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https://www.readbyqxmd.com/read/27904828/dent-s-disease-complicated-by-nephrotic-syndrome-a-case-report
#1
Guohua He, Hongwen Zhang, Shanshan Cao, Huijie Xiao, Yong Yao
Dent's disease is an X-linked recessive proximal tubular disorder that mostly affects male patients in childhood or early adult life. The condition is caused by mutations in the CLCN5 (Dent disease 1) or OCRL (Dent disease 2) genes located on chromosome Xp11.22 and Xq25, respectively. In most male patients, proteinuria is subnephrotic but may reach nephrotic levels. Here, we report the first case of Dent's disease complicated by nephrotic syndrome. Dent's disease should be considered in the differential diagnosis of nephrotic syndrome, and especially in male patients with early onset of nephrotic syndrome...
November 2016: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/27904154/circadian-rhythm-of-blood-pressure-and-the-renin-angiotensin-system-in-the-kidney
#2
REVIEW
Naro Ohashi, Shinsuke Isobe, Sayaka Ishigaki, Hideo Yasuda
Activation of the intrarenal renin-angiotensin system (RAS) has a critical role in the pathophysiology of the circadian rhythm of blood pressure (BP) and renal injury, independent of circulating RAS. Although it is clear that the circulating RAS has a circadian rhythm, reports of a circadian rhythm in tissue-specific RAS are limited. Clinical studies evaluating intrarenal RAS activity by urinary angiotensinogen (AGT) levels have indicated that urinary AGT levels were equally low during both the daytime and nighttime in individuals without chronic kidney disease (CKD) and that urinary AGT levels were higher during the daytime than at nighttime in patients with CKD...
December 1, 2016: Hypertension Research: Official Journal of the Japanese Society of Hypertension
https://www.readbyqxmd.com/read/27904113/fanconi-syndrome-associated-with-hyponatremia-in-two-patients-with-legionella-pneumonia
#3
Akihiro Ryuge, Yasuhiko Ito, Taishi Yamakawa, Hitoshi Tanaka, Hirotoshi Yasui, Shuko Mashimo, Kenshi Watanabe, Rie Nomura, Nobukazu Suganuma, Shoichi Maruyama
Legionella pneumophila is a cause of community-acquired pneumonia that is reported to induce electrolyte disorders, including hyponatremia, hypokalemia, and hypophosphatemia. We herein report two Japanese men with Legionella pneumonia and hyponatremia and hypophosphatemia. These findings were associated with an elevation of urinary low-molecular-weight tubular protein, including urinary β2-microglobulin, N-acetyl-β-D-glucosaminidase, the fractional excretion of phosphate and uric acid, and the presence of glycosuria and panaminoaciduria, suggesting that their electrolyte disorders had been caused by Fanconi syndrome...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27903492/angiotensinogen-import-in-isolated-proximal-tubules-evidence-for-mitochondrial-trafficking-and-uptake
#4
Bryan A Wilson, Nildris Cruz-Diaz, Yixin Su, James C Rose, TanYa M Gwathmey, Mark C Chappell
The renal proximal tubules are a key functional component of the kidney and express the angiotensin precursor angiotensinogen; however, it is unclear the extent that tubular angiotensinogen reflects local synthesis or internalization. Therefore, the current study established the extent that angiotensinogen is internalized by proximal tubules and the intracellular distribution. Proximal tubules were isolated from the kidney cortex of male sheep by enzymatic digestion and a discontinuous Percoll gradient. Tubules were incubated with radiolabeled 125I-angiotensinogen for 2 hrs at 37°C in serum/phenol-free DMEM/F12 media...
November 30, 2016: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/27900972/failure-to-thrive-and-nephrocalcinosis-due-to-distal-renal-tubular-acidosis-a-rare-presentation-of-pediatric-lupus-nephritis
#5
Madhumita Nandi, Mrinal Kanti Das, Sukanta Nandi
A 9-year-old female child was initially diagnosed of having nephrocalcinosis with distal renal tubular acidosis (dRTA) while investigating for short stature. She later on developed features of nephrotic syndrome (NS) while on treatment for RTA. Investigation for the cause of NS revealed very strong serological evidence in favor of systemic lupus erythematosus (SLE). Histopathological confirmation could not be done due to bilateral severely contracted kidneys. There are a few case reports of dRTA as the presentation of SLE, but nephrocalcinosis with dRTA with subsequent manifestation of SLE has hitherto not been reported in literature...
