuchi, Keiichi Sumida, Noriko Hayami, Naoki Sawa, Kenmei Takaichi, Takeshi Fujii, Kenichi Ohashi, Shinichi Akiyama, Shoichi Maruyama, Yoshifumi Ubara
A 95-year-old woman was admitted to our hospital for evaluation of bilateral lower-limb edema persisting for 3 months. Serum creatinine was 1.55 mg/dl, and urinary protein excretion was 9.1 g/day. Renal biopsy revealed stage 1 membranous glomerulonephritis (MGN) with immunoglobulin G4-dominant staining. This patient did not have any underlying disease such as infection with hepatitis B or C virus or malignancy, and anti-phospholipase A2 receptor (PLA2R) antibody was detected in the serum. Accordingly, idiopathic MGN was diagnosed...
May 2016: Case Reports in Nephrology and Dialysis
Katrina E Wardrope, Lynn Manson, Wendy Metcalfe, Eoin D O Sullivan
The anti-CD20 monoclonal antibody rituximab is associated with rare but significant adverse events, notably posterior reversible encephalopathy syndrome (PRES) and acute respiratory distress syndrome (ARDS). We report a case of concomitant ARDS and PRES developing after rituximab therapy for treatment of cryoglobulinaemic vasculitis. There are 7 reported cases of PRES complicating rituximab use. PRES onset varied from immediate to 21 days after administration. All patients recovered completely, and rituximab was reintroduced in half of the cases...
January 2016: Case Reports in Nephrology and Dialysis
Camilla Grøndahl, Søren Rittig, Johan Vestergaard Povlsen, Kostantinos Kamperis
Acute postinfectious glomerulonephritis (PIGN) affects children typically after upper respiratory tract or skin infections with streptococci but can complicate the course of other infections. In children, it is generally a self-limiting disease with excellent prognosis. This paper reports a previously healthy 4-year-old boy who experienced a protracted course of PIGN with persisting episodes of gross haematuria, proteinuria, decreased complement C3c levels but normal P-creatinine levels. Due to the protracted course and the nephrotic-range proteinuria, a renal biopsy was performed 6 months after the initial presentation and the overall pathology was consistent with acute endocapillary glomerulonephritis...
January 2016: Case Reports in Nephrology and Dialysis
Yoichi Iwafuchi, Tetsuo Morioka, Takashi Morita, Toshio Yanagihara, Yuko Oyama, Naoya Morisada, Kazumoto Iijima, Ichiei Narita
A common renal phenotype of paired box protein 2 (PAX2) mutations is renal coloboma syndrome. We report a single family with diverse renal phenotypes associated with PAX2 mutation. The proband presented steroid-resistant focal segmental glomerulosclerosis with optic coloboma, whereas his two sons showed severe renal hypoplasia with end-stage renal disease, with or without optic coloboma. In all three cases, a heterozygous PAX2 genetic mutation was identified (exon 2; NM_003987.3:c.76dupG, p.Val26Glyfs*28). Based on histopathological findings of the proband, we hypothesized that autophagic dysfunction was associated with the pathophysiology of the focal segmental glomerulosclerosis with PAX2 mutation...
January 2016: Case Reports in Nephrology and Dialysis
Minh-Ha Tran, Cynthia Chan, Whitney Pasch, Philip Carpenter, Hirohito Ichii, Clarence Foster
Focal segmental glomerulosclerosis (FSGS) causes glomerular lesions that can progress to end-stage renal disease. It is suspected to be caused by a circulating factor that is amenable to plasmapheresis removal and exhibits a risk for recurrence in the renal allograft. We present two patients with FSGS recurrence in their allograft kidneys diagnosed by biopsy after significant proteinuria developed in the posttransplant setting. Treatment with therapeutic plasma exchange induced long-term remission in both patients...
January 2016: Case Reports in Nephrology and Dialysis
Özgür Can, Başak Boynueğri, Ali Murat Gökçe, Ebru Özdemir, Ferhat Ferhatoğlu, Mustafa Canbakan, Gülizar Manga Şahin, Mesut İzzet Titiz, Süheyla Apaydın
Brown tumors are focal bone lesions, encountered in patients with uncontrolled hyperparathyroidism. They can be located in any part of the skeleton. Clinically significant lesions in the craniofacial bones are rare. Craniofacial involvement may cause facial disfiguration and compromise social ease of the patient and normal functions, such as chewing, talking, and breathing. In this case report, we present a patient with a brown tumor of the craniofacial bones provoked by secondary hyperparathyroidism and review the last 10 years of craniofacial brown tumors associated with secondary hyperparathyroidism in the English literature...
