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tubular disease

A J Kim, H Ro, J H Chang, J Y Jung, W K Chung, Y H Park, H H Lee
Besides the initial description of IgG4-related pancreatic disease, other sites are now commonly involved. However, occurrence of IgG4-related disease is rare in organ transplanted patients. A 57-year-old man who received a kidney transplantation presented with recurrent dyspnea on exertion. A computed tomography scan of the chest revealed bilateral interlobular septal thickening and multiple tubular and branching small nodular lesions in the right upper lobe, and mass-like consolidation of the left middle lobe...
October 2018: Transplantation Proceedings
S Uday, S Sakka, J H Davies, T Randell, V Arya, C Brain, M Tighe, J Allgrove, P Arundel, R Pryce, W Högler, N J Shaw
OBJECTIVES: Hypophosphataemic rickets (HR) is usually secondary to renal phosphate wasting but may occur secondary to reduced intake or absorption of phosphate. We describe a series of cases of HR associated with the use of Neocate® , an amino-acid based formula (AAF). METHODS: A retrospective review of cases with HR associated with AAF use presenting to centres across the United Kingdom. RESULTS: 10 cases were identified, over a 9 month period, all associated with Neocate® use...
September 28, 2018: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
Shaoqun Shu, Jiefu Zhu, Zhiwen Liu, Chengyuan Tang, Juan Cai, Zheng Dong
BACKGROUND: Acute kidney injury (AKI) may lead to the development of chronic kidney disease (CKD), i.e. AKI-CKD transition, but the underlying mechanism remains largely unclear. Endoplasmic reticulum (ER) stress is characterized by the accumulation of unfolded or misfolded proteins in ER resulting in a cellular stress response. The role of ER stress in AKI-CKD transition remains unknown. METHODS: In this study, we examined ER stress in the mouse model of AKI-CKD transition after unilateral renal ischemia-reperfusion injury (uIR)...
October 9, 2018: EBioMedicine
Heike Biebermann, Gunnar Kleinau, Dirk Schnabel, Detlef Bockenhauer, Louise C Wilson, Ian Tully, Sarah Kiff, Patrick Scheerer, Monica Reyes, Sarah Paisdzior, John W Gregory, Jeremy Allgrove, Heiko Krude, Michael Mannstadt, Thomas J Gardella, Mehul Dattani, Harald Jüppner, Annette Grüters
Context: The alpha-subunit of the stimulatory G-protein (Gαs) links numerous receptors to adenylyl cyclase. Gαs, encoded by GNAS, is expressed predominantly from the maternal allele in certain tissues. Thus, maternal heterozygous loss-of-function mutations cause hormonal resistance, as in pseudohypoparathyroidism type Ia, while somatic gain-of-function mutations cause hormone-independent endocrine stimulation, as in McCune-Albright Syndrome. Objective: We here report two unrelated boys presenting with a new combination of clinical findings that suggest both gain and loss of Gαs function...
October 11, 2018: Journal of Clinical Endocrinology and Metabolism
Petter Bjornstad, Sunita K Singh, Janet K Snell-Bergeon, Julie A Lovshin, Yuliya Lytvyn, Leif E Lovblom, Marian J Rewers, Genevieve Boulet, Vesta Lai, Josephine Tse, Leslie Cham, Andrej Orszag, Alanna Weisman, Hillary A Keenan, Michael H Brent, Narinder Paul, Vera Bril, Bruce A Perkins, David Z I Cherney
OBJECTIVE: Our aim was to define the relationships between plasma biomarkers of kidney injury and intrarenal hemodynamic function (glomerular filtration rate [GFR], effective renal plasma flow [ERPF], renal vascular resistance [RVR]) in adults with type 1 diabetes (T1D). METHODS: The study sample consisted of patients with longstanding T1D (≥50 years duration); 44 Diabetic Kidney Disease (DKD) resistors (eGFR >60mL/min/1.73m2 and <30mg/day urine albumin excretion) and 22 with DKD, in addition to 73 controls...
October 12, 2018: Diabetes, Obesity & Metabolism
Krithika Hariharan, Harald Stachelscheid, Bella Rossbach, Su-Jun Oh, Nancy Mah, Kai Schmidt-Ott, Andreas Kurtz, Petra Reinke
Human pluripotent stem cells (hPSCs) provide a source for the generation of defined kidney cells and renal organoids applicable in regenerative medicine, disease modeling, and drug screening. These applications require the provision of hPSC-derived renal cells by reproducible, scalable, and efficient methods. We established a chemically defined protocol by application of Activin A, BMP4, and Retinoic acid followed by GDNF, which steered hPSCs to the renal lineage and resulted in populations of SIX2+ /CITED1+ metanephric mesenchyme- (MM) and of HOXB7+ /GRHL2+ ureteric bud (UB)-like cells already by 6 days...