November 2016: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/27900941/the-kidney-in-sickle-hemoglobinopathies%C3%A2
#6
Medha Airy, Garabed Eknoyan
With improvements in the care of patients with sickle hemoglobinopathies, sickle cell disease (SCD) has evolved from a disease that was fatal in childhood into one in which most survive past their 5th decade and some into old age. As a result, the renal complications of sickle hemoglobinopathies, which are age dependent, have emerged as a common and serious complication of SCD. Approximately 14 - 18% of mortality in SCD is attributed to chronic kidney disease (CKD), which develops in 1/3 of individuals with SCD and progresses to end-stage renal disease in 4 - 18% of them...
November 30, 2016: Clinical Nephrology
https://www.readbyqxmd.com/read/27900407/do-inhibitors-of-crystallisation-play-any-role-in-the-prevention-of-kidney-stones-a-critique
#7
REVIEW
William G Robertson
A critical examination of data in the literature and in as yet unpublished laboratory records on the possible role of so-called inhibitors of crystallisation in preventing the formation of calcium-containing kidney stones leads to the following conclusions. So-called inhibitors of spontaneous "self-nucleation" are unlikely to play any role in the initiation of the crystallisation of CaOx or CaP in urine because excessive urinary supersaturation of urine with respect to these salts dominates the onset of "self-nucleation" within the normal time frame of the transit of tubular fluid through the nephron (3-4 min)...
November 29, 2016: Urolithiasis
https://www.readbyqxmd.com/read/27900255/adenoid-cystic-carcinoma-of-the-floor-of-the-mouth-a-rare-presentation
#8
Chandresh Jaiswara, Neeraj Kumar Dhiman, Akhilesh Kumar Singh, Naresh Kumar Sharma, Vishal Verma, Arun Pandey
Adenoid cystic carcinoma (ACC) is an unusual salivary gland malignancy that remains poorly understood. It is a slow growing but aggressive neoplasm with a tendency for recurrence. It is characterized by the proliferation of ductal (luminal) and myoepithelial cells in cribriform, tubular, solid, and cystic forms. Standard treatment, including surgery with postoperative radiation therapy, has attained reasonable local control rates, but distant metastases do not allow any improvement in the survival rate. The understanding of the molecular mechanisms driving ACC is quite rudimentary...
November 2016: Journal of Oral Biology and Craniofacial Research
https://www.readbyqxmd.com/read/27900025/pbx3-promotes-gastric-cancer-invasion-and-metastasis-by-inducing-epithelial-mesenchymal-transition
#9
Shuanhu Wang, Chenglong Li, Wenbin Wang, Chungen Xing
The overexpression of pre-leukemia transcription factor 3 (PBX3) in tumors plays an important role in invasion, metastasis and proliferation in a variety of human cancer types. Tumor metastasis and angiogenesis significantly contribute to the progression of cancer and create challenges for cancer therapy. In the present study, reverse transcription-polymerase chain reaction demonstrated that PBX3 was upregulated in gastric cancer (GC) tissues and Transwell assay revealed that the overexpression of PBX3 promoted GC invasion and metastasis in vitro...
November 2016: Oncology Letters
https://www.readbyqxmd.com/read/27899231/carvedilol-exacerbate-gentamicin-induced-kidney-mitochondrial-alterations-in-adult-rat
#10
Luís Félix, M M Oliveira, Romeu Videira, Elisabete Maciel, Nuno D Alves, Fernando M Nunes, Anabela Alves, José M Almeida, M Rosário M Domingues, Francisco P Peixoto
Gentamicin is an aminoglycoside antibiotic widely used to treat many types of bacterial infections. Although its properties, his clinical use is limited due to the occurrence of nephrotoxicity, which has been related to mitochondrial dysfunction. Carvedilol, an antihypertensive drug with strong antioxidant properties, has been tested in order to prevent gentamicin nephrotoxicity. This study aimed to test this hypothesis using a rat model of gentamicin-induced nephrotoxicity. Animals were treated subcutaneously with DMSO (control) (0...
