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https://www.readbyqxmd.com/read/29924239/tuberous-sclerosis-complex-review-based-on-new-diagnostic-criteria
#1
Larissa Karine Leite Portocarrero, Klícia Novais Quental, Luciana Paula Samorano, Zilda Najjar Prado de Oliveira, Maria Cecília da Matta Rivitti-Machado
Tuberous sclerosis complex is a multisystemic, autosomal dominant genetic disorder with complete penetrance, that can evolve with hamartomas in multiple organs, such as skin, central nervous system, kidney and lung. Due to the wide phenotypic variability, the disease is often not recognized. Tuberous sclerosis complex affects one in 10,000 newborns and most patients are diagnosed during the first 15 months of life. The diagnostic criteria for tuberous sclerosis were reviewed in 2012, at the second International Tuberous Sclerosis Complex Consensus Conference...
June 2018: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/29909504/anderson-fabry-disease-in-heart-failure
#2
REVIEW
M M Akhtar, P M Elliott
Anderson-Fabry disease is an X-linked lysosomal storage disorder caused by mutations in the GLA gene that result in deficiency of the enzyme alpha-galactosidase A. The worldwide incidence of Fabry's disease is reported to be in the range of 1 in 40,000-117,000, although this value may be a significant underestimate given under recognition of symptoms and delayed or missed diagnosis. Deficiency in alpha-galactosidase A causes an accumulation of neutral glycosphingolipids such as globotriaosylceramide (Gb3) in lysosomes within various tissues including the vascular endothelium, kidneys, heart, eyes, skin and nervous system...
June 16, 2018: Biophysical Reviews
https://www.readbyqxmd.com/read/29906338/a-nutraceutical-diet-based-on-lespedeza-spp-vaccinium-macrocarpon-and-taraxacum-officinale-improves-spontaneous-feline-chronic-kidney-disease
#3
Alessandro Di Cerbo, Tommaso Iannitti, Gianandrea Guidetti, Sara Centenaro, Sergio Canello, Raffaella Cocco
Chronic kidney disease is characterized by structural and/or functional impairment of one or both kidneys persisting for more than 3 months. In cats, chronic kidney disease can frequently occur in animals aged over 9 years with an incidence of approximately 10%. Thirty-four client-owned, neutered cats, suffering from stage II-III chronic kidney disease and diagnosed according to the International Renal Interest Society guidelines were randomly assigned to receive either a control diet (n = 17) or a nutraceutical diet (ND; n = 17) for 90 days...
June 2018: Physiological Reports
https://www.readbyqxmd.com/read/29901634/high-dose-fulvestrant-as-third-line-endocrine-therapy-for-breast-cancer-metastasis-to-the-left-kidney-a-case-report-and-literature-review
#4
Dandan Xia, Huiyu Wang, Runjie Wang, Chaoying Liu, Junying Xu
RATIONALE: Endocrine therapy plays an important role in the treatment of patients with hormone receptor-positive breast cancer. Renal metastasis of breast cancer is rare in clinical practice. PATIENT CONCERNS: We present here a 54-year-old woman with breast cancer after first line chemotherapy and second line endocrinotherapy (i.e., toremifene & exemestane) failure. DIAGNOSES: The patient was rarely diagnosed breast cancer metastasis to the kidney and a positive hormone status (ER and PR) but was negative for human epidermal factor receptor 2 (HER2)...
June 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29900001/transfusion-management-of-incident-dialysis-patients-in-canada-a-prospective-observational-study
#5
Aminu K Bello, Christine M Ribic, Serge H Cournoyer, Mercedeh Kiaii, Martine LeBlanc, Melanie Poulin-Costello, David N Churchill, Norman Muirhead
Background: Several studies have demonstrated harm associated with using erythropoiesis-stimulating agents (ESA) to achieve higher hemoglobin (Hb) levels. Subsequently, more conservative use of ESAs has changed anemia therapy in patients with chronic renal failure. Objective: The objectives were to identify transfusion rates in hemodialysis (HD) patients during the first year of therapy, to identify factors associated with the probability of transfusion, describe reasons for the transfusions, and identify the Hb values associated with each transfusion...
