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Case Reports in Nephrology and Dialysis

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https://www.readbyqxmd.com/read/28612007/erdheim-chester-disease-presenting-with-secondary-hypertension-as-a-result-of-bilateral-proximal-renal-artery-stenosis-a-case-report
#1
Farid Arman, Hania Shakeri, Niloofar Nobakht, Anjay Rastogi, Mohammad Kamgar
Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis presenting most commonly with bone and central nervous system symptoms, including but not limited to bone pain and diabetes insipidus. We present a known case of ECD, which was referred for secondary hypertension workup and diagnosed with severe, proximal, bilateral renal artery stenosis.
May 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28612006/cisplatin-therapy-does-not-worsen-renal-function-in-severe-antenatal-bartter-syndrome
#2
Thomas R Welch, David R Shaffer, Darren R Feldman
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 of malignancy...
May 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28612005/renal-limited-lupus-like-nephritis-how-much-of-a-lupus
#3
Asmaa S Abu Maziad, Jose Torrealba, Mouin G Seikaly, Jared R Hassler, Allen R Hendricks
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...
May 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28612004/extracranial-aneurysms-in-2-patients-with-autosomal-recessive-polycystic-kidney-disease
#4
Daniel S Elchediak, Anne Marie Cahill, Emma E Furth, Bernard S Kaplan, Erum A Hartung
Unlike autosomal dominant polycystic kidney disease (ADPKD), autosomal recessive polycystic kidney disease (ARPKD) is not generally known to be associated with vascular abnormalities. Only 4 cases of ARPKD patients with intracranial aneurysms have been reported previously. 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...
May 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28612003/discontinuation-of-peritoneal-dialysis-after-late-initiation-of-eculizumab-in-a-case-of-familial-atypical-hemolytic-uremic-syndrome-a-case-report
#5
Rafael Alonso Valente, Giannina Elena García Rodríguez, Yanina García Marcote, Manuel Fidalgo Díaz, Vanesa Becerra Mosquera, Daniel Novoa García, Teresa Cordal Martínez, Cándido Díaz Rodríguez
BACKGROUND: Atypical hemolytic-uremic syndrome is caused by a thrombotic microangiopathy and manifests itself with hemolytic anemia, thrombocytopenia, and organ ischemia. Its etiology is a mutation affecting the genes encoding for proteins of the complement system. Early treatment with eculizumab (8.6 months from the moment of presentation), a humanized monoclonal antibody against complement, is shown to be effective in controlling symptoms and reversing organ damage. We present a patient with a mutation not previously described in the literature...
January 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28503551/a-case-of-fibrillary-glomerulonephritis-with-fibril-deposition-in-the-arteriolar-wall-and-a-family-history-of-renal-disease
#6
Kentaro Watanabe, Kentaro Nakai, Nozomi Hosokawa, Shuhei Watanabe, Keiji Kono, Shunsuke Goto, Hideki Fujii, Shigeo Hara, Shinichi Nishi
Herein, we report a case of fibrillary glomerulonephritis (FGN). FGN usually shows non-amyloidal fibrils in the mesangium and glomerular capillary walls on electron microscopy. Inherited cases of FGN have been reported in only 3 families, and the suspected genetic form was autosomal dominant. In the present case, the deposition of microfibrils in the arteriolar wall as well as the glomerulus is unique. Our patient's father died of nephrotic syndrome, and his elder brother had a biopsy-proven glomerulopathy...
January 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28203565/tubular-dysfunction-mimicking-dent-s-disease-in-2-infants-born-with-extremely-low-birth-weight
#7
Midori Awazu, Mie Arai, Shoko Ohashi, Hirotaka Takahashi, Takashi Sekine, Kazushige Ikeda
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...
January 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28203564/neurotoxicity-following-the-ingestion-of-bilimbi-fruit-averrhoa-bilimbi-in-an-end-stage-renal-disease-patient-on-hemodialysis
#8
Camille Pereira Caetano, Cinara Barros de Sá, Bruno Antônio Paixão Faleiros, Marcelo Fonseca Coutinho Fernandes Gomes, Edna Regina Silva Pereira
INTRODUCTION: The toxic effects of the ingestion of star fruit (Averrhoa carambola) in chronic kidney disease patients are well described in the literature. Recently, the compound caramboxin has been isolated, explaining the mechanisms of its neurotoxicity. Bilimbi fruit belongs to the family Oxalidaceae, Averrhoa bilimbi species, and exhibits similar biochemical characteristics to star fruit. OBJECTIVE: To report the case of a patient with chronic kidney disease who developed a seizure disorder after the ingestion of bilimbi fruit...