November 26, 2016: Experimental and Toxicologic Pathology: Official Journal of the Gesellschaft Für Toxikologische Pathologie
https://www.readbyqxmd.com/read/27898408/sulodexide-protects-contrast-induced-nephropathy-in-sprague-dawley-rats
#11
Qing Zhao, Jianyong Yin, Zeyuan Lu, Yiwei Kong, Guangyuan Zhang, Binghui Zhao, Feng Wang
BACKGROUND: Sulodexide is a powerful antithrombin agent with reno-protective property. However, whether it has beneficial effects on Contrast-Induced Nephropathy (CIN) remained elusive. In the current study, we evaluated the therapeutic effects of Sulodexide on CIN and investigated the potential mechanisms. METHODS: CIN model was induced by intravenous injection of indomethacin, followed by Ioversol and L-NAME. Sprague-Dawley rats were divided into 4 groups: control group, CIN group, CIN+vehicle group (CIN rats pretreated with vehicle) and CIN+ Sulodexide (CIN rats pretreated with Sulodexide)...
November 30, 2016: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/27898354/vasitis-mimicking-an-amyand-s-hernia-a-case-report
#12
Juan Manuel Romero Marcos, Santiago Baena Bradaschia, José María Muñoz Pérez, José Andrés Cifuentes Ródenas
INTRODUCTION: Amyand's hernia is an inguinal hernia containing the caecal appendix. It is usually an intraoperative finding, although it can be diagnosed preoperatively with radiologic examinations, which would show a tubular structure inside the inguinal canal. PRESENTATION OF CASE: A male patient presented to the emergency department complaining of abdominal pain in the right lower quadrant. He had been orchidectomized during his childhood due to cryptorchidism, and had been under antibiotic treatment a week before due to a suspected gonorrhoea...
November 22, 2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27897190/versatile-sensitive-liquid-chromatography-mass-spectrometry-implementation-of-10%C3%A2-%C3%AE-m-ot-columns-suitable-for-small-molecules-peptides-and-proteins
#13
T Vehus, H Roberg-Larsen, J Waaler, S Aslaksen, S Krauss, S R Wilson, E Lundanes
We have designed a versatile and sensitive liquid chromatographic (LC) system, featuring a monolithic trap column and a very narrow (10 μm ID) fused silica open tubular liquid chromatography (OTLC) separation column functionalized with C18-groups, for separating a wide range of molecules (from small metabolites to intact proteins). Compared to today's capillary/nanoLC approaches, our system provides significantly enhanced sensitivity (up to several orders) with matching or improved separation efficiency, and highly repeatable chromatographic performance...
November 29, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27896392/tubular-and-genetic-disorders-associated-with-kidney-stones
#14
REVIEW
Nilufar Mohebbi, Pietro Manuel Ferraro, Giovanni Gambaro, Robert Unwin
This concise review summarizes our current understanding and the recent developments in genetics and related renal tubular disorders that have been linked with, or have been shown to be causal in, renal stone disease. The aim is to provide a readily accessible quick and easy update for urologists, nephrologists and endocrine or metabolic physicians whose practice involves the diagnosis and management of nephrolithiasis. An important message is to always consider a seemingly rare, and usually genetic, cause of kidney stones, since some of these are emerging as more common than originally thought, especially in adult clinical practice in which a family history of stones is a common finding...
November 28, 2016: Urolithiasis
https://www.readbyqxmd.com/read/27895157/beneficial-effects-of-myo-inositol-oxygenase-deficiency-in-cisplatin-induced-aki
#15
Rajesh K Dutta, Vinay K Kondeti, Isha Sharma, Navdeep S Chandel, Susan E Quaggin, Yashpal S Kanwar
Overexpression of the proximal tubular enzyme myo-inositol oxygenase (MIOX) induces oxidant stress in vitro However, the relevance of MIOX to tubular pathobiology remains enigmatic. To investigate the role of MIOX in cisplatin-induced tubular AKI, we generated conditional MIOX-overexpressing transgenic (MIOX-TG) mice and MIOX-knockout (MIOX(-/-)) mice with tubule-specific MIOX overexpression or knockout, respectively. Compared with cisplatin-treated wild-type (WT) mice, cisplatin-treated MIOX-TG mice had even greater increases in urea, creatinine, and KIM-1 levels and more tubular injury and apoptosis, but these effects were attenuated in cisplatin-treated MIOX(-/-) mice...