2018: Canadian Journal of Kidney Health and Disease
https://www.readbyqxmd.com/read/29896258/resveratrol-provides-benefits-in-mice-with-type-ii-diabetes-induced-chronic-renal-failure-through-ampk-signaling-pathway
#6
Haiyan Guo, Linyun Zhang
Type II diabetes-induced ischemic injuries are known to lead to the rapid degeneration of the kidneys as a result of chronic renal failure. Chronic renal failure is a condition, which typically manifests with symptoms including cardiovascular system and left ventricular hypertrophy, atherosclerosis as well as arterial and aortic stiffness. Resveratrol is a multifunctional compound that has been reported to produce beneficial outcomes for patients with type-II diabetes due to prevention of oxidative stress and apoptosis...
July 2018: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29895012/continuous-blood-purification-ameliorates-multiple-organ-failure-through-inhibiting-the-activation-of-the-p38-mapk-signaling-pathway-in-a-rat-model
#7
Lan Ling, Qian-Kuan Wen, Shan-Hong Zhang, Li-Da Zhi, Hong Li, Gang Li, Wen-Jia Zhang
BACKGROUND/AIMS: Multiple organ failure (MOF) is a primary threat to the survival of patients with systemic inflammation. Blood purification is employed in the treatment of MOF, as an artificial kidney or artificial liver. This study focuses on the effects of continuous blood purification (CBP) on ameliorating MOF through regulating the p38 mitogen-activated protein kinase (MAPK) signaling pathway in a rat model. METHODS: A rat model of MOF was successfully established by endotoxin injection after hemorrhagic shock resuscitation...
June 7, 2018: Kidney & Blood Pressure Research
https://www.readbyqxmd.com/read/29864754/treatment-of-a-patient-with-psychotic-disorder-undergoing-haemodialysis-a-case-report
#8
Arijana Turčin
We report a case of a 35-year old male patient that was in treatment for chronic kidney failure for 20 years, with a personal history of unsuccessful kidney transplantation and undergoing continuous haemodialysis. He had to be treated psychiatrically for the first time because of exacerbation of psychotic symptoms. He was hospitalized twice in succession, because of the severity of the psychotic symptoms that included imperative auditory hallucinations that also led to a serious suicidal attempt. The prescribed psychiatric therapy had to be adjusted in type, dosage and especially the time of administration as his psychiatric status fluctuated every two days in sync with the wash-out of medication due to haemodialysis...
June 2018: Psychiatria Danubina
https://www.readbyqxmd.com/read/29850311/nontraumatic-exertional-rhabdomyolysis-leading-to-acute-kidney-injury-in-a-sickle-trait-positive-individual-on-renal-biopsy
#9
Kalyana C Janga, Sheldon Greenberg, Phone Oo, Kavita Sharma, Umair Ahmed
A 26-year-old African American male with a history of congenital cerebral palsy, sickle cell trait, and intellectual disability presented with abdominal pain that started four hours prior to the hospital visit. The patient denied fever, chills, diarrhea, or any localized trauma. The patient was at a party at his community center last evening and danced for 2 hours, physically exerting himself more than usual. Labs revealed blood urea nitrogen (BUN) level of 41 mg/dL and creatinine (Cr) of 2.8 mg/dL which later increased to 4...
2018: Case Reports in Nephrology
https://www.readbyqxmd.com/read/29849940/clinical-characteristics-and-diagnosis-of-a-rare-case-of-systemic-al-amyloidosis-a-descriptive-study
#10
Pengguo Chen, Zhaohan Wang, Hui Liu, Donglin Liu, Zhibin Gong, Jin Qi, Jianfang Hu
Systemic amyloidosis is a rare disease involving multiple organs. It is difficult to establish diagnosis as the symptoms is diverse and non-specific. And without specific therapy the prognosis is very poor. We analyzed detailed clinical and laboratorial data of a 53-year-old male patient. The characteristic features included refractory pleural effusion, extraordinary hepatomegaly and cardiac failure. The illness lasted 9 months and therapy period spanned 4 months. Fine needle biopsy of liver, lung, heart, pancreas and kidney was performed...
May 11, 2018: Oncotarget
https://www.readbyqxmd.com/read/29848533/severe-babesia-microti-infection-presenting-as-multiorgan-failure-in-an-immunocompetent-host
#11
Juan G Ripoll, Mahrukh S Rizvi, Rebecca L King, Craig E Daniels
A previously healthy 67-year-old farmer presented to an outside hospital after a 2-week history of non-specific respiratory symptoms. A certain diagnosis was not initially apparent, and the patient was discharged home on a regimen for presumed chronic obstructive pulmonary disease exacerbation. He re-presented to the emergency department with shock and hypoxaemic respiratory failure requiring prompt intubation and fluid resuscitation. He was then transferred to our institution due to multiorgan failure. On arrival, the patient demonstrated refractory shock and worsening acute kidney injury, severe anaemia and thrombocytopaenia...