October 11, 2018: Cellular and Molecular Life Sciences: CMLS
M Sidhu, L Brady, G D Vladutiu, M A Tarnopolsky
Pathogenic variants in the PGAM2 gene are associated with glycogen storage disease type X (GSDX) and is characterized by exercise induced muscle cramping, weakness, myoglobinuria, and often tubular aggregates in skeletal muscle. We report here a patient diagnosed with GSDX at 52 years of age with a normal increase in post-exercise lactate with both anaerobic and aerobic exercise. Genetic testing found two novel PGAM2 variants (c.426C > A, p.Tyr142Ter and c.533delG, p.Gly178Alafs*31).
December 2018: Molecular Genetics and Metabolism Reports
John J Bissler, J Christopher Kingswood
Tuberous sclerosis complex (TSC) is a tumor predisposition syndrome with significant renal cystic and solid tumor disease. It commonly causes several types of cystic disease and benign tumors (angiomyolipomata) in the kidneys that can both lead to significant premature loss of glomerular filtration rate. The main risks of angiomyolipomata, severe bleeding, loss of renal function, and pulmonary lymphangioleiomyomatosis, can be ameliorated by active surveillance and preemptive therapy with mTOR inhibitors. The cystogenic mechanism may involve primary cilia, but also appears to also involve a majority of normal tubular cells and may be driven by a minority of cells with mutations inactivating both their TSC1 or TSC2 genes...
October 11, 2018: American Journal of Medical Genetics. Part C, Seminars in Medical Genetics
Min Shi, Liang Ma, Ping Ina Fu
Accumulating evidences indicated that obesity and metabolic syndrome were independent risk factors for the development and progression of kidney diseases. Apart from inflammation, lipotoxicity, and hemodynamic factors, adipokines have been proposed to play crucial roles in the relationship between kidney diseases and metabolic disorders. As one of key adipokines, fatty acid binding protein 4 (FABP4), which is mainly expressed in adipocytes and macrophages, has recently been shown to be associated with renal dysfunction and kidney damage...
October 8, 2018: Current Medicinal Chemistry
Marie-Michèle Gaudreault-Tremblay, Catherine Litalien, Natalie Patey, Aicha Merouani
Rationale: Acute tubulointerstitial nephritis (ATIN) in children is most commonly due to allergic drug reactions. In neonates, diagnosis of ATIN is clinically suspected and a kidney biopsy is not routinely performed. Presenting concern: A 17-day-old newborn presented with vomiting and dehydration, along with anuric acute kidney injury, severe electrolyte disturbances, hypocomplementemia, and thrombocytopenia. Abdominal ultrasound revealed bilateral nephromegaly and hepatosplenomegaly...
2018: Canadian Journal of Kidney Health and Disease
Takashi Tsukakoshi, Lisheng Lin, Takashi Murakami, Junko Shiono, Isho Izumi, Hitoshi Horigome
Gitelman syndrome (GS) is an inherited renal tubular disorder characterized by hypokalemic metabolic alkalosis, hypomagnesemia, and low urinary calcium excretion. While it is considered a benign disease, severe ventricular arrhythmia and sudden cardiac death related to the prolongation of the QT interval have been reported in rare cases. Herein we report a 13-year-old girl with GS who presented with persistent prolongation of the QT interval, even after being treated for hypokalemia and hypomagnesemia. Genetic analysis identified SCN5A H558R polymorphism, which modulates the function of myocardial sodium channel, and SLC12A3 A588V mutation, which causes GS...
October 10, 2018: International Heart Journal
Peng Wang, Man-Li Luo, Erwei Song, Zhanmei Zhou, Tongtong Ma, Jun Wang, Nan Jia, Guobao Wang, Sheng Nie, Youhua Liu, FanFan Hou
Transforming growth factor-β (TGF-β) is a well-established central mediator of renal fibrosis, a common outcome of almost all progressive chronic kidney diseases. Here, we identified a poorly conserved and kidney-enriched long noncoding RNA in TGF-β1-stimulated human tubular epithelial cells and fibrotic kidneys, which we termed TGF-β/Smad3-interacting long noncoding RNA ( lnc-TSI ). Lnc-TSI was transcriptionally regulated by Smad3 and specifically inhibited TGF-β-induced Smad3 phosphorylation and downstream profibrotic gene expression...
October 10, 2018: Science Translational Medicine
Fang Fang, Xiaohan Hu, Xiaomei Dai, Sanfeng Wang, Zhenjiang Bai, Jiao Chen, Jian Pan, Xiaozhong Li, Jian Wang, Yanhong Li
BACKGROUND: Research on acute kidney injury (AKI) has focused on identifying early biomarkers. However, whether AKI could be diagnosed in the absence of the classic signs of clinical AKI and whether the condition of subclinical AKI, identified by damage or functional biomarkers in the absence of oliguria or increased serum creatinine (sCr) levels, is clinically significant remains to be elucidated in critically ill children. The aims of the study were to investigate the associations between urinary cystatin C (uCysC) levels and AKI and mortality and to determine whether uCysC-positive subclinical AKI is associated with adverse outcomes in critically ill neonates and children...