November 28, 2016: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/27895154/kidney-tubular-ablation-of-ocrl-inpp5b-phenocopies-lowe-syndrome-tubulopathy
#16
Kazunori Inoue, Daniel M Balkin, Lijuan Liu, Ramiro Nandez, Yumei Wu, Xuefei Tian, Tong Wang, Robert Nussbaum, Pietro De Camilli, Shuta Ishibe
Lowe syndrome and Dent disease are two conditions that result from mutations of the inositol 5-phosphatase oculocerebrorenal syndrome of Lowe (OCRL) and share the feature of impaired kidney proximal tubule function. Genetic ablation of Ocrl in mice failed to recapitulate the human phenotypes, possibly because of the redundant functions of OCRL and its paralog type 2 inositol polyphosphate-5-phosphatase (INPP5B). Germline knockout of both paralogs in mice results in early embryonic lethality. We report that kidney tubule-specific inactivation of Inpp5b on a global Ocrl-knockout mouse background resulted in low molecular weight proteinuria, phosphaturia, and acidemia...
November 28, 2016: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/27894621/glomerulocystic-kidney-presenting-as-a-unilateral-kidney-mass-in-a-newborn-with-tuberous-sclerosis-report-of-a-case-and-review-of-the-literature
#17
Miguel Rito, Rafael Adame Cabrera
Glomerular cysts are defined as a 2-3 times dilation of Bowman spaces and their presence in at least 5% of the glomeruli defines the kidneys as glomerulocystic (GCK). The association between cystic kidney disease and the tuberous sclerosis complex (TSC) is well known, but its presentation as a unilateral mass with glomerulocystic pattern is rare. We describe a case of an infant with a prenatal diagnosis of TSC, with a renal mass that was believed to be a renal tumor. A four-month-old infant with maternal history of TSC and prenatally diagnosed subependymal nodules and a right renal mass underwent nephrectomy...
November 18, 2016: Pathology, Research and Practice
https://www.readbyqxmd.com/read/27892560/solution-phase-synthesis-of-1d-tubular-polymers-via-preorganization-polymerization
#18
Hao Yu, Robert Häner
The synthesis of one-dimensional tubular polymers via a preorganization-polymerization approach is presented. Prior to polymerization, photo-reactive monomers self-assemble into one-dimensional tubular structures in aqueous medium. The supramolecular polymers are subsequently converted into covalent polymers by light-induced anthracene dimerization.
November 28, 2016: Chemical Communications: Chem Comm
https://www.readbyqxmd.com/read/27889732/distal-renal-tubular-acidosis-associated-with-celiac-disease-and-thyroiditis
#19
Amit Kumar Satapathy, Sapna Mittal, Vandana Jain
BACKGROUND: Association of distal renal tubular acidosis (RTA) with autoimmune diseases is extremely rare in children. CASE CHARACTERISTICS: 12-year-old girl with distal RTA. Despite resolution of acidosis on bicarbonate, she continued to have poor growth and delayed puberty. Investigations revealed autoimmune thyroiditis and celiac disease. OUTCOME: Levothyroxine and gluten-free diet were initiated. Child gained height and weight and had onset of puberty after gluten withdrawal...
November 15, 2016: Indian Pediatrics
https://www.readbyqxmd.com/read/27888661/use-of-vimentin-immunocytochemical-staining-for-evaluation-of-atypical-cells-in-voided-urine-samples
#20
Margareta Strojan Fležar, Irena Srebotnik-Kirbiš, Helena Gutnik
BACKGROUND: Cytomorphology of exfoliated atypical reactive/repair renal tubular cells (RRTC) can resemble atypical urothelial cells thus suggesting a differential diagnostic question of urothelial neoplasia in urinary cytology. Vimentin expression has been shown in RRTC and used for differentiation from atypical urothelial cells. METHODS: The institutional computer database was searched for urinary cytology cases with vimentin immunocytochemical staining (2008-2012)...
November 26, 2016: Diagnostic Cytopathology
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