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Marina Flotats-Bastardas, Daniel Ebrahimi-Fakhari, Ludwig Gortner, Martin Poryo, Michael Zemlin, Alfons Macaya-Ruiz, Sascha Meyer
Tuberous sclerosis complex (TSC) is a genetic disease with a significant morbidity and mortality. We conducted a retrospective analysis of two cohorts (Vall d'Hebron University Hospital [HVH], Barcelona, Spain, 1982-2015, and at Saarland University Medical Center [UKS], Homburg, Germany, 1998-2015) to assess prevalence and treatment of TSC associated manifestations and to evaluate if the follow-up was in line with published recommendations. This was considered if more than 15% of patients did not receive adequate examination with regard to potential organ involvement...
March 20, 2018: Neuropediatrics
María Del Pino, Amado Andrés, Ana Ávila Bernabéu, Joaquín de Juan-Rivera, Elvira Fernández, Juan de Dios García Díaz, Domingo Hernández, José Luño, Isabel Martínez Fernández, José Paniagua, Manuel Posada de la Paz, José Carlos Rodríguez-Pérez, Rafael Santamaría, Roser Torra, Joan Torras Ambros, Pedro Vidau, Josep-Vicent Torregrosa
Fabry disease (FD) is a rare, X-linked disorder caused by mutations in the GLA gene encoding the enzyme α-galactosidase A. Complete or partial deficiency in this enzyme leads to intracellular accumulation of globotriaosylceramide (Gb3) and other glycosphingolipids in many cell types throughout the body, including the kidney. Progressive accumulation of Gb3 in podocytes, endothelial cells, epithelial cells, and tubular cells contribute to the renal symptoms of FD, which manifest as proteinuria and reduced glomerular filtration rate leading to renal insufficiency...
March 16, 2018: Kidney & Blood Pressure Research
Judy Tan, Anita Mehrotra, Girish N Nadkarni, John Cijiang He, Erik Langhoff, James Post, Carlos Galvao-Sobrinho, Henry C Thode, Rajeev Rohatgi
BACKGROUND: Chronic kidney disease (CKD) patients who live far (>30 miles) from their nephrologist experience lower rates of clinic visit adherence, limited access to treatment, and higher rates of hospitalization and mortality than patients who live in close proximity to their nephrologist. Strategies to minimize disparities between urban and remotely located CKD patients are needed. The purpose of this study was to determine whether adherence to clinic visits and clinical outcomes in the remote management of CKD via telenephrology is comparable to in-person conventional care...
March 20, 2018: American Journal of Nephrology
Dimitrios Chanouzas, Alexander Small, Richard Borrows, Simon Ball
BACKGROUND: The measurement of CMV specific cellular immunity in organ transplant recipients could contribute additional acuity to serology based, CMV infection risk stratification, facilitating optimisation of immunosuppression and anti-viral prophylaxis. METHODS: A pilot study of renal transplant recipient (RTR's) responses in the T-SPOT.CMV ELISPOT based assay. 108 RTR's were recruited 3 months post-transplantation, immediately prior to the cessation of stratified anti-viral prophylaxis, used in recipients from seropositive donors...
2018: PloS One
Zbigniew Zdrojewski
Recognizing the antiphospholipid syndrome (APS) in patients with lupus (SLE) can be difficult and therefore underestimated. Detection of antiphospholipid antibodies (aPL) in each patient with SLE should be done as a rule. Introduction of the new classification criteria of this syndrome will certainly improve the recognition of APS in the future. The Sapporo APS classification criteria (1998) were replaced by the Sydney criteria in 2006. Based on the most recent criteria, classification with APS requires one clinical and one laboratory manifestation...
2018: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
Chengcheng Zhu, Kimberly Kallianos, Travis Henry, Michael D Hope
No abstract text is available yet for this article.
April 2018: Radiology
Ghaneh Fananapazir, Robert Benzl, Michael T Corwin, Ling-Xin Chen, Junichiro Sageshima, Susan L Stewart, Christoph Troppmann
Purpose To determine whether the predonation computed tomography (CT)-based volume of the future remnant kidney is predictive of postdonation renal function in living kidney donors. Materials and Methods This institutional review board-approved, retrospective, HIPAA-compliant study included 126 live kidney donors who had undergone predonation renal CT between January 2007 and December 2014 as well as 2-year postdonation measurement of estimated glomerular filtration rate (eGFR). The whole kidney volume and cortical volume of the future remnant kidney were measured and standardized for body surface area (BSA)...