January 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28203563/unsuccessful-treatment-with-abatacept-in-recurrent-focal-segmental-glomerulosclerosis-after-kidney-transplantation
#9
Tilde Kristensen, Per Ivarsen, Johan Vestergaard Povlsen
Recurrence of focal segmental glomerulosclerosis (FSGS) after renal transplantation occurs in up to 20-50% of FSGS patients and is associated with inferior allograft survival. Treatment of both primary FSGS as well as recurrent FSGS after transplantation with plasma exchange and immunosuppression is often unsuccessful and remains a major challenge as the disease still leads to end-stage renal disease and decreased graft survival. Previous case reports have described patients with recurrent FSGS who were successfully treated with a B7-1 inhibitor (abatacept) inducing partial or complete remission...
January 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28101502/pregnant-woman-with-atypical-hemolytic-uremic-syndrome-delivered-a-healthy-newborn-under-eculizumab-treatment
#10
Erol Demir, Halil Yazici, Yasemin Ozluk, Isin Kilicaslan, Aydin Turkmen
Pregnancy-associated thrombotic microangiopathy is a very rare condition; however, it significantly increases fetal or maternal morbidity and mortality. Pregnancy may trigger atypical hemolytic uremic syndrome (aHUS) or thrombotic thrombocytopenic purpura. The risk for pregnancy-associated aHUS is highest during the second pregnancy. The outcome is usually poor with 50-60% mortality; renal dysfunction and hypertension are the rule in those who survive the acute episode. After the development of complement regulation mechanisms and aHUS pathogenesis, eculizumab has been widely used as a first-line treatment in aHUS...
September 2016: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/27904866/sj%C3%A3-gren-syndrome-related-membranous-glomerulonephritis-progressing-to-membranoproliferative-glomerulonephritis
#11
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
https://www.readbyqxmd.com/read/27904865/giant-true-brachial-artery-aneurysm-after-hemodialysis-fistula-closure-in-a-renal-transplant-patient
#12
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
https://www.readbyqxmd.com/read/27904864/nephrotic-range-proteinuria-and-peripheral-edema-in-a-child-not-only-idiopathic-nephrotic-syndrome
#13
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
https://www.readbyqxmd.com/read/27781207/reversal-of-gastric-bypass-resolves-hyperoxaluria-and-improves-oxalate-nephropathy-secondary-to-roux-en-y-gastric-bypass
#14
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
https://www.readbyqxmd.com/read/27781206/a-case-of-transforming-growth-factor-%C3%AE-induced-gene-related-oculorenal-syndrome-granular-corneal-dystrophy-type-ii-with-a-unique-nephropathy
#15
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
https://www.readbyqxmd.com/read/27781205/a-unique-cause-of-proteinuria-in-pregnancy-class-ii-lupus-nephritis-with-concomitant-minimal-change-disease
#16
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
https://www.readbyqxmd.com/read/28101501/classical-presentation-of-acute-pyelonephritis-in-a-case-of-brucellosis
#17
Wadha Alfouzan, Sara Al-Sahali, Hawra'a Sultan, Rita Dhar
Although Brucella species is known to affect almost all organs in humans, renal involvement presenting as acute pyelonephritis remains a rare entity in brucellosis. We report the case of a female patient who presented with symptoms of fever with chills, right loin pain and dysuria in the emergency room. Blood cultures drawn at the time of admission grew Brucella spp., but no organisms were isolated from urine culture although urinalysis data was indicative of urinary tract infection. Empiric therapy with piperacillin/tazobactam plus gentamicin relieved her symptoms...
May 2016: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/27722157/maintenance-hemodialysis-using-native-arteriovenous-fistula-in-a-patient-with-severe-generalized-recessive-dystrophic-epidermolysis-bullosa
#18
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
https://www.readbyqxmd.com/read/27504450/coexistence-of-acute-crescent-glomerulonephritis-and-igg4-related-kidney-disease
#19
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
https://www.readbyqxmd.com/read/27390744/phospholipase-a2-receptor-positive-idiopathic-membranous-glomerulonephritis-with-onset-at-95-years-case-report
#20
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
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