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Case Reports in Nephrology and Dialysis

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https://www.readbyqxmd.com/read/29594148/c3-glomerulopathy-and-atypical-hemolytic-uremic-syndrome-two-important-manifestations-of-complement-system-dysfunction
#1
Ravneet Bajwa, John A DePalma, Taimoor Khan, Anmol Cheema, Sheila A Kalathil, Mohammad A Hossain, Attiya Haroon, Anne Madhurima, Min Zheng, Ali Nayer, Arif Asif
The advances in our understanding of the alternative pathway have emphasized that uncontrolled hyperactivity of this pathway causes 2 distinct disorders that adversely impact the kidney. In the so-called atypical hemolytic uremic syndrome (aHUS), renal dysfunction occurs along with thrombocytopenia, anemia, and target organ injury to multiple organs, most commonly the kidney. On the other hand, in the so-termed C3 glomerulopathy, kidney involvement is not associated with thrombocytopenia, anemia, or other system involvement...
January 2018: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29594147/oxcarbazepine-therapy-for-complete-central-diabetes-insipidus
#2
Basmah Abdallah, Spencer Hodgins, Daniel Landry, Michael O'shea, Gregory Braden
Oxcarbazepine and carbamazepine cause hyponatremia by unknown mechanisms. We describe a patient with complete central diabetes insipidus and seizures who developed worsening hyponatremia when her dose of oxcarbazepine was increased. The patient maintained a normal serum sodium level and has had appropriately concentrated urine for 5 years on just oxcarbazepine, despite undetectable antidiuretic hormone (ADH) levels. This suggests that oxcarbazepine (or one of its metabolites) may stimulate collecting tubule V2 receptor-G protein complex independent of ADH, resulting in increased renal tubular water reabsorption...
January 2018: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29594146/early-conversion-from-tacrolimus-to-belatacept-in-a-highly-sensitized-renal-allograft-recipient-with-calcineurin-inhibitor-induced-de-novo-post-transplant-hemolytic-uremic-syndrome
#3
Vasishta S Tatapudi, Bonnie E Lonze, Ming Wu, Robert A Montgomery
Background: Kidney transplantation is the first-line therapy for patients with end-stage renal disease since it offers greater long-term survival and improved quality of life when compared to dialysis. The advent of calcineurin inhibitor (CNI)-based maintenance immunosuppression has led to a clinically significant decline in the rate of acute rejection and better short-term graft survival rates. However, these gains have not translated into improvement in long-term graft survival. CNI-related nephrotoxicity and metabolic side effects are thought to be partly responsible for this...
January 2018: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29594145/exit-site-infection-due-to-mycobacterium-chelonae-in-an-elderly-patient-on-peritoneal-dialysis
#4
Arata Hibi, Takahisa Kasugai, Keisuke Kamiya, Chiharu Ito, Satoru Kominato, Toshiyuki Miura, Katsushi Koyama
Nontuberculous mycobacteria (NTM) are rarely isolated from peritoneal dialysis (PD)-associated catheter infections. However, NTM infection is usually difficult to treat and leads to catheter loss. Prompt diagnosis is essential for appropriate treatment. A 70-year-old Japanese man who had been on PD for 2 years and with a medical history of 2 episodes of exit site infections (ESIs) due to methicillin-resistant Staphylococcus aureus was admitted to the hospital due to suspected ESI recurrence. However, Gram staining of the pus revealed no gram-positive cocci...
January 2018: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29594144/an-unexpected-case-of-scurvy-in-a-peritoneal-dialysis-patient
#5
Raymonda El Khoury, Mitchell Warren, Saba Ali, James L Pirkle
This case describes an obese adult male peritoneal dialysis patient who presented with a pruritic follicular rash. Nutrient deficiency was not suspected initially in this case because there was no history of protein-calorie malnutrition, but the patient reported a diet devoid of fruits and vegetables and had not been taking his dialysis vitamin as prescribed. Skin biopsy showed follicular hyperkeratosis with fragmented hair shafts and corkscrew hairs consistent with scurvy. After supplementation with ascorbic acid 500 mg twice daily for 2 weeks, the rash resolved completely...
September 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29594143/a-case-of-crescentic-glomerulonephritis-complicated-with-hypocomplementemic-urticarial-vasculitis-syndrome-and-anca-associated-vasculitis
#6
Marenao Tanaka, Norihito Moniwa, Tomohiro Mita, Toshiyuki Tobisawa, Tamaki Matsumoto, Atsushi Mochizuki, Tomohisa Yamashita, Toshiyuki Yano, Masato Furuhashi, Tetsuji Miura
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...