January 2016: Case Reports in Nephrology and Dialysis
Shuhei Watanabe, Keiji Kono, Hideki Fujii, Kentaro Nakai, Shunsuke Goto, Shinichi Nishi
We encountered 2 cases of hypophosphatemia due to Legionella pneumonia. Both cases showed increased urinary phosphate excretion and renal tubular dysfunction, which ameliorated with recovery from Legionella pneumonia. Serum fibroblast growth factor-23 level was suppressed, whereas serum 1,25(OH)2 vitamin D and parathyroid hormone levels were normal. Delayed elevation of serum 1,25(OH)2 vitamin D levels was observed with improvement in renal tubular function. These findings suggested hypophosphatemia might be mediated by renal tubular dysfunction...
January 2016: Case Reports in Nephrology and Dialysis
Dean Markić, Kristian Krpina, Juraj Ahel, Antun Gršković, Josip Španjol, Nino Rubinić, Mauro Materljan, Ivana Mikolašević, Lidija Orlić, Sanjin Rački
We report a case of a kidney-transplanted patient with urolithiasis treated with mini-percutaneous laser lithotripsy. The patient presented with renal dysfunction and graft hydronephrosis. Diagnostic procedures revealed ureterolithiasis as a cause of obstruction, and percutaneous nephrostomy was inserted as a temporary solution. Before surgery, the stone migrated to the renal pelvis. Mini-percutaneous laser lithotripsy was successfully performed, and during surgery, all stone fragments were removed. Six months after successful treatment, the patient has good functioning and stone-free graft...
January 2016: Case Reports in Nephrology and Dialysis
Chia Wei Teoh, Avnesh S Thakor, Joao G Amaral, Dimitri A Parra, Elizabeth A Harvey, Damien G Noone
BACKGROUND: Cuffed, double-lumen, tunneled haemodialysis catheters are a common means of vascular access in paediatric haemodialysis, particularly in infants. Haemodialysis catheter fracture with distal embolization is a rare complication. CASE REPORT: A 2-year-old boy was receiving chronic haemodialysis via a right internal jugular cuffed, double-lumen, tunneled haemodialysis catheter, inserted 3 months previously. He was asymptomatic and was incidentally found to have had embolization of a fractured catheter tip into a segmental branch of the left pulmonary artery...
January 2016: Case Reports in Nephrology and Dialysis
Yoichi Iwafuchi, Tetsuo Morioka, Takashi Morita, Kanako Watanabe, Yuko Oyama, Ichiei Narita
Nephrotic syndrome without hematuria due to infection-related glomerulonephritis is uncommon. The present report describes a case of nephrotic syndrome due to infection-related glomerulonephritis without hematuria and hypertension in an older child. A 14-year-old boy was referred to our hospital because of a 5-day history of fever, nausea, weight gain and recent leg edema without hypertension. Laboratory data showed nephrotic-range proteinuria, hypoalbuminemia, mild hypocomplementemia and acute renal injury without hematuria...
January 2016: Case Reports in Nephrology and Dialysis
Anthony Valeri, Bobby Lee, John Duffy, Robin Ferrer, Ronald Vilotta
Installation of the Nephros Dual Stage Ultrafilter (DSU) added to a conventional hemodialysis unit to achieve ultrapure dialysate was tested in a group of 23 stable outpatients on chronic hemodialysis. Comparing the 6-month period prior to the installation of the filters (as baseline) to the 6-month period after the installation of the filters, we found a significant 40% reduction in the darbepoetin dose needed to maintain a stable hemoglobin level (p < 0.001). In addition, surrogate inflammatory markers, WBC count and serum albumin level, showed small but statistically significant improvements (p = 0...
January 2016: Case Reports in Nephrology and Dialysis
Jinxian Huang, Ling Wu, Xiaoyan Huang, Yan Xie, Jinquan Yu, Jin Yang, Huiqiong Fang, Lijun Zhang
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and anti-glomerular basement membrane (GBM) disease are two separate diseases, while sometimes they can coexist together. The exact mechanisms are not clear, but due to the rapid progression and poor prognosis, prompt and aggressive treatment is usually required. We treated with steroids combined with cyclophosphamide and rituximab an 84-year-old man with ANCA-associated vasculitis and anti-GBM disease who had prior pulmonary fibrosis and a coexisting anterosuperior mediastinal mass...
January 2016: Case Reports in Nephrology and Dialysis
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