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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
Satoshi Takasaki, Kunihiko Maeda, Kensuke Joh, Shu Yamakage, Sachiko Fukase, Toshiyuki Takahashi, Masayuki Suzuki, Akira Matsunaga, Takao Saito
Lipoprotein glomerulopathy (LPG) is characterized by histopathological features showing intra-glomerular lipoprotein thrombi and type III hyperlipoproteinemia (HLP), with heterozygote mutation of apolipoprotein (apo) E gene. On the other hand, as another renal lipidosis with type III HLP, apoE2 homozygote-related glomerulopathy (apoE2-GN) showing foamy macrophages has been reported. The case of a 25-year-old man who had LPG by clinical behavior and gene analysis, but demonstrated atypical histopathological features with a substantial amount of foamy macrophage infiltration in the glomeruli, is presented...
September 2015: Case Reports in Nephrology and Dialysis
Rumina Zaman, Alec Maggi, Sudeep K Rajpoot, Divya-Devi Joshi
Glomerulocystic kidney disease (GCKD) is a rare condition comprising heritable and non-heritable types [Oh et al.: Nephron 1986;43:299-302]. Hepatoblastoma is a sporadically occurring tumor of embryonal origin that is associated with overgrowth syndrome and renal cysts. A concurrent presentation of GCKD with hepatoblastoma was first described in 1989 [Rao et al.: Jpn J Surg 1989;19:583-585]. We report the simultaneous presentation of hepatoblastoma and GCKD in a 5-month-old child and explore the probability of insulin-like growth factors, insulin-like growth factor-binding protein and Beckwith-Wiedemann gene mutation as a putative cause...
September 2015: Case Reports in Nephrology and Dialysis
Akinari Sekine, Eiko Hasegawa, Rikako Hiramatsu, Koki Mise, Keiichi Sumida, Toshiharu Ueno, Masayuki Yamanouchi, Noriko Hayami, Tatsuya Suwabe, Junichi Hoshino, Naoki Sawa, Kenmei Takaichi, Kenichi Ohashi, Takeshi Fujii, Yoshifumi Ubara
Renovascular lesions of lupus nephritis (LN) were classified into five categories by D'Agati in Heptinstall's Pathology of the Kidney, with thrombotic microangiopathy (TMA) and clinical thrombotic thrombocytopenic purpura (TTP) being combined. We encountered 2 cases with histological LN (class III and lass V), and they presented with clinical features of TTP, such as acute renal failure, microangiopathic hemolytic anemia, thrombocytopenia, fever, and central neurologic symptoms. Immunosuppressive therapy with plasmapheresis was performed in both patients...
September 2015: Case Reports in Nephrology and Dialysis
Aya Shima, Takaichi Suehiro, Misaki Takii, Hiroyasu Soeda, Makoto Hirakawa
Ceftriaxone (CTRX) is a third-generation cephalosporin widely used for the treatment of bacterial infections in patients with renal disease because of its excretion by both renal and hepatic mechanisms. Biliary pseudolithiasis is a known CTRX-associated complication; however, there have been no studies of this adverse event in adult patients receiving maintenance hemodialysis. Here we report the case of a 79-year-old Japanese woman with end-stage renal disease (ESRD) receiving maintenance hemodialysis who developed CTRX-induced pseudolithiasis...
September 2015: Case Reports in Nephrology and Dialysis
Jin Hae Kim, Ji Won Kim, Young Nam Kim, Hye In Kim, Jun Young Kim, Ghee Young Kwon, Kihyun Kim, Hye Ryoun Jang
Monoclonal gammopathy with undetermined significance (MGUS) carries a risk of progression to multiple myeloma, and progression is usually diagnosed with changes in M-protein or bone marrow biopsy. We report a case of 62-year-old female patient showing MGUS progression to multiple myeloma without significant changes in M-protein but diagnosed by kidney biopsy. During the follow-ups, azotemia and tubular proteinuria were aggravated without elevation of M-protein. Kidney biopsy showed intratubular and glomerular inclusions associated with plasma cell dysplasia...
September 2015: Case Reports in Nephrology and Dialysis
Jumpei Hasegawa, Junichi Hoshino, Tatsuya Suwabe, Noriko Hayami, Keiichi Sumida, Koki Mise, Toshiharu Ueno, Naoki Sawa, Atsushi Wake, Kenichi Ohashi, Takeshi Fujii, Kazuho Honda, Kenmei Takaichi, Yoshifumi Ubara
A 65-year-old woman was admitted to our hospital for the evaluation of rapidly progressive renal dysfunction with serum creatinine of 2.7 mg/dl and urinary protein of 1.5 g daily. C-reactive protein (CRP) was 0.1 mg/dl. Kidney-limited intravascular large B-cell lymphoma (IVL) localized to the glomerular capillaries was diagnosed because the intraglomerular cells were positive for CD20 and CD79a, while there was no positivity in the extraglomerular kidney and extrarenal organs. Treatment with rituximab, cyclophosphamide, hydroxydaunomycin, vincristine, and prednisolone was started, and the patient has since been doing well...
May 2015: Case Reports in Nephrology and Dialysis
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