May 30, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29843773/paraquat-induced-acute-kidney-injury-and-lung-fibrosis-a-case-report-from-bangladesh
#12
Ishrat Tahsin Isha, Z H M Nazmul Alam, Bidur Kanti Shaha, Md Shafiqul Bari, Mohammad Zabed Jillul Bari, Fazle Rabbi Chowdhury
BACKGROUND: Since Bangladesh government issued a ban on the use of highly toxic WHO Class I pesticides, annual consumption of herbicides like Paraquat have been sharply increasing in the markets. Paraquat poisoning is an emerging public health threat and its high mortality rate is responsible for a significant number of deaths. Diagnostic limitations and unavailable sample at presentation have resulted in under-reporting and lack of awareness among the treating physicians, making Paraquat poisoning one of the most neglected toxicological emergencies...
May 30, 2018: BMC Research Notes
https://www.readbyqxmd.com/read/29797247/nutritional-treatment-of-advanced-ckd-twenty-consensus-statements
#13
Adamasco Cupisti, Giuliano Brunori, Biagio Raffaele Di Iorio, Claudia D'Alessandro, Franca Pasticci, Carmela Cosola, Vincenzo Bellizzi, Piergiorgio Bolasco, Alessandro Capitanini, Anna Laura Fantuzzi, Annalisa Gennari, Giorgina Barbara Piccoli, Giuseppe Quintaliani, Mario Salomone, Massimo Sandrini, Domenico Santoro, Patrizia Babini, Enrico Fiaccadori, Giovanni Gambaro, Giacomo Garibotto, Mariacristina Gregorini, Marcora Mandreoli, Roberto Minutolo, Giovanni Cancarini, Giuseppe Conte, Francesco Locatelli, Loreto Gesualdo
The Italian nephrology has a long tradition and experience in the field of dietetic-nutritional therapy (DNT), which is an important component in the conservative management of the patient suffering from a chronic kidney disease, which precedes and integrates the pharmacological therapies. The objectives of DNT include the maintenance of an optimal nutritional status, the prevention and/or correction of signs, symptoms and complications of chronic renal failure and, possibly, the delay in starting of dialysis...
May 24, 2018: Journal of Nephrology
https://www.readbyqxmd.com/read/29795560/zika-virus-infection-in-the-veterans-health-administration-vha-2015-2016
#14
Patricia L Schirmer, Aaron Wendelboe, Cynthia A Lucero-Obusan, Russell A Ryono, Mark A Winters, Gina Oda, Mirsonia Martinez, Sonia Saavedra, Mark Holodniy
BACKGROUND: Zika virus (ZIKV) is an important flavivirus infection. Although ZIKV infection is rarely fatal, risk for severe disease in adults is not well described. Our objective was to describe the spectrum of illness in U.S. Veterans with ZIKV infection. METHODOLOGY: Case series study including patients with laboratory-confirmed or presumed positive ZIKV infection in all Veterans Health Administration (VHA) medical centers. Adjusted odds ratios of clinical variables associated with hospitalization and neurologic complications was performed...
May 2018: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/29789935/potential-use-of-stem-cells-as-a-therapy-for-cystinosis
#15
REVIEW
Celine J Rocca, Stephanie Cherqui
Cystinosis is an autosomal recessive metabolic disease that belongs to the family of lysosomal storage disorders (LSDs). Initial symptoms of cystinosis correspond to the renal Fanconi syndrome. Patients then develop chronic kidney disease and multi-organ failure due to accumulation of cystine in all tissue compartments. LSDs are commonly characterized by a defective activity of lysosomal enzymes. Hematopoietic stem and progenitor cell (HSPC) transplantation is a treatment option for several LSDs based on the premise that their progeny will integrate in the affected tissues and secrete the functional enzyme, which will be recaptured by the surrounding deficient cells and restore physiological activity...