March 20, 2018: Radiology
Gerard K Nguyen, Vincent M Mellnick, Aldrin Kay-Yuen Yim, Amber Salter, Joseph E Ippolito
Purpose To determine if sex differences in abdominal visceral fat composition, measured by using computed tomography (CT), and tumor glucose metabolism, measured by gene expression, can help predict outcomes in patients with clear cell renal cell carcinoma (RCC). Materials and Methods This retrospective cohort study included 222 patients with clear cell RCC from The Cancer Imaging Atlas. By using CT, body fat was segmented into subcutaneous fat and visceral fat areas (VFAs) and normalized to total fat to obtain the relative VFA (rVFA) and relative subcutaneous fat area...
March 20, 2018: Radiology
Tarini Ullal, Adam Birkenheuer, Shelly Vaden
Babesiosis is a hemoprotozoal tick-borne disease that is commonly associated with thrombocytopenia and anemia; however, renal involvement has been documented in dogs. The purpose of this retrospective study was to document azotemia and proteinuria in dogs infected with Babesia sp. and to describe the response to antiprotozoal therapy. The electronic database of the North Carolina State University Vector Borne Disease Laboratory was searched to identify dogs who were diagnosed with babesiosis and to determine if they had proteinuria and/or azotemia...
March 20, 2018: Journal of the American Animal Hospital Association
Nick X Bommer, Serena E Brownlie, Linda R Morrison, Marge L Chandler, James W Simpson
Three juvenile male Irish wolfhound littermates presented with marked polyuria and polydipsia. The four female siblings were apparently unaffected. Diagnostic testing revealed glucosuria with normoglycemia, generalized aminoaciduria, hypokalemia and metabolic acidosis consistent with Fanconi syndrome. Renal ultrasonographic and histologic findings are presented. Cases were managed with a supplementation regimen based on a treatment protocol for Fanconi syndrome in basenjis. These dogs did not have angular limb deformities as documented previously in juvenile canine siblings with Fanconi syndrome...
March 20, 2018: Journal of the American Animal Hospital Association
Luciano A Favorito
No abstract text is available yet for this article.
March 2018: International Braz J Urol: Official Journal of the Brazilian Society of Urology
Piotr Czempik, Daniel Cieśla, Piotr Knapik, Łukasz Krzych
BACKGROUND: Acute kidney injury (AKI) in critically ill patients has a deleterious impact on the prognosis, especially when renal replacement therapy (RRT) is required. This issue has not yet been investigated in the intensive care setting in Poland. OBJECTIVES: The aim of the study was to evaluate the short-term outcomes of AKI-RRT subjects, based on a large registry population. MATERIAL AND METHODS: This observational multicenter study covered 100 demographic and clinical variables from the Silesian Registry of ICUs regarding 15,030 adult patients hospitalized between October 2011 and December 2014...
March 16, 2018: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
Ulkem Kocoglu Barlas, Hasan Serdar Kıhtır, Nilufer Goknar, Melike Ersoy, Nihal Akcay, Esra Sevketoglu
BACKGROUND: Hemolytic uremic syndrome (HUS) is a clinical syndrome characterized by hemolytic anemia, thrombocytopenia, and acute kidney injury. Atypical hemolytic uremic syndrome (aHUS) is a devastating disease with significant mortality and high risk of progression to end-stage kidney disease. It is mostly caused by dysregulation of the alternative complement pathway. Cobalamin C (Cbl C) defect is a genetic disorder of cobalamin metabolism and is a rare cause of HUS. CASE-DIAGNOSIS/TREATMENT: We present a 6-month-old male infant who was admitted to the pediatric intensive care unit (PICU) due to restlessness, severe hypertension, anemia, respiratory distress, and acute kidney injury...
March 20, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Zhihui Lu, Nana Song, Bo Shen, XiaLian Xu, Yi Fang, Yiqin Shi, Yichun Ning, Jiachang Hu, Yan Dai, Xiaoqiang Ding, Jianzhou Zou, Jie Teng
BACKGROUND: The hepatocyte growth factor (HGF) target pathway plays pivotal renoprotective roles after acute kidney injury (AKI). Syndecan-1 serves as the co-receptor for HGF. Shedding of syndecan-1 is involved in various pathological processes. Thus, we hypothesized that IRI induced syndecan-1 shedding and inhibiting syndecan-1 shedding would protect against kidney injury by potentiating activation of the HGF receptor c-Met. METHODS: Expression of syndecan-1 and its sheddases were observed in kidneys of sham and I/R mice...
