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- 1. ウィルムス腫瘍の症状、診断、および病期分類 presentation diagnosis and staging of wilms tumor
- 2. ウィルムス腫瘍の治療および予後 treatment and prognosis of wilms tumor
- 3. 微細欠失症候群（1番～11番染色体） microdeletion syndromes chromosomes 1 to 11
- 4. ゲノム疾患：概要 genomic disorders an overview
- 5. 6ヵ月以下の乳幼児における体重減少の評価 evaluation of weight loss in infants six months of age and younger
- Valsartan slows the progression of diabetic nephropathy in db/db mice via a reduction in podocyte injury, and renal oxidative stress and inflammation.
- Zhou G, Cheung AK, Liu X1, Huang Y1.Author information 1†Division of Nephrology and Hypertension, Department of Internal Medicine, University of Utah School of Medicine, Salt Lake City, UT 84108, U.S.A.AbstractHigher doses of AngII (angiotensin II) blockers are intended to optimize albuminuria reduction rather than for blood pressure control in chronic kidney disease. However, the long-term renoprotection of high-dose AngII blockers has yet to be defined. The present study sought to determine whether doses of ARB (AngII receptor blocker) that maximally reduce proteinuria could slow the progression of glomerulosclerosis in the uninephrectomized db/db mouse, a model of Type 2 diabetes. Untreated uninephrectomized db/db mice had normal blood pressure, but developed progressive albuminuria and mesangial matrix expansion between 18 and 22 weeks of age, which was associated with increased renal expression of TGFβ1 (transforming growth factor β1), PAI-1 (plasminogen-activator inhibitor-1), type IV collagen and FN (fibronectin). Treatment with valsartan in the drinking water of db/db mice from 18 to 22 weeks of age, at a dose that was determined previously to maximally reduce proteinuria, prevented the increases in albuminuria and the markers of renal fibrosis seen in untreated db/db mice. In addition, WT-1 (Wilms tumour protein-1)-immunopositive podocyte numbers were found to be lower in the untreated glomeruli of mice with diabetes. The expression of podocin and nephrin were continually decreased in mice with diabetes between 18 and 22 weeks of age. These changes are indicative of podocyte injury and the administration of valsartan ameliorated them substantially. Renal expression of TNFα (tumour necrosis factor α), MCP-1 (monocyte chemoattractant protein-1), Nox2 (NADPH oxidase 2), p22phox and p47phox and urine TBARS (thiobarbituric acid-reacting substance) levels, the markers of renal inflammation and oxidative stress, were increased during disease progression in mice with diabetes. Valsartan treatment was shown to reduce these markers. Thus high doses of valsartan not only reduce albuminuria maximally, but also halt the progression of the glomerulosclerosis resulting from Type 2 diabetes via a reduction in podocyte injury and renal oxidative stress and inflammation.
- Clinical science (London, England : 1979).Clin Sci (Lond).2014 May;126(10):707-20. doi: 10.1042/CS20130223.
- Higher doses of AngII (angiotensin II) blockers are intended to optimize albuminuria reduction rather than for blood pressure control in chronic kidney disease. However, the long-term renoprotection of high-dose AngII blockers has yet to be defined. The present study sought to determine whether dose
- PMID 24195695
- Clear cell sarcoma of the kidney demonstrates an embryonic signature indicative of a primitive nephrogenic origin.
- Karlsson J1, Holmquist Mengelbier L, Ciornei CD, Naranjo A, O'Sullivan MJ, Gisselsson D.Author information 1Department of Clinical Genetics, Lund University, University and Regional Laboratories, Lund, Sweden.AbstractClear cell sarcoma of the kidney (CCSK) is a tumor affecting children with a median age of 3 years at diagnosis. The cell of origin of CCSK is unknown and data on the molecular changes giving rise to CCSK is scarce. This has hindered the identification of positive diagnostic markers and development of molecularly targeted treatment protocols for CCSK. We have characterized a panel of CCSK to gain information regarding its molecular profile and possible origin. High-resolution genomic analysis with single nucleotide polymorphism array of 37 tumors did not reveal any clues to the mechanisms behind tumor development as remarkably few genetic imbalances were found. Gene expression analysis revealed a highly characteristic gene signature, enriched for pathways involved in embryonic development, including kidney formation. The presence of markers for two different developmental lineages in the embryonic kidney was therefore investigated in the tumor cells. FOXD1 which identifies cells giving rise to stromal elements, and CITED1, a marker for cells primed for nephrogenic epithelial differentiation, were both highly expressed in CCSK. In addition, the early embryonic marker OSR1 was expressed at higher levels in CCSK than in Wilms tumor, normal fetal kidney or adult kidney. As this marker discriminates the intermediate mesoderm from other mesodermal structures, our study could suggest that CCSK arises from a mesodermal cell type that retains the capacity to initiate differentiation towards both nephrons and stroma, but remains locked in a primitive state. © 2014 Wiley Periodicals, Inc.
- Genes, chromosomes & cancer.Genes Chromosomes Cancer.2014 May;53(5):381-91. doi: 10.1002/gcc.22149. Epub 2014 Feb 1.
- Clear cell sarcoma of the kidney (CCSK) is a tumor affecting children with a median age of 3 years at diagnosis. The cell of origin of CCSK is unknown and data on the molecular changes giving rise to CCSK is scarce. This has hindered the identification of positive diagnostic markers and development
- PMID 24488803
- Non-activated protein C rescue treatment in Wilms tumour associated hepatic sinusoidal obstructive syndrome.
- De Leonardis F1, Koronica R, Bruno SD, Santoro N.Author information 1Division of Paediatric Haematology-Oncology, Department of Pediatrics, Bari, Italy.AbstractHepatic sinusoidal obstructive syndrome (HSOS) is a frequent complication in patients undergoing haematopoietic stem cell transplant (HSCT), and more rarely, in paediatric patients receiving conventional chemotherapy for solid tumours. Its diagnosis relies on a combination of clinical signs and symptoms such as hepatomegaly, jaundice, weight gain and fluid retention. HSOS treatment is primarily based on supportive care and anti-fibrinolytic agents. Here we report two patients affected by Wilms tumour who developed life-threatening HSOS that failed to respond to conventional treatment. Both patients recovered after receiving aggressive supportive treatment that included administration of non-activated protein C (Ceprotin®-Baxter). Pediatr Blood Cancer 2014;61:940-941. © 2013 Wiley Periodicals, Inc.
- Pediatric blood & cancer.Pediatr Blood Cancer.2014 May;61(5):940-1. doi: 10.1002/pbc.24859. Epub 2013 Nov 26.
- Hepatic sinusoidal obstructive syndrome (HSOS) is a frequent complication in patients undergoing haematopoietic stem cell transplant (HSCT), and more rarely, in paediatric patients receiving conventional chemotherapy for solid tumours. Its diagnosis relies on a combination of clinical signs and symp
- PMID 24281860
- PRESENTATION Children with Wilms tumor typically present with an asymptomatic abdominal mass. It is not uncommon for the tumor to be discovered by a parent while bathing the child or by a relative who notices a protuberant ...
- Wilms tumor is a type of kidney cancer that occurs in children. ... Calling your health care provider Call your health care provider if you discover a lump in your child's abdomen, blood in the urine, or other symptoms of ...
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