May 22, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29778671/international-study-of-comparative-health-effectiveness-with-medical-and-invasive-approaches-ischemia-trial-rationale-and-design
#16
David J Maron, Judith S Hochman, Sean M O'Brien, Harmony R Reynolds, William E Boden, Gregg W Stone, Sripal Bangalore, John A Spertus, Daniel B Mark, Karen P Alexander, Leslee Shaw, Jeffrey S Berger, T Bruce Ferguson, David O Williams, Robert A Harrington, Yves Rosenberg
BACKGROUND: Prior trials comparing a strategy of optimal medical therapy with or without revascularization have not shown that revascularization reduces cardiovascular events in patients with stable ischemic heart disease (SIHD). However, those trials only included participants in whom coronary anatomy was known prior to randomization and did not include sufficient numbers of participants with significant ischemia. It remains unknown whether a routine invasive approach offers incremental value over a conservative approach with catheterization reserved for failure of medical therapy in patients with moderate or severe ischemia...
April 21, 2018: American Heart Journal
https://www.readbyqxmd.com/read/29772737/distal-colon-motor-dysfunction-in-mice-with-chronic-kidney-disease-putative-role-of-uremic-toxins
#17
Elsa Hoibian, Nans Florens, Laetitia Koppe, Hubert Vidal, Christophe O Soulage
Although gastrointestinal complications are a common feature of patients with chronic kidney disease (CKD), the impact of uremia on bowel motility remains poorly understood. The present study was, therefore, designed to investigate the impact of uremia on gut motility. Kidney failure was induced in mice by chemical nephrectomy using an adenine diet (0.25% w/w ). Gastrointestinal transit time and colon motility were explored in vivo and ex vivo . Colons from control mice were incubated with uremic plasma or uremic toxins (urea, indoxyl-sulfate or p-cresyl-sulfate) at concentrations encountered in patients with end-stage renal disease...
May 16, 2018: Toxins
https://www.readbyqxmd.com/read/29770103/outbreak-of-seoul-virus-among-rats-and-rat-owners-united-states-and-canada-2017
#18
J L Kerins, S E Koske, J Kazmierczak, C Austin, K Gowdy, A Dibernardo
What is already known about this topic?: ​: Seoul virus, a type of hantavirus, is carried by Norway rats. Humans become infected through contact with virus shed in rat urine or droppings, or inhalation of virus particles in dust from contaminated bedding. Infected rats do not develop disease, but humans can experience symptoms ranging from mild influenza-like illness to severe disease with kidney failure and death. Although infections have been previously reported in humans after contact with wild rats, Seoul virus infections had not been reported in pet rats in the United States or Canada...
February 1, 2018: Canada Communicable Disease Report, Relevé des Maladies Transmissibles Au Canada
https://www.readbyqxmd.com/read/29764427/col4a5-and-lama5-variants-co-inherited-in-familial-hematuria-digenic-inheritance-or-genetic-modifier-effect
#19
Konstantinos Voskarides, Gregory Papagregoriou, Despina Hadjipanagi, Ioanelli Petrou, Isavella Savva, Avraam Elia, Yiannis Athanasiou, Androulla Pastelli, Maria Kkolou, Michalis Hadjigavriel, Christoforos Stavrou, Alkis Pierides, Constantinos Deltas
BACKGROUND: About 40-50% of patients with familial microscopic hematuria (FMH) caused by thin basement membrane nephropathy (TBMN) inherit heterozygous mutations in collagen IV genes (COL4A3, COL4A4). On long follow-up, the full phenotypic spectrum of these patients varies a lot, ranging from isolated MH or MH plus low-grade proteinuria to chronic renal failure of variable degree, including end-stage renal disease (ESRD). METHODS: Here, we performed Whole Exome Sequencing (WES) in patients of six families, presenting with autosomal dominant FMH, with or without progression to proteinuria and loss of renal function, all previously found negative for severe collagen IV mutations...
May 16, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29761573/using-fat-to-fight-disease-a-systematic-review-of-non-homologous-adipose-derived-stromal-stem-cell-therapies
#20
REVIEW
Marjorie E Bateman, Amy L Strong, Jeffrey M Gimble, Bruce A Bunnell
OBJECTIVE: The objective of this review is to describe the safety and efficacy of ASC and SVF in treating common diseases and the next steps in research that must occur prior to clinical use. EVIDENCE REVIEW: Pubmed, Ovid Medline, Embase, Web of Science, and the Cochrane Library were searched for articles about use of SVF or ASC for disease therapy published between 2012 and 2017. One meta-analysis, 2 randomized controlled trials, and 16 case series were included, representing 844 human patients...
May 15, 2018: Stem Cells
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