October 10, 2018: Critical Care: the Official Journal of the Critical Care Forum
Eoghainín Ó hAinmhire, Haojia Wu, Yoshiharu Muto, Erinn L Donnelly, Flavia G Machado, Lucy X Fan, Monica Chang-Panesso, Benjamin D Humphreys
Gli1-positive resident mesenchymal stem cell-like cells are the predominant source of kidney myofibroblasts in fibrosis but investigating Gli1-positive myofibroblast progenitor activation is hampered by the difficulty of isolating and propagating primary cultures of these cells. Using a genetic strategy with positive and negative selection, we isolated Kidney-Gli1 (KG1) cells that maintain expression of appropriate mesenchymal stem cell-like cell markers, respond to hedgehog pathway activation and display robust myofibroblast differentiation upon treatment with TGFb...
October 10, 2018: American Journal of Physiology. Renal Physiology
Tomáš Seeman, Michael Pohl, Ulrike John
BACKGROUND: Proteinuria is a common complication in adults with autosomal dominant polycystic kidney disease (ADPKD) and serves as a risk factor for progression. However, proteinuria has rarely been examined in children with ADPKD and the type of proteinuria has not yet been investigated. The aim of the study was to assess the prevalence and to analyse the types of proteinuria in children with ADPKD. METHODS: Children with ADPKD followed-up in our tertiary centres during the years 2012-2013 were investigated in a cross-sectional study...
October 2018: Minerva Pediatrica
Wei Yih Hee, Leila M Blackman, Adrienne R Hardham
Mastigonemes, tripartite tubular hairs on the anterior flagellum of Phytophthora zoospores, are instrumental for disease dissemination to new host plants. A previous study showed that PnMas2 was part of the tubular shaft of Phytophthora parasitica mastigonemes. In the current study, genes encoding two related proteins, PnMas1 and PnMas3, were identified in the genome of P. parasitica. PnMas1 interacts with PnMas2 and also occurs along the mastigoneme shaft. RNA-Seq analyses indicate that PnMas1 and PnMas2 genes have similar expression profiles both in vitro and in planta but that PnMas3 is expressed temporally prior to PnMas1 and PnMas2 during asexual development and plant infection...
October 9, 2018: Protoplasma
Mohammad Hadi Dehghani, Samira Tajik, Ahmad Panahi, Mostafa Khezri, Ahmad Zarei, Zoha Heidarinejad, Mahmood Yousefi
Cadmium is a heavy metal toxic that enters water resources through industrial, household, agricultural waste and non-sanitary landfill of urban and industrial wastes. Pollution of water resources by cadmium increases incidence of diseases including Itai-Itai, kidney disorders, cancer, chromosome effects and kidney tubular damages in low exposures. The aim of this study is to study the efficiency of a new poly urea-formaldehyde adsorbent in the removal cadmium ions from aqueous solutions. The effect of different variables such as initial pH, contact time, initial concentration of cadmium and test of real wastewater samples were evaluated...
2018: MethodsX
Jiming Yin, Yangjia Wang, Jing Chang, Bin Li, Jia Zhang, Yu Liu, Song Lai, Ying Jiang, Huihua Li, Xiangjun Zeng
The epithelial-mesenchymal transition (EMT) of podocytes had been reported to be involved in the glomerular fibrosis in diabetic kidney diseases, which was regulated by TGFβ and NFκB pathways. And apelin, an adipokine which is upregulated in diabetic kidney diseases, was reported to be negatively correlated to TGFβ in polycystic kidney disease and attenuate EMT in renal tubular cells. Therefore, it is hypothesized that apelin might inhibit the EMT of podocytes through downregulating the expression and activation of TGFβ/Smad pathway in diabetic kidney diseases...
October 9, 2018: Cell Death & Disease
Desmond Yh Yap, Susan Yung, Tak Mao Chan
Immunosuppressive therapies for lupus nephritis (LN) have improved significantly over the past few decades, resulting in growing number of choices for treatment individualization and improved renal and patient outcomes. Corticosteroids combined with mycophenolate or cyclophosphamide induces a satisfactory response in a high proportion of Asian and Caucasian patients, but the rate of improvement varies considerably between patients. Relatively low disease flare rate was observed in Chinese patients receiving low-dose prednisolone and mycophenolate maintenance...
October 2018: Nephrology
Yangyang Zuo, Youhua Liu
Wnt/β-catenin is an evolutionarily conserved, developmental signalling pathway that regulates embryogenesis, injury repair and pathogenesis of human diseases. Dysregulated activation of Wnt/β-catenin is associated with the development and progression of renal fibrotic lesions after injury. Wnt are induced and β-catenin is activated in various models of experimental chronic kidney disease (CKD) and in human nephropathies. Recent findings indicate that pro(renin) receptor is an amplifier of Wnt/β-catenin by acting as a downstream target and an obligatory component for its signal transduction...
October 2018: Nephrology
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