September 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29594142/rare-bacteria-infecting-the-heart-and-affecting-the-kidney-of-a-young-child
#7
Gurinder Kumar, Alyaa Saeed Al Ali, Namrata Gulzar Bhatti
Rare bacteria can lead to infective endocarditis, which may lead to renal involvement as severe glomerulonephritis. We report our experience of a 12-year-old child who presented with infective endocarditis and blood culture-grown Gemella morbillorum - a rarely reported bacteria. The clinical picture was further complicated with severe glomerulonephritis. Renal biopsy was suggestive of crescentic glomerulonephritis. The child was managed with antibiotics, steroids, and plasmapheresis and responded well to the treatment...
September 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29457022/a-case-report-of-compound-heterozygous-cyp24a1-mutations-leading-to-nephrolithiasis-successfully-treated-with-ketoconazole
#8
Emma Davidson Peiris, Raghav Wusirika
CYP24A1 is an enzyme that inactivates vitamin D. Loss-of-function mutations in this enzyme are rare but have been linked with idiopathic infantile hypercalcemia as well as adult-onset nephrocalcinosis and nephrolithiasis. Genetic testing for this mutation should be considered in the presence of calciuria, elevated serum calcium, elevated 1,25-dihydroxyvitamin D, and suppressed parathyroid hormone. We present a case with these lab findings as well as an elevated 25-hydroxyvitamin D/24,25-dihydroxyvitamin D ratio in whom compound heterozygous CYP24A1 mutations were found...
September 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29457021/difficult-renal-pathological-classification-in-a-case-of-pediatric-nephrotic-syndrome
#9
Hiroshi Yamaguchi, Atsutoshi Shiratori, Taku Nakagawa, Kyoko Kanda, Shigeo Hara, Norishige Yoshikawa, Ryojiro Tanaka
The underlying histopathology is very important in determining patient management, as the histopathology usually has direct repercussions on the treatment response and clinical course. However, the impact of the method used to assess renal biopsies, i.e., light microscopy (LM), immunofluorescence (IF), and electron microscopy (EM), on the occurrence of a difficult biopsy classification in the native kidneys of pediatric nephrotic patients is unknown. A 12-month-old Japanese boy was diagnosed with nephrotic syndrome (NS); he was administered prednisolone (60 mg/m2 /day), and a continuous albumin infusion was started...
September 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29457020/infective-endocarditis-associated-with-streptococcal-toxic-shock-syndrome-due-to-streptococcus-dysgalactiae-subsp-equisimilis-infection-in-a-hemodialysis-patient
#10
Momoko Kan, Yasukiyo Mori, Nao Kishimoto, Hiroki Shibutani, Yuno Tomita, Tomoko Hagino, Eiko Ohira, Masahiro Karakawa
The risk of infective endocarditis in chronic hemodialysis patients is markedly higher than that in the general population. We report the first case of a hemodialysis patient with infective endocarditis caused by Streptococcus dysgalactiae subsp. equisimilis (SDSE) who presented with streptococcal toxic shock syndrome. In the last decade, there has been an increase in the incidence of SDSE infections. Therefore, it is important to recognize SDSE as a possible causative agent of infective endocarditis in an immunocompromised population, such as hemodialysis patients...
September 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29177155/novel-de-novo-avpr2-variant-in-a-patient-with-congenital-nephrogenic-diabetes-insipidus
#11
Shivani Joshi, Per Brandstrom, Niels Gregersen, Søren Rittig, Jane Hvarregaard Christensen
Early diagnosis and treatment of congenital nephrogenic diabetes insipidus (CNDI) are essential due to the risk of intellectual disability caused by repeated episodes of dehydration and rapid rehydration. Timely genetic testing for disease-causing variants in the arginine vasopressin receptor 2 ( AVPR2 ) gene is possible in at-risk newborns with a known family history of X-linked CNDI. In this study, a Swedish male with no family history was diagnosed with CNDI at 6 months of age during an episode of gastroenteritis...
September 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29464179/leukocyte-cell-derived-chemotaxin-2-associated-renal-amyloidosis-a-case-report
#12
Gagandeep Kaur, Babitha Bijin, Kamron Saleem, Benjamin Sarsah, Bijin Thajudeen
Amyloidosis is a disorder characterized by the deposition of abnormal protein fibrils in tissues. Leukocyte cell-derived chemotaxin 2-associated amyloidosis is a recently recognized entity and is characterized by a distinctive clinicopathologic type of amyloid deposition manifested in adults by varying degrees of impaired kidney function and proteinuria. There are only a limited number of cases reported in the literature. We present a 64-year-old Hispanic female with a history of hypertension who was referred for chronic kidney disease management...