March 20, 2018: Transplantation
Hillary J Braun, Nancy L Ascher
No abstract text is available yet for this article.
March 20, 2018: Transplantation
Lisa B VanWagner, Samantha Montag, Lihui Zhao, Norrina B Allen, Donald M Lloyd-Jones, Arighno Das, Anton I Skaro, Samuel Hohmann, John J Friedewald, Josh Levitsky
BACKGROUND: In the general population, even mild renal disease is associated with increased cardiovascular (CV) complications. Whether this is true in liver transplant recipients (LTR) is unknown. METHODS: This was a retrospective cohort study of 671 LTR (2002-2012) from a large urban tertiary care center and 37,322 LTR using Vizient hospitalization data linked to the United Network for Organ Sharing. The MDRD4 equation estimated GFR (eGFR). Outcomes were 1-year CV complications (death/hospitalization from myocardial infarction, heart failure, atrial fibrillation, cardiac arrest, pulmonary embolism or stroke) and mortality...
March 20, 2018: Transplantation
Lee Ena, Jong Seung Lim, Ji Yeon Son, Yu Jin Park, Yong Hee Lee, Ji Yeong Kim, Seung Jun Kwack, Byung Mu Lee, Mee-Young Ahn, Hyung Sik Kim
Triclosan (TCS), a common antimicrobial ingredient, is present in many consumer products, including soaps, shampoos, and toothpaste. Owing to its widespread use, potential adverse effects on animals and humans may arise from lifetime exposure, but data on chronic prepubertal exposure of TCS are still lacking. The aim of the present study was to investigate the influence of subchronic TCS exposure (0.25, 25, 250, or 750 mg/kg) on target organ toxicity in prepubertal male rats. After daily administration of TCS to rats by oral gavage for 60 d, a significant reduction in body weight and relative weights of liver, kidneys, testes, and adrenal glands was observed in the 750-mg/kg (high dose) group...
March 20, 2018: Journal of Toxicology and Environmental Health. Part A
Delia De Lisi, Ugo De Giorgi, Cristian Lolli, Giuseppe Schepisi, Vincenza Conteduca, Cecilia Menna, Giuseppe Tonini, Daniele Santini, Alberto Farolfi
To date, results of combination therapy studies have shown no meaningful clinical benefit over monotherapy and an unacceptably high degree of toxicity in the treatment of metastatic renal cell carcinoma (RCC), with the exception of a combination of immune-checkpoint inhibitors and the association of lenvatinib with everolimus. Lenvatinib is a potent multi-targeted tyrosine kinase inhibitor that targets VEGFR pathways. Everolimus inhibits primarily mTORC1 complex, a downstream effecter of the intracellular PI3K/AKT/mTOR pathway...
March 20, 2018: Expert Opinion on Drug Metabolism & Toxicology
Pablo Roa Rojas, Hernán Arango Fernández, Martha Rebolledo Cobos, Jonathan Harris Ricardo
Beckwith-Wiedemann syndrome is a rare congenital condition, characterized by presenting macroglossia, defects of the abdominal wall, hemihypertrophy, omphalocele, neonatal hypoglycemia, umbilical hernia, hepatomegaly, cardiac abnormalities, among others. Macroglossia occurs in 90% of cases, causing a problem in chewing, swallowing, phonation and breathing, resulting in a closure of the upper airway. The therapeutic option of choice is partial glossectomy. We present a 2-month-old pediatric patient with Beckwith-Wiedemann syndrome and area blockage due to severe macroglossia; in the medical history, congenital heart disease, interatrial communication, persistent ductus arteriosus, symptomatic epilepsy, renal failure, hypoglycemia, tracheotomy and gastrostomy, due to airway collapse and dysphagia...
April 1, 2018: Archivos Argentinos de Pediatría
Miguel A Marín Gabriel, Ana M Malalana Martínez, Ibone Olza Fernández
Lithium therapy is currently a cornerstone of treatment for mothers who suffer bipolar disorders. Those who wish to breastfeed their children are often told they have to decide whether modifying the treatment for their disorder or even avoiding lactation. Lithium administration during breastfeeding has been described to produce certain side effects such as thyroid disorders, hypothermia and hypotonia. To our knowledge, there are few publications where infants have no laboratory abnormalities. Here we present the case of an infant without renal or thyroid alteration while he was breastfed...
April 1, 2018: Archivos Argentinos de Pediatría
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