May 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29034246/tolvaptan-for-the-treatment-of-enlarged-polycystic-liver-disease
#13
Hiroki Mizuno, Junichi Hoshino, Tatsuya Suwabe, Keiichi Sumida, Akinari Sekine, Yoichi Oshima, Masahiko Oguro, Kyohei Kunizawa, Masahiro Kawada, Rikako Hiramatsu, Noriko Hayami, Eiko Hasegawa, Masayuki Yamanouchi, Naoki Sawa, Kenmei Takaichi, Yoshifumi Ubara
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...
May 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28966924/a-case-of-lipoprotein-glomerulopathy-with-apoe-chicago-and-apoe-glu3lys-treated-with-fenofibrate
#14
Hitoshi Kodera, Yasuhide Mizutani, Satoshi Sugiyama, Toshio Miyata, Takashi Ehara, Akira Matsunaga, Takao Saito
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 of apoE Chicago (Arg147Pro) and apoE (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...
May 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28868300/aa-amyloidosis-and-atypical-familial-mediterranean-fever-with-exon-2-and-3-mutations
#15
Junko Yabuuchi, Noriko Hayami, Junichi Hoshino, Keiichi Sumida, Tatsuya Suwabe, Toshiharu Ueno, Akinari Sekine, Masahiro Kawada, Masayuki Yamanouchi, Rikako Hiramatsu, Eiko Hasegawa, Naoki Sawa, Kenmei Takaichi, Takeshi Fujii, Kenichi Ohashi, Kiyoshi Migita, Takao Masaki, Yoshifumi Ubara
A 54-year-old Japanese man presented with recurrent abdominal pain, fever lasting >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...
May 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28868299/tubuloreticular-inclusions-in-the-absence-of-systemic-lupus-erythematosus-and-hiv-infection-a-report-of-three-pediatric-cases
#16
Ayah Elmaghrabi, Elizabeth Brown, Ei Khin, Jared Hassler, Allen R Hendricks
Tubuloreticular inclusions (TRIs) are subcellular structures located within the cisternae of endoplasmic reticulum. Formation of TRIs has been linked to the exposure of excess interferon (IFN), either from endogenous or exogenous sources. In renal disease, TRIs have been most commonly associated with systemic lupus erythematosus (SLE), and human immunodeficiency virus-associated nephropathy (HIVAN). Case reports of patients with renal biopsies showing TRIs without underlying SLE or HIV are infrequent in adults, and to our knowledge none have been reported in children...
May 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28868298/case-report-of-spontaneous-remission-of-biopsy-proven-idiopathic-immune-complex-mediated-membranoproliferative-glomerulonephritis
#17
Rehan Shah, Mark S Segal, Michael J Wilkowski
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...
May 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28868297/a-toddler-presenting-with-pulmonary-renal-syndrome
#18
Florence A Aeschlimann, Rae S M Yeung, Ronald M Laxer, Diane Hebert, Ashley Cooper, Rose Chami, Damien Noone
Pulmonary renal syndrome refers to an association of pulmonary and glomerular disease and includes disorders, such as the ANCA-associated vasculitides, anti-glomerular basement membrane antibody disease, systemic lupus erythematosus, and IgA vasculitis (Henoch-Schönlein purpura). We present the medical history of a 26-month-old boy with an extensive purpuric rash, involving the limbs, trunk, and face, who developed clinically significant pulmonary hemorrhage and renal involvement. Rapid recognition of this rare but potentially life-threatening condition is crucial...
May 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28868296/hemodialysis-arteriovenous-access-occlusion-using-the-amplatzer-vascular-plug-in-patients-with-intractable-arm-edema
#19
Michele Di Filippo, Danilo Barbarisi, Doriana Ferrara, Stefania Brancaccio, Luca Del Guercio, Renata Bracale, Alfredo Capuano, Giovanni Esposito, Umberto Marcello Bracale
OBJECTIVES: Vascular occlusion of hemodialysis arteriovenous access (AVA) using an Amplatzer vascular plug (AVP; St. Jude Medical, St. Paul, MN, USA) is an arising and alternative practice in selected patients; however, few reported cases can be found in the literature. Herein, we report on our experience with endovascular treatment of complicated AVA. MATERIALS AND METHODS: From September 2015 to December 2016, 3 patients at our clinic underwent an occlusion of hemodialysis AVA with 2 different Amplatzer vascular plugs: 2 patients with type II and 1 patient with type IV...
May 2017: Case Reports in Nephrology and Dialysis
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
#